Myotonic Disorders/SMA/IBM

Question 1

Describe polymyositis.

Answer 1

chronic inflammation of mms, onset in adulthood, B proximal weakness noted in upper legs

Question 2

What are some clinical signs of polymyositis?

Answer 2

Onset: weeks to months; IBM: months to years, hard rising from chair, raising arms above one’s head

Question 3

What diagnostic findings will you see with polymyositis?

Answer 3

+mm biopsy, increased CK and EMG findings; Rx with corticosteroids (IBM does not respond to steroids)

Question 4

Describe dermatomyositis.

Answer 4

connective tissue disease related to polymyositis ‘systemic inflammation of skin and mms’ skin rash and proximal mm weakness

Question 5

What are signs of proximal mm weakness?

Answer 5

Hard rising from seated position w/o help, hard going up stairs and raising arms above head, hard to comb hair

Question 6

What are diagnostic findings will you see with dermatomyositis?

Answer 6

increased ESR, +mm biopsy and EMG

Rx: prednisone, IV immunoglobin, plasmapherisis

Question 7

Describe inclusion body myositis.

Answer 7

slow progressive weakness and wasting distally; proximal weakness: onset-months to years, quads and mms that make fist affected early on

Question 8

What are some clinical findings of inclusion body myositis?

Answer 8

freq: trip&falling(foot drop), weak up stairs(weak quads), turning door knobs and grip key(weak long finger flexors)

Question 9

What are some diagnostic findings of inclusion body myositis?

Answer 9

Increased CK 10x>normal, + EMG findings and mm biopsy

Question 10

Describe spinal mm atrophy

Answer 10

death of neuron cells in anterior horn of spinal cord and system wide mm wasting. No sensory affects; PROXIMAL LE mms 1st-mms UE, spine and neck
Clinically present with hypotonia and areflexia