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59 Cards in this Set
- Front
- Back
LOWER motor neuron lesion, UPPER motor neuron lesion, or BOTH?
Spinal Muscular Atrophy |
LOWER
*UPPER too (brainstem motor nuclei) |
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LOWER motor neuron lesion, UPPER motor neuron lesion, or BOTH?
POLIOmyetlitis/PostPOLIO syndrome |
LOWER
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LOWER motor neuron lesion, UPPER motor neuron lesion, or BOTH?
AMYOTROPHIC LATERAL SCLEROSIS |
BOTH
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LOWER motor neuron lesion, UPPER motor neuron lesion, or BOTH?
Primary Lateral Sclerosis |
UPPER
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UPPER motor neuron sign or LOWER motor neuron sign?
HYPOreflexia |
LOWER
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UPPER motor neuron sign or LOWER motor neuron sign?
Spasticity |
UPPER
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UPPER motor neuron sign or LOWER motor neuron sign?
Flaccidity |
LOWER
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UPPER motor neuron sign or LOWER motor neuron sign?
UP-going toes |
UPPER
Positive Babinski |
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UPPER motor neuron sign or LOWER motor neuron sign?
FASCICULATIONS |
LOWER
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How do you test for recruitment of the DIAPHRAGM?
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- MONOpolar needle
- Between 8th-9th INTERCOSTAL SPACE (anterior axillary line) - Intercostals are recruited during EXPIRATION - Diaphragm recruited during INSPIRATION |
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FASCICULATIONS on EMG are indicative of which MOTOR NEURON DISEASES?
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ALS
- also, poliomyelitis or postpolio syndrome - SMA in the TONGUE |
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Which MOTOR NEURON DISEASE would you see CRD's in? |
SMA type THREE - may also see fibs/PSW's Also in ALS |
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Pattern of inheritance for SMA type I?
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Autosomal RECESSIVE
(Acute Werdnig Hoffman) |
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Pattern of inheritance for SMA type II?
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Autosomal RECESSIVE
(Chronic Werdnig Hoffman) |
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Pattern of inheritance for SMA type III?
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Autosomal recessive or dominant
(Kugelberg Welander) |
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Age of onset for SMA type I?
Disease progression? |
3-6 months
- death by 2-3 years old (acute Werdnig Hoffman) |
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Age of onset for SMA type II?
Disease progression? |
2-12 months
- death by 10 years old - WC by 2-3 years old (chronic Werdnig Hoffman) |
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Age of onset for SMA type III?
Disease progression? |
2-15 years
- NORMAL life expectancy - WC by age 30 (Kugelberg-Welander) |
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Death from SMA type I & II is usually due to what?
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RESPIRATORY FAILURE
(acute & chronic Werdnig Hoffman) |
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Tongue fasciculations should cause you to include what in your differential diagnosis?
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SMA
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Patients with SMA show what abnormality in blood work?
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Increased CPK
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What will you see on muscle Bx with SMA?
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Hypertrophic/atrophic fibers
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An infant that NEVER sits independently has HYPOtonia, is not reaching his/her milestones, FROG-LEGGED position, with tongue fasciculations. What does he/she have?
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SMA I (acute werdnig hoffman)
*facial muscles are affected the least |
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An infant that CAN sit independently but has HYPOtonia, progressive weakness, KYPHOSCOLIOSIS, EQUINUS deformity of the feet, with tongue faciculations. What does he/she have?
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SMA II (chronic werdnig hoffman)
*watch for progressive PULMONARY involvement *will likely need assistive devices for standing/walking |
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What is the pattern of weakness seen in Kugelberg-Wellander disease? |
SMA II - SYMMETRIC: lower then upper |
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Which motor neuron disease may mimic DUCHENNE clinically?
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SMA III (Kugelberg Wellander)
- Gower's sign d/t lower limb weakness - Calf pseudohypertrophy *in contrast SMA III has good long-term survival -dependent mostly on respiratory function |
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Typical onset of ALS?
Which sex is more prone to develop it? |
60's
MEN |
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In terms of survival is it better to develop ALS at a YOUNGER age or an OLDER age?
- What other factors predict survival? |
YOUNGER
- SEVERITY of onset - Pulmonary function |
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What are the first signs of ALS typically?
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ASYMMETRIC weakness, atrophy, FASCICULATIONS
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3, 5, & 10 year mortality for ALS?
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3 years - 50%
5 years - 70% 10 years - 90% |
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Sensation& what two muscle groups are typically spared in ALS?
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Extrocular muscles
Bowel/Bladder |
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What electrodiagnostic signs will point to a diagnosis of ALS?
