Skeletal Muscle and Peripheral Nerve Pathology II Flashcards by Matt Stanton

botulism

clostridium botulinum neurotoxin

-blocks release of ACh from presynaptic neurons

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canned food

botulism

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double vision, blurry vision, slurred speech, difficulty swallowing, dry mouth, muscle weakness (shoulders to body)

botulism

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type I muscle fibers

aerobic
high lipid
oxidative

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one slow fat red ox

type 1 muscle fiber
slow - aerobic exercise
high lipid
red colored - high myoglobin
oxidative

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ATPase at differnt pH levels

shows type 1 or type 2 fibers - in checkerboard pattern

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perifascicular atrophy

dermatomyositis

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corticosteroid use

type 2 fiber atrophy and sparing of type 1 fibers

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reinnervation

switch in fiber type

increase in motor unit size - more myofibers innervated by individual axon

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type grouping

of muscle fibers
-with ongoing denervation and reinnervation

loss of checkerboard

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skeletal m disorder

either myopathy or neurogenic issue

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neurogenic injury

fiber type grouping and grouped atrophy**

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creatine kinase

released with segmental myofiber degeneration

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exercise

myofiber hypertrophy

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cytoplasmic inclusions

seen in several primary forms of myopathy

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three primary inflammatory myopathies

polymyositis
dermatomyositis
inclusion body myositis

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lymphocytes in atrophic muscle fibers

inflammatory myopathy

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dermatomyositis

systemic autoimmune disease
proximal muscle weakness and skin changes

swollen hands and lesions on knuckles

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dermatomyositis autoAbs

anti-M2 - gottron papule and heliotrope rash (around eyes)
anti-Jo1 - lung disease, nonerosive arthritis, mechanic hand rash
anti-P155/P140 - paraneoplastic anc juvenile

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gottron papules and heliotrope rash

dermatomyositis hand lesions

-with anti-Mi2

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interstitial lung disease, nonerosive arthritis, mechanic hand rash

dermatomyositis

-with anti-Jo1 - worse prognosis

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paraneoplastic and juvenile dermatomyositis

anti-P155/P140

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perifascicular atrophy

dermatomyositis

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proximal muscle weakness, myalgias, elevation in serum creatine kinase, heliotrope rash, gottron papules, dysphagia

dermatomyositis

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heliotrope rash

around eyes | -with dermatomyositis

association with dermatomyositis

interstitial lung disease rapidly progressive and can lead to death also - cardiac involvement

polymyositis

like dermatomyositis - but no skin lesions has myalgia and weakness inflammation in muscles

endomysial mononuclear inflammatory cells in muscles

polymyositis

age of dermatomyositis and polymyositis

30-40yo

age of inclusion body myositis

>60yo

quadriceps progressive muscle weakness in patient 65yo

inclusion body myositis

nuclei in middle of muscle cell

inclusion body myositis

tx of polymyositis and dermatomyositis

corticosteroids

tx of inclusion body myositis

respond poor to steroids/immunosuppressants

common complication of statins

myopathy within 6 weeks - check CK levels if weakness - RTO for eval

toxic myopathies

statins chloroquine, hydroxychloroquine thyrotoxic alcohol

acute toxic rhabdomyolysis, myoglobinuria, renal failure

with binge drinking alcohol

ethanol myopathy

type II fibers

inherited skeletal m disease

RyR - malignant hyperthermia AD NEM and AR NEM - childhood weakness - floppy infant XL, Xq28, AD, and AR - severe congenital hypotonia - floppy infan and poor prognosis with X-linked form

malignant hyperthermia

mutation in RyR

muscular dystrophies

most X-linked | mutations in dystrophin**

duchenne and becker muscular dystrophy

differ in amount of loss of dystrophin function both LOF mutations of dystrophin - on X chromosome over time - disease progression - fatty replacement

dystrophin

mutated in muscular dystrophy

dystrophin stain

absent in duchenne | reduced in becker

walking delayed and can't keep up with peers

duchenne dystrophy see marked elevation of creatine kinase during first decade of life - then falls as muscle mass loss

pseudohypertrophy

enlargement of lower leg muscles with weakness | -fat replacement

crawl around, hands to stand up, hands on hip to stand

muscular dystrophy - duchennes or becker

myotonic dystrophy

auto dominant | -skeletal m weakness, cataracts, endocrinopathy, cardiomyopathy

myotonia

sustained involuntary contraction of muscles

mutation in myotonic dystrophy

expansion of CTG triple repeats in 3' non-coding region of DMPK gene

association with myotonic dystrophy

hirsutism

symptoms with exercise or fasting

lipid or glycogen metabolism diseases

bad with exercise

mitochondrial myopathies

mcardles disease

can't break down sugars

ragged red fiber and phonograph record

myopathies due to inborn errors of metabolism

DDx for infantile hypotonia

primary disease of skeletal muscle abnormality of brain neuronopathy - spinal muscular atrophy

spinal muscular atrophy

rare hypertrophy of muscle fibers with surrounding atrophic fibers

periodic paralysis

ion channel myopathies can be hyperK, hypoK, or normoK

RYR1 mutation

malignant hyperthermia -anesthetic trigger hypermetabolic state tetany and excessive heat production

rimmed vacuoles

inclusion body myositis

MPNST

malignant peripheral nerve sheath tumor

peripheral nerve sheath tumor

schwannoma neurofibroma malignant peripheral nerve sheath tumor

loss of merlin

schwannoma

benign | cell differentiation and often arise from peripheral nerves

schwannoma histo

dense eosinophilic antoni A areas and loose pale antoni B areas also hyalinized blood vessels

veracay bodies

antoni A area where tumor cell nuclei align with areas of anuclear zone in schwannoma

tinnitus and hearing loss

schwannoma at CPA - cerebellopontine angle CN VIII aka acoustic neuroma

neurofibroma

benign nerve sheath tumor heterogenous composition neoplastic schwann cells admixed with perineural like cells, fibroblasts, mast cells, CD34 spindle cells

NF1

associated with neurofibromas multiple** superficial cutaneous neurofibromas diffuse neurofibroma - large plaquelike elevation of skin plexiform - deep - nerve roots or large nerves

MPNST

high grade tumors divergent differentiation - focal areas that exhibit other lines of differentiation triton tumor

triton tumor

MPNST malignant peripheral nerve sheath tumor

cafe au lait spots

with neurofibromatosis type I

b/l CN VIII schwannoma and multiple meningiomas

neurofibromatosis type II

NF2

associated with schwannomas

Skeletal Muscle and Peripheral Nerve Pathology II Flashcards

(74 cards)