Pathoma - Skeletal Muscle/Neuromuscular Junction Flashcards
Dermatomyositis - what is it?
inflammatory condition of skin and skeletal muscle
Dermatomyositis - etiology
unknown- some association with carcinoma
Dermatomyositis - clinical presentation
bilateral proximal muscle weakness (can’t comb hair or climb stairs)
rash on eyelids
malar rash may be seen
Dermatomyositis - Lab values
increased creatine kinase (CK)
+ ANA
+ anti-Jo1
Dermatomyositis - treatment
corticosteroids
Dermatomyositis - muscle biopsy
perimysial inflammation w. perifasicular atrophy
Polymyositis - what is it?
inflammation of skeletal muscle (no skin involvement)
Polymyositis - muscle biopsy
endomysial inflammation
X-linked muscular dystrophy (Duchenne’s) - what is it?
degenerative disorder w/ muscle wasting
X-linked (Duchenne’s) muscular dystrophy - cause
dystrophin deletion
X-linked muscular dystrophy - clinical presentation
proximal muscle weakness at 1 year age
progresses distally
calf pseudohypertrophy
X-linked muscular dystrophy - lab values
increased creatine kinase (CK)
Becker muscular dystrophy - cause
dystrophin mutation
Becker muscular dystrophy - clinical presentation
milder disease
presents later
Myasthenia gravis - cause
autoAb’s against post-synaptic Ach receptors
Myasthenia gravis - clinical presentation
women
worsens w/ use
ptosis
improves with anticholinesterase agents
Myasthenia gravis - association
thymic hyperplasia or thymoma
Lambert-Eaton syndrome - cause
autoAb’s against pre-synaptic Ca2+ channels (also impairs Ach release)
Lambert Eaton - association
Paraneoplastic syndrome (small cell carcinoma most common)
Lambert Eaton - clinical presentation
improves w/ use
eyes not affected
anticholisterase agents don’t help
improves w/ tumor removal