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SINGLE FIBER = increasd JITTER & fiber DENSITY
Low rate rep stim = increased DECREMENT NCS = normal EMG = membrane instability, LDLA MUAPs, CRDs (NEUROPATHIC) |
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What agent slows disease PROGRESSION in ALS?
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Rilutek - riluzole antiglutamate
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What pathologic organism is responsible for Poliomyelitis?
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PICORNAVIRUS
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What are some PSEUDOBULBAR signs associated with ALS?
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- Difficulty chewing
- Dysphagia - Difficulty speaking - Emotional outbursts |
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What percent of patients with Poliomyelitis have SEVERE disability, MILD disability, & COMPLETE RECOVERY?
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Severe = 25, Mild = 25%, Complete recovery = 50%
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What factors predict increased mortality due to poliomyelitis in ADULTS?
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10% mortality in adults with
- BULBAR signs - RESPIRATORY involvement |
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What electrodiagnostic signs will point to a diagnosis of POLIOMYELTIS?
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Normal SNAP (sensory is spared)
Normal or decreased CMAP NEUROPATHIC EMG = LDLA MUAPs, membrane instability |
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What is the pathologic difference between Poliomyelitis & Post Polio Syndrome?
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Polio = degeneration of anterior horn cell
Post Polio = death of anterior horn cell (burned out) |
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The Halstead-Ross Criteria are used to diagnose which Motor Neuron disease? What are they?
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POST POLIO SYNDROME
1. Previous Dx of polio 2. Recovery of fxn 3. 15 year disease stability 4. Return of Sx (weakness, atrophy) 5. No other medical problems to explain |
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What electrodiagnostic signs will point to a diagnosis of POLIOMYELTIS?
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SNAP = normal
CMAP = abnormal EMG = GIANT MUAPs, membrane instability SINGLE FIBER = increased jitter, density, & blocking (heavy collateral sprouting) |
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GIANT MUAPs on EMG point to a diagnosis of which motor neuron disease?
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POST POLIO
- severe loss of anterior horn cells - remaining motor units become huge due to heavy collateral sprouting |
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ALS or Post polio?
Low rate rep stim shows INCREASED decrement? |
ALS
*LRRS is normal in post polio |
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Weakness + INTACT sensory + UPPER motor neuron signs = ?
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ALS
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Weakness + INTACT sensory + LOWER motor neuron signs + defect in MUSCLE + NO PAIN =
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Myopathy or inclusion body myositis
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Weakness + INTACT sensory + LOWER motor neuron signs + defect in MUSCLE + PAIN =
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Polymyositis/dermatomyositis
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Weakness + INTACT sensory + LOWER motor neuron signs + defect in ANTEROR HORN CELL =
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Poliomyelitis
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Weakness + INTACT sensory + LOWER motor neuron signs + defect in NMJ =
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MG or lambert eaton
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Weakness + ALTERED sensory + UPPER motor neuron signs =
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Tumor, syrinx, MS (cerebrum, brain stem, spinal cord)
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Weakness + ALTERED sensory + LOWER motor neuron signs = |
peripheral neuropathy, plexopathy, radiculopathy |
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Critical illness polyneuropathy is primarily axonal or demyelinating?
Motor or sensory? |
AXONAL LOSS
Both motor and sensory PERIPHERAL POLYNEUROPATHY |
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1 month old with weak cry, generalized hypotonia, symmetric weakness of lower>upper limbs, frog legged position when supine, diaphragmatic breathing (BELL-SHAPED torso), facial weakness, & tongue fasciculations
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SMA I
*preserved MSR's does NOT exclude the diagnosis, although they are usually absent |
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What type of SMA has a characteristic BASELINE TREMOR on ekg?
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SMA II
*especially in limb leads |
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Caused by abnormality in FRATAXIN (protein) |
Friedreich's ataxia - both upper and lower motor neuron lesions - sparing of motor neurons of spinal cord |
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What is the liklihood that a patient with Friedreich's ataxia will develop scoliosis?
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almost 100%!!!
*usually before age 10 |
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Wheelchair reliance is imminent when what muscle group becomes less than 3/5 and time to ambulate 30 feet is greater than ___ seconds?
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KNEE EXTENSION
time to ambulate >12 seconds |
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If surgical management of scoliosis is to be pursued it must occur prior to the patients FVC reaching what level?
Why? |
A forced vital capacity <35% of normal is a contraindication to scoliosis surgery d/t increased perioperative morbidity.
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In general what is the most effective treatment for progressive scoliosis?
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spinal ARTHRODESIS (fusion)
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When should you start using spinal orthotics to manage spinal deformity in Duchenne Muscular Dystrophy?
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NEVER -they are are ineffective and do not change the natural history of the curve
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