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1711 Cards in this Set
- Front
- Back
|
How many human chrmosomes?
|
46
|
|
What percentage of newborns have chromosomal abnormalitites?
|
0.5%
|
|
What are the 2 most frequent numerical chromosomal abnormalities?
|
trisomy
monsomy |
|
What is trisomy 21?
|
Down's syndrome
|
|
How palmar creases in Downs?
|
1
|
|
Brushfield spots on iris is found in what syndrome?
|
Down's
|
|
Clinodactaly (incurving) of 5th finger occurs in what syndrome?
|
Down's
|
|
Ventral septal defect occurs in what syndrome?
|
Down's
|
|
A 3rd fontanelle occurs in what syndrome?
|
Down's
|
|
hypotonia occurs in what syndrome?
|
Down's
|
|
Increased space between 1st and 2nd toes occurs in what syndrome?
|
Down's
|
|
Upward slant of palpebral fissures occurs in what syndrome?
|
Down's
|
|
Low set auricles occur in what syndrome?
|
Down's
|
|
What syndrome is trisomy 18?
|
Edward's Syndrome
|
|
Intrauterine growth retardation is characteristic of what syndrome?
|
trisomy18
|
|
A small mouth is characteristic of what syndrome?
|
trisomy 18
|
|
Rocker-bottom feet is found in what syndrome?
|
Edward's
|
|
Abnormal overlapping of fingers is found in what syndrome?
|
Edward's
|
|
A prominent occiput is found in what syndrome?
|
Edward's
|
|
Failure to thrive is found in what syndrome?
|
Edward's
|
|
Hypoplastic finger nails is found in what syndrome?
|
Edward's
|
|
What syndrome is Trisomy 13?
|
Patau's
|
|
Intrauterine growth retardation in all three parameters is found in what syndrome?
|
Trisomy 13
|
|
Coloboma of iris (keyhole-shaped pupil) is found in what syndrome?
|
Trisomy 13
|
|
Capillary hemangioma is found in what syndrome?
|
Trisomy 13
|
|
Polydactly is found in what syndrome?
|
Trisomy 13
|
|
Cleft lip and palate is found in what syndrome?
|
Patau's
|
|
Skin defect of skull is found in what syndrome?
|
Trisomy 13
|
|
Hyperconvex nails is found in what syndrome?
|
Trisomy 13
|
|
What is 45,x syndrome?
|
Turner's syndrome
|
|
What is the numeration for Turner's syndrome?
|
45,X
|
|
Short stature is found in what syndrome?
|
Turner's
|
|
Triangle face is found in what syndrome?
|
Turner's
|
|
Abnormal shape of ears and webbed neck is found in what syndrome?
|
Turner's
|
|
Broad "shield" chest is found in what syndrome?
|
45,X
|
|
Wide-set nipples ifs found in what syndrome?
|
45,X
|
|
Congenital lymphedema of hands and feet is found in what syndrome?
|
45,X (Turner's)
|
|
Shortened 4th and 5th metacarpals and metatarsals is found in what syndrome?
|
Turner's
|
|
Cubitus valgus is found in what syndrome?
|
Turner's
|
|
Primary amenorrhea is found in what syndrome?
|
Turner's
|
|
Coarctation of the aorta is found in what syndrome?
|
Turner's
|
|
Normal IQ is found in what syndrome?
|
Turner's
|
|
Infertility is found in what syndrome?
|
Turner's
|
|
What is the numerical designation of Klinefelter's syndrome?
|
47, XXY
|
|
What syndrome is 47,XXY?
|
Klinefelter's syndrome
|
|
Tall stature is found in what syndrome?
|
Klinefelter's
|
|
Post-puberty small testicles is found in what syndrome?
|
Klinefelter's
|
|
Gynecomastia is found in what syndrome?
|
Klinefelter's syndrome
|
|
Eunuchoid build is found in what syndrome?
|
Klinefelter's
|
|
Increased risk for mild mental retardation is found in what syndrome?
|
Klinefelter's
|
|
Infertility can be found in what two syndromes?
|
Turner's and Klinefelter's
|
|
During what week range is amniocentesis done?
|
14-16 weeks
|
|
During what week range is chorionic villi testing done?
|
9-10 weeks
|
|
What teratogenic infectious agents have the mnemonic TORCHES?
|
Toxoplasmosis
Other (varicella or parvovirus) Rubella Cytomegalovirus HErpes/HIV Syphilis |
|
What anticonvulsant is known as a teratogen?
|
valproic acid
|
|
What anti-coagulant is known as a teratogen?
|
warfarin
|
|
What vitamin's derivatives are teratogens?
|
Vitamin A
|
|
What is the average growth in the first year?
|
10''
|
|
Birth length doubles, triples, or quadruples by age 13?
|
triples
|
|
Birth weight doubles, triples, or quadruples by end of first year?
|
triples
|
|
How many fonatnelles at birth?
|
6
|
|
What is last fontanelle to close?
|
anterior
|
|
Persistent primitive reflexes are the earliest markers of what?
|
abnormal neurological maturation
|
|
What tone predominates in a newborn?
|
flexor tone
|
|
What age: in prone turns head to side?
|
newborn
|
|
What age: automatic reflex walking?
|
newborn
|
|
What age: rounded spine when held sitting?
|
newborn
|
|
What age: hands fisted?
|
newborn
|
|
What age: grasp reflex?
|
newborn
|
|
What age: ability to fix and follow bright object?
|
newborn
|
|
What age: turning head to rattle or voice?
|
newborn
|
|
What age: cry?
|
newborn
|
|
What age: reflex stage?
|
newborn
|
|
What age: basic trust vs. basic mistrust?
|
newborn
|
|
What age: does not differentiate between self and mother?
|
newborn
|
|
What age head midline?
|
4 months
|
|
What age head held when pulled to sit?
|
4 months
|
|
What age in prone position lifts head to 90 degrees?
|
4 months
|
|
What age turns to supine?
|
4 months
|
|
What age hands mostly open?
|
4 months
|
|
What age hands fisted?
|
newborn
|
|
What age midline hand play?
|
4 months
|
|
What age crude palmar grasp?
|
4 months
|
|
What age fascinated by own face in mirror?
|
4 months
|
|
Whta age recognizes bottle?
|
4 months
|
|
What age turns to voice and bell consistently?
|
4 months
|
|
What age laughs and squeals?
|
4 months
|
|
What age responsive vocalization?
|
4 months
|
|
What age blows bubbles?
|
4 months
|
|
What age shows circular reaction, the interesting result of action motivates its repetition?
|
4 months
|
|
What age developing a basic sense of trust?
|
4 months
|
|
What age maintains sitting?
|
7 months
|
|
What age may lean on arms?
|
7 months
|
|
What age rolls to prone?
|
7 months
|
|
What age bears all weight, bounces when held erect?
|
7 months
|
|
What age cervical lordosis?
|
7 months
|
|
What age intermediate grasp?
|
7 months
|
|
What age transfers cube from hand to hand?
|
7 months
|
|
What age bangs objects?
|
7 months
|
|
What age differentiates between familiar person and stranger?
|
7 months
|
|
What age holds bottle?
|
7 months
|
|
What age looks for dropped object?
|
7 months
|
|
What age talks to mirror image?
|
7 months
|
|
What age uses single-word and double consonant vowel combinations?
|
7 months
|
|
What age begins to differentiate between mother and self-individuation. Sense of belonging to a central person?
|
5 months
|
|
What age creeps on all 4s?
|
10 months
|
|
What age pivots in sitting?
|
10 months
|
|
What age stand momentarily, cruises?
|
10 months
|
|
What age slight bow leg?
|
10 months
|
|
What age increased lumbar lordosis, acute lumbosacral angulation?
|
10 months
|
|
What age pincer grasp, mature thumb to index grasp?
|
10 months
|
|
What age bangs 2 cubes held in hands?
|
10 months
|
|
What age plays peek-a-boo?
|
10 months
|
|
What age finger feeds?
|
10 months
|
|
What age chews with rotary movement?
|
10 months
|
|
What age shouts for attention?
|
10 months
|
|
What age initiates speech sounds?
|
10 months
|
|
What age waves bye-bye?
|
10 months
|
|
What age uses "mama" and "dada" with meaning?
|
10 months
|
|
What age inhibits behavior to "no?"
|
10 months
|
|
What age can retrieve an object hidden from view?
|
10 months
|
|
What age practicing phase of separation-individuation?
|
10 months
|
|
What age walks alone, arms in high guard or mid-guard?
|
14 months
|
|
What age wide-base, excessive knee and hip flexion?
|
14 months
|
|
What age foot contact on entire side?
|
14 months
|
|
What age slight valgus of knees and feet?
|
14 months
|
|
Whta age pelvic tilt and rotation?
|
14 months
|
|
What age piles 2 cubes?
|
14 months
|
|
What age scibbles spontaneously?
|
14 months
|
|
What age holds crayons full length in palm?
|
14 months
|
|
What age casts objects?
|
14 months
|
|
What age uses spoon with over pronation and spilling?
|
14 months
|
|
What age removes a garment?
|
14 months
|
|
What age uses single words and understands simple commands?
|
14 months
|
|
What age differentiates available behaviors patterns for new ends, i.e. pulls rug on which is a toy
|
14 months
|
|
What age is rapprochement phase - individuation and ambivalence to mother?
|
14 months
|
|
What age is stage of autonomy vs. shame and doubt?
|
14 months
|
|
What age is pleasure in control of muscle and sphincter?
|
14 months
|
|
What age arms at low guard?
|
18 months
|
|
What age mature supporting base and heel strike?
|
18 months
|
|
What age seats self in chair?
|
18 months
|
|
What age walks backwards?
|
18 months
|
|
What age emerging hand dominance?
|
18 months
|
|
What age crude release?
|
18 months
|
|
What age holds crayon butt end in palm?
|
18 months
|
|
What age dumps raisin from bottle spontaneously?
|
18 months
|
|
What age initiates housework?
|
18 months
|
|
What age carries, hugs doll?
|
18 months
|
|
What age drinks from cup neatly?
|
18 months
|
|
What age points to named body part?
|
18 months
|
|
What age identifies 1 picture?
|
18 months
|
|
What age says "no"
|
18 months
|
|
What age uses jargon?
|
18 months
|
|
What age capable of insight?
|
18 months
|
|
What age capable of problem solving by mental combinations?
|
18 months
|
|
What age begins running?
|
2 years
|
|
What age walks up and down stairs alone?
|
2 years
|
|
What age jumps on both feet in place?
|
2 years
|
|
What age hand dominance is usual?
|
2 years
|
|
What age build 8-cube tower?
|
2 years
|
|
What age initiates vertical line?
|
2 years
|
|
What age places pencil shaft between thumb and fingers?
|
2 years
|
|
What age draws with arm and wrist action?
|
2 years
|
|
What age pulls on garment?
|
2 years
|
|
What age uses spoon well?
|
2 years
|
|
What age opens door turning knob?
|
2 years
|
|
What age feeds doll with bottle or spoon?
|
2 years
|
|
What age toilet training usually begun?
|
2 years
|
|
What age two-word phrases are common?
|
2 years
|
|
What age uses verbs?
|
2 years
|
|
What age refers to self by name?
|
2 years
|
|
What age uses me, mine?
|
2 years
|
|
What age follows simple directions?
|
2 years
|
|
What age able to evoke and obect or event not present?
|
2 years
|
|
What age object performance established?
|
2 years
|
|
What age comprehends symbols?
|
2 years
|
|
What age runs well?
|
3 years
|
|
What age pedals tricycle?
|
3 years
|
|
What age broad jumps?
|
3 years
|
|
What age walks up stairs alternating feet?
|
3 years
|
|
What age imitates 3-cube bridge?
|
3 years
|
|
What age copies circles?
|
3 years
|
|
What age uses overhand throw with anteroposterior arm and motion?
|
3 years
|
|
What age catches with extended arms hugging against body?
|
3 years
|
|
What age most children toilet trained day and night?
|
3 years
|
|
What age pours from pitcher?
|
3 years
|
|
What age unbutton; washes and dries hands and face?
|
3 years
|
|
What age parallel play?
|
3 years
|
|
What age can take turns?
|
3 years
|
|
What age can be reasoned with?
|
3 years
|
|
What age three word sentences are usual?
|
3 years
|
|
What age uses future tense?
|
3 years
|
|
What age asks who, what, where?
|
3 years
|
|
What age folows prepositional commands?
|
3 years
|
|
What age gives full name?
|
3 years
|
|
What age may stutter, eager?
|
3 years
|
|
What age identifies sex of self?
|
3 years
|
|
What age recognizes 3 colors?
|
3 years
|
|
What age capable of deferred limitation symbolic play?
|
3 years
|
|
What age drawing of graphic images, mental images, and verbal evocation of event?
|
3 years
|
|
What age range is the preoperation period?
|
2-7 years
|
|
What age range stage of initiative vs. guilt?
|
3-5
|
|
What age deals with issue of genital sexuality?
|
3 years
|
|
What age walks down stairs alternating feet?
|
4 years
|
|
What age hops on 1 foot?
|
4 years
|
|
What age plantar arches developing?
|
4 years
|
|
What age sits up from supine without rotating?
|
4 years
|
|
What age handles a pencil by a finger and wrist action, like adults?
|
4 years
|
|
What age copies a cross?
|
4 years
|
|
What age draws a frog-like person with head and extremities?
|
4 years
|
|
What age throws underhand?
|
4 years
|
|
What age cuts with scissors?
|
4 years
|
|
What age cooperative play-sharing and interacting?
|
4 years
|
|
What age imaginative make-believe play?
|
4 years
|
|
What age dresses and undresses with supervision distinguishing front and back of clothing, and buttoning?
|
4 years
|
|
What age buttons clothing?
|
4 years
|
|
What age does simple errands outside of home?
|
4 years
|
|
What age gives connected account of experience?
|
4 years
|
|
What age asks why, when, how?
|
4 years
|
|
What age uses past tense, adjectives, and adverbs?
|
4 years
|
|
What age knows opposite analogies?
|
4 years
|
|
What age repeats 4 digits?
|
4 years
|
|
What age skips, tiptoes?
|
5 years
|
|
What age balances 10-seconds on each foot?
|
5 years
|
|
What age hand dominance expected?
|
5 years
|
|
What age draws man with head, body, and extremities?
|
5 years
|
|
What age throws with diagonal arm and body rotation?
|
5 years
|
|
What age catches with hand?
|
5 years
|
|
What age creative play?
|
5 years
|
|
What age competitive team play?
|
5 years
|
|
What age uses fork for stabbing food?
|
5 years
|
|
What age brushes teeth?
|
5 years
|
|
What age self-sufficient in toileting?
|
5 years
|
|
What age dresses without supervision except tying shoelaces?
|
5 years
|
|
What age fluent speech?
|
5 years
|
|
What age misarticulation of some sounds may persist?
|
5 years
|
|
What age gives name, age, and address?
|
5 years
|
|
What age defines concrete nouns-classification and use?
|
5 years
|
|
What age follows 3 part commands?
|
5 years
|
|
What age number concept to 10?
|
5 years
|
|
What is the age range of industry vs. inferiority?
|
5-adolescence
|
|
What age adjusts to the inorganic laws of the tool world?
|
5 years
|
|
What age rides bicycle?
|
6 years
|
|
What age roller skates?
|
6 years
|
|
What age prints alphabet?
|
6 years
|
|
What age letter reversals still acceptable?
|
6 years
|
|
What age mature catch and throw of ball?
|
6 years
|
|
What age teacher is important authority?
|
6 years
|
|
What age uses fork appropriately?
|
6 years
|
|
What age uses knife for spreading?
|
6 years
|
|
What age uses knife for spreading?
|
6 years
|
|
What age plays table games?
|
6 years
|
|
What age shows mastery of grammar?
|
6 years
|
|
What age uses proper articulation?
|
6 years
|
|
What age eats with fork and knife ?
|
7 years
|
|
What age combs hair?
|
7 years
|
|
What age is responsible for grooming?
|
7 years
|
|
What age range capable of concrete operational thought?
|
7-adolescence?
|
|
What age capable of logical thought?
|
7 years
|
|
During what trimester do congenitial limb deficiencies occur?
|
1st trimester
|
|
On what day of gestation does mesodermal formation of the limb occur?
|
day 26
|
|
What are 2 risk factors for congenital limb deficiency?
|
thalidomide
maternal diabetes |
|
What is the preferred classification system for pediatric limb deficiencies?
|
International Society for Prosthetics and Orthotics (ISPO)
|
|
ISPO classifies limb deficiencies in what two ways?
|
transverse or longitudinal
|
|
What limb deficiency classification has a distal portion?
|
longitudinal
|
|
What limb deficiency has not distal limb portion?
|
transverse
|
|
Longitudinal deficiencies are named after what?
|
the bone that is affected
|
|
Transverse deficiencies are named after what?
|
the segment beyond which there is no skeletal portion
|
|
What is amelia?
|
absence of limb
|
|
What is absence of limb?
|
amelia
|
|
What is meromelia?
|
partial absence of limb
|
|
What is partial absence of limb?
|
meromelia
|
|
What is hemimelia?
|
absence of half of a limb
|
|
What is absence of half of a limb?
|
hemimelia
|
|
What is phocomelia?
|
flipper-like appendage attached to trunk
|
|
What is a flipper-like appendage attached to trunk?
|
phocomelia
|
|
What is acheiria?
|
missing hand or foot
|
|
What is a missing hand or foot?
|
acheria
|
|
What is adactyly
|
absent metacarpal or metatarsal
|
|
What is an absent metacarpal or metatarsal?
|
adactyly
|
|
What is aphalangia?
|
absent finger or toe
|
|
What is in an absent finger or toe?
|
aphalangia
|
|
What is the name of the classification that descirbes deficiencies as either terminal or intercalary?
|
Frantz
|
|
What is an upper extremity amelia in the ISPO system?
|
transverse upper arm, total
|
|
What is a fibula hemimelia in the ISPO system?
|
longitutinal fibular deficiency
|
|
What is an upper extremtiy phocomelia in the ISPO system?
|
longitudinal total humerus, ulna; carpal, metacarpal, phalangeal
|
|
What is a transverse upper arm, total in the classic classification?
|
upper extremity amelia
|
|
What is a longitudinal fibular deficiency in the classic classification?
|
fibula hemimelia
|
|
What is the incidence of congenital upper extremity deficiency?
|
4.1 per 10,000 live births
|
|
What are the 3 limb deficiencies that do have heriditary implications?
|
*deficiencies of hand and feet
*central ray deficiencies *adactyly involving the first 4 digits with the 5th intact |
|
What is the TAR syndrome?
|
Thrombocytopenia with Absence of Radius
|
|
What is Fanconi's syndrome?
|
UE limb deficiency and anemia and leukopenia developing at 5-6 years of age
|
|
What is Holt-Oram Syndrome?
|
congenital heart disease, tetralogy of Fallot and UE limb deficieny
|
|
What syndrome is Thrombocytopenia with Absence of Radius
|
TAR syndrome
|
|
What syndrome is UE limb deficiency and anemia and leukopenia developing at 5-6 years of age?
