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190 Cards in this Set

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differentiate the increased tone from spastic UMN weakness from parkinson's cogwheel rigidity
spastic UMN weakness is velocity dependent unlike cogwheel rigidity which is not
what is the exception to most muscle diseases having proximal muscle weakness more than distal
myotonic dystrophy
what is usually elevated in myopathies
creatine kinase
EMG findings in myopathies
small motor unit potential - because the muscle fibers in each motor unit are randomly lost resulting in fewer muscle fibers per motor unit
differentiate dermatomyositis and polymyositis
polymyositis - T cells directed against the muscle fibers
dermatomyositis - complement mediated vasculopathy against the muscle capillaries, associated with a skin rash
characteristics of myotinic dystrophy
autosomal dominant
myotonia
wasting of temporal muscle
frontal balding
conduction abnormalities
CTG trinucleotide repeat on chormosome 19
differentiate dermatomyositis and polymyositis
polymyositis - T cells directed against the muscle fibers
dermatomyositis - complement mediated vasculopathy against the muscle capillaries, associated with a skin rash
characteristics of DMD
x-linked recessive
pseudohypertrophy of calves
very high CK levels
absent dystrophin
deletion or duplication on Xp21
severe proximal leg and pelvic girdle weakness
decreased number of ACh receptors available for binding at the NMJ
myasthenia gravis
symptoms associated with MG
ocular - double vision and ptosis
bulbar - dysphagia
fatiguing extremity weakness
diagnosis of myasthenia gravis
tensilon test (edrophonium)
elevated AChE receptor antibodies
EMG reveals a decremental response
what should be checked in a patient with myasthenia gravis
thymoma
treatment of myasthenia gravis
pyridostigmine
associated with a decreased release of ACh from presynaptic terminal
symptoms
botulism - nicotinic and muscarinic receptors are affected - rapidly progressive descending paralysis and mydriasis and constipation
antibody directed against the nicotinic acetylcholine receptor
myasthenia gravis
associatd with infant ingestion of honey
infantile botulism
EMG reveals an incremental response
Lambert-Eaton syndrome
antibodies against the presynaptic voltage gated calcium channels
Lambert-Eaton
symptoms associated with Lambert-Eaton
leg weakness
dry mouth
absent reflexes - increase briefly after muscle activation
neoplasm associated with Lambert-Eaton
small cell lung cancer
cranial nerve associated with lacrimation and salivation
CN VII
what nuerotransmitters initiates sweat gland action
ACh (muscarinic) even though it's a sympathetic response T1-L2
is the pupil affected in myasthenia gravis
No, because only nicotinic postganglionic ACh receptors are affected
TCA associated with atropine-like side effects
amitriptyline
it is normal for some elderly individuals to have diminished ankle jerk reflexes
**
causes of peripheral neuropathy
DANG THERAPIST
D - DM
A- alcohol
N - B12 deficiency
G - Guillain-Barre syndrome
T - trauma
H - hereditary (charcot-marie-tooth)
E - endocrine
R - rheumatologic
A - amyloidosis
P - porphyria
I - infections
S -sarcoidosis
T - toxins (phenytoin, INH without B6, arsenic, lead, vincristine)
acute inflammatory demyelinating polyradiculoneuropathy
Guillain-Barre syndrome
symptoms of Guillain-Barre syndrome
ascending paralysis
bilateral facial weakness
unstable blood pressure
no reflexes
sensory loss
flaccid weakness
diagnosis of Guillain-Barre
high CSF protein
conduction block and slowed conduction velocity by EMG
differentiate the cells responsible for remyelination of Guillain-Barre and multiple sclerosis
GBS - schwann cells
MS - oligodendrocytes
where are sodium channels at the highest density in a neuron
the initial segment and at the node of Ranvier
how are muscle stretch reflexes initiated
activation of muscle spindles - monosynaptic reflex
result of compression of the median nerve across the wrist
carpal tunnel syndrome
causes numbness of the hand (digits 1-3) and awakens patients from sleep
carpal tunnel syndrome
