Demyelination

Question 0

Myopathy workup

Answer 0

• EMG: strength may be normal, motor units will be small
• blood -> elevated CK
• myopathy confirmed with muscle biopsy/genetic testing

Question 1

Myopathy presentation

Answer 1

• weakness, often symmetric with NO sensory loss

- inherited: DMD, autoimmune inflammatory: polymyositis, dermatomyocytis

Question 2

DMD

Answer 2

• myopathy
• calf hypertrophy (connective tissue)
• biopsy reveals highly variable fiber size: existing motor unit compensate for decaying ones
• dystrophin mutation

Question 3

Inflammatory myopathies

Answer 3

• More common in adults

- presents with proximal weakness and muscle pain

Question 4

Dermatomyositis

Answer 4

• Grotton’s sign (rash over extensor surface of digits)
• heliotrope rash on skin exposed to sun
• perifasciular atrophy

Question 5

NMJ disease (presentation/diagnosis)

Answer 5

• weakness, no numbness (looks like myopathy)
• myasthenia gravis most common form lambert eaton second most
• diagnosis: repetitive stimulation results in decrement or increment
• ID Abs

Question 6

Myasthenia gravis vs lambert eaton

Answer 6

• MG: anti-ACh receptor Abs

- LE: anti-presynaptic Ca channels

Question 7

Myasthenia gravis presentation

Answer 7

• lots of confusing eye stuff (Diplopia, Ptosis)
• slurred speech
• dysphagia
• weakness in arms and legs
• fatigue
• difficulty breathing

Question 8

Generalized neuropathy

Answer 8

• progressive weakness and burning pain -> numbness, often symmetric
• hyporeflexia
• often start at feet and ascend
• Diabeetus, HIV renal failure
• EMG reveals Large motor units (compensatory resp precedes paralysis)

Question 9

Trt of diabetic neuropathy

Answer 9

• anticonvulsants: gabapentin/pregabalin
• SNRIs
• antidepressants

Question 10

Diabetic neuropathy: 4 symptoms

Answer 10

• gait problems
• orthostatic hypotension
• gastric paresis
• foot ulcers

Question 11

Focal neuropathy

Answer 11

• present with numbness and often severe weakness
• carpal tunnel (nerve compression: compression most common neuropathy) 1st common, ulnar compression 2nd.
• workup is neuro EMG
• can result in eventual thenar atrophy

Question 12

Peroneal nerve pinch

Answer 12

Anteriolateral paresthesis
Foot drop (slaps as they walk)

Question 13

Bell’s palsy

Answer 13

• lesion of CN VII
• often idiopathic
• steroids can help
• recovery typically occurs completely

Question 14

Radiculopathy

Answer 14

• pain/numbness/tingling in nerve root distribution
• sometimes weakness
• usually asymmetric
• pattern is key
• biceps: C5/6, triceps: C 7/8, knee: L2/4, ankle: S1/2 (reflex is lost in ridiculopathy)

Question 15

ALS

Answer 15

• upper and motor neuron signs
• CSPT AND ventral horn hit
• EMG will show denervation in all muscle groups
• diagnosis by rule out: polyrediculopathy
• can trt (sort of) with glutamate antagonist rulizole

Question 16

Spinal muscular atrophy

Answer 16

• like ALS, but only lower motor neuron signs
• SMN1 defect
• 1/6000 are effected; 1/40 are carriers

Question 17

Guillane-barre syndrome (presentation, diagnosis, trt)

Answer 17

• polyneuropathy
• ascending paralysis
• EMG and CSF analysis (elevated protein without cells)
• potential resp compromise
• IVIg and plasmapheresis