|
Fanconi's syndrome
|
|
What syndrome is UE limb deficiency, congenital heart disease, ASD, and tetralogy of Fallot?
|
Holt-Oram Syndrome
|
|
What is Baller-Gerold Syndrome?
|
UE limb deficiency and craniosynostosis
|
|
What syndrome is UE limb deficiency and craniosynostosis?
|
Baller-Gerold Syndrome
|
|
What is VACTERL syndrome?
|
Vertebral defects
Anal atresia Cardiac defects Tracheo-Esophogeal fistula Renal dysplasia Limb deficiency |
|
What is the most common congential limb deficiency?
|
left terminal transradial deficiency
|
|
At what developmental milestone and approx. month age range is the first fitting for a prosthetic in a child with a left terminal transradial deficiency?
|
sitting balance
6-7 months |
|
What is the initial prosthesis in a left terminal transradial prosthesis?
|
passive mit
|
|
At what month age range is the child provided with an improved prosthesis for a terminal transradial?
|
11-13 months
|
|
What are the 3 developmental milestones for an improved prosthesis in a terminal transradial?
|
walking
grasping 5 minute attention span |
|
What are 2 design features of an initial transradial prosthesis?
|
*self suspending
*supracondylar socket |
|
By what age range can a child operate all types of prosthetic components and controls?
|
4-5 years old
|
|
What are the two indications for the krukenberg procedure?
|
absent hand
visual impairment |
|
What procedure separates the ulna and radius in the forearm in a child with a missing hand?
|
Krukenberg procedure
|
|
The Krukenberg procedure is used primarily for unilateral or bilateral missing hands?
|
bilateral
|
|
What are the 2 possible suspensions for a transhumeral deficiency?
|
harness
silicone suction |
|
What is the developmental milestone for addition of active terminal devices in a transhumeral deficiency?
|
walking
|
|
At what age range can a child use a body-powered hook?
|
2-3 years old
|
|
What what age range can a child use a body-powered elbow?
|
4-5 years
|
|
An infant will have greater difficulty rolling over with a transradial or transhumeral prosthesis?
|
transhumeral
|
|
Amputation revision is required in what percentage of UE limb deficiencies?
|
10%
|
|
What procedure attaches a toe to the residual limb?
|
Vilke procedure
|
|
What is the most common lower extremity deficiency?
|
fibular longitudinal deficiency (fibular hemimelia)
|
|
What is the percentage of fibular longitudinal deficiency that is bilateral?
|
25%
|
|
What amputation is performed with a severe leg-length discrepency caused by a unilateral fibular longitudinal deficiency?
|
Syme's amputation (ankle disarticulation with attachement of the distal heal pad to the end of the tibia)
|
|
What is the incidence of longitudinal deficiency of the tibia?
|
1 in 1,000,000 births
|
|
What is the treatment of choice for a longitudinal deficiency of the tibia?
|
knee disarticulation
|
|
What is the inheritance pattern of a partial tibial deficiency?
|
autosomal dominant
|
|
What percentage of partial tibial deficiency is herditary?
|
30%
|
|
What proceudure is done if there is a long tibial segment remaining in a partial tibial deficiency?
|
synostosis with the fibular and amputation of the foot
|
|
What is PFFD?
|
partial proximal femoral focal deficiency
|
|
What is the incidence of PFFD?
|
1 in 50,000 births
|
|
What is the postion the PFFD is held in ?
|
flexion, external rotation, and abduction
|
|
What is the Van Ness rotation?
|
rotating the foot 180 degress so ankle motion can control the prosthesis
|
|
What condition is an indication for the Van Ness Rotation?
|
Partial proximal femoral focal deficiency
|
|
At what developmental milestone is a child fitted with a LE prosthesis? month age range?
|
pull up to standing; 9-10 months
|
|
What age is normal heel-to-toe gait?
|
2 years old
|
|
At what developmental milestone can a child attain prosthetic heel-strike to toe-off gait? approx age?
|
one-legged standing; 5 years old
|
|
At what age (months) is a knee joint added to a prosthesis in a child with partial proximal femoral focal deficiency?
|
18 months
|
|
What is the most commonly prescribed foot for the child amputee?
|
solid ankle cushion heel
|
|
For what age group is a fluid controlled knee reserved?
|
adolescents
|
|
What two knees are used for childhood prosthetics?
|
single axis
polycentric |
|
At what age is a suction socket prescribed?
|
5 years old
|
|
What a complication of a patellar tendon-bearing prosthesis in a child?
|
dislocated patella
|
|
What type of prosthesis and cuff are used for a child BKA?
|
patellar tendon-bearing prosthesis with a supracondylar cuff
|
|
What is a sign of an ill-fitting prosthesis in a child?
|
gait deviation
|
|
How often is a prosthesis replaced ages 1-5?
|
annually
|
|
How often is a prosthesis replaced in ages 5-12?
|
every 2 years
|
|
What is the most common cause of pediatric amputations?
|
trauma
|
|
What percentage of pediatric amputations are lower limb?
|
60%
|
|
What is the most frequent cause of disease-related amputation in children?
|
tumors
|
|
What are the two most common tumors causing amputation in children?
|
*Ewing's sarcoma
*osteogenic sarcoma |
|
What complication of fracture non-union can cause pediatric amputations?
|
neurofibromatosis
|
|
Why is a joint disarticulation preferred over a trans-metaphyseal or a trans-diaphyseal ampution?
|
to preserve the epiphyseal growth plate
|
|
What is the most common complication after amputation in the immature child?
|
terminal overgrowth at the transected end
|
|
Terminal overgrowth as a complication after pediatric amputation occurs most frequently on what 4 bones in order of frequency?
|
humerus, fibula, tibia, femur
|
|
How do amputees preserve their energy expenditure?
|
decreasing walking speed
|
|
What is the youngest age to use a power wheelchair?
|
3 years old
|
|
What is the traditional age range for use of a power wheel chair?
|
5-6 years old
|
|
Do congenital limb-deficient children develop phantom pain or sensation?
|
no
|
|
Do children with congenital limb deficiencies who undergo conversion to surgical amputation of the limb experience phantom pain or sensation?
|
no
|
|
What condition is characterized by adduction of the forefoot on the hindfoot, with the heel in normal position or slightly valgus?
|
metatarsus varus
|
|
What is metatarus varus?
|
What condition is characterized by adduction of the forefoot on the hindfoot, with the heel in normal position or slightly valgus
|
|
What is the cause of metatarsus varus?
|
uterine positioning
|
|
By what age range have most cases of metatarsus varus resolved?
|
3-4
|
|
What are the 3 associated deformities of club foot?
|
*plantar flexion
*heel varus *forefoot varus |
|
What is the incidence of club foot?
|
1 per 1,000
|
|
What is talipes equinovarus?
|
club foot
|
|
What is the treatment of a rigid metatarsus varus?
|
splinting
|
|
What is the deformity of talipes calcaneovalgus?
|
foot eversion
ankle dorsiflexion |
|
Is flat foot normal in infants?
|
yes
|
|
Cavus foot is associated with what 3 neurologic conditions?
|
poliomyelitis
Charcot-Marie-Tooth Friederich's ataxia |
|
Poliomyelitis, Charcot-Marie-Tooth, and Friederich's ataxia are associated with foot condition?
|
cavus foot
|
|
What is cavus foot?
|
high longitudinal arch
|
|
What condition is usually seen in disorders of motor weakness such as CMT or pes cavus foot deformity?
|
claw toes
|
|
What is bow leg?
|
genu varum
|
|
What is the postion of claw toes?
|
metatarsophalangeal joints are hyperextended and interphalangeal joints are flexed
|
|
Infants generally have genu varus or genu valgus?
|
genu varum (bow leg)
|
|
By 12-18 months infants are genu varus or genu valgus?
|
genu valgus
|
|
What is Blount's disease?
|
tibia vara
|
|
Where is the abnormality causing tibia vara?
|
medial portion of the proximal tibial growth plate
|
|
What is tibia vara called?
|
Blount's disease
|
|
What is the most common cause of leg bowing in the young child?
|
Blount's disease (tibia vara)
|
|
In what subset of children is tibia vara most common?
|
obese children who walk at 9-10 months
|
|
What is the most common racial group for tibia vara?
|
African-American
|
|
What is the treatment of Blount's disease?
|
osteotomy of proximal tibia and fibula
|
|
What are the 3 conditions of developmental dysplasia of the hip?
|
hip subluxation
hip dislocation acetabular dysplasia |
|
When is hip dislocation diagnosed?
|
birth
|
|
Hip dislocation is more common in what type of delivery?
|
breech
|
|
What is the incidence of hip dislocation?
|
1 per 1,000 birth
|
|
Hip dislocation is more common in males or females?
|
females
|
|
If the mother had a history of dislocated hip, the risk to the baby is increased to what ratio if breech?
|
1 in 15
|
|
If the mother had a history of dislocated hip, the risk to the baby is increased to what ratio if nonbreech?
|
1 in 25
|
|
What two conditions have an increased incidence of hip dysplasia at birth?
|
metatarsus adductus
torticollis |
|
Metatarsus adductus and torticollis predispose to what other congenital disorder?
|
hip dysplasia
|
|
What are the 2 names of the test for hip dysplasia at birth?
|
Galeazzi or Allis test
|
|
With knees and hips flexed a lower level of the flexed knee indicates what condition?
|
hip dysplasia
|
|
With knees and hips flexed a lower level of the flexed knee indicates what condition much less common than hip dysplasia?
|
congenital short femur
|
|
What two tests are conducted for congenital instability of the hips?
|
Barlow and Ortolani tests
|
|
What imaging study is now done in conjunction with the Barlow or Ortolani test for congenital hip instability?
|
ultrasound
|
|
What test is used to determine if a dislocated hip can be readily dislocated?
|
Barlow test
|
|
What is the purpose of the Barlow test?
|
To determine if a dislocated hip can be readily dislocated
|
|
What is the purpose of the Ortolani test?
|
To determine if a dislocated hip can be readily reduced
|
|
If a hip remains dislocated for several weeks what hip ROM may become limited on the affected side?
|
hip abduction
|
|
What is the sign of a positive Ortolani test?
|
palpable "clunk" as the hip reduces
|
|
What are the 2 treatments for hip instability?
|
Pavlik harness or hip spica cast
|
|
The Pavlik harness or hip spica cast maintain hip reduction in what degree range of flexion?
|
90-120 degrees of hip flexion
|
|
What position and degree range does the pavlik harness or hip spica cast maintain the hip in?
|
90-120 degrees of flexion
|
|
How long are the pavlik harness or hip spica cast used to treat hip instabiility?
|
3-4 months
|
|
What is a complication of forced hip abduction in the brace or pavlik harness?
|
avascular necrosis of the hip
|
|
What is a ROM precaution of the pavlik harness or hip spica cast?
|
no forced abduction
|
|
What other range of motion is limited by the pavlik harness or hip spica cast?
|
adduction
|
|
What is the incidence of congenital tortecollis?
|
1 in 250
|
|
What side, right or left, comprises 75% of congenital torticollis?
|
right side
|
|
What is the olive sign?
|
a soft nontender enlargement in the sternocleidomastoid
|
|
What does the "olive" sign represent?
|
congenital torticollis
|
|
At what month range does congenital torticollis subside?
|
4-6 months
|
|
What is plagicephaly?
|
orbital assymetry
|
|
What 4 secondary deformities can occur with congenital torticollis?
|
*flattening of the ipsilateral face
*contralateral occipital flattening *orbital asymmetry *ipsilateral hip dysplasia |
|
What are 3 differential diagnoses for congenital torticollis?
|
*muscular fibrosis
*cervical hemivertebra *atlantoaxial rotary subluxation |
|
In right torticollis what direction is the head tilted?
|
right shoulder
|
|
In right torticollis what direction is the chin rotated to?
|
left
|
|
In left torticollis what direction is the head tilted?
|
left shoulder
|
|
In left torticollis what direction is the chin rotated to?
|
right
|
|
What is the most common cause of congenital torticollis?
|
sternocleidomastoid fibrosis
|
|
Sternocleidomastoid fibroisis is the most common cause of what congenital disorder?
|
congenital torticollis
|
|
The SCM is enlarged or smaller on the side toward which the head is laterally tilted?
|
enlarged on the side toward which the head is tilted
|
|
What condition can result from a cervical hemivertabrae?
|
congenital cervical scoliosis
|
|
X-rays of congential torticollis reveal rotation of what two vertabrae?
|
C1-C2
|
|
What is the mainstay treatment of congenital torticollis?
|
stretching
|
|
On what side of the crib is a mobile put for an infant with right torticollis?
|
right of the crib
|
|
On what side of the crib is a mobile put for an infant with left torticollis?
|
left of the crib
|
|
Failure to regain full cervical ROM in congenital torticollis will lead to what complication?
|
persistent facial assymetry
|
|
Surgery is performed most successfully in congenital torticollis if the child is less than what age?
|
12
|
|
What is the finding in subluxation of the radial head (nursemaid's elbow)?
|
the radial head and neck are displaced distal to the annular ligament
|
|
What two motions reduces a subluxation of the radial head?
|
supination and extension of the forearm
|
|
Is the xray normal in subluxation of the radial head?
|
yes
|
|
What is little leaguer's elbow?
|
medial epicondylitis
|
|
What is the cause of medial epicondylitis?
|
repetitive stress on the apophysis of the medial humeral epicondyle ossification center
|
|
What is the stress vector causing medial epicondylitis?
|
valgus stress on the elbow
|
|
What is Osgood-Schatter's disease?
|
traction apophysitis of the anterior tibial tubercle
|
|
What is a traction apophysitis of the anterior tibial tubercle?
|
Osgood Schlatter's disease
|
|
Where is the knee pain in Osgood-Schlatter's, anterior or posterior knee?
|
anterior knee
|
|
What is the pathology of Osgood-Schlatter's disease?
|
inflammation/microfractures of the apophyseal cartilage between the tibial tubercle and the secondary ossification center of the tibial tuberosity where the patella tendon attaches
|
|
Where is the tenderness in Osgood-Schlatter's disease?
|
directly over the tibial tubercle
|
|
What are the 2 activity restrictions for Osgood-Schlatter's disease?
|
no deep knee bending
no knee flexion |
|
What is the most common cause of limping and pain in the hip of children?
|
transient toxic synovitis of the hip
|
|
What are two other causes of nontraumatic hip pain in children?
|
*Avascular necrosis of the proximal femur (Legg-Calve-Perthes disease)
*Epiphyiolysis (slipped captial femoral epiphyisis (SCFE) |
|
What is the most common hip disorder of preadolescent and adolescent children?
|
Slipped Capital Femoral Epiphysis (SCFE)
|
|
What is a risk factor for SCFE?
|
obesity
|
|
In what gender is SCFE more common?
|
boys
|
|
In what gender is Legg-Calve-Perthes more common?
|
boys
|
|
What is the incidence of nontraumatic hip pain in children?
|
1 in 750
|
|
What ROM is limited in acute transient toxic synovitis?
|
limited hip internal rotation
|
|
How long does acute transient toxic synovitis take to resolve?
|
3-5 days
|
|
What is the xray finding in acute transient toxic synovitis?
|
normal
|
|
What is the age range for acute transient toxic synovitis?
|
3-6 years old
|
|
What gender is affected more frequently in acute transient toxic synovitis?
|
boys
|
|
Where is the pain in Legg Calves Perthes?
|
groin with radiation to anterior thigh
|
|
What 3 hip ROMs are limited in Legg Calves Perthes?
|
internal rotation
extension abduction |
|
What are 2 xray finding in Legg Calves Perthes?
|
*small ossified femoral head
*widening of hip joint space |
|
What are 2 risk factors of SCFE?
|
*obesity
*delayed secondary sex characteristics |
|
What is the resting position of the hip apparent in SCFE?
|
external rotation
|
|
What are 2 hip ROM limitations in SCFE?
|
internal rotation
abduction |
|
What are 2 xray findings in SCFE?
|
*growth plate wider and irregular
*narrow epiphysis |
|
What is the preferred treatment for SCFE?
|
surgical pinning
|
|
What is the age range for SCFE?
|
9-15 years old
|
|
In what what racial group is SCFE more common?
|
blacs
|
|
In what gender is SCFE more common?
|
boys
|
|
What is the prognosis in SCFE?
|
variable
|
|
What are two criteria for a good prognosis in Legg Calve Perthes disease?
|
*< 6 years old
< 50% femoral head involvement |
|
Involvement of what part of the femoral head indicates a poorer prognosis in Legg Calve Perthes?
|
lateral femoral head
|
|
What percent of children have a second episode of acute transient toxic synovitis?
|
<10%
|
|
What is the inheritence pattern of idiopathic scoliosis?
|
autosomal dominant
|
|
When is the most common onset of idiopathic scoliosis?
|
adolescence
|
|
What is the curve pattern in idiopathic infantile scoliosis?
|
left thoracolumbar
|
|
What is the curve pattern in idiopathic juvenile scoliosis?
|
right thoracic or double curve
|
|
What is the curve pattern in idiopathic adolescent scoliosis?
|
right thoracic
|
|
What is the male:female ratio in adolescent scoliosis?
|
1:1
|
|
Whose scoliosis worsens more commonly, males or females?
|
females, 8-10x more frequently
|
|
30% of infants with congenital scoliosis may have one of what 2 other congenital deformities?
|
*unilateral renal agenisis
*spinal cord abnormality |
|
Congenital scoliosis is an additional finding in what acronym syndrome?
|
VATER
|
|
Acquired scoliosis is most commonly seen in what 4 congenital disorders?
|
cerebral palsy
spinal bifida muscular dystrophy spinal muscular atrophy |
|
Scoliosis is uncommon in children with who are able to do what activity?
|
walk
|
|
In what form of cerebral palsy is acquired scoliosis most common?
|
spastic quadreplegia
|
|
What two activities is a spastic quadraplegic CP patient with acquired scoliosis unable to perform?
|
stand or walk
|
|
At what progression milestone in Duchenne's muscular dystophy is acquired scoliosis more common?
|
When the child becomes wheelchair bound
|
|
Acquired scoliosis is very common in children with spina bifida who do not have neurologic function below what level?
|
Thoracolumbar level
|
|
What is the Adams's test? What is a positive test?
|
a forward bending test; positive if there is a prominence on the posterior trunk corresponding to the convex side
|
|
What xray view is used to measure a cobb angle?
|
PA
|
|
To measure the cobb angle a straight line is drawn along the superior endplate of what vertabrae?
|
most tilted cephalad vertabrae
|
|
To measure the cobb angle a straight line is drawn along the inferior endplate of what vertabrae?
|
most tilted inferior caudal vertabrae
|
|
At what degree of angulation curve range do pulmonary function test abnormalities appear in scoliosis?
|
50-60%
|
|
What is the most common pulmonary function test abnormality in scoliosis?
|
decreased vital capacity
|
|
There is a decreased sized hemithorax on the concave or convex side of scoliosis?
|
convex side
|
|
What idiopathic scoliosis degree range requires bracing?