results in weakness of the hand and numbness of digits 4 and 5
ulnar neuropathy at the elbow
differentiate radial neuropathies at the spiral groove or above
saturday night palsy - at the spiral groove - results in normal tricep function because the nerve has already gone of proximally - both result in a wrist drop
where does the peroneal nerve get compressed resulting in weak foot dorsiflexion
fibular head
3 causes of foot drop
1. peroneal neuropathy
2. L5 radiculopathy
3. ALS
spontaneous discharge of individual muscle fibers
fibrillations
spontaneous discharge of individual motor units
fasciculations
what results in a large amplitude of MUP
anything that involves the LMN axons (anterior horn cells, root, plexus, peripeheral nerve) because re-innervation and recruitment of additional motor units
what results in small MUPs
myopathies - because there is random, scattered drop out of individual muscle fibers
characteristics of a LMN injury
fibrillation acutely and later a LARGE motor unit potential
lesions of the nerve roots
symptoms
radiculopathies:
both motor and sensory symptoms
painful shooting pain in the limb and dermatomal distribution
which dermatome lies below the C4 dermatoms
T2
nerve roots associated with the following reflexes:
biceps
brachioradialis
triceps
patellar
archilles
biceps - C5,6
brachioradialis - C5,6
triceps - C7
patellar - L2-4
Achilles - S1,2
most common cervical radiculopathy - presents with neck pain and often radiates into the hand
C7 radiculopathy
C5 radiculopathy
weakness
sensory
reflexes
weakness - deltoids, infraspinatous, supraspinatous, brachioradialis
sensory - shoulder, lateral arm/forearm
reflex- biceps, brachioradialis
C7 radiculopathy
weakness
sensory
reflexes
weakness - triceps, pronator teres, finger extensors
sensory - palm/digits 2-4
reflex- triceps
C8/T1 radiculopathy
weakness
sensory
reflex
weakness - hand
sensory - 5th digit, medial forearm
reflex - none
most common lumbosacral radiculopathy - presents with back pain which radiates down the back of the leg
S1 radiculopathy
L2-4 radiculopathy
weakness
sensory
reflex
weakness - quadriceps, hip flexors, thigh adductors
sensory - anterior thigh, medial lower leg
reflex - patellar
L5 radiculopathy
weakness
sensory
reflex
weakness - foot drop, foot inversion, knee flexion, hip abduction
sensory - lateral leg, dorsal foot
reflex - normal
S1 radiculopathy
weakness
sensory
reflex
weakness - gastrocnemius, gluteus maximus
sensory - posterior leg, plantar foot
reflex - achilles
upper trunk lesion
Erb's palsy (C5,6 nerve roots) - waiter's tip position
lower trunk lesion
C8/T1 nerve roots - Klumpke's palsy (hand weakness), thoracic outlet syndrome, pancoast tumor (horner's syndrome)
lesion of long thoracic nerve
scapular winging
meralgia paresthetica
lateral femoral cutaneous neuropathy - associated with wearing a tight belt and overweight individuals
acute and severe lumber plexopathy with weakness and sensory loss in the lumbar plexus distribution and abdominal pain in a hospitalized post-op patient
retroperitoneum hemorrhage due to excessive anticoagulation with IV herparin therapy
two main anterior horn cell diseases
ALS
polio
symptoms associated with ALS
UMN - corticospinal and corticobulbar tract degeneration - pseudobulbar palsy
LMN - anterior horn cell degeneration and brain stem motor nuclei (nucleus ambiguus and hypoglossal nucleus) - bulbar palsy
fasciculations
viral disease associated with fasciculations, normal sensation, absent reflexes in affected area
polio virus
corticospinal tract
precentral gyrus - corona radiata - posterior limb of the internal capsule - cerebral peduncle - basis pontis - medullary pyramid - pyramidal decussation - lateral spinal cord
common features of spinal cord lesions (myelopathy)
UMN findings blow the level of lesion
LMN findings seen at the level of the lesion
Brown-Sequard syndrome
ipsilateral spastic motor deficits
ipsilateral dorsal column deficits
contralateral pain and temperature deficits
what is spared in a central spinal cord syndrome
sacral nerves - most lateral in the spinothalamic pathway
produces bilateral pain and temperature deficits in the arms, usually in the cervical region
syringomyelia
