Term
| What are signs of upper versus lower motor lesions |
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Definition
Upper: Spasticity, Hyperreflexia
Lower: Atrophy, Hyporeflexia |
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Term
| What sensations are carried by Small and large sensory fibers |
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Definition
Small: Pain and Temp
Large: Vibration and Proprioception |
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Term
| What are the components of the motor unit |
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Definition
| Lower motor neuron motor axon, neuromuscular junction, and muscle fibers |
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Term
| How does a nerve conduction study appear in axonal damge and in demyelinating disease |
|
Definition
Axonal Damage -> decreased myelin: Slow Velocity
Axon Loss: Low amplitude |
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Term
| How does myopathy and chronic denervation appear on EMG |
|
Definition
Myopathy: Small motor units with fast recruitment
Chronic Denervation: Large motor units with reduced recruitment |
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Term
| What is the presentation of ALS and how is it treated |
|
Definition
Upper/Lower motor neuron lesions:
Progressive Weakness w/ Atrophy
Bulbar dysfuction
Respiratory Weakness->cause of death
Treatment: Riluzole(anti-glutamate) |
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Term
| What is the most likely mutation causing ALS |
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Definition
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Term
| What is the major mutation in Spinal Muscular Atrophy |
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Definition
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Term
| What are the different types of SMA and their presentations? |
|
Definition
I)Werdig-Hoffman:Can't sit, die @< 2
Onset: <6 months,
II)Difficulty sitting, die in 2-3 decade
Onset:6-18 months
III)Kugelberg-Welander: Don't die
Onset: >18 months
IV)Few deficits
Onset: Adult |
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Term
| What is Spinal Muscular Atrophy |
|
Definition
| Hereditary disease with progressive lower motor neuron dysfunction |
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Term
| What is Bulbospinal Muscular Atrophy(kennedy disease) and what are some typical signs and the prognosis? |
|
Definition
X-linked recessive disease of the androgen receptor
Signs
Proximal Muscle Weakness
Facial Fasciculations
Gynecomastia
Prognosis: Weakness but normal life expectancy |
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Term
| What are the signs of Radiculopathies |
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Definition
Pain/Paresthesias, numbness, weakness
Dermatomal and myotomal distribution |
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Term
| What are the motor and sensory deficits of C6, C7, L4, L5 and S1 Radiculopathies and which is the most common |
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Definition
C6: Biceps
Sensory:Lateral forearm and thumb and index finger
C7: Wrist and Elbow extension
Sensory: Middle and index finger
L4: Quads
Sensory: Anterior thigh and medial lower limb
L5: Foot drop
Sensory: Lateral lower leg and foot dorsum
S1: Plantar Flexion and knee flexion
Sensory: posterior leg and sole of foot |
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Term
| Which limb plexus is more often injured in trauma |
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Definition
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Term
| What are the signs of Radial, Peroneal, Median and Ulnar Neuropathy, lateral cutaneous branch of femoral |
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Definition
Radial: Wrist drop
Peroneal: Foot Drop
Lat cutaneous: Meralgia Paresthetica (pain paresthesias, numbness over anterior/lateral thigh)
Median: Thenar atrophy, lateral 3 and 1/2 digits
Ulnar: Intrinsic hand muscle weakness, and medial boarder of the hand |
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Term
| What is the cause of mononeuritis multiplex |
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Definition
| Vasculitis caused by autoimmune or CT disease |
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Term
| What are the symptoms and treatment of Guillian-Barre |
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Definition
Signs
Paresthesia and numbness
Prox/Distal Weakness
Hyporeflexivity
Bulbar Dysfunction
Autonomic Dysfunction
Treatment: IVIG, Plasmaphoresis |
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Term
| What are the acute and chronic causes of demyelinating sensory polyneuropathy |
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Definition
Acute: GBS(AIDP)
Chronic: CIDP |
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Term
| What is the triad of the miller-fischer variant of Guillian Barre |
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Definition
| Ataxia, Arreflexia, Opthalmoplegia |
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Term
| What is the appearance of the CSF in Guilian Barre and what infections are seen typically before GBS onset? |
|
Definition
Cytoalbuminologic: high protein without cells
Infections: CMV,HIV, EBV, Jejuni |
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Term
| What is the presentation of Chronic Inflammatory Demyelinating Polyneuropathy and how is it treated? |
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Definition
Gradual Prox->Distal Weakness/Sensory
Episodes of relapse and remission
Tx: IVIG, Plasmaphoresis, Steroids |
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Term
| What is the cause, age of onset and signs of Charcot Marie Tooth Disease |
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Definition
Cause: PMP-22 mutations
Age:10-40
Presentation
Distal weakness
Non-painful numbness
High Arches and Hammer toes |
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Term
| What are the types of Charcot Marie Tooth Dissease(CMTD) and how are they inherited and do they cause axonal or demyelinating disease? |
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Definition
CMT1: Demyelinating AD
CMT2: Axonal AD or AR
CMT4: Demyelinating AR |
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Term
| What is the cause of hereditary neuropathy with liability to Pressure Palsy |
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Definition
Deletion of PMP-22
CMT 1: PMP-22 Duplication |
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Term
| What is the treatment for neuropathic pain |
|
Definition
1st Line
Tricyclc antidepressants
Anticonvulsants
SNRI's
Lidocaine
2nd Line
Muscle Relaxants
Canabinoids
Alpha 2-Agonists
NMDA Blockers
3rd Line
NSAIDs
Opiods
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Term
| what is the most common anti-body and cancer associated with paraneoplastic neuropathies |
|
Definition
Anti-Hu Antibody
Small Cell Lung Cancer
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Term
| Most common cause of neuropathy in the US? |
|
Definition
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