|
20-40 degrees
|
|
What neuromuscular scoliosis degree range requires surgery?
|
20-40 degrees
|
|
Surgery is indicated in cerebral palsy scoliosis at what degree?
|
60 degrees
|
|
What is the radiographic criterion for Scheurmann's disease?
|
3 consecutive vertabrae are wedged >5 degrees
|
|
Whta is the preferrred brace for Scheurmann's disease?
|
TLSO
|
|
What are two other x-ray findings in Scheurmann's disease?
|
*Schmorl's nodes
*irregular endplates |
|
What is the presenting symptom in spondylolisthesis?
|
painful lumbar flexion
|
|
What form of spondylolisthesis is most common in adults?
|
degenerative
|
|
What is the most common level for pediatric spondylolisthesis?
|
L5-S1
|
|
What are the two most frequent types of spondylolisthesis in children?
|
dysplastic
isthmic |
|
What is the most common type of pediatric spondylolisthesis?
|
isthmic
|
|
Isthmic spondylolisthesis occurs at the site of a previous what?
|
pars spondylolysis
|
|
What type of spondylolisthesis is associated with severe neurologic deficits?
|
dysplastic
|
|
What is the defect in dysplastic spondylolisthesis?
|
lengthening of lamina
|
|
Spondylolisthesis is more common in males or females?
|
males
|
|
Spondylolisthesis progresses more commonly in males or females?
|
females
|
|
How much more common (multiple range) is spondylolisthesis is males than females?
|
2-4x
|
|
In children with spondylolisthesis what percentage slippage requires fusion?
|
>50%
|
|
What type of spondylolisthesis is more likely to cause compression on the cauda equina?
|
dysplastic
|
|
What is the most common type of connective tissue disease in children?"
|
juvenile rheumatoid arthritis
|
|
What are the two diagnostic criteria of juvenile rheumatoid arthritis?
|
arthritis >6 weeks
onset<16 years old |
|
What are the 5 types of juvenile arthritis?
|
*polyarticular RF negative
*polyarticular RF positive *pauciarticular Type 1 *pauciarticular Type 2 *Systemic onset juvenile arthritis |
|
What is the most common subtype of juvenile arthritis?
|
pauciarticular type 1 (early onset) 30% of all children with JRA
|
|
What are the two diagnostic criteria for polyarticular JRA?
|
5 or more joints involved in
first 6 months |
|
What percentage of all JRA is polyarticular RF negative?
|
25%
|
|
Polyarticular JRA is more common in boys or girls?
|
girls
|
|
What is the main complaint in polyarticular RF negative?
|
stiffness
|
|
What joint is involved in 50% of children with polyarticular JRA?
|
hip
|
|
Hip involvement is common in what subtype of polyarticular JRA?
|
RF negative
|
|
What is the hip pathology causing late disability in children with polyarticular RF negative?
|
erosion of the femoral head
|
|
Polyarticular RF positive is symmetric or asymmetric?
|
symmetric
|
|
What is the minimum age of onset for polyarticular RF positive?
|
11 years old
|
|
What are two findings other than symmetric involvement in RF positive polyarticular RF positive?
|
subcutaneous nodules
erosions |
|
What is the most common type of JRA?
|
pauciarticular
|
|
What is the maximum number of joints affected in pauciarticular JRA?
|
4
|
|
What are the two subtypes of pauciarticular JRA?
|
Type 1 early onset
Type 2 late onset |
|
Pauciarticular Type 1 is more common in boys or girls?
|
girls
|
|
What is the maximum age of onset for pauciarticular type 1?
|
4 years old
|
|
Pauciarticular type 1 accounts for what percentage of all children with JRA?
|
30%
|
|
What is the primary ocular complication in pauciarticular type 1?
|
iridocyclitis
|
|
What is the demographic (gender and age range) of pauciarticular JRA type 2?
|
boys 9-10 years old
|
|
With what haplotype is pauciarticular type 2 associated in 90% of cases?
|
HLA-B27
|
|
What is a late complication of pauciarticular type 2 with HLA-B27 positive?
|
ankylosing spondylitis
|
|
What is Still's disease?
|
systemic-onset JRA
|
|
What is the least common form of JRA?
|
systemic-onset JRA
|
|
Large or small joints are most often involved with Still's disease?
|
small joints
|
|
What are 3 constitutional symtoms of Still's disease?
|
*spiking fevers
*rash *hepatosplenomegaly |
|
What is a cardiac complication of Still's disease
|
pericarditis
|
|
In systemic-onset JRA what symptoms can precede arthritis?
|
systemic symptoms i.e.
acute spiking fevers hepatosplenomegaly lymphadenopathy myalgia fatigue pericarditis |
|
What JRA involves the SI joint?
|
pauciarticular type II (i.e. HLA-B27)
|
|
What percentage of Iridocyclitis in pauciarticular type 1 is chronic?
|
50% chronic
|
|
What percentage range of Iridocyclitis in pauciarticular type 2 is acute?
|
10-20% acute
|
|
What is the only JRA that is RA positive?
|
polyarticular RF positive
|
|
What 2 JRAs are ANA negative?
|
pauciarticular type 2 and systemic-onset (Still's Disease)
|
|
Whta 3 JRA can have a positive ANA?
|
polyarticular RF neg
polyarticular RF pos pauciarticular type 1 |
|
What JRA has the highest likelihood of ANA positive?
|
polyarticular RF pos
|
|
What JRA has the highest likelihood of an ultimate morbidity of severe arthritis?
|
polyarticular RF pos
|
|
What is the HLA of polyarticular RF pos?
|
HLA DR4
|
|
What JRA has a risk of vision loss?
|
pauciarticular type 1
|
|
Systemic features of RA are more common in adults or children?
|
children
|
|
What is the only JRA that is predominately males?
|
pauciarticular type 2
|
|
Large joint are invovled more frequently with children of adults with RA?
|
children
|
|
What is the wrist deviation of children with JRA?
|
ulnar deviation at the wrist
|
|
What wrist loss of ROM is present with JRA?
|
loss of wrist extension
|
|
What is the finger deviation of of children with JRA?
|
radial deviation of the fingers
|
|
At what joint does radial deviation of the fingers occur in children with JRA?
|
MCP
|
|
What is finger deformity in children with JRA?
|
finger flexion
|
|
What is more common in children with RA, bursitis or tenosynovitis?
|
tensynovitis
|
|
Rheumatoid nodule occur more frequently in adults or children with RA?
|
adults
|
|
The cervical spine is involved more frequently in adults or children with RA?
|
children
|
|
Periarticular bone demineralization is seen radiographically once what percentage of demineralazation occurs?
|
50%
|
|
What percentage of children improve with NSAID therapy?
|
50%
|
|
What are the 3 NSAIDs approved for use in children?
|
naproxen
ibuprofen tolmentin |
|
What percentage range of children receiving gold salts showed improvement?
|
60-70%
|
|
What are 3 side effects of gold salts?
|
rash
proteinuria bone marrow suppression |
|
What is more efficacious oral or IM gold?
|
IM
|
|
What anti-malarial drug is most commonly used for JRA?
|
hydroxychloroquine
|
|
What is the JRA remission percentage range with D-penicillamine?
|
60-70%
|
|
What are the two mechanisms of action of cyclosporine?
|
*blocks production of interleukin-2
*blocks synovial T-cells |
|
What med is used for children with refractory JRA disease?
|
azothioprine
|
|
What med can reduce the use of steroids?
|
azothioprine
|
|
Do systemic steroids reduce symptoms or cause remission, or both?
|
only reduce symptoms
|
|
Reye's syndrome is more of risk with aspirin use for JRA when there is also an infection with what two viral agents?
|
varicella
influenza |
|
What is a CNS complication of ibuprofen?
|
asceptic meningitis
|
|
What is a skin complication of naproxen?
|
cutanea tarda
|
|
What is a cognitive complication of indomethacin?
|
poor attention span
|
|
What is an ocular side effect of hydroxycholoquine?
|
macular degeneration
|
|
What is an immune side effect of d-penicillamine?
|
bone marrow suppression
|
|
Use of what medication class should be avoided with methotrexate use?
|
NSAIDs
|
|
NSAIDs should not be used with methotrexate because of an increased risk of what side effect?
|
bone marrow suppression
|
|
What is the cause of joint pain?
|
stretching of the joint capsule
|
|
What pediatric pain questionnaire takes into account the child's cognitive level of development?
|
Varni/Thompson
|
|
What is the ideal water temperature range for JRA heat treatments?
|
90-100 degrees F
|
|
What is the depth of heat penetration using a hot pack?
|
1 cm
|
|
What is the degree range and bias (flexion or extension) of wrist splinting in children with wrist RA?
|
15-20 degrees extension
|
|
Subluxation of the atlantoaxial joint can occur in RA with the erosion of what ligament?
|
transverse ligament
|
|
The erosion of the cervical spine transverse ligament can lead to the subluxation of what joint in children with RA?
|
atlanto-axial joint
|
|
The TMJ is involved in what percentage of children with JRA?
|
50%
|
|
What 2 shoulder ROMs are limited in children with JRA?
|
abduction and internal rotation
|
|
What shoulder ROM is limited in adults with RA?
|
external rotation
|
|
What percentage of the normal elbow flexion ROM is required for ADLs?
|
>90%
|
|
What wrist ROM is lost early in JRA?
|
wrist extension
|
|
What bias wrist contracture develops in children with JRA?
|
flexion contracture
|
|
Hyperextension (swan-neck deformity) of the PIP is more common in adults or children with RA?
|
adults
|
|
What deformity is the PIP hyperextension?
|
swan-neck
|
|
What is the Swan Neck deformity?
|
PIP hyperextension
|
|
What deformity is flexion at the PIP and hyperextension at the DIP?
|
Boutannaire's deformity
|
|
What is Boutannaire's deformity?
|
flexion at the PIP and hyperextension at the DIP
|
|
At what joint does radial deviation of the fingers occur?
|
MCP
|
|
What are the two ROM biases for hip flexion contracture in children with JRA?
|
internal rotation and adduction
|
|
What are the two ROM biases for hip flexion contracture in adults with RA?
|
external rotation and abduction
|
|
What joint is involved in 50% of children with polyarticular RF neg JRA?
|
hip
|
|
What degree of knee flexion minimizes intra-articular pressure?
|
30 degrees
|
|
What is the gait of a child with JRA with foot/ankle involvement?
|
flat foot gait
|
|
What part of the gait cycle is affected by MTP involvement?
|
push off
|
|
What joint in the foot is most commonly affected by JRA?
|
MTP
|
|
What ACR class has limited ability to perform ADLs?
|
Class IV
|
|
What fraction of children with JRA go into remission?
|
2/3rds
|
|
What is the percentage range of death from JRA in children?
|
2-4%
|
|
Involvement of what joint indicates a poor outcome?
|
hip
|
|
Involvement of small or large joints indicates a poor outcome?
|
small joints
|
|
Early or late age onset of disease in JRA indicates a poor outcome?
|
late
|
|
RF-positive or negative indicates a poor outcome in JRA?
|
RF positive
|
|
Timely or delay in treatment indicates a poor outcome in JRA?
|
delay in treatment
|
|
Juvenile onset seronegative spondyloarthropathies are more common in boys or girls?
|
boys
|
|
What are the 4 seronegative spondyloarthropathies?
|
*anklyosing spondylitis
*reactive arthritis (Reiter's) *arthritis with IBS *psoriatic arthritis |
|
What is the haplotype associated with seronegative spondyloarthropathies?
|
HLA-B27
|
|
Above what age is anklyosing spondylities more common?
|
8 years old
|
|
What pecentage of white patients with AS are HLA-B27 positive?
|
90%
|
|
What percent children with AS have peripheral joint involvement?
|
82%
|
|
What peripheral joints are most commonly invovled in juvenile AS?
|
lower extremities, especially the hip
|
|
What feature of AS occurs more commons in children than adults?
|
enthesitis
|
|
What is enthesitis?
|
pain at the insertion of tendon to bone
|
|
What is pain at the insertion of tendon to bone?
|
enthesitis
|
|
What are 3 types of symptoms of seronegative spondyloarthropathies?
|
arthritis
enthesitis tenosynovitis |
|
What joint involvement is necessary for a diagnosis of juvenile AS?
|
x-ray evidence bilateral SI joint involvement
|
|
What is an indicator of poor outcome in juvenile AS?
|
hip involvement
|
|
What is the triad of reactive arthritis (Reiter's syndrome)?
|
assymetric arthritis
urethritis conjunctivitis |
|
Is the arthritis is Reiter's syndrome symmetric or assymetric?
|
assymetric
|
|
What two joint are most commonly involved in reactive (Reiter's) arthritis?
|
knee or ankle
|
|
What is the most common gender and age for reactive arthritis?
|
boys greater than 8 years old
|
|
What 5 organisms most commonly are involved with reactive arthritis?
|
chlamydia
salmonella shigella yersinia |
|
What percentage range of children with UC or Crohn's suffer from arthritis?
|
10-20%
|
|
Is psoriatic arthritis symmetric or assymetric?
|
assymetric
|
|
What are 3 associated symptoms of psoriatic arthritis?
|
nail pitting
hyperkeratosis anterior uveitis |
|
What is associated with a poor functional outoome in psoriatic arthritis?
|
positive ANA
|
|
What percent of SLE begins in childhood?
|
20%
|
|
What is the male:female ratio of SLE?
|
4.5:1
|
|
How many of the 11 diagnostic criteria must be present for a diagnosis of SLE?
|
at least 4
|
|
What 2 rashes are diagnositic criteria of SLE?
|
discoid rash
malar rash |
|
What 4 positive immunoserology tests are diagnositic criteria of SLE?
|
LE cells
antinative DAN antibodies anti-SM antibodies false positive test for syphilis |
|
What 4 "itis"es are diagnostic criteria of SLE?
|
nephritis
pericarditis or pleuritis non-erosive arthritis encephalitis |
|
Is the arthritis of SLE erosive or nonerosive?
|
nonerosive
|
|
Is positive or negative ANA a diagnoistic criterion of SLE?
|
positive ANA
|
|
What ulceration is a diagnostic criterion of SLE?
|
oral or nasal mucocutaneous ulceration
|
|
What eye condition is a diagnostic criterion of SLE?
|
photosensitivty
|
|
What blood disorder is a diagnostic criterion of SLE?
|
cytopenia
|
|
List all 11 diagnostic criteria of SLE?
|
discoid rash
malar rash cytopenia positive ANA pericarditis or pleuritis encephalopathy nephritis arthritis positive immonserology (LE cells, false positive test for syphlis photosensitivity oral of nasal mucocutaneous ulceration |
|
What fraction of children have a malar rash?
|
1/3
|
|
What is a malar rash?
|
erythematous rash over the bridge of the nose and cheeks
|
|
What is the most important of the SLE diagnostic criteria in determining outcome in children?
|
nephritis
|
|
What percentage of children with SLE have nephritis?
|
75%
|
|
Can joint deformities develop in children with SLE?
|
yes, nonerosive joint deformities
|
|
What 6 conditions of SLE are associated with a poor outcome?
|
*hematuria
*proteinura *hypertension *pulmonary hypertension *chronic active disease *biopsy-proven proliferative glomerulonephritis |
|
What three structures are involved in juvenile dermatomyositis?
|
muscle
skin subcutaneous tissues |
|
What are 3 clinical features of juvenile dermatomyositis?
|
*vasculitis
*calcinosis *no association with malignancy in childhood |
|
What is the most common age range for juvenile dermatomyositis?
|
5-14 years old
|
|
What gender is more commonly affected with juvenile dermatomyositis?
|
girls
|
|
Weakness in juvenile dermatomyositis is proximal or distal?
|
proximal
|
|
What is the classic rash of juvenile dermatomyositis?
|
periorbital heliotropic rash
|
|
What is the EMG finding in juvenile dermatomyositis?
|
inflammatory myopathy
|
|
What enzymes are elevated in juvenile dermatomyositis?
|
muscle enzymes
|
|
What are 8 common constitutional symptoms associated with juvenile dermatomyositis?
|
fever
malaise muscle tenderness weight loss arthritis dyspnea dyspahgia EKG changes |
|
What is the mainstay treatment of juvenile dermatomyositis?
|
steroids
|
|
Over what time frame is the steroid taper in juvenile dermatomyositis?
|
2 years
|
|
What is the purpose of physical therapy in juvenile dermatomyositis?
|
to prevent contractures
|
|
What is the pathological hallmark of scleroderma?
|
fibrosis
|
|
What is the average age of onset range for scleroderma?
|
8-10 years old
|
|
What is the localized from of scleroderma?
|
mophea
|
|
Mophea is only present in what tissue?
|
skin
|
|
What is CREST syndrome?
|
Calcinosis
Raynaud's Esophogeal dysfunction Sclerodactyly Telangiectasia |
|
What is the organism causing Lyme disease?
|
borrelia burgdorferi
|
|
What class of microbe is borrelia burgdorferi?
|
spirochete
|
|
What tick transmits borrelia burgdorferi?
|
deer tick
|
|
What is the name of the deer tick that transmits borrelia burgdorferi?
|
ixodes dammini
|
|
Erythema migrans is characeristic of what condition?
|
lyme disease
|
|
What is the appearance eythema migrans?
|
round red skin lesion with central clearing
|
|
What are 5 initial constitutional symptoms of lyme's disease?
|
fever, fatigue, headache, myalgia, and stiff neck
|
|
What are 3 late phase conditions of lyme's disease?
|
arthritis, cardiac, and neuorlogical disease
|
|
What is the cardiac condition in lyme's disease?
|
heart block
|
|
What percent of children develop a chronic inflammatory arthritis in lyme's disease?
|
10%
|
|
What are 3 antibiotics used to treat lyme's disease?
|
doxycylcine
amoxicillin erythromycin |
|
What antibiotic (and delivery method IV or p.o.) is used to treat late-stage Lyme's disease?
|
IV ceftriaxone
|
|
What is the age of onset of rheumatic fever?
|
>4 years old
|
|
What is the male:female ratio of rheumatic fever?
|
1:1
|
|
What 4 joints are most commonly in rheumatic fever?
|
knees, elbows, wrists, ankles
|
|
What are 5 associated findings in rheumatic fever/
|
carditis
fever rash chorea nodules |
|
Rheumatic fever is often preceded by what infection?
|
streptococcal infection
|
|
Diagnoisis of rheumatic fever is made by what criteria?
|
Jones criteria
|
|
What is the EKG change in rheumatic fever?
|
prolonged PR interval
|
|
What 5 conditions are considered the "major" in the Jones criteria?
|
carditis
polyarthritis chorea erythema marginatum subcutaneous nodules |
|
Prognosis in rheumatic fever is based on what organ involvement?
|
cardiac involvement
|
|
What two meds are the mainstays of rheumatic fever treatment?
|
salicylates
corticosteriods |
|
Septic arthritis occurs in children under what age?
|
2 years old
|
|
What gender is affected more by septic arthritis?
|
boys
|
|
What joint is affected by the transient synovitis of 3-10 year old boys?
|
hip
|
|
Where is the referred pain in the transient synovitis of 3-10 year old boys?