anterior spinal artery occlusion
all function below level of infarction may be lost producing spastic weakness and loss of pain and temperature - posterior column is preserved
posterior spinal artery occlusion
pure loss of posterior column function below the level of the lesion - stomping gait
demyelination of the posterior columns
CST demyelination
small fiber peripheral neuropathy
B12 deficiency
what is used to detect B12 deficiency
anemia associated with high MCV
high levels of homocystine and methylmalonic acid
motor nucleus for CN IX and X
nucleus ambiguus
degeneration of this nucleus in ALS results in bulbar palsy
Nucleus ambiguus
lateral medullary syndrome
occlusion of the vertebral artery or PICA:
STT - contralateral P and T
spinothalamic tract - ipsilateral
horner's syndrome
nucleus ambiguus
vestibular nuclei
inferior cerebellum
nucleus solitarious
CN VII - taste
CN IX - blood pressure
CN X - lungs
important for induction of sleep
medial medullary syndrome
contralateral spastic weakness and loss of vibration/proprioception
ipsilateral atrophy of tongue
lesion of the lateral vestibular nuclei (midbrain or pons)
decerebrate posturing
acoustic schwannoma
cerebellopontine angle mass
CN VIII - hearing loss, dizzness, tinnitus
CN VII and V later resulting in diminished corneal reflex
ipsilateral ataxia from compression of the cerebellum
3 trigeminal nuclei
mesencephalic - proprioceptive information
principal - tactile and pressure
spinal - pain and temperature
corneal reflex
afferent CN V, efferent is bilateral CN VII
differentiate CN for visceral senation and taste
visceral - CN V
taste - CN VII
UMN lesion of CN VII
lower facial weakness, but not upper because it has bilateral representation
LMN lesion of CN VII
Bell's palsy - upper and lower facial weakness
caudal RAS
important for induction of sleep, raphe nucleus, serotonin
CN III palsy
eyes is deviated down and laterally
ptosis
mydriasis
Weber's syndrome
infarction of the midbrain cerebral peduncle and the 3rd nerve as it travels by
ipsilateral 3rd nerve palsy and contralateral hemiplegia
DDX of CN III palsy
Weber's syndrome
uncal herniation
posterior communicating aneurysm
DM - ischemic lesion of 3rd nerve
cavernous sinus thrombosis
CNs involvedin cavernous sinus thrombosis
CN III, IV, VI, and V1
1st, 2nd, and 3rd order of the sympathetic pathway - lesions of each that can result in Horner's syndrome
1st - hypothalamus to T1-2 (pontine hemorrhage, PICA stroke)
2nd - T1-2 to superior cerivcal ganglion (pancoast tumor)
3rd - SCG to pupil/blood vessels/sweat glands (carotid dissection, cluster headache)
miosis
ptosis
anhydrosis
Horner's syndrome
a lesion of the optic nerve results in what finding
afferent pupillary defect
weakness in superior oblique muscle with difficulty looking down and in - patients tilt their head
trochlear nerve palsy
3 conditions that restrict vertical eye movements
thalamic hemorrhages
progressive supranuclear palsy
pineal tumor
tract important in head turning and localize visual and auditory information
tectospinal tract
how does the inferior olivary nucleus connect with the cerebellum
climbing fibers
all of inputs are called mossy fibers
the source of the outflow from the cerebellar hemisphers via the VL nucleus of the thalamus to the motor cortex
dentate
chronic alcoholism can result in degeneration where
anterior lobe of the cerebellum - resulting in gait ataxia
lesion of the central tegmental tract
palatal myoclonus
mollaret's triangle
red nucleus - (central tegmental) -inferior olive - (climbing fibers) - cerebellum - (superior cerebellar peduncle) - red nucleus
which spinocerebellar tract crosses the midline twice
ventral spinocerebellar tract
source of outflow from the vermis and flocculonocular lobe to the vestibular nuclei
fastigial cerebellar nucleus
important in coordinating proximal muscles
paravermis
this area coordinates the vestibular system and midline truncal musculature
vermis
what part of the cerebellum do childhood medulloblastomas typically affect
midline cerebellar vermis (flocculonocular lobe)
Wernicke's encephalopathy
encephalopathy
ophthalmoplegia - bilateral CN VI
gait ataxia
Friedreich's ataxia
