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Myopathies Flashcards

1
Q

inflammatory myopathies

A

heterogenous group of muscle diseases
symmetric upper and lower PROXIMAL muscle weakening
also neck, abs, upper 1/3 esophagus, diaphragm, thoracic muscles, sphincter ani
Sx: GI (REFLUX, incontinence, intestinal bleeding), ILD, arthritis, HEART (myocarditis, CAD, arrhythmia)
can occur with other autoimmune CT disease
associated with: BREAST CA, ADENOCARCINOMA (DM more than PM)
lab: elevated CK, abnormal electromyograms
Tx: corticosteroids, cytotoxic drugs

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2
Q

dermatomyositis

A

more in females
5-15 yrs, 45-60 yrs
SKIN
bigger association with: BREAST CA and ADENOCARCINOMA
perivascular infiltrates: PERIMYSIAL, CD4 T cells (macrophages, dendritic cells, B cells)

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3
Q

anti-Jo-1

A

myopathy

associated with arthritis

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4
Q

antisynthetase syndrome

A

anti-Jo-1

Sx: MYOSITIS, non-erosive symmetric polyarthritis of SMALL joints, MECHANIC’s HANDS

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5
Q

amyopathic dermatomyositis

A

Sx: DM type rash, NO myositis (can develop late), SEVERE ILD

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6
Q

juvenile dermatomyositis

A

peak onset: 6-11 years

Sx: CALCINOSIS, cutaneous ULCERATION, LIPODYSTROPHY

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7
Q

inclusion body myositis

A

MEN over 50
histo: sarcoplasmic and nuclear INCLUSIONS with RIMMED VACUOLES
Sx: insidious muscle weakness in over MONTHS to YEARS localized to THIGH and FINGER FLEXORS
PROXIMAL LOWER extremities, DISTAL UPPER extremities
RESISTANT to GLUCOCORTICOIDS
ENDOMYSIAL, CD8 T cells (and macrophages), mononuclear inflammatory cells surround and invade non-necrotic muscle fibers
MHC CLASS I

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8
Q

polymyositis

A

more common in females
late teens, older 50-60 yrs
ENDOMYSIAL, CD8 T cells (and macrophages), mononuclear inflammatory cells surround and invade non-necrotic muscle fibers
MHC CLASS I
complement, Ab, cytokines cause endothelial damage, hypoxia, capillary loss leads to loss of skeletal muscle fibers

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9
Q

anti-Mi2

A

Ab: nuclear helicase

dermatomyositis

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10
Q

Ab against tRNA synthetases

A

associated with ILD

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11
Q

anti-PM-Scl

A

associated with overlap syndrome

includes features of scleroderma, mild muscle disease, arthritis, limited skin sclerosis

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12
Q

polymyalgia rheumatica (PMR)

A

over 50
AM STIFFNESS/PAIN SHOULDER/HIP: disuse leads to atrophy and weakness
bursitis (subdeltoid, subacromial), tendon inflammation, pitting edema in distal extremities
lab: elevated ESR
association: temporal giant cell arteritis (15%), carpal tunnel (10-15%) due to tenosynovitis
Tx: glucocorticoid (doesn’t respond then NOT PMR)

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13
Q

temporal (giant cell) arteritis

A

older, females
necrotizing inflammation of large sized arteries originating from aorta
GRANULOMA
Sx: headache, jaw claudication, visual, systemic, arthralgia, tender swollen temporal artery, upper respiratory
complication: AORTIC ANEURYSM (wide mediastinum)
association: polymyalgia rheumatica (50%)
IFN-y, macrophages, giant cells, MMPs
lab: elevated ESR, hepatic enzymes: AST, ALKALINE PHOSPHATASE
Dx: biopsy
Tx: glucocorticoids ASAP (so don’t go BLIND)

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14
Q

ESR (sed rate)

A

measures rate RBC gall through plasma in 1 hr
distance between the top of plasma level and the top of the sedimented RBC is ESR
indirect measure of fibrinogen levels, influenced by RBC properties

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15
Q

C-reactive protein (CRP)

A

direct measure of serum CRP
more SENSITIVE than ESR
wider range

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16
Q

X-linked muscular dystrophy

A

male, BIG CALVES, GOWER sign
DYSTROPHIN gene
Duchenne: deletion
Becker: mutated

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17
Q

Gower’s sign

A

use hands to stand up and climb body over legs

18
Q

peripheral neuropathy

A

distal muscle wasting, decreased sensation, depressed reflexes

19
Q

motor neuron disease

  1. pattern of weakness
  2. fasciculations
  3. reflexes
  4. sensory loss
A
  1. weakness: variable, usually symmetric (exception ALS)
  2. reflexes: variable, decreased in most (exception ALS)
  3. fasciculations
  4. no sensory loss
20
Q

polyneuropathy

  1. pattern of weakness
  2. fasciculations
  3. reflexes
  4. sensory loss
A
  1. weakness: distal > proximal
  2. fasciculations sometimes
  3. reflexes: decreased/absent
  4. sensory loss
21
Q