|
thigh or knee
|
|
Bacterial septic arthritis accounts for what percentage of all childhood arthritis?
|
6.5%
|
|
Bacterial septic arthritis in children in usually monoarticular or polyarticular?
|
monoarticular
|
|
What are the two most common pathogens in bacterial septic arthritis?
|
haemopholis influenzae
staph aureus |
|
What is the most common organism in newborn septic arthritis?
|
staph aureus
|
|
What is the most common organism in 2 months-2 years old septic arthrtis?
|
h. influenzae
|
|
What is the most common organism in the older than 2 years septic arthritis?
|
staph aureus
|
|
What is the most common organism causing septic arthritis in sexually active adolescents?
|
gonococcal disease
|
|
Hemophilias are associated with defecienies of what three factors?
|
factors VIII, IX, or XI (8,9,11)
|
|
On what chromosome are factors 8,9, and 11 carried?
|
X chromosome
|
|
Hemophilia A is a deficiency of what factor?
|
factor VIII
|
|
Hemophilia B is also call what?
|
Christmas hemophilia
|
|
Hemophilia B is a deficiency of what factor?
|
factor IX
|
|
Hemophilia C is a deficiency of what factor?
|
factor XI
|
|
Deficiency of factor VIII is what hemophilia?
|
A (classic)
|
|
Deficiency of factor IX is what hemophilia?
|
B (christmas)
|
|
Deficiency of factor XI is what hemophilia?
|
C
|
|
What is the hallmark sign of hemophilia?
|
hemarthrosis
|
|
Can hemarthrosis in hemophilia occur spontaneously?
|
yes
|
|
What are three symptoms of hemarthrosis?
|
pain, swelling, and decreased ROM
|
|
What are three joint changes associated with repeated hemarthroses?
|
osteoporosis
muscle atrophy fixed, non-functional joint |
|
What is the mainstay treatment of hemophilia A during bleeding episodes?
|
Factor VIII replacement
|
|
What are two local treatment during a hemophilia bleeding episode?
|
cold and pressure
|
|
Early treatment with factor VIII can prevent what two poor joint outcomes?
|
disability and deformity
|
|
Severe hemophilia is defined as factor activity less than what percentage?
|
<1%
|
|
Moderate hemophilia is defined as factor acitivity greater than what percentage?
|
>1%
|
|
Mild hemophilia is defined as factor activity greater than what percentage?
|
>5%
|
|
What percentage of hemophiliacs are currently infected with HIV from factor transfusions?
|
30%
|
|
What forms of hepatitis are transmissilble by clotting factor concentrate?
|
hepatitis B and C
|
|
What is Kawasaki's disease?
|
systemic vasculitis
|
|
What is the most common age range for Kawasaki's disease?
|
<4 years old
|
|
What are the 9 diagnostic criteria of Kawasaki's disease?
|
high fever>5 days
strawberry tongue red, chapped lips pharyngeal erythema conjunctival injection edema of hands or feet erythema of palms or soles truncal rash cervical lymphadenopathy |
|
Where are the 5 erythemas of Kawasaki's disease?
|
eyes (conjunctiva)
lips tongue throat palms, soles |
|
Where is the edema in Kawasaki's?
|
hands or feet
|
|
Greater than how long (days) must the fever last in the diagnositic criteria of Kawasaki's?
|
>5 days
|
|
What is the #1 cause of nonmotor vehicle deaths in children ages 1 through 4?
|
burns
|
|
What is the #2 cause of nonmotor vehicle death in children ages 4-14?
|
burns
|
|
Children younger than 4 have a higher risk of death in burns independent of what factor?
|
burn size
|
|
What type of injury is an important predictor of burn mortality?
|
inhalation injury
|
|
What type of burns is the single most common type of pediatric burn injury?
|
scald burn
|
|
With scald burn there is increased suspicion of what?
|
child abuse or neglect
|
|
What percentage of burns are a result of abuse?
|
8%
|
|
What is the rule of "9"s for children?
|
9% is taken from the legs and added to the head of a child<1 year old. For each subsequent year 1% is returned to the legs unitil age 9.
|
|
What is a partial thickness burn?
|
epidermis plus partial elements of the dermis
|
|
What is a full-thickness burn?
|
all epidermal and dermal elements
|
|
Name 2 characteristics of a full-thickness burn
|
anesthetic, avascular
|
|
What color is a full-thickness burn?
|
white
|
|
What color is a partial-thickness burn?
|
red (shallower) or white (deeper), depending on depth
|
|
What total body surface area is an indication for a burn hospitalization?
|
20%
|
|
What total body surface area in children or the elderly is an indication for burn hospitalization?
|
10%
|
|
What percentage full-thickness is an indication for burn hospitalization?
|
5%
|
|
Burns to any of what 6 areas is an indication for a burn hospitalization?
|
eyes, face, hands, feet, genitalia
|
|
What type of pulmonary burn is an indication for hospitalization?
|
inhalation
|
|
What cause of burn is an indication for hosptilization?
|
elecrical
|
|
Comfortable positions for burn patients promote what poor outcome?
|
contractures
|
|
What is the contracture preventing position for neck burns?
|
extension
|
|
What is the contracture preventing position for anterior axilla burns?
|
90 degress abduction, neutral rotation
|
|
What is the contracture preventing position for posterior axilla burns?
|
shoulder flexion
|
|
What is the contracture preventing position for elbow/forearm burns?
|
elbows extended, forearm supinated
|
|
What is the contracture preventing position for wrist burns?
|
15-20 degrees extension
|
|
What is the contracture preventing position for MCP burns?
|
70-90 degrees flexion
|
|
What is the contracture preventing position for IP burns?
|
full-extension
|
|
What is the contracture preventing position for palmar burns?
|
all joints full extension, thumb radially abducted
|
|
What is the contracture preventing position for chest burns?
|
straight, no lateral or anterior flexion
|
|
What is the contracture preventing position for hip burns?
|
extension, 10 degrees abduction, neutral rotation
|
|
What is the contracture preventing position for knee burns?
|
extension
|
|
What is the contracture preventing position for ankle burns?
|
90 degrees dorsiflexion
|
|
When is splinting initiated in the pediatric burn patient?
|
skin tightening
|
|
Do small children tend to lose strength or joint mobility with extended joint immobilization?
|
no
|
|
What type of ROM PT is recommended for children with burns, passive, active, or active assisted ROM?
|
active or active assisted
|
|
What burn-treatment modality can cause premature closure of epiphyseal plates?
|
ultrasound
|
|
What 2 modalities are used as additions to scar therapy?
|
fluidotherapy
paraffin |
|
What should not be used by neck burn patients while lying supine?
|
pillows
|
|
Where is the most common location for decubiti in children with neck burns?
|
occiput
|
|
What is the most common burn-related contracture in pediatric burn patients?
|
axilla
|
|
What splint is used for successfully in pediatric axilla burns?
|
airplane splint
|
|
What is the correct splint position for dorsal hand burns?
|
MCP flexion 70-90 degrees, IP extension, radial abduction of thumb
|
|
What is the correct splint position for volar hand burns?
|
MCP/IP full extension, fingers abducted, palmar abduction of thumb
|
|
What is the correct splint position for dorsal foot burns?
|
ankle and toes plantar flexion
|
|
What is the correct position for sole or foot burns?
|
ankle dorsiflexion, toes neutral
|
|
Cancer is what number cause of death in the US?
|
#2
|
|
What is the most common type of cancer in children?
|
leukemia
|
|
In what brain fossa do 50% of pediatric brain cancers develop?
|
posterior
|
|
What is the second most common type of childhood cancer?
|
brain tumors
|
|
What location pediatric brain tumor presents with increased ICP and seizures?
|
supratentorial
|
|
Supratentorial brain tumors present with what 2 symptoms?
|
increased ICP and seizures
|
|
What location tumors present with increased ICP and cerebellar signs?
|
cerebellar and 4th ventricle tumors
|
|
Cerebellar and 4th ventricle tumors present with what 2 signs?
|
increased ICP and cerebellar signs
|
|
What are 3 symptoms of brain stem tumors?
|
ataxia
CN palsies hemiparesis |
|
In what decade is the peak incidence of Hodgkin's disease?
|
3 decade
|
|
What is the most common presentation of Hodgkin's disease in children?
|
painless cervical adenopathy
|
|
What virus MAY cause Burkitt's lymphoma?
|
Epstein-Barr
|
|
What 2 bones are usually affected by Burkitt's lymphoma?
|
facial bones
mandible |
|
What type of cells massively proliferate in Burkitt's lymphoma?
|
lymphoid cells
|
|
From what 2 cell types does neuroblastoma develop?
|
sympathetic ganglia
adrenal medulla |
|
What is the third most common pediatric neoplasm in children <5years old?
|
neuroblastoma
|
|
What age is the peak incidence of neuroblastoma?
|
2 years old
|
|
Presentation of a neuroblastoma in what area is associated with a poor prognosis?
|
abdominal area
|
|
Neuroblastoma is the third most common pediatric neoplasm in what age group?
|
< 5 years old
|
|
What are the 3 most common etiologies of abdominal masses in early childhood?
|
hydronephrosis
neuroblastoma Wilm's tumor |
|
Where does a Wilm's tumor orginate?
|
kidney
|
|
What is the most common malignant soft tissue tumor in children?
|
rhabdomyosarcoma
|
|
With what condition is rhabdomyosarcoma associated?
|
neurofibromatosis
|
|
In what two areas is rhabdomyosarcoma commonly found?
|
head and neck
|
|
How is rhabdomyosarcoma diagnosed?
|
biopsy
|
|
Metastasis to what structure is uncommon in children?
|
bone
|
|
What are the 2 most common bone tumors in children?
|
#1 osteosarcoma
#2 Ewing's sarcoma |
|
What is the most common age range for osteosarcoma or Ewing's?
|
> 10 years old
|
|
What is the most common site of osteosarcoma?
|
distal femur
|
|
What are the 2nd and 3rd most common sites for osteosarcoma?
|
proximal tibia
proximal humerus |
|
What part of long bones is the most common site for osteosarcoma?
|
metaphysis
|
|
Ewing's sarcoma most common occurs in what part of the bone?
|
diaphysis
|
|
What is the most common malignant ocular tumor?
|
retinoblastoma
|
|
What is the age range for >90% of retinoblastomas?
|
< 5 years old
|
|
What is the most common type of pediatric leukemia?
|
actue lymphoblastic leukemia
|
|
What is the peak age range for pediatric leukemia?
|
2-5 years old
|
|
What are 2 risk factors for childhood leukemia?
|
chromosomal abnormalities
immune deficiency |
|
What two tumors occur commonly in the epihysis?
|
chondoblastoma
giant cell tumor |
|
What 5 tumors (malignant and benign) occur in the metaphysis?
|
osteosarcoma
aneurysmal bone cyst chondromyxoid fibroma osteochondroma unicameral bone cyst |
|
What 2 tumors (malignant and benign) occur in the diaphysis?
|
Ewing's sarcoma
eosinophilic granuloma |
|
What is the leading cause of death in childrn > 1 year old in the US?
|
TBI
|
|
What is the annual incidence of TBI in children (per 100,000)?
|
185/100,000
|
|
What is the death rate of children (per 100,000) from TBI?
|
10/100,000
|
|
What is the second leading cause of death in children in US?
|
leukemia
|
|
What is the leading and second leading cause of TBI in children?
|
#1 MVA
# 2 falls |
|
After direct impact, what force is the cause of brain injury?
|
initial deceleration or shearing forces
|
|
Shearing forces cause what 2 types of brain injury?
|
diffuse axonal injury
multiple punctate hemorrhages |
|
What injury is NOT indicative of the severity of brain injury?
|
skull fracture
|
|
What is a contrecoup injury?
|
cerebral contusion distant from point of impact
|
|
What neurological developmental issue in children may result is a greater risk of shearing injury?
|
incomplete myelination
|
|
Brain injury secondary to increased rotational forces is a result of what fact of the pediatric population?
|
relatively large head
|
|
What percentage of children who sustain a TBI also suffer other injuries?
|
50%
|
|
What percentage range of TBI children also have a SCI?
|
5-10%
|
|
What are the 2 most common etiologies of pediatric brachial plexus injuries?
|
trauma
obstetrical complications |
|
What nerve roots are damaged in an upper trunk brachial plexopathy?
|
C5 C6
|
|
What is the mechanism of injury in an upper trunk brachial plexopathy?
|
sudden traction to the neck
|
|
What is an upper trunk brachial plexopathy also called?
|
Erb-Duchenne palsy
|
|
What is a lower trunk brachial plexopathy also called?
|
Klumpke paralysis
|
|
What is the mechanism of injury in Klumpke's paralysis?
|
violent upward pull of shoulder
|
|
What 2 nerve roots are damaged in Klumpke's paralysis?
|
C8 and T1
|
|
What is the most common cause of Klumpke's paralysis?
|
fall onto hyperabducted shoulder
|
|
What syndrome is associated with Klumpke's paralysis?
|
Horner's syndrome
|
|
What ganglion may be damageed in Klumpke's paralysis?
|
superior cervical sympathetic ganglion
|
|
What mechanism of injury is rare in Klumpke's paralysis?
|
obstetric
|
|
What are 2 signs of Klumpke's paralysis?
|
small hand muscle atrophy
claw hand deformity |
|
In Erb's palsy shoulder adduction is caused by weakness in what two muscles?
|
deltoid and suprapinatus weakness
|
|
In Erb's palsy shoulder internal rotation is caused by weakness in what 2 muscles?
|
teres minor and infraspinatus weakness
|
|
In Erb's palsy elbow extension is caused by weakness in what 2 muscles?
|
bicep and brachioradialis weakness
|
|
In Erb's palsy forearm pronation is caused by weakness in what 2 muscles?
|
supinator and brachioradialis weakness
|
|
In Erb's palsy wrist flexion is cuased by weakness in what 2 muscles?
|
extensor carpi radialis longus and extensor carpi radialis brevis weakness
|
|
What is the position of the arm in Erb's palsy?
|
Waiter's tip
|
|
What is the position of the shoulder in Erb's palsy?
|
adducted and internally rotated
|
|
What is the position of the elbow in Erb's palsy?
|
extended
|
|
What is the position of the forearm in Erb's palsy?
|
prontated
|
|
What is the position of the wrist in Erb's palsy?
|
flexed
|
|
A GCS of what range is considered severe and the patient is comotose?
|
3-8
|
|
What is the GCS for a mild injury?
|
13-15
|
|
What is the GCS for a moderate injury?
|
9-12
|
|
What is a higher (less severe injury) in GCS? extension or flexion to pain stimulus?
|
flexion
|
|
What is the GCS for spontaneous eye opening?
|
4
|
|
What is the GCS for eye opening to speech?
|
3
|
|
What is the GCS for eye opening to pain?
|
2
|
|
What is the GCS for no eye opening?
|
1
|
|
What is the GCS for verbal response oriented?
|
5
|
|
What is the GCS for verbal response confused conversation (irritable cry)?
|
4
|
|
What is the GCS verbal response inappropriate words (cries to pain)?
|
3
|
|
What is the GCS verbal response incomprehensible speech (moans to pain)?
|
2
|
|
What is the GCS verbal response none?
|
1
|
|
What is the best upper limb motor response obeys commands?
|
6
|
|
What is the best upper limb motor response localizes pain (withdraws to touch)?
|
5
|
|
What is the GCS best upper limb motor response withdraws to pain?
|
4
|
|
What is the GCS best upper motor response flexion to pain?
|
3
|
|
What is the GCS best upper motor response extension to pain?
|
2
|
|
What is the GCS best upper motor response none?
|
1
|
|
What is the infant modification for GCS oriented?
|
coos, babbles
|
|
What is the infant modification for GCS confused conversation?
|
irritable cry, consolable
|
|
What is the infant modification for GCS inappropriate words?
|
cries to pain
|
|
What is the infant modification for GCS incomprehensible sounds?
|
moans to pain
|
|
What is the infant modification best upper limb motor response for obeys commands?
|
normal movements
|
|
What is the infant modification best upper limb motor response for localizes pain?
|
withdraws to touch
|
|
What deficit is more common in children than adults post TBI?
|
dystonia
|
|
Damage to olfactory bulb and tracts, temporal lobes, or subfrontal areas can results in what sensory deficit?
|
anosmia
|
|
Basilar skull fracture is associated with what CN injury?
|
CN VIII (vestibulocochlear)
|
|
CN VIII can be injury in what location skull fracture?
|
basilar skull fracture
|
|
Deficits in what 2 areas are the largest cause of disability in pediatric TBI?
|
cognitive and communication
|
|
Children with what psychomotor condition are more likely to suffer TBI?
|
hyperactive
|
|
Agitation may result from damage to what 2 brain areas?
|
frontal lobes
subcortical areas |
|
Damage to frontal lobes or subcortical areas may result in what symptom?
|
agitation
|
|
In what timeframe (months) does the majority of improvement in IQ take place after TBI?
|
first 4 months
|
|
Deficits in impulse control and disinhibition can result from damage to what area?
|
frontal lobe
|
|
Where is ADH produced?
|
hypothalamus
|
|
What are 3 signs of SIADH?
|
decreased urinary output
hyponatremia decreased serum osmolarity |
|
What condition overwhelmingly affects more girls than boys post TBI?
|
precocious puberty
|
|
Cerebral salt wasting syndrome directly affects what function?
|
renal tubular function
|
|
What condition directly affects renal tubular function?
|
cerebral salt-wasting syndrome
|
|
Diabetes insipidus is marked by excessive or deficient production of ADH?
|
deficiency of ADH
|
|
Diabetes insipidus is marked by excessive or deficient water loss?
|
excessive water loss (hypovolemia)
|
|
What are 3 complications of prolonged intubation in pediatric TBI?
|
*tracheal stenosis
*tracheomalacia *vocal cord injury |
|
What is central autonomic dysfunction?
|
unexplained hyperthermia, HTN, diaphoresis, rigidity, decerebrate posturing, and tachypnea after TBI
|
|
What percent of children with TBI are affected by central autonomic dysfunction?
|
14%
|
|
Dysfunction of what 2 brain areas are thought to play are role in central autonomic dysfunction?
|
hypothalmus
brainstem |
|
What 2 outcomes are worse in patients with central autonomic dysfunction after TBI?
|
cognitive and motor
|
|
Heterotopic ossification is more common after TBI in children older than what age?
|
11
|
|
What are three risk factors for pediatric post-TBI heterotopic ossification?
|
>11 years old
more severe injury two or more extremity fractures |
|
What joints are most commonly affected by HO post TBI?
|
hips, knees
|
|
Is HO associated with a good or poor outcome in pediatric TBI?
|
poor
|
|
What is the definition of post-traumatic epilepsy in pediatric TBI?
|
two or more late (1-2 weeks post injury) seizures after TBI
|
|
Is prophylaxis with anti-epileptic drugs indicated in pediatric TBI?
|
no
|
|
What are two causes of ventricular system enlargement after pediatric TBI?
|
cerebral atrophy
obstruction of CSF flow (hydrocephalus) |
|
What percent of hospital costs in pediatric TBI is due to inpatient rehab?
