Pathology: Muscle and peripheral nerve disease Flashcards
what to ask for in myositis history?
tryptophan in herbal preparations
myophagia is feature of what?
polymyositis
in polymyositis, what can you see in the muscle on microscopy?
regenerating fibres
what is histocompatibility in poly myositis?
autoimmune against MHC-class I sarcolemmal membrane
any myophagia in inclusion-body myositis?
nope
where is the inflmmatory cell infiltration in inclusion-body myositis??
interstitium
with rimmed vacuoles
what is the inclusion-body myositis bodies?
amyloid
a-beta protein
what is dermatomyositis linked with
malignancy
dermatomyositis juvenile form presents how?
more systemic features: lethargy
not associated with malignancy
dermatomyositis pathophysiology?
complement: MAC attack complex is expressed in blood vessels due to autoimmune
2 kinds of structural myopathies? cause by?
dystrophies
protein aggregate myopathies
by genetics
what protein affected in Duchenne muscular dystrophy
dystrophin
what is dysferlin?
protein important structure in muscle
seen in limb girdle muscular dystrophy
Pompe’s disease is? what do you see?
acid maltase disease
see lots of vacuoles filled with glycogen
what is McArdle’s disease? myophosphorylase deficiency? see what clinically?
get fatigue quickly but get second wind as other glycogen recpetors are still active
carnitine deficiency what kind of muscle disease?
lipid storage diseases
mitochondrial myopathies cause?
defect in mitochondrial DNAs
mitochondrial myopathies in only muscles?
nope, can seen in brain too, presents with seizures
mitochondrial myopathies, muscle biopsy tell-tale sign?
ragged-red fibres
neurogenic disorders, where can pathology be?
upper-motor neuron, anterior horn cells
long tracts
peripheral nerves
3 conditions central neurogenic disorders?
MND
ALS
spinal muscular atrophy
3 peripheral neuropathy types
axonal degen
demyelination
mixed axonal degen and demyelination
3 main features of denervation?
group atrophy
type grouping
target fibres
axonal neuropathy process post injury?
- wallerian degeneration: distal from injury and proximal to first node of ranvier
- axonal regeneration from axon sprouting