atrophy of spinocerebellar pathway
CST degeneration
posterior column degeneration
peripheral neuropathy
GAA trinucleotide on chromosome #9
seizure mediation with side affects of ataxia, nystagmus, and lethargy if taken in excess
phenytoin
which cranial nerve is used to smell salts
CN V
causing for anosmia
head trauma
parkinson's
olfactory groove meningioma
most epileptic part of the brain
uncus - foul odor (burnt rubber)
monocular vision loss
decreased visual acuity
pain behind the eye
afferent pupillary defect
optic neuritis (MS)
pituitary adenoma (prolactinoma) and vision loss
optic chiasm lesion
bitemporal hemianopsia
anterior choroidal stroke
contralateral hemianopsia with central break-line zone
contralateral hemiplegia
hemisensory loss
optic radiation lesion
MCA stroke
contralateral homonymous hemianopsia
PCA stroke and vision loss
contralateral homonymous hemianopsia with macular sparing
thalamic nuclei:
VPL
VPM
LGB
MGB
VL
DM
VPL - somatosensory
VPM - facial sensory and taste
LGB - primary visual
MGB - primary auditory
VL - primary motor cortex from cerebellum
DM - from amygdala to prefrontal cortex
lesion of the subthalamus
hemiballimus
consists of the pineal gland and the habenular nuclei
epithalamus
pinealmoas
can cause hydrocephalus or vertical gaze palsy
contains the descending motor fibers from the motor cortex to the brain stem (corticobulbar tract)
genu
occlusion of the lenticulostriate artery from the MCA
pure motor stroke - posterior limb of the internal capsule
occlusion of the thalamogeniculate artery from the PCA
pure sensory stroke - posterior limb of the internal capsule
lesion of the genu
lower facial weakness
pseudobulbar palsy
multiple subcortical strokes that destroy a large percentage of the corticobulbar tract - dyarthria, dysphagia, emotional incontinence
conduction aphasia
lesion of the arcuate fasciculas - cannot repeat
Right hemonymous hemianopsia
alexia without agraphia
Left PCA stroke involving the splenium of the corpus callosum
connects the hippocampal formations and temporal lobe structures
anterior commissure
accomoodation is normal in patients with an INO
Multiple sclerosis
inhibits the peripheral degradation of levodopa
carbidopa
side effects of levodopa
nausea
hallucinations
dyskinesias
dopamine agonists
ropinirole
pramipexole
side affects of DA agonists
nausea
hallucinations
gambling
sleep attacks
compulsive behavior
medication used for treatment of tremor in parkinson's
anticholinergic:
trihexyphendyl
benztropine
drugs that cause parkinsonism
drugs that block DA:
antiemetics - metoclopramide and prochlorperazine
neuroleptics - haloperidol
unique feature of progressive supranuclear palsy that sets apart from parkinson's
vertical supranuclear palsy
early falling
Shy-Dragar syndrome
multisystem atrophy
parkinsonism with profound autonomic insufficiency
trinucleotide CAG repeat on chromosome #4
Huntington's disease
Wilson's disease
defect of copper metabolism
any movement disorder in a young individual
proximal tremor
kaiser-Fleischer ring
eye looking away from the hemiparesis
MCA stroke
eyes looking at the hemiparesis
pontine stroke or hemorrhage
posterior left MCA stroke
supplies the supramarginal and angular gyrus - Gerstmann's syndrome
agraphia, acalculia, finger agnosia, right left confusion
contralateral leg motor and sensory deficits, bladder function is also impaired
ACA stroke
most common lacunar stroke
pure motor stroke - lenticulostriate artery
Amaurosis Fugax
transient loss of vision in one eye due to TIA - extracranial carotid artery disease
four most common locations for hypertensive hemorrhage
putamen - contralateral hemiplegia and hemisensory loss
thalamus - contralateral hemisensory loss and hemiplegia with decreased vertical eye movement
pons - coma and pinpoint pupils
cerebellum - occpital headaches, ipsilateral ataxia and nystagmus
used for abortive therapy of heacaches
sumatriptan - may cause coronary artery vasospasm and should not be used in patients with coronary artery disease and uncontrolled HTN
treatment of cluster headache
high flow oxygen
drug of choice for primary generalized epilepsy
valproic acid
what happens if you occlude the cerebral aqueduct