|
47%
|
|
At 1 year what is the difference between children with minor TBI and age matched controls?
|
nothing
|
|
What are two indicators of poor outcome in pediatric TBI?
|
coma for > 90 days
profound brain injury |
|
What has a better prognosis, anoxic or traumatic brain injury?
|
traumatic
|
|
What percent of children unconcious for more than 90 days eventually regain conciousness?
|
75%
|
|
What cause of TBI predicts a worse survival and cognitive outcome in pediatric TBI?
|
abused children
|
|
What is a predictor of worse outcome for children with diffuse TBI?
|
younger age
|
|
Is CP a progressive or non-progressive lesion?
|
non progressive
|
|
Up to what age can CP occur?
|
up to age 3
|
|
What is the leading cause of childhood disability?
|
CP
|
|
What is the incidence of CP per 1000 births?
|
2-3
|
|
What percent range of CP occurs during the prenatal period?
|
70-80%
|
|
What prenatal deficiency can lead to diplegia?
|
iodine
|
|
What prenatal ingestion can lead to quadraplegia?
|
organic mercury
|
|
Premature birth at less than how many weeks is a risk factor for CP?
|
<32 weeks
|
|
What is the most comon antecedent of CP?
|
prematurity
|
|
Blood vessels and capillaries in the germinal matrix in the watershed zone next to what stucture are vulnerable to hemorrhage?
|
lateral ventricles
|
|
In the postnatal period what type of childabuse is a risk factor for CP?
|
shaken baby syndrome
|
|
What is a sign of shaken baby syndrome?
|
retinal hemorrhage
|
|
What two toxins are a risk factor for postnatal CP?
|
lead
organophosphates |
|
What congenital heart disease is a risk factor for postnatal CP?
|
tetralogy of fallot
|
|
Name 3 stroke syndromes with hemiplegia that are postnatal risk factors for CP?
|
sickle cell
AVM rupture Congenital heart disease (tetralogy of Fallot) |
|
What are the 4 acquired congenital infections (TORCH) that are prenatal CP risk factors?
|
toxoplasmosis
rubella CMV herpes |
|
What are 3 maternal causes/diseases for prenatal CP?
|
seizures
hyperthyroidism mental retardation |
|
What is the most common reason for a prenatal hypoxic-ischemic injury?
|
idiopathic
|
|
What are three maternal risk factors of prenatal CV?
|
multiple pregnancies
maternal bleeding maternal drug use |
|
What 3 complications of full term labor/birth are perinatal CP risk factors?
|
placenta previa
placental abruption meconium aspiration |
|
What LFT abnormality is a risk for perinatal CP?
|
hyperbilirubinemia
|
|
What type of CP can hyperbilirubinemia cause?
|
athetoid
|
|
In what two brain areas is bilirubin deposited leading to athetoid CP?
|
CN nuclei
basal ganglia |
|
What is the neurological damage from bilirubin called?
|
kernicterus
|
|
Is the bilirubin in kernicterus conjugated or unconjugated?
|
unconjugated
|
|
What are 3 causes of kernicterus?
|
Rh incompatibility
G6PD deficiency ABO incompatability |
|
Birth trauma to the brain usually causes what type of CP?
|
spastic hemiplegia
|
|
What brain infection is a risk factor for postnatal CP?
|
meningitis
|
|
What postnatal condition is a risk factor for CP?
|
anoxia causing anoxic encephalopathy
|
|
What are the three types of CP?
|
spastic
dyskinetic mixed type |
|
What is the most common type of CP?
|
spastic
|
|
Is spastic CP pyramidal or extrapyramidal?
|
pyramidal
|
|
Is dyskinetic CP pyramidal or extra-pyramidal?
|
extra-pyramidal
|
|
Spastic CP patients exhibit UMN or LMN symptoms?
|
UMN symptoms
|
|
Name 3 UMN signs or symptoms.
|
spasticity
hyperreflexia babinski |
|
What are the 5 subtypes of spastic CP?
|
spastic monoplegia
spastic diplegia spastic triplegia spastic quadraplegia spastic hemiplegia |
|
What is the most common type of CP?
|
spastic diplegia
|
|
What is the usual risk factor of those with spastic diplegia?
|
prematurity
|
|
What percent of all CP is spastic diplegia?
|
75%
|
|
Is spastic diplegia primarily UE or LE?
|
LE
|
|
What is the first sign of diplegia CP?
|
hypotonia
|
|
What type of hemorrhage is seen in diplegia CP?
|
intraventricular
|
|
At what gestational week age range is intraventricular hemorrhage seen in diplegia?
|
28-32 weeks
|
|
What are two brain MRI findings in spastic diplegia?
|
perventricular leukomalacia
posthemorrhagic porencephaly |
|
Where is the pathological damage causing LE spasticity in diplegic CP?
|
damage to pyrimidal fibers within the internal capsule
|
|
What 3 LE muscles are most commonly affected in diplegic CP?
|
hip flexors
hip adductors gastrocnemius |
|
What are the two gait patterns seen in diplegic CP?
|
scissoring gait with toe walking
|
|
What are two common eye findings in diplegia?
|
strabismus
visual defects |
|
What percent of diplegic CP have cognitive impairment?
|
30%
|
|
What percent range of diplegic CP have seizures?
|
20-30%
|
|
What extremities are affected in spastic triplegia?
|
bilateral LEs and one UE
|
|
What is the gait pattern of spastic triplegia?
|
scissoring with toe walking
|
|
What is a common cause of spastic quadraplegia?
|
difficult delivery with evidence of perinatal asphyxia
|
|
What percentage of spastic quadraplegia has a prenatal origin?
|
50%
|
|
What is the classic brain MRI finding in a preterm spastic quadraplegia?
|
periventricular leukomalacia
|
|
In spastic quadraplegia what type of posturing is seen?
|
opisthotonic posturing
|
|
In what percentage of spastic quadraplegia are seizures present?
|
50%
|
|
In spastic hemiplegia what is usually more involved, the arm or the leg?
|
the arm
|
|
In spastic hemiplegia is the lesion usually unilateral or bilateral?
|
unilateral
|
|
What is the common brain MRI finding in premature spastic hemiplegia?
|
periventricular leukomalacia
|
|
At what month age range is hemiparesis evident in spastic hemiplegia?
|
4-6 months old
|
|
What is the first sign of spastic hemiplegia?
|
hypotonia
|
|
What is an early sign of spastic hemiplegia?
|
premature preferential hand dominance
|
|
What is the average age of walking is spastic hemiplegia?
|
24 months
|
|
Sensory deficits in spastic hemiplegia occur on the ipsi or contralateral side?
|
ipsilateral side
|
|
In spastic hemiplegia, what type of motor deficits are common, gross, fine, or perceptual?
|
perceptual motor deficits
|
|
What are the three extrapyramidal deficits seen in dyskinetic CP?
|
tone
postura coordination |
|
What are slow, writhing movements of the extremities?
|
athetosis
|
|
What are abrupt, irregular jerky movements of the head and neck and extremities?
|
chorea
|
|
What is the combination of athetois and choreiform movements?
|
choreoathetoid
|
|
What movements predominate in choreathetoid?
|
athetoid
|
|
What is a slow rhthymic movement with tone changes generally found in the trunk and extremities?
|
dystonia
|
|
What is uncoordinated movements associated with nystagmus, dysmetria, and wide-based gait?
|
ataxia
|
|
Ataxia increases or decreases with purposeful movement?
|
increases with purposeful movement
|
|
In ataxia, muscle tone is normal or abnormal during sleep?
|
normal
|
|
What are some pseudobulbar signs?
|
dysarthria
dysphasia drooling oromotor dyskineias |
|
Dysarthria, dysphasia, drooling, and oromotor dyskineias are examples of what kind of involvement?
|
pseudobulbar
|
|
What are two associations seen with sensineuronal hearing loss in dyskinetic CP?
|
hyperbilirubinemia and neonatal jaundice
|
|
Hyperbilirubinemia and neonatal jaundice are associcated with what complication of dyskinetic CP?
|
sensorineuronal hearing loss
|
|
Sensorineuronal hearing loss is seen in what type of CP?
|
dyskinetic CP
|
|
What is the common type of mixed CP?
|
spastic athetoid
|
|
What brain artery is usually involved in spastic hemiplegia CP?
|
middle cerebral artery
|
|
Hemisphere dominance can be switched up until what age?
|
6 years old
|
|
What is the most common presentation of spastic hemiplegia?
|
failure to use involved hand
|
|
Diplegic CP is a result of the hypoperfusion of what area?
|
germinal matrix of the periventricular region
|
|
What brain area involved in diplegic CP resides in the border zone between penetrating branches of the major vessels?
|
periventricular white matter
|
|
The periventricular white matter is traveresed by what fibers?
|
descending fibers of the motor cortex
|
|
In the corona radiata UE fibers are lateral or medial?
|
lateral
|
|
In the corona radiata LE fibers are lateral or medial?
|
medial
|
|
What is the main brain MRI finding in diplegic CP?
|
periventricular leukomalacia
|
|
A large lesion in diplegic CP will involve what extremities?
|
upper and lower extremities
|
|
What CP category has the highest incidence of significant disability?
|
spastic quadraplegia
|
|
What is the cause of hemiplegia, ischemia, focal injury, or hypoxia?
|
focal injury
|
|
What is the cause of diplegia, ischemia, focal injury, or hypoxia?
|
ischemia
|
|
What is the cause of quadraplegia, ischemia, focal injury, or hypoxia
|
hypoxia
|
|
What is the usual specific cause of spastic quadraplegia?
|
perinatal asphyxia
|
|
Where is the major injury in spastic quadraplegia?
|
bilateral cortical zones
|
|
What motor cortex is more severely injured in spastic quadraplegia, medial or lateral?
|
medial motor cortex
|
|
What percentage of spastic quadraplegics are independent in ambulation and modified ADLs?
|
25%
|
|
What percentage of spastic quads require assisted ambulation?
|
50%
|
|
What percentage of spastic quads are completely disables?
|
25%
|
|
What is a poor prognostic indicator in spastic quads?
|
muscle tone
|
|
What are two early manifestations of extensor hypertonicity in spastic quads?
|
opisthotonus and precocious head raising
|
|
Lower extremities in spastic quad CP develop flexor or extensor spasticity?
|
extensor
|
|
In what type of CP are hip dislocations and scoliosis more common?
|
spastic quadraplegia
|
|
Upper or lower motor neuron signs are present in all 4 extremities in spastic quad CP?
|
UMN
|
|
What percentage of dyskinetic CP patients walk?
|
50%
|
|
Most dyskinetic CP patients who walk do so after what age?
|
3 years old
|
|
What gaze is paralyzed in dyskinetic CP?
|
upward gaze
|
|
What is the muscle tone at birth of children with dyskinetic CP?
|
hypotonic
|
|
What sensory deficit is common in dyskinetic CP?
|
sensorineural deafness
|
|
Seizures are present in what percentage of dyskinetic CP?
|
25%
|
|
What movement disorder finding is common in dyskinetic CP?
|
athetosis
|
|
In what 2 types of CP is strabismus most common?
|
quadraplegic and diplegic
|
|
What 2 brain structures are injured in dyskinetic and mixed CP?
|
basal ganglia and thalamus
|
|
What is the mechanism of injury in dyskinetic CP?
|
diffuse anoxia resulting in hypoxia of the basal ganglia and thalamus
|
|
Where are the initial symptoms in dyskinetic CP?
|
involuntary movements of the hands and fingers
|
|
What is the age range in dyskinetic CP when abnormal movements are seen in all 4 extremities?
|
18 months - 3 years
|
|
What percentage of children with dyskinetic CP have normal intelligence?
|
78%
|
|
In what type of CP is the UE more invovled than the LE?
|
dyskinetic
|
|
What are two complications of non-ambulatory dyskinetic CP patients?
|
hip dysplasia and scoliosis
|
|
What is tension athetosis?
|
limb movement increases tone
|
|
In what type of CP is tension athetosis seen?
|
dyskinetic CP
|
|
What 3 pseudobulbar symptoms are seen in dyskinetic CP?
|
drooling
dysarthria oromotor dyskinesias |
|
In what type of CP are pseudobulbar symptoms seen?
|
dyskinetic CP
|
|
What are the DTRs in dyskinetic CP?
|
normal to slightly increased
|
|
Is muscle tone normal or increased during sleep in dyskinetic CP?
|
normal
|
|
In what type of CP is nystagmus seen?
|
ataxic CP
|
|
What is an eye finding in ataxic CP?
|
nystagmus
|
|
What are the two features of the scissoring gait pattern?
|
hips flexed and adducted
knees flexed with valgus |
|
What two muscles are weak in spastic hemiplegia?
|
hip flexion and ankle dorsiflexion
|
|
What LE muscle is overactive in spastic hemiplegia?
|
posterior tibialis
|
|
Hip hiking or hip circumduction are seen in what type of CP?
|
spastic hemiplegia
|
|
What is the foot postion in stance phase in spastic hemiplegia?
|
supinated
|
|
What is the best indicator of ambulation in CP?
|
independent sitting by age 2
|
|
What is a poor prognostic sign in CP?
|
persistence of 3 or more primitive reflexes at 18-24 months
|
|
What type of CP is primarily congenital?
|
spastic hemiplegia
|
|
What type of CP is usually mild?
|
spastic monoplegia
|
|
What is the overall percentage of mental retartdation in CP?
|
50%
|
|
What type of CP has the highest rate of mental retardation?
|
spastic quadraplegia
|
|
What 2 types of CP have the lowest rate of mental retardation?
|
spastic hemiplegia and diplegia
|
|
What type of CP has the highest incidence of seizures?
|
spastic quadraplegia
|
|
What is the overall incidence of seizure in CP?
|
50%
|
|
What is the most common seizure type in CP?
|
grand mal with tonic clonic convulsions
|
|
What type of anopsia can occur in hemiplegic CP?
|
homonymous hemianopsia
|
|
Homonymous hemianopsia occurs in what type of CP?
|
spastic hemiplegia
|
|
What is the percentage rate range of strabismus in CP?
|
25-60%
|
|
In what two types of CP is strabismus most common?
|
diplegia and quadraplegia
|
|
What is the most common cause of sensory neural hearing loss in CP?
|
kernicterus
|
|
In what percentage of CP is sensorineural hearing loss found?
|
12%
|
|
What percentage of athetoids have some dysarthria?
|
most
|
|
What percentage of bilateral spastic CPs have dysarthria?
|
50%
|
|
What is a good indicator of intellectual potenital in CP?
|
2-3 word sentences by age 3
|
|
What are 3 causes of defective speech in CP?
|
*psuedobulbar palsy
*supranuclear spastic paralysis *dyskinetic incoordination of muscles innervated by lower CNs |
|
What pulmonary functions may be decreased in CP?
|
vital capacity
aerobic working capacity |
|
What type of pulmonary disease is seen in scoliosis?
|
restrictive pulmonary disease
|
|
What are two GI problems seen in CP?
|
GERD, constipation
|
|
Oromotor problems are seen in what two types of CP?
|
spastic quadraplegia and dyskinetic
|
|
What is a oral side effect of phenytoin?
|
gingival hyperplasia
|
|
What are 4 behavioral problems seen in CP?
|
ADD, distractability, impulse control, hyperkinesis
|
|
What are 3 causes of sensorineural hearing loss in CP?
|
TORCH, kernicterus, and medications
|
|
What percentage of children with CP survive to adulthood?
|
90%
|
|
What are 3 factors that reduce the likelihood of independent living in CP?
|
mental retardation
seizures wheelchair dependency |
|
The Individuals with Disabilities Act mandates early intervention for those children who demonstrate developmental delays until what age?
|
3 years old
|
|
What school of PT is most widely used in CP?
|
Bobath (neurodevelopmental treatment -NDT)
|
|
What are two primary goals of Bobath (NDT)?
|
normalize tone
inhibit primitive reflexes |
|
What two deformities doos an AFO control?
|
equinus or equinovarus
|
|
A full length foot plate discourages what ROM?
|
toe flexion
|
|
What kind of support discourages stimulation to a particularly reflexogenic area of the foot?
|
metatarsal support
|
|
Nerve or motor point blocks are used to decrease spasticity most commonly in what 3 muscles?
|
adductor, hamstring, and gastrocnemius
|
|
What gait pattern does a nerve or motor point block reduce?
|
scissoring gait
|
|
What foot deformity does a nerve or motor point block correct?
|
equinovarus
|
|
How long is a phenol or alcohol block effective (month range)?
|
3-6 months
|
|
What deformity complication can occur as a result of a tibial nerve block?
|
calcaneo-valgus foot
|
|
What are two expected effects of an obturator nerve block?
|
reduce scissored gait
reduce adductor tone |
|
What are two expected effects of a sciatic block to the medial hamstrings (semimembranosus and semitendinosus)?
|
reduce crouch gait
reduce internal rotation deformities |
|
What block is used to reduce scissoring gait and reduce adductor tone?
|
obturator block
|
|
What block is used to reduce crouch gait and reduce internal rotation deformities?
|
sciatic branch block to the semimembranosus and semitendinosus
|
|
What are two expeceted effects of tibial blocks?
|
reduce plantar flexion tone
improve tolerance of AFOs |
|
What block is used to reduce plantar flexion and improve tolerance of AFOs?
|
tibial block
|
|
What is one expected effect of a femoral nerve block?
|
reduce spastic recurvatum
|
|
What block is used to reduce spastic recurvatum?
|
femoral nerve block
|
|
How does botulinum toxin work?
|
irreversibly blocks presynaptic release of ACh into the neuromuscular junction
|
|
When is the onset of botulinum toxin, in hour range?
|
24-72 hours
|
|
When, in weeks, is the peak effect of botulinum toxin?
|
2 weeks
|
|
What is a common side effect of a phenol or alcohol block?
|
dysesthesia
|
|
What neurosurgical procedure is used to decrease spasticity?
|
selective posterior rhizotomy
|
|
How does a selective posterior rhizotomy decrease spasticity?
|
decreases excitatory sensory input to the motor neurons
|
|
Which roots are stimulated in a selective posterior rhizotomy?
|
dorsal roots
|
|
What are 6 patient selection criteria for a selectvie posterior rhizotomy?
|
lack of dystonia or athetosis
functional strength selective motor control ages 3-8 lack of significant contractures cognitive preservation |
|
What are 5 complications of selective posterior rhizotomy?
|
hyptonia
weakness bladder dysfunction hip dislocation lordosis |
|
Why is hip dislocation a complication of selective posterior rhizotomy?
|
sparing of L1 leads to unbalanced hip flexor spasticity
|
|
Why is lordosis is complication of selective nerve rhizotomy?
|
sparing of L1
|
|
What two complications of selective posterior rhizotomy are temporary?
|
hypotonia
bladder dysfunction |
|
What LE spasticity can be used by a CP child to stand?
|
extensor spasticity
|
|
What structure is exposed in a selective posterior rhizotomy?
|
cauda equina (through a laminectomy)
|
|
On what receptors, and where, does of baclofen act?