diseases of neuromuscular junction

  1. pattern of weakness
  2. fasciculations
  3. reflexes
  4. sensory loss
A
  1. weakness: proximal > distal, often extraocular
  2. no fasciculations
  3. reflexes: normal in MG, decreased in lambert-eaton and botulism
  4. no sensory loss
22
Q

myopathy

  1. pattern of weakness
  2. fasciculations
  3. reflexes
  4. sensory loss
A
  1. weakness: proximal > distal
  2. no fasciculations
  3. reflexes: normal initially, decreased later
  4. no sensory loss
23
Q

anterior horn disease

  1. serum muscle enzymes
  2. nerve conduction studies
  3. electromyography
  4. repetitive nerve stimulation test
  5. muscle biopsy
A
  1. normal
  2. normal
  3. decreased number motor units, evidence of denervation and re-innervation
  4. normal, decremental response can occur
  5. denervation: atrophic angular and target fibers, fiber type grouping, group atrophy
24
Q

peripheral neuropathy

  1. serum muscle enzymes
  2. nerve conduction studies
  3. electromyography
  4. repetitive nerve stimulation test
  5. muscle biopsy
A
  1. normal
  2. slow or low amplitude CMAPs and SNAPs
  3. decreased number motor units, evidence of denervation and re-innervation
  4. normal
  5. denervation: atrophic angular fibers, fiber type grouping, group atrophy
25
myopathy 1. serum muscle enzymes 2. nerve conduction studies 3. electromyography 4. repetitive nerve stimulation test 5. muscle biopsy
1. increased 2. normal 3. normal/increased motor unit number recruitment, short duration/low amplitude frequency polyphasic 4. normal 5. myopathic (necrosis, storage material, inflammation)
26
neuromuscular transmission defects 1. serum muscle enzymes 2. nerve conduction studies 3. electromyography 4. repetitive nerve stimulation test 5. muscle biopsy
1. normal 2. normal 3. normal or small motor units, variability or MUAPs 4. CMAPs at low are of stimulation, decrease amplitude, increment at fast rates in presynaptic disorders 5. normal or type II atrophy
27
CMAPs
compound muscle action potential
28
SNAPs
sensory nerve action potentials
29
Duchenne's muscular dystrophy
lethal, children X linked recessive DYSTROPHIN deletion: excessive Ca enters and activates proteases that cause muscle atrophy Sx: progressive muscle weakness, pseudo hypertrophy of calves, abnormal heart, low IQ, wing scapula, lordosis lab: CK Tx: potentially morpholinos
30
Becker's muscular dystrophy
milder, live longer X-linked recessive DYSTROPHIN mutation Sx: lordosis, calf hypertrophy
31
myotonic dystrophy
AD increased CGT repeats on chromosome 19: encodes protein kinase Sx: percussion and grip myotonia, FACIAL, NECK, DISTAL weakness, cataracts, bald, DM, sleep apnea, megaesophagus/colon, heart conduction defects, mental retardation in newborns of myotonic mothers
32
proximal myotonic dystrophy
similar to myotonic dystrophy PROXIMAL weakness chromosome 3
33
metabolic myopathies
disorders of glycogen, lipid metabolism, or mitochondrial respiratory chain Sx: muscle weakness, exercise intolerance, muscle pain, myoglobinuria
34
McArdle's
muscle PHOSPHORYLASE deficiency: glycogen can not break down in exercise to produce ATP, lactate, pyruvate for oxidative metabolism lab: lack of increase LACTATE with ischemic exercise Sx: muscle cramps and stiffness on exercise relieved by rest, COKE colored URINE lab: elevated CK
35
infantile acid maltase deficiency
floppy baby | congenital
36
endocrine myopathies
PROXIMAL, non-specific biopsy: type II atrophy | cushing's syndrome, hypothyroidism (muscle spasms, high CK, obese), hyperparathyroidsim (muscle weakness)
37
toxic myopathies
muscle fiber atrophy, VACUOLIZATION, mitochondrial dysfunction, fiber necrosis ethanol, anesthetics, cholesterol lowering drugs, steroids, narctoics, herbicides, insecticides, flame retardants, etc.
38
myasthenia gravis
Ab destroy postsynaptic ACh receptors Sx: PTOSIS (increased with sustained upward gaze), diplopia, fluctuating muscle weakness WORSENS with ACTIVITY CMAP: low Tx: thymectomy, immunotherapy, anti cholinesterase drugs, steroids, plasma exchange
39
Eaton-lambert
Ab to presynaptic Ca channel: no ACh release Sx: DRY MOUTH, PROXIMAL limb weakness, metallic taste, ANTICHOLINERGIC symptoms, REDUCED REFLEXES, UNREACTIVE PUPILS (impaired sympathetic function) IMPROVE with ACTIVITY association: small cell lung CA Tx: remove CA, 3,4 diaminopyridine, immunosuppressive: steroids, azathioprine, plasmapheresis
40
aminopyridine
affects voltage gated potassium channels, prolongs depolarization and improves neuromuscular transmission Tx: eaton lambert

Musculoskeletal (13 decks)

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