normal size 4th ventricle with large 3rd and lateral ventricles
associated with xanthochromia
subarachnoid hemorrhage
Kluver-Bucy syndrome
head trauma affecting the bilateral medial temporal lobes involving the amygdala resulting in docility with decreased fear response
Papez circuit
hippocampus - fornix - mammilary bodies - thalamis fasciculus - anterior nucleus of the thalamus - cingulate gyrus - hippocampus
what are schwann cells and PNS neurons associated with
neural crest origin
where do microglia originate from
mesoderm
what do CNS neurons and ependymal cells originate from
neuroectoderm
this cells maintain the BBB, form reactive gliosis in response to injury, and have GFAP marker
astrocytes
what cells fuse to form multinucleated giant cells in CNS in HIV-infected individual
microglia
these cells look like fried eggs on H&E staining in CNS
oligodendrocytes
these cells are destroyed in Guillain-Barre syndrome
Schwann cells
what two neurotransmitters are decreased in Huntington's disease
ACh
GABA
3 specialized brain areas with fenestrated capillaries and no BBB
area postrema - vomiting
OVLT - osmotic sensing
neurohypophysis - ADH/oxytocin release
differentiate the later and ventromedial hypothalamus
lateral - hunger
ventromedial - satiety
*both are affected by leptin, leptin inhibits hunger
what hormones do the supraoptic nucleus and paraventricular nucleus of the hypothalamus make
supraoptic - ADH
paraventricular - oxytocin
differentiate the lateral and medial geniculate nucleus of the thalamus
lateral - CN II input for vision
medial - superior olive and inferior colliculus input for hearing
differentiate the VPL and VPM of the thalamus
VPL - spinothalamic and dorsal column input sensory
VPM - trigeminal and gustatory sensory
5 components of the limbic system
cingulate gyrus
hippocampus
fornix
mamillary bodies
septal nucleus
breakdown of the spinal nerve
31 total:
8 cerival
12 thoracic
5 lumbar
5 sacral
1 coccygeal
what disease can be caused by a defect in superoxide dismutase 1 (SOD1)
ALS
differentiate function of superior and inferior colliculi
superior - conjugate vertical gaze center
inferior - auditory
what can a pinealoma damage
superior colliculus resulting in paralysis of vertical gaze center
differentiate where V1, V2, and V3 exit the skull
V1 - superior orbital fissure
V2 - foramen rotundum
V3 - foramen ovale
what exit the superior orbital fissure
CN III, IV, V1, and VI
CN associated with the cavernous sinus
CN III, IV, V1, V2, and VI
muscle that opens the jaw
lateral pterygoid
muscles that close the jaw
masseter
temporalis
medial pterygoid
differentiate LMN and UMN lesion of the facial nerve
UMN lesion - contralateral paralysis of the lower face only
LMN lesion - ipsilateral paralysis of upper and lower face
most common primary brain tumor
glioblastoma multiforme
this brain tumor is associated with pseudopalisading and areas of necrosis and hemorrhage
glioblastoma multiforme
this brain tumor is associated with being S-100 positive
schwannoma
this brain tumor is associated with fried egg cells that are often calcified
oligodendroglioma
GFAP positive childhood tumor with rosenthal fibers
pilocytic astrocytoma
PNET tumor with rosettes and small blue cells
medulloblastoma
tumor most commonly found in the 4th ventricle with characteristeic perivascular pseudorosettes
ependymoma
this brain tumor is most often cerebellar and associated with von Hippel-Lindau syndrome
hemangioblastoma
associated with port-wine stains (nevus flammeus), leptomeningeal angiomas, and pheochromocytomas
Sturge-Weber syndrome
lysosomal storage disease due to arylsulfatase A deficiency that causes demyelination
metachromatic leukodystrophy
hereditary motor and sensory neuropathy
Charcot-Marie-Tooth disease
unilateral headache with photophobia and aura of neurologic symptoms before headache
migraine
unilateral repetitive brief headache characterized by periorbital pain with ipsilateral lacrimation and rhinorrhea
cluster headache
differentiate peripheral and central vertigo
peripheral - inner ear etiology (semicircular canal, vestibular nerve, Meniere's disease)
central - brain stem or cerebellar