|
GABA receptors in the spinal cord
|
|
What are 3 side effects of baclofen?
|
weakness, fatigue, confusion
|
|
What is a precaution of baclofen?
|
reduces seizure threshold
|
|
What is the drug of choice to reduce spasticy in SCI and MS?
|
baclofen
|
|
Baclofen is the drug of choice for what 2 conditions?
|
SCI and MS
|
|
Where is the site of action of dantrolene?
|
intrafusal and extrafusal skeletal muscle fibers
|
|
What is the mode of action of dantolene?
|
decreases release of calcium from sarcoplastic reticulum
|
|
What is a precaution of dantrolene?
|
hepatotoxicity
|
|
What is the drug of choice for spasticity of cerebral origin?
|
dantrolene
|
|
Dantrolene is the drug of choice for spasticity of what origin?
|
cerebral origin
|
|
Where are the three sites of action of benzodiazepines?
|
brainstem
reticular formation spinal cord |
|
What is the mode of action of benzodiazepines?
|
increase GABA binding thereby increasing presynaptic inhibition
|
|
What is a precaution of benzodiazepines?
|
tolerance and dependence
|
|
In what 2 conditions are benzodiazepines most helpful?
|
incomplete SCI and immediate post op in children with CP
|
|
Is clonidine an agonist or antagonist?
|
agonist
|
|
Where are the three sites of action of clonidine?
|
brain
brain stem substantia gelatinosa |
|
What are 3 side effects of clonidine?
|
bradycardia
hypotension depression |
|
Where is the site of action of tizanidine?
|
adrenergic receptors in spine and brain
|
|
What are the 2 modes of action of clonidine?
|
inhibits short latency of motor neurons; augmentation of presynaptic inhibition
|
|
What is the mode of action of tizanidine?
|
prevents release of excitatory amino acids from presynaptic terminal of spinal interneurons
|
|
What are 3 side effects of tizanidine?
|
dry mouth
sedation dizziness |
|
What are two precautions of tizanidine?
|
orthostatic hypotension
hallucination |
|
What are 4 conditions where tizanidine is helpful?
|
dystonia
torticollis blepharospasm stabismus |
|
Where is the site of action of botulinum toxin?
|
presynaptically at the neuromuscular junction
|
|
What is the mode of action of botulinum toxin?
|
prevents release of ACh into the NMJ by binding irreversibly to the presynaptic membranes of the ACh
|
|
What are 3 side effects of botulinum toxin?
|
weakness
cramping pain |
|
What are two precautions of botulinum toxin?
|
antibody formation
respiratory arrest |
|
Where is the site of action of phenol?
|
motor end plate of a peripheral nerve
|
|
How does phenol affect the nerve?"
|
denatures protein and causes axonal degeneration
|
|
What are 3 side effects of phenol?
|
dysesthesias
anesthesia cardiac arrhythmia |
|
What is one of the most common musculoskeletal complaints of adults with CP?
|
neck pain
|
|
Neck pain is more common in spastic or dyskinetic CP adults?
|
dyskinetic
|
|
What are 4 indicators of successful employment in CP patients?
|
*IQ>80
*ambulation with or without assistive device *speech hard to understand to understandable *hand use normal to requiring assistance |
|
What are 3 indicators of unemployable CP patients?
|
IQ<50
nonambulatory and nonoral requires assistance using hand |
|
What is the stimulus for the Moro (startle) reflex?
|
sudden neck extension - feeling of falling
|
|
What is the Moro response?
|
Shoulder abduction and extension then arm flexion and adduction
|
|
At what month range is the Moro suppressed?
|
4-6 months
|
|
In what 2 conditions does the Moro reflex persist?
|
CNS pathology
static encephalopathy |
|
What is the stimulus in the rooting reflex?
|
stroking around lips or mouth
|
|
What is the rooting response?
|
moving mouth and head toward stimulus in search of nipple
|
|
When, in months, is the rooting reflex suppressed?
|
4 months
|
|
What is the stimulus in the positive supporting reflex?
|
light pressure on plantar surface
|
|
What is the response in the positive supporting reflex?
|
legs extend for partial support of body weight
|
|
When is the positive supporting reflex suppressed (in months range)
|
3-5 months
|
|
What is the stimulus for the asymmetric tonic neck reflex?
|
head turning to side
|
|
What is the response of the asymmetric tonic neck reflex?
|
extremities extend on face side; flex on occiput side ("fencer position")
|
|
What is the age month range when the asymmetric tonic neck reflex is suppressed?
|
6-7 months
|
|
In what condition does the asymmetric neck reflex persist?
|
static encephalopathy
|
|
What are two possible stimuli in the symmetric tonic neck reflex?
|
neck extension
neck flexion |
|
What is the response to the neck flexion stimulus in the symmetric tonic neck reflex?
|
arms flex, legs extend
|
|
What is the response to the neck extension stimulus in the symmetric tonic neck reflex?
|
arms extend, legs flex
|
|
At what month range is the symmetric tonic neck reflex suppressed?
|
6-7 months
|
|
What is the stimulus for the palmar grasp reflex?
|
pressure on palm or stretching finger flexors
|
|
What is the response of the palmar grasp reflex?
|
flexion of all fingers
|
|
At what month age range is the palmar grasp reflex suppressed?
|
5-6 months
|
|
In what condition is the palmar grasp reflex absent?
|
LMN paralysis
|
|
In what condition does the palmar grasp reflex persist?
|
spasticity
|
|
What is the stimulus in the plantar grasp reflex?
|
pressure on sole distal to the metatarsal heads
|
|
What is the response in the plantar grasp reflex?
|
flexion of all toes
|
|
In what condition is the plantar grasp reflex absent?
|
LMN paralysis
|
|
In what condition does the plantar grasp reflex persist?
|
spasticity
|
|
What is the stimulus in the automatic neonatal walking reflex?
|
vertical support plantar contact
|
|
What is the response in automatic neonatal walking reflex?
|
alternating automatic steps with support
|
|
When is the automatic neonatal walking reflex suppressed in month range?
|
3-4 months
|
|
In what condition is the automatic neonatal walking reflex absent?
|
LMN paralysis
|
|
What is the stimulus in the placement or placing reflex?
|
contact on dorsum of foot or hand
|
|
What is the response in the placement or placing reflex?
|
extremity flexion to place hand or foot over an obstacle
|
|
By what age (years) is the placement or placing reflex suppressed?
|
age 1
|
|
When is the plantar grasp reflex suppressed, in months range?
|
12-14 months, when walking is acheived
|
|
In what 2 conditions is the placement or placing reflex absent?
|
LMN paralysis and LE spasticiy
|
|
What is the stimulus in the neck righting or body derotational reflex?
|
neck rotation in supine position
|
|
What is the response in the neck righting or body derotational reflex?
|
sequential body rotation from shoulder to pelvis toward direction of face
|
|
By what age in months is the neck righting or body derotational reflex suppressed?
|
4 months or when replaced by volitional rolling
|
|
What is the stimulus in the tonic labyrinthine reflex?
|
head postion in space - strongest at 45 degrees from horizontal supine or prone
|
|
What is the response to the supine stimulus (45 degrees head) in the tonic labyrinthine reflex?
|
extensor tone
|
|
What is the response to the prone stimulus (45 degrees head) in the tonic labyrinthine reflex?
|
flexor tone
|
|
When, in month range, is the tonic labyrinthine reflex suppressed?
|
4-6 months
|
|
What reflex is associated with scissoring?
|
positive supporting
|
|
What reflex is an early sign of lower extremity spasticity?
|
positive supporting
|
|
What is the second most common childhood abnormality after CP?
|
spina bifida
|
|
What is another name for spina bifida?
|
myelodysplasia
|
|
What is the most common childhood disorder of the spine?
|
spina bifida
|
|
In what country is the incidence of spina bifida the highest?
|
British Isles
|
|
In what country is the incidence of spina bifida the lowest?
|
Japan
|
|
Is spina bifida more common in males or females?
|
females
|
|
What season of conception is most associated with spina bifida?
|
spring
|
|
What two anticonvulsants are associated with spina bifida?
|
valproic acid
carbemazepine |
|
What supplement reduces the incidence of spina bifida?
|
folic acid
|
|
When does neurulation take place in utero?
|
3-4 weeks
|
|
When is the post-neurulation phase (in week range)?
|
4-7 weeks
|
|
On what gestation day is the neural tube defect thought to occur?
|
day 26
|
|
By what day is the spinal cord formed?
|
day 53
|
|
What 2 serum and amniotic fluid measurement are used for prenatal spina bifida diagnosis?
|
alpha-fetoprotein and acetylcholinesterase
|
|
What is the reliability percentage of AFP?
|
80%
|
|
During what week range is AFP measurement most reliable?
|
weeks 13-15
|
|
When is aminotic testing of AFP most accurate (week range)?
|
weeks 16-18
|
|
Does amniocentesis detect closed neural tube defects without leakage of CSF?
|
no
|
|
When (week range) is ultrasound most reliable?
|
weeks 16-24
|
|
What are the two major types of spina bifida?
|
spina bifida occulta
spina bifida cystica |
|
What is a sign of spina bifida occulta in 50% of children with spina bifida occulta?
|
pigmented nevus, angioma, hirsute patch, dimple, or dermal sinus on the overlying skin
|
|
In what two spinal segments does spina bifida occulta usually occur?
|
lumbar and sacral
|
|
What form of spina bifida is associated with Arnold Chiari formations?
|
spina bifida cystica
|
|
What type of spina bifida herniates to the surface?
|
spinal bifida cystica
|
|
What term refers to any neural tube defect that is open the environment?
|
spina bifida aperta
|
|
Name 3 lesions that may be seen in spina bifida cystica
|
meningocele
myelomengingocele myelocele |
|
Is the motor paralysis seen in spina bifida cystica UMN or LMN?
|
LMN
|
|
What are the two spinal levels most commonly involved in spina bifida occulta?
|
L5 and S1
|
|
In what percentage range of the population is spina bifida occulta a normal variant?
|
5-10%
|
|
What are the two contents of a meningocele?
|
meninges
spinal fluid |
|
What two spinal segments are most commonly affedcted by meningocele?
|
lumbar
sacral |
|
In less than what percentage of spina bifida cystica does meningocele occur?
|
<10%
|
|
What are the neurologic signs in meningocele?
|
none
|
|
What are the 3 contents of a myelomeningocele?
|
meninges
spinal cord spinal fluid |
|
What is an associated finding in myelomenigocele?
|
arnold chiari malformation
|
|
What is a complication of the Arnold-Chiari malformation seen in over 90% of cases?
|
hydrocephalus
|
|
What is the most common type of spina bifida cystica?
|
myelomeningocele
|
|
What level range controls trunk flexion, abdominals and lower trunk extensors?
|
T6-12
|
|
What 3 muscle functions are controlled by T6-12?
|
trunk flexion
abdominals lower trunk extensors |
|
What type of paralysis occurs with a lesion at T6-12?
|
flaccid paralysis
|
|
What two spinal postions occur when a lesion is T6-12?
|
kyphosis
scoliosis |
|
What is the hip and knee position when a lesion is at T6-12?
|
flexion
|
|
The frog leg postion occurs when the lesion is at what level range?
|
T6-12
|
|
What is the foot deformity when the lesion is T6-12?
|
equinus
|
|
Early hip dislocation occurs when the lesion is at what level range?
|
L1-L3
|
|
L1-L3 lesions are associated with what 2 hip contractures?
|
hip flexion and adduction contractures
|
|
L1-L3 lesions are associated with what knee contractures?
|
knee flexion contracture
|
|
Name 3 contractures associated with a lesion at L1-L3.
|
hip flexion
hip adduction knee flexion |
|
Hip flexion, hip adduction, and knee flexion contractures are associated with lesions at what spinal range?
|
L1-L3
|
|
At what spina bifida levels is the bowel and bladder dysfunction?
|
all
|
|
Iliopsoas, hip flexion, hip adduction, quadriceps, and knee extension is controlled by what range spinal levels?
|
L1-L3
|
|
What level range spina bifida is associated with late hip dislocation?
|
L4-L5
|
|
Calcaneovarus or calcaneus foot (ankles dorsiflexed) is a result of what spina bifida level range?
|
L4-L5
|
|
What foot deformity is associated with a L4 L5 spina bifida level?
|
calcaneovarus or calcaneus foot (ankles dorsiflexed)
|
|
A L4-L5 spina bifida level is associated with an early or late hip dislocation?
|
late
|
|
A L1-L3 spina bifida level is associated with an early or late hip dislocation?
|
early
|
|
What knee contracture is associated with a L4-L5 spina bifida level?
|
knee extension contracture
|
|
What level range spina bifida is associated with pes cavus?
|
S1-S2
|
|
Clawing of the toes is associated with what spina bifida level range?
|
S1-S4
|
|
What is an Arnold Chiari II malformation?
|
downward displacement of the medulla and brainstem through the foramen magnum
|
|
Through what foramen does an Arnold Chiari II malformation herniate?
|
forament magnum
|
|
During what week of birth does an Arnold Chiari become symptomatic?
|
first week
|
|
What shunt is placed for a Arnold Chiari malformation?
|
ventriculoperitoneal
|
|
What is the most common complication following shunt placement?
|
infection
|
|
What is the second most common complication following an Arnold Chiari shunt placement?
|
obstruction
|
|
Hydrocephalus is correlated with defects most common in what spinal segment?
|
thoracic
|
|
What is a peripheral joint condition associated with spina bifida?
|
charcot joints
|
|
What are the 3 most common symptoms of a tethered cord?
|
weakness, scoliosis, and pain
|
|
What two benign spinal tumors are associated with spina bifida?
|
lipoma
fibrolipoma |
|
What is a tethered cord?
|
abnormal attachment of cord at distal end
|
|
What is associated with a saggital cleaveage of the spinal cord (diastematomyelia)?
|
bony spur
|
|
In what spinal segment is syringomyelia most common?
|
cervical spine
|
|
What type of cells line a syrinx?
|
glial cells
|
|
What is the imaging study of choice for a syrinx?
|
MRI
|
|
The frog leg postion occurs when the lesion is at what level range?
|
T6-12
|
|
What is the foot deformity when the lesion is T6-12?
|
equinus
|
|
Early hip dislocation occurs when the lesion is at what level range?
|
L1-L3
|
|
L1-L3 lesions are associated with what 2 hip contractures?
|
hip flexion and adduction contractures
|
|
L1-L3 lesions are associated with what knee contractures?
|
knee flexion contracture
|
|
What is the incidence percentage range of syringomyelia?
|
5-40%
|
|
In what spinal segement does a syrinx most commonly occur?
|
cervical spine
|
|
What are 6 signs/symptoms of a syrinx?
|
*deterioration of neurologic function
*pain *temperature sensory deficits *spasticity *hyperreflexia |
|
What is the most common single cause of death in spina bifida?
|
central respiratory dysfunction
|
|
What are 3 symptoms of central respiratory dysfunction in spina bifida?
|
stidor
central apnea aspiration |
|
What are 3 visual symptoms of spina bifida?
|
strabismus
lateral rectus palsy nystagmus |
|
What are 3 urinary malformations seen in spina bifida?
|
renal hypoplasia
horseshoe kidney solitary kidney |
|
Where are the 3 most common areas of skin breakdown in a non-ambulatory spina bifida patient?
|
sacrum
ischial tuberosities greater trochanters |
|
What is the most common area a skin breakdown in an ambulatory spina bifida patient?
|
soles of feet
|
|
What percentage of myelomeningocele patients have urinary dysfunction?
|
>90%
|
|
What type bladder is seen in myelomeningocele?
|
neurogenic
|
|
What urinary fibers are in the T10-L2 levels?
|
sympathetic adrenergic innervation
|
|
What urinary fibers are in the S2-4 levels?
|
parasympathetic cholinergic innervation
|
|
What urinary fibers are in S2-S5 leveles?
|
somatic innervation through the pudendal plexus
|
|
What type of bladder is seen most commonly in thoracic spinal bifida?
|
hypertonic (spastic) bladder
|
|
What type of bladder is seen in sacral lesions?
|
hypotonic (flaccid) bladder
|
|
Hypertonic (spastic bladder) is seen in lesions of what spinal segment?
|
thoracic
|
|
Hypotonic (flaccid) bladder is seen in lesions of what spinal segment?
|
sacral
|
|
Proteus urinary tract infections in spina bifida is associated with what condition?
|
calcinosis
|
|
Calcinosis in spina bifida is seen with what urinary tract infectious organism?
|
proteus
|
|
Voluntary somatic motor and sensory nerve supply to the external anal sphincter occurs via what spinal level range?
|
S2-4
|
|
Through what plexus do the S2-4 nerves supply voluntary somatic motor and sensory nerve supply to the external anal sphinctger?
|
pudendal plexus
|
|
What percentage of spina bifida children have fecal incontinence?
|
80%
|
|
In lesions above what level can an intact spinal reflex arc maintain sphincter tone despite absent rectal sensation?
|
above L2
|
|
What is the most common delayed self-care task in myelomeningocele?
|
independence in toileting
|
|
What 2 reflexes have been associated with a greater chance of bowel continenece?
|
bulbocavenosus
anocavernosus |
|
With what function has the bulbocavernosus or anocavernosus reflex been associated?
|
bowel continence in spina bifida
|
|
In spina bifida increased pressure on the hypothalamus can lead to what condition?
|
precocious puberty
|
|
In what percentage range is precocious puberty seen in spina bifida?
|
10-20%
|
|
Short stature in spina bifida is a result of what hormone deficiency?
|
growth hormone
|
|
What is the treatment for short stature in spina bifida?
|
gonadotropin releasing hormone
|
|
In spina bifida, what is the relationship between IQ and level of lesion?
|
Inverse relationship: higher the lesion, lower the IQ
|
|
Concentration and attention deficits are to what condition in spina bifida?
|
hydrocephalus
|
|
Does hydrocephalus alone exclude normal cognitive function?
|
no
|
|
What complication of hydrocephalus can lead to cognitive deficits?
|
meningitis
|
|
In spina bifida, higher scores are acheived on verbal tasks, written, math, or visual motor activities?
|
verbal tasks
|
|
When is the cystic lesion in spina bifida usually repaired?
|
first day of life
|
|
By what age are nearly all shunts in spina bifida revised?
|
age 5
|
|
What percentage range of spina bifida patients require shunting for hydrocephalus?
|
75-85%
|
|
Neurogenic bladder are seen in nearly all spina bifida patients, except those with what level lesions?
|
very low sacral
|
|
At what residual volume should intermitttent catheterization begin?
|
20ml or more
|
|
Boys can become independant in self-cath by what age?
|
5 years old
|
|
At what age is IVP recommended in a spina bifida patient?
|
2 years old
|
|
What class of meds is used in spina bifida to reduce detrusor contractions?
|
anticholinergics
|
|
What class of meds is used in spina bifida to increase outflow resistence?
|
alpha adrenergic agents
|
|
In what spinal segement are most spinal deformities in spina bifida?
|
thoracic
|
|
What is the cause of paralytic scoliosis?
|
loss of truncal support
|
|
What is a possible cause of rapidly progressive scoliosis in spina bifida?
|
tethered cord
|
|
What are two common complications of paralytic scoliosis?
|
hip dislocation
pelvic obliquity |
|
What hip condition is not treated as long as there is no restriction of ROM?
|
bilateral hip dislocation
|
|
What is the treatment for a gibbus deformity?
|
kyphectomy
|
|
Knee flexion contractures up to what degree are well tolerated in the ambulatory spina bifida patients?
|
up to 20 degrees
|
|
What are 3 complications of untreated unilateral hip dislocations?
|
pelvic obliquity
difficulty sitting decubitii |
|
What knee contractures are most common in thoracolumbar spina bifida patients?
|
flexion or extension contractures
|
|
What is the procedure performed for severe tibial torsion in spina bifida?
|
tibial osteotomy
|
|
What are 5 foot deformities commonly seen in spina bifida?
|
equinus
equinovarus calcaneal cavus rocker bottom |
|
What foot deformity is seen in upper lumbar or thoracic lesions?
|
rigid club foot
|
|
What procedure is performed for calcaneus foot deformity?
|
posterior transfer of tibialis anterior
|
|
Older than what age is a posterior transfer of tibialis anterior performed?
|
>5 years old
|
|
What is the surgical treatment for equinus deformity?
|
achilles tendon lengthening
|
|
What procedure is used to correct severe claw toe deformity and pes cavus?
|
flexor tenodesis or transfer and plantar fasciotomy
|
|
Flexor tenodesis or transfer and plantar fasciotomy are used to treat what two foot deformiites seen in spina bifida?
|
claw toe deformity and pes cavus
|
|
Achilles tendor lengthening is used to treat what spina bifida foot deformity?
|
equinus deformity
|
|
Posterior transfer of tibialis anterior is used to treat what spina bifida foot deformity?
|
calcaneus foot deformity
|
|
At what age is a bowel program started in spina bifida?
|
2 years old
|
|
What chemical allergy develops commonly in spina bifida patients?
|
latex allergy
|
|
What 2 tests are used to demostrate a latex allergy?
|
serum IgE antigen specific for rubber
radioallergosorbent test |
|
What is the increased risk of anaphylaxis in the operating room for children with spina bifida (Nx)?
|
500x
|
|
During the first 6 months, what development is normal is spina bifida?
|
motor development
|
|
At what month range do development delays become obvious?
|
6-12 months
|
|
By what month do children with thoracic lesions roll?
|
18 months
|
|
What is the lowest lesion that allows trunk control?
|
T12
|
|
A child can sit normally if what level is spared?
|
L4-5
|
|
What spine segment lesions require assistive devices for passive standing?
|
thoracic
|
|
If what level is spared may a child with spina bifida use an AFO?
|
L3
|
|
When do children with low lumbar lesions walk?
|
2 years old
|
|
What is the gait pattern of a child with a low lumbar lesion?
|
trendelenburg lurch and gastrocnemius limp
|
|
What is the mental age range required to learn crutch walking?
|
2-3 years old
|
|
Crutch walking can be acheived in what level lesions?
|
low thoracic and upper lumbar lesions
|
|
By what age range can low thoracic and upper lumbar lesion spina bifida patients be expected to crutch walk?
|
4-5 years old
|
|
What orthotic is used for spastic plantar flexors?
|
AFO
|
|
What percentage range of thoracic lesions acheive functional community ambulation?
|
0-33%
|
|
What percentage of high lumbar lesions acheive functional community ambulation?
|
31%
|
|
What percentage range of low lumbar lesions acheive functional community ambulation?
|
38%
|
|
What percentage of sacral lesions patients acheive functional ambulation?
|
100%
|
|
What percentage of lumbar lesions acheive functional community ambulation by the age 31?
|
95%
|
|
What are two early predictors of ambulation in spina bifida?
|
sitting balance and motor level
|
|
What are three unfavorable predictors of ambulation in
|
deformities of the spine
deformities of the LEs obesity |
|
When is wheelchair training begun in spina bifida?
|
during 2nd year
|
|
By what age is referral to preschool programs mandated?
|
3 years old
|
|
What is the unemployment rate percentage range of patients with spina bifida?
|
25-50%
|
|
What is a common complication among pregnant women with spina bifida?
|
premature labor
|
|
What is the highest level where male sexual function is preserved?
|
L5
|
|
What is the incidence of spina bifida with one affected parent?
|
4%
|
|
What is the cause of death in 40% of spina bifida patients between 5-30 years old?
|
urologic causes
|
|
Are neuromuscular diseases in children primarily UMN or LMN?
|
LMN
|
|
What is a characterstic LE muscular finding in Duchenne's muscular dystrophy and Becker's muscular dystrophy?
|
pseudohypertrophy of the gastroc
|
|
What is the leg appearance of a patient with hereditary motor sensory neuropathy?
|
stork or champagne bottle leg appearance
|
|
In what 3 conditions is hepatosplenomegaly seen?
|
metabolic myopathy
acid maltase deficiency types 3 and 4 glycogenosis |
|
What abdominal finding is characteristic in metabolic myopathy, acid maltase deficiency and types 3 and 4 glycogenosis?
|
hepatosplenomegaly
|
|
What is Gower's sign?
|
inability to rise from sitting postion on floor normally
|
|
The inability to rise from sitting postion on floor normally is called what?
|
Gower's sign
|
|
What muscle group is weak in Gower's sign?
|
pelvic girdle muscles (proximal muscle weakness)
|
|
In Gower's sign what is weak, hip flexors or extensors?
|
hip extensors
|
|
Describe the myopathic gait.
|
hyperlordotic, waddling gait
|
|
What two muscle groups are weak in a myopathic gait?
|
back and hip extensors
|
|
Why is hyperlordosis the compensatory postion in a myopathic gait?
|
It helps position the center of gravity posterior to the hip and stabilizes the hip in extension.
|
|
What is the postion of the ankle on initial contact during stance phase of a myopathic gait?
|
plantar flexion
|
|
Plantar flexion in myopathic gait during mid and late stance phase allows maintenance of knee extension and knee stability and places the center of gravity anterior or posterior to the knee?
|
posterior to the knee
|
|
What is the gait pattern of paralytic muscle disease?
|
toe walking
|
|
Toe walking is characteristic of what tpe of muscular disease?
|
paralytic muscular disease
|
|
In Duchenne's MD, the ankle usually develops a plantar flexion or dorsiflexion contracture?
|
plantar flexion contracture
|
|
How much more powerful are plantar flexors than dorsiflexors?
|
6x
|
|
A trendelenburg gait is produced by weakness of what muscle?
|
hip abductor weakness (gluteus medius)
|
|
In a trendelenburg gait, the trunk is bent laterally over the stance or swing phase hip?
|
stance phase hip joint
|
|
A steppage gait is caused by weakness of what muscles? Leading to what ankle position?
|
ankle dorsiflexion weakness
plantar flexion |
|
To clear the plantar flexed foot, in a steppage gait what is the position of the knee and hip?
|
hyperflexed knee and hip
|
|
Is Duchenne's x or y linked?
|
x linked
|
|
Where is the chromosome abnormality in Duchenne's?
|
Xp21
|
|
What protein is deficient in Duchenne's?
|
dystrophin
|
|
What % presence of dystrophin is diagnostic of Duchenne's?
|
<3%
|
|
What are 6 cardinal signs of Duchenne's?
|
waddling, lordotic gait
abnormal run Gower's sign inability to hop proximal muscle weakness calf hypertrophy |
|
What is a cardiac condition seen in Duchenne's?
|
cardiomyopathy
|
|
What lab value is grossly elevated in Duchenne's?
|
CK
|
|
What is the EMG pattern of Duchenne's
|
myopathic
|
|
What are 4 presenting symptoms of Duchenne's?
|
delay in walking
abnormal gait frequent falling difficulty climbing stairs |
|
In what muscle group is weakness first seen in Duchenne's?
|
neck flexors
|
|
What appears first pelvic or shoulder weakness?
|
pelvic weakness
|
|
In Duchenne's what is weaker, ankle dorsiflexion or plantar flexion?
|
dorsiflexion
|
|
In Duchenne's what is weaker ankle everters or inverters?
|
ankle everters
|
|
In Duchenne's what is weaker knee flexors or extensors?
|
knee extensors
|
|
In Duchenne's what is weaker hip flexors or extensors?
|
hip extensors
|
|
In Duchenne's what is weaker, hip adductor or abductors?
|
hip abductors
|
|
In Duchenne's what % of muscle power is lost by age 6?
|
50%
|
|
In Duchenne's what is average age of wheelchair dependency?
|
10 year old
|
|
In Duchenne's what muscle develop contractures, flexors or extensors?
|
flexors
|
|
Between what ages do 50% of Duchennes develop scoliosis?
|
12-15 years old
|
|
What pulmonary function test is a predictor of spinal deformity severity and restrictive pulmonary compromise?
|
forced vital capacity
|
|
Forced vital capacity is predictive of what two metrics in Duchenne's?
|
spinal deformity
restrictive pulmonary compromise |
|
An FVC of less than what % of normal is a contraindication to spinal arthordesis because of morbidity?
|
40%
|
|
In what two cardiac muscle cell types is dystrophin present?
|
myocardial cells
Purkinje cells |
|
In Duchenne's almost all patient demostrate EKG abnormalities in ages older than what?
|
>13 years old
|
|
At what age is cardiomyopathy first noticed in Duchenne's?
|
>10 years old
|
|
What 2 cardiac conditions are poor prognostic indicators in Duchenne's?
|
cardiomyopathy
systolic dysfunction |
|
Name 5 EKG abnormalities in Duchenne's?
|
Q waves in lateral leads
elevated ST poor R wave progression increased R/S ratio conduction defects |
|
Does Becker's have a later or earlier onset than Duchenne's?
|
later
|
|
Can the amount of dystrophin be normal in Becker's?
|
Yes
|
|
What is the abnormality of the dystrophin in Becker's?
|
increased or decreased molecular weight resulting in abnormal function
|
|
What are two presenting symptoms of Becker's?
|
difficulty running
cramps on exercise |
|
What are 3 cardinal signs of Becker's?
|
proximal muscle weakness
calf hypertrophy waddling gait, lordosis |
|
What is the EMG pattern of Becker's?
|
myopathic
|
|
What does the ultrasound show in Becker's?
|
increased echo
|
|
What is the CK in Becker's?
|
grossly elevated
|
|
Foci of atrophic fibers in Becker's resemble what abnormality?
|
denervation
|
|
What is the chromosome locus of Becker's?
|
Xp21
|
|
What muscle groups develop contracturs in Congenitial muscular dystrophy?
|
flexors
|
|
What are 3 ages stages of onset of congenital muscular dystrophy?
|
at birth
infancy early childhood |
|
What are 4 presenting symptoms of congenital muscular dystrophy?
|
hypotonia and weakness
fixed deformities variable sucking and swallowing delayed motor milstones |
|
What are 3 cardinal clinical signs of congenital muscular dystrophy?
|
general hypotonia or weakness
fixed deformities in relation to uterine position variable weakness or contractures |
|
What are 4 associated features of congenital muscular dystrophy?
|
intellectual retardation
dislocation of hips scoliosis hydrocephalus and fundal changes |
|
What type of fundal changes are seen in congenital muscular dystrophy?
|
Santavuori type
|
|
In Japan, what intellectual retardation type is seen in congenital muscular dystrophy?
|
Fukuyama type
|
|
What is the cause of death in congenital muscular dystrophy?
|
respiratory infection
|
|
Can CK be normal in congenital MD?
|
yes
|
|
What does muscle ultrasound show in congenital MD?
|
increased echo
|
|
What is the EMG pattern seen in congenital MD?
|
myopathic pattern
|
|
What is the inheritence pattern of congenital MD?
|
autosomal recessive
|
|
What are the two chromosomes of congenital MD?
|
9q 31-33
6q |
|
What is the typical foot deformity of congenital MD?
|
equinovarus
|
|
What are three weak muscles initially in fascioscapulohumeral dystrophy?
|
oculi
zygomaticus orbicularis ori |
|
What 4 muscles are spared in FSH?
|
masseter, temoporalis, extraocular, and pharyngeal muscles
|
|
What sensory deficit is more commn in FSH than normal?
|
sensory neural hearing deficit
|
|
What are 3 body habitus abnormalities seen in FSH (2 of the upper back and 1 of the spine)?
|
posterior and lateral scapular winging
high riding scapula hyperlordosis |
|
What respiratory muscles are more affected in FSH, inspiratory or expiratory muscles?
|
expiratory muscle weakness
|
|
What is the cognitive deficit in FSH?
|
none
|
|
What is the lifespan of FSH?
|
normal
|
|
What is a cardinal sign of FSH?
|
can't whistle
|
|
What is the chromosome for FSH?
|
4q35
|
|
What is the inheritence pattern of FSH?
|
autosomal dominant
|
|
What autosomal recessive MD resembles Becker's or Duchenne's?
|
limb girdle syndrome
|
|
What two muscles groups are predominately weak in Limb Girdle?
|
shoulder and pelvic girdle muscles
|
|
What are 3 presenting symptoms of Limb Girdle?
|
difficulty running, with gait, and climbing stairs
|
|
What happens to muscles upon exercise with limb girdle?
|
cramping
|
|
Name 3 cardinal symptoms in limb girdle.
|
abnormal gait
lordotic posture difficulty hopping and rising from floor |
|
What is the EMG pattern of limb girdle?
|
myopathic
|
|
What is the CK elevation range in limb girdle?
|
mild to gross
|
|
What is the gene in limb girdle?
|
15q
|
|
What is the inheritence pattern in limb girdle?
|
autosomal recessive
|
|
What disorder is at 19q13.1?
|
central core myopathy
|
|
What is the inheritence pattern of central core?
|
autosomal dominant
|
|
What anesthetic emergency is common in central core?
|
malignant hyperthermia
|
|
Malignant hyperthermia is common in what myopathy?
|
central core
|
|
What muscle fiber type is predominant in central core?
|
type 1
|
|
What is the oxidative and glycolytic description of type 1 fibers?
|
high oxidative
low glycolytic |
|
There is a lack of what type fibers in central core?
|
type II
|
|
A relative lack of type II fibers is characteristic of what myopathy?
|
central core
|
|
There is a relative lack of what enzymes in central core?
|
glycolytic
|
|
Is the weakness in central core progressive or non-progressive?
|
non-progressive
|
|
What is the inheritence pattern of minicore?
|
autosomal recessive
|
|
What muscle fibers predominate in minicore?
|
type 1
|
|
Is minicore progressive or non-progressive?
|
non-progressive
|
|
Is weakness in minicore predominantly proximal or distal?
|
proximal
|
|
The diaphragm is weak in what myopathy?
|
minicore
|
|
What is the respiratory risk in minicore?
|
noctural hypoventilation
|
|
What disease is carried on 1q21-q23?
|
nemaline myopathy
|
|
What is nemaline myopathy also called?
|
red-body myopathy
|
|
What is the inheritence pattern of nemaline?
|
autosomal recessive
|
|
What are two presenting symptoms of nemaline?
|
hypotonia and proximal weakness
|
|
What are the 3 possible inheritence patterns of myotubular?
|
autosomal dominant
autosomal recessive x-linked recessive |
|
What is the x-linked chromosome of myotubular?
|
Xq28
|
|
Where is the muscle cell nucleus in myotubular, peripheral or central?
|
central
|
|
Where is the muscle cell nucleus usually found, peripherally or centrally?
|
peripherally
|
|
What is the most common type of centronuclear myopathy?
|
myotubular
|
|
What are 4 presenting symptoms of autosomal myotubular myopathy?
|
hypotonia
delay in motor milestones ptosis weakness of external ocular muscles |
|
Severe neonatal hypotonia, dysphagia, and vent support at birth are symptoms of what myopathy?
|
x-linked myotubular myopathy
|
|
What myopathy presents with ptosis, external ocular muscle weakness, delay in motor milestones, and generalized proximal and distal weakness?
|
autosomal myotubular myopathy
|
|
What myopathy is characterized by delayed relaxation of muscles after contraction?
|
myotonic myopathies
|
|
What disease is characterized by myotonia, muscle weakness/wasting, cataracts, premature balding, cardiomyopathy, conduction defects, gonadal atrophy, and dementia?
|
myotonic muscular dystrophy
|
|
What are 2 characteristic facial feature of myotonic muscular dystrophy?
|
long thin face
temporal and masseter wasting |
|
Is the weakness in myotonic muscular dystrophy more proximal or distal?
|
distal
|
|
What muscular dystrophy has a characteristic long face and temporal and masseter wasting?
|
myotonic muscular dystrophy
|
|
What are two other names for myotonic muscular dystrophy?
|
steinert's disease or dystrophia myotonica
|
|
What are the 3 intial weaknesses in myotonic muscular dystrophy?
|
anke dorsiflexors, evertors, and inverters, and hand muscles
|
|
What dystrophy presents with hand and ankle weakness?
|
myotonic muscular dystrophy
|
|
What are 4 cardinal clinical signs of myotonic MD?
|
sustained grip myotonia
tongue percussion myotonia inability to bury eyelashes frontal balding, hatchet facies |
|
Sustained grip myotonia is characteristic of what disease?
|
myotonic MD
|
|
Percussion myotonia of the tongue is characteristic of what disease?
|
myotonic MD
|
|
Prognosis in myotonic MD depends on what two conditions?
|
cardiomyopathy
respiratory problems |
|
What onset is most common in myotonic MD?
|
adult onset
|
|
What form of myotonic MD is most severe?
|
congenital
|
|
What are the two EMG patterns seen in myotonic MD?
|
myotonia and myopathy
|
|
What are two finding on EKG in myotonic MD?
|
conduction defects
arrhythmia |
|
What are 3 findings on muscle biopsy in myotonic MD?
|
dystrophic changes
internal nuclei sarcoplasmic masses |
|
What is the inheritence pattern of myotonic MD and what is the chromosome?
|
autosomal dominant 19q13
|
|
Inability to bury eyelashes is characteristic of what disease?
|
myotonic MD
|
|
What is another name for myotonica congenita?
|
Thompsen's disease
|
|
In what myotonia disorder is myotonia the only or predominant feature?
|
myotonia congenita
|
|
What is the only or predominant feature in myotonia congenita?
|
myotonia
|
|
What 3 conditions/activities exacerbate symptoms in myotonia congenita?
|
rest
inactivty cold |
|
What condition is characterized by inability to release a grip?
|
myotonia congenita
|
|
In myotonia congenita muscle atrophy or hypertrophy is seen?
|
muscle hypertrophy
|
|
Sustained eye closure in an infant is a feature of what disease?
|
myotonia congenita
|
|
Stiffness in myotonia congenita is manifest after what activities or condition?
|
cold weather
upon waking period of rest after sustained activity |
|
What myotonia features myotonia following fright?
|
myotonia congenita
|
|
Myotonia congenita can overlap with what other condition is some families?
|
hyperkalemic periodic paralysis
|
|
What is the EMG pattern in myotonia congenita?
|
myotonic discharges
|
|
What are the findings on muscle biopsy in myotonia congenita?
|
normal
|
|
What is the gene in myotonia congenita?
|
7q35
|
|
What are 4 pharmacologic agents used during disabling symtoms of myotonia congenita?
|
quinine
procainamide phenytoin steroids |
|
What disease is characterized by hypotonia, dwarfism, diffuse bone disease, narrow palpebral fissures, and blepharospasm?
|
Schwartz-Jampel syndrome
|
|
Name 6 features of Schwartz-Jampel syndrome?
|
flat facies
micrognathia blepharospasm narrow palpebral fissures hypotonia dwafism |
|
What disease is characterized by severe hypotonia at birth, facial weakness, arthrogryposis, and swollowing difficulties?
|
congenital myotonic dystrophy
|
|
What disease does the mother of babies with congenital myotonic dystrophy have?
|
congenital myotonic dystrophy
|
|
What are 3 presenting symtoms of congenital myotonic dystrophy?
|
floppiness
poor sucking and swallowing respiratory insufficiency |
|
What are three associated features of congenital myotonic dystrophy?
|
talipes equinovarus
hydramnios thin ribs |
|
What is a cardiac feature of congenital myotonic dystrophy?
|
ventricular dilation with or without periventricular hemorrhage
|
|
What is a respiratory complication of congenital myotonic dystrophy?
|
respiratory insufficiency
|
|
Who is always affected in congenital myotonic dystrophy?
|
mother
|
|
At what milestone achievment is orthopedic intervention for talipes started?
|
standing
|
|
What is the deformity at the subtalar joint in talipes?
|
inversion
|
|
At what joint is there inversion in talipes?
|
subtalar
|
|
What is the deformity in talipes at the talonavicular joint?
|
adduction
|
|
In talipes at what joint is there an adduction deformity?
|
talonavicular
|
|
In talipes what is the deformity at the ankle joint?
|
plantarflexion
|
|
Another word for plantar flexion at the ankle.
|
equinus
|
|
What is the cause of transient neonatal myasthenia?
|
ACh receptor antibodies transferred from mother to fetus
|
|
What are 3 signs of transient neonatal myasthenia?
|
difficulty feeding
weakness hypotonia |
|
What is the duration (in weeks) of transient neonatal myasthenia?
|
3 weeks
|
|
What form of myasthenia has a high titer of ACh receptor antibodies?
|
juvenile myasthenia
|
|
What is the demographic of juvenile myasthenia?
|
adolescent girls
|
|
What are 4 clinical features of juvenile myasthenia?
|
ptosis
opthalmoplegia facial weakness swallowing difficulty |
|
What is the NCS hallmark of juvenile myasthenia?
|
fatigability of muscle after stimulation of peripheral nerve at 4 or 10 Hz
|
|
Response to what medication is used to confirm juvenile myasthenia?
|
IV edrophonium
|
|
What type of EMG is used to evaluate juvenile myasthenia?
|
single fiber EMG
|
|
What is the clinical hallmark of single fiber EMG in juvenile myasthenia?
|
minature endplate potentials
|
|
What two medications are used to treat neonatal myasthenia?
|
prostigmine or pyridostigmine
|
|
What medication is used to treat congenital or infantile myasthenia?
|
pyridostigmine
|
|
What are 5 treatments for juvenile myasthenia with antibodies to ACh receptors?
|
prostigmine, plasma exchange, thymectomy, qod steroid therapy, and azothioprine
|
|
In autoimmune myasthenia gravis, what is more affected proximal or distal muscles?
|
proximal
|
|
In autoimmune myasthenia what is more affected lower limbs or upper limbs?
|
upper limbs
|
|
What are 5 common presenting symptoms of autoimmune MG?
|
opthalmoparesis, ptosis, facial weakness, dysphagia, and proximal weakness
|
|
Response to what medication is used to confirm the diagnosis of autoimmune MG?
|
IV edrophonium
|
|
What class of medication is edrophonium?
|
anticholinesterase
|
|
What EMG study is used diagnose autoimmune MG?
|
repetitive nerve stimulation
|
|
What is the hallmark finding of repetitive nerve stimulation in the diagnosis of MG?
|
10% decrement of CMAP amplitudes at stimulation rates of 2-5 Hz over a train of 5 stimuli
|
|
What receptors are detected in 90% of patients with generalized MG and 50% with ocular myasthenia?
|
anticholinesterase receptors
|
|
What is the onset period for infantile botulism (days to months)
|
10 days - 6 months
|
|
What are 4 hallmark symptoms of infantile botulism?
|
acute onset hypotonia, dysphagia, constipation, and weak cry
|
|
What is the gag reflex in infantile botulism? increased or decreased
|
decreased
|
|
In infantile botulism are DTRs increased, decreased, or normal?
|
normal
|
|
What are 3 eye findings in infantile botulism?
|
ophthalmoplegia, ptosis, and pupillary dilation
|
|
In what bodily sample is the toxin of infantile botulism found?
|
stool
|
|
What is the agent causing infantile botulism?
|
clostridium botulinum toxin
|
|
What is another name for Guillan Barre?
|
Acute inflammatory demylinating polyradiculopathy (AIDP)
|
|
What are two prodrome infectons of AIDP?
|
upper respiratory or GI infection
|
|
What are 4 infectious agents causing the prodrome infections leading up to Guillan Barre (AIDP)?
|
mycoplasma, CMV, EBV, and Campylobacter jejuni
|
|
Is the onset weakness in GB distal or proximal?
|
distal
|
|
Is the onset weakness in AIDP UE or LE?
|
LE
|
|
What is the most common paralysis in GB?
|
facial paralysis
|
|
Is the facial paralysis in AIDP UMN or LMN?
|
LMN
|
|
When (in weeks) does paralysis peak in GB?
|
2 weeks
|
|
When in months is maximum recovery?
|
7 months
|
|
What are two treatments for AIDP in children?
|
plasma exchange and IV immune globulin
|
|
What is the prognosis in children without treatment in AIDP?
|
good
|
|
What is CIDP?
|
chronic inflammatory demyelinating polyradiculoneuropathy
|
|
What is the course in CIDP?
|
chronic relapsing
|
|
What are the H and F wave latencies in CIDP: normal , absent, or prolonged?
|
absent or prolonged
|
|
What are the conduction velocities in CIDP: slower, faster, or unchanged?
|
slower conduction velocities
|
|
What is the distal latency in CIDP: normal or prolonged?
|
prolonged
|
|
What does the NCS show in CIDP?
|
conduction block
|
|
What is the CMAP pattern in the EMG of CIDP?
|
temporal dispersion
|
|
What is HMSN?
|
hereditary motor sensory neuropathy
|
|
What is the prevalence of HMSN?
|
1 per 2500
|
|
What is the most common form of HMSN?
|
charcot marie tooth (HMSN 1)
|
|
What is the characteristic nerve biopsy finding in Charcot Marie Tooth (HMSN 1)?
|
onion bulb
|
|
What type of neuropathy on EMG is Charcot Marie Tooth?
|
hypertrophic demyelinating
|
|
What is the gene for Charcot Marie Tooth?
|
17p11.2-12
|
|
What type of neuropathy on EMG is Charcot Marie Tooth II?
|
axonal neuropathy
|
|
What is another name for Charcot Marie Tooth II?
|
HMSN 2
|
|
What are the amplitudes in Charcot Marie Tooth 2 on NCS: increased, decreased, or normal?
|
decreased amplitudes
|
|
What compartment of the leg atrophies in Charcot Marie Tooth II: anterior or posterior compartment?
|
anterior
|
|
What HMSN has a stork leg appearance?
|
HMSN 2 (Charcot Marie Tooth II)
|
|
What Charcot Marie Tooth has LESS involvement of the hands?
|
Type II
|
|
When is the onset of HMSN 3?
|
infancy or early childhood
|
|
What has an earlier age of onset Charcot Marie Tooth I or II?
|
CMT I
|
|
What is HMSN 3 also called?
|
Dejerine Sottas disease
|
|
What is the inheritence pattern of HMSN 4?
|
autosomal recessive
|
|
What is HMSN 4 also called?
|
Refsum's disease
|
|
Where is the initial weakness in Refsum's disease?
|
distal lower extremities
|
|
Where does weakness appear in HMSN 4 after the distal lower extremities?
|
distal upper extremities
|
|
Is the weakness in GB (AIDP) ascending or descending?
|
ascending from distal LEs
|
|
What are the DTRs in Refsum's?
|
decreased DTRs
|
|
What is the neurpathy caused by arsenic?
|
axonal or demylenating
|
|
In what 4 bodily samples can arsenic be measured?
|
blood, hair, nails, urine
|
|
What is the most common toxic neuropathy?
|
lead
|
|
Where the predominant weakness in lead toxicity: UEs or LEs?
|
LEs
|
|
What are 7 clinical findings in lead toxicity?
|
anorexia
nausea vomiting clumsiness seizures mental status changes papilledema |
|
What is the neurpathy of mercury toxicity?
|
distal motor axonal neuropathy
|
|
Use of what topical cleaning agent can result in mercury toxicity?
|
ammonia
|
|
What are 4 clinical findings in mercury toxicity?
|
encephalopathy
absent DTRs ataxic gait distal sensory paresthesias |
|
Exposure to what chemical may result in organophosphate toxicity?
|
insecticides or lubricants
|
|
What are 4 symptoms of organophosphate toxicity?
|
encephalopathy
sweating diarrhea constricted pupils |
|
What is a late effect of organophosphate toxicty?
|
motor polyneuropathy
|
|
What is the chemical involved in glue-sniffing?
|
n-hexane
|
|
What is the neuropathy of glue-sniffing?
|
distal motor and sensory demyelinating polyneuropathy
|
|
What toxic neuropathy has the following clinical features: distal weakness, absent DTRs, and foot drop?
|
vincristine
|
|
What are 3 clinical features of vincristine toxicity?
|
distal weakness, absent DTRs, and foot drop
|
|
What is the neurpathy of vincristine toxicity?
|
pure motor axonal polyneuropathy
|
|
What metabolic neuropathy involves peroneal innervated musculature?
|
end stage renal disease
|
|
Is the sensory neuropathy of end stage renal disease dermatomal or stocking glove?
|
stocking glove
|
|
What is the neuropathy of end stage renal disease?
|
distal motor and sensory polyneuropathy
|
|
What are 4 findings in the neuropathy of end stage renal disease?
|
loss of sensation, vibratory sense, and distal weakness, especially the musculature innervated by the peroneal nerve
|
|
What is the neuropathy of diabetes?
|
distal motor and sensory polyneuopathy
|
|
Is diabetic neuropathy axonal, demyelinating, or both?
|
axonal and demyelinating
|
|
What is the distribution of diabetic neuropathy?
|
stocking-glove
|
|
What motor neuron disorder shows both UMN and LMN signs/symptoms?
|
spinal muscular atrophy
|
|
What two cell types degenerate in spinal muscular atrophy?
|
anterior horn cells of the spinal cord and brainstem motor nuclei
|
|
What brain tract is spared in spinal muscular atrophy?
|
pyramidal tract
|
|
What chromosome is linked to spinal muscular atrophy?
|
5q13
|
|
What is the inheritence pattern of all 3 spinal muscular atrophy subtypes?
|
autosomal recessive
|
|
What is SMA type I also called?
|
Werdnig-Hoffman disease
|
|
What are 3 presenting symptoms of Werdnig-Hoffman?
|
*hypotonia
*generalized symmetrical weakness *sucking and swallowing difficulty |
|
In Werdnig-Hoffman what is weaker, UE or LEs?
|
lower extremities
|
|
What is more affected in Werdnig-Hoffman proximal or distal muscles?
|
proximal
|
|
What is the leg postion when supine of Hoffman Werdnig?
|
Frog-leg i.e. hip abducted and externally rotated
|
|
What 2 muscle weaknesses causes the diaphragmatic breathing seen in Werdnig-Hoffman (SMA I)?
|
abdominal and intercostal weakness
|
|
Fasciculations of what structure occur in 60% of patients with Werdnig-Hoffman?
|
tongue
|
|
What 2 muscles are spared in Werdnig-Hoffman?
|
extraocular and myocardium
|
|
Does the preservation of DTRs exclude the diagnosis of SMA?
|
no
|
|
What is the appearance of the abdomen in Werdnig-Hoffman?
|
protuberant abdomen
|
|
What is the shape of the chest in Werdnig-Hoffman?
|
Bell-shaped chest
|
|
What is the breathing pattern in Werdnig-Hoffman?
|
diaphragmatic breathing
|
|
What is the position of the legs in SMA-I?
|
internally rotated arms- jug handle posture
|
|
Within what time span do the majority of patients with SMA-I die?
|
within first year
|
|
What is the most commoncause of death in SMA-I?
|
pneumonia
|
|
Is the weakness in Werdnig-Hoffman progressive or non-progressive?
|
non-progressive
|
|
What is the motor nerve conduction velocity in SMA I?
|
normal or reduced
|
|
What is the CK in Werdnig Hoffman?
|
normal
|
|
What is the EMG pattern in Werdnig-Hoffman?
|
denervation
|
|
What type muscle fibers are seen in large cluster in SMA-I muscle biopsy?
|
Type I
|
|
What SMA is characterized by the ability to sit unsupported?
|
SMA II
|
|
Is an SMA II patient able to stand?
|
no
|
|
What is age of onset range in months of SMA II?
|
6-12 months
|
|
Where is the predominant weakness in SMA II: UEs or LEs?
|
LEs
|
|
What are two presenting symptoms of SMA II?
|
leg weakness
inability to stand or walk |
|
Is the weakness in SMA II symmetrical or asymmetrical ?
|
symmetrical
|
|
Is the weakness in SMA II distal or proximal?
|
proximal
|
|
What two structures in SMA II have tremors or fasciculations?
|
tongue and hands
|
|
What is an associated spinal feature of SMA II?
|
scoliosis
|
|
What two limbs have excessive joint laxity in SMA II?
|
hands and feet
|
|
What SMA can have advanced intellect?
|
SMA II
|
|
Is weakness in SMA II progressive or non-progressive?
|
non-progressive
|
|
Prognosis in SMA II is dependent of what function?
|
respiratory
|
|
What is the CK in SMA II?
|
normal
|
|
What does the ultrasound show in SMA II?
|
increased echo
|
|
What does the EMG show in SMA II?
|
denervation and re-innervation
|
|
What muscle fibers predominate in SMA II muscle biopsy?
|
type I
|
|
What is the most mild form of SMA?
|
type III or Kugelberg-Welander syndrome
|
|
What is SMA III also called?
|
Kugelberg-Welander
|
|
Werdnig-Hoffman patient can never acheive what motor milestone?
|
sit
|
|
SMA II patient will never acheive what motor milestone?
|
stand
|
|
Kugelberg-Welander (SMA III) will never acheive what motor milestone?
|
stand alone
|
|
What 2 muscle groups are predominately affected in SMA III?
|
hip extensors and abductors
|
|
What are two features of the gait of SMA III?
|
waddling and pelvic drop
|
|
What are two features of the posture of SMA III?
|
lumbar lordosis and anterior pelvic tilt
|
|
What spinal deformity is common in SMA III?
|
scoliosis
|
|
What is the age of onset range in SMA III (months to age category)?
|
18 months to late teenage
|
|
What is a common presenting complaint of SMA III?
|
difficults running, jumping, or climbing stairs
|
|
What are two vaiable clinical signs in SMA III?
|
hand tremor and tongue fasciculations
|
|
What CP sign is seen in SMA III?
|
Gower's sign
|
|
What are the survival prospects of SMA III? on what function does survival depend?
|
good; depends on respiratory function
|
|
What does the ultrasound show in SMA III?
|
increased echo
|
|
What is the CMAP amplitude in SMA III: increased, decreased, or normal?
|
decreased
|
|
What muscle fiber type predominates in SMA III upon biopsy?
|
type I
|
|
What is the EMG pattern seen in SMA III?
|
denervation and re-innervation
|
|
What is the CK level in SMA III?
|
normal or moderately elevated
|
|
Before what age is the onset of Friedrich's ataxia?
|
<20 years old
|
|
What brain structure degenerates in Friedrich's ataxia?
|
cerebellum
|
|
What is the chromosome in Friedrich's ataxia?
|
9q21
|
|
What is the inheritence pattern of Friedrich's ataxia?
|
autosomal recessive
|
|
What is the presenting symptom in Friedrich's ataxia?
|
gait ataxia
|
|
Is Friedrich's ataxia ascending or desceding?
|
ascending
|
|
What are 7 signs/symptoms of worsening in Friedrich's ataxia?
|
weakness
atrophy dysarthria proprioceptive loss nystagmus scoliosis fatigue |
|
What intracellular body is the source of the abnormal protein in Friedrich's ataxia?
|
mitochondria
|
|
What is the name of the abnormal protein in Friedrich's?
|
frataxin
|
|
What is the prevalence of scoliosis in Friedrich's?
|
100%
|
|
What produces more stress on muscle fibers: eccentric (lengthening) or concentric (shortening) contractions?
|
eccentric (lengthening) contractions
|
|
What type of exercise program is prescribed in Duchenne's: maximal or submaximal?
|
submaximal
|
|
What is a Duchenne's resting heart rate compared to controls?
|
increased resting heart rate
|
|
What is a Duchenne's peripheral O2 utilization compared to controls?
|
decreased
|
|
What is a Duchenne's CV capacity compared to controls?
|
decreased
|
|
What is imminent when knee extension strength is <3/5 (antigravity)?
|
wheelchair reliance
|
|
What lower extremity strength testing is used to determine imminent wheelchair use?
|
knee extension strength < 3/5
|
|
Wheelchair use is imminent when time to ambulate 30 feet is greater than what time (seconds)?
|
12 seconds
|
|
What 2 interventions can prolong ambulation in Duchenne's?
|
bracing and surgery following loss of independent ambulation
|
|
Is there any evidence for the efficacy of prophylactic surgery in Duchenne's to prolong independent ambulation?
|
no
|
|
What two metrics are used to determine corrective scoliosis surgery in neuromuscular disease in children?
|
curvature <35 degrees
VC 35% |
|
Scoliosis surgery is recommended prior to vital capacity falling below what percentage of normal?
|
35%
|
|
Scoliosis surgery is recommended prior to a curve becoming greater than what degree?
|
35 degrees
|
|
A forced vital capacity less than what percentage range of normal may preclude scoliosis surgery due to perioperative morbidity?
|
30-40%
|
|
30-40% of what pulmonary function test is a contraindication to corrective scoliosis surgery?
|
forced vital capacity
|
|
Are spinal orthotics helpful in the scoliosis of Duchenne's?
|
no
|
|
What scoliosis treatment is ineffective in Duchenne's?
|
spinal orthotics
|
|
What spinal surgical procedure is most effective in the treatment of progressive scoliosis in neuromuscular disease in children?
|
spinal arthrodesis
|
|
Spinal bracing is used to improve what activity in children with neuromuscular disease who are unable to walk?
|
sitting balance
|
|
What is the most common cause of mortality in childhood neuromuscular disease?
|
pulmonary complications
|
