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Criteria for Myopathy?
- muscle ache/weakness
- CK are 10X normal
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3 Infectious Polyneuropathies?
- VZV
- Diptheria
- Mycobacterium Leprae
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What is albuminocytological Dissociation?
WHere is it seen?
normal cell count but elevated protein count
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MCC of death of NMJ and inflammatory demyelinating Neuropathy?
respiratory failure
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Charcot Marie-Tooth Disease affects what ?
- Hereditary Motor and Sensory Neuropathy
- peroneal muscle group--atrophy..inverted bottle..thigh muscle are bulky but peroneal arent..
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Charcot-Marie tooth Disease affects what nervous system?
peripheral
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What is deficient in Charcot-Marie-Tooth Disease?
structural protein-connexin involved in function of peripheral nerve
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What 2 pathogenesis are responsible for Diabetic neuropathy?
ischemia to nerve bc of arteriosclerosia and sorbitol accumulation casing osmotic rupture of cells
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How are Polymyositis and dermatomyositis different from inclusion body myositis?
- Polymyositis and dermatomyositis involve proximal muscle groups i.e shoulder and pelvic girdle
- Inclusion body involves distal asymmetrical myopathy
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What myopathy has ANA and Anti-Jo1 antibodies?
Dermatomyositis
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What is the difference between Polymyositis and dermatopolymyositis?
Both involve myopthay of proximal muscles by dermatomyositis has a rash
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WHich of the myopathies is associated with ca of lung, stomach, ovary?
Dermatomyositis
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Periendomysial and perifascicular lymphocytic infiltrate is seen in what two myopathies?
Polymyositis and Dermatomyositis
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Cytoplasmic vacuoles, eosinophilic granules and amyloid are present in what Myotpathy?
Inclusion Body Myositis
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Myotonic Dystrophy presents how?
How is it inherited?
Whats seen on microscopy?
How is it elicited?
- stifness
- CTG repeats
- Ring fibrs
- Percussion of thenar eminence
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4 Trinucleotide repeat disease?
- Friedrich's Ataxia
- Mytonic dystrophy
- Huntingtons
- Fragile X syndrome
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What is the origin of Myesthenia gravis antibodies against ach receptor?
Thymoma/THymic hyperplasia
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In terms of presentation how is Myesthenia gravis different from Lambert eaton syndrome?
- MG-symptoms worsen with use
- LES-symptoms improve with use
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What is the origina of Lambert eaton syndrome antibodies?
Small cell carcinoma
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Diagnosis of MG?
Tx?
- endrophonium
- Neostigmine, steroids, thymectomy
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In terms of muscles affected what is the difference between MG and LES?
- MG--facial muscles
- LeS-proximal--shoulder girdle
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How does ETOH binge drinking cause myopathy?
rhabdomyolysis---myoglobinuria
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What 2 drugs can induce myopathy?
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What endocrine disorder can cause myopathy?
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What substance can cause myopathy?
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In floopy infant with marked hypotonia, what happens to CK levels?
unchanged
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Red ragged fibers are seen with what myopathies?
Mitchondrial Neuropathy
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2 conditions that have mitochondrial inheritance?
Leiber's hereditary optic neuropathy--degeneartion of ganglion cells in the retina nd their axons
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Duchenes and Beckers muscular dystrophies are inherited how?
X linked inheritance
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A pt using hands to get up is called what?
Gower's sign
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Calf pseudohypertrophy is seen in what condition?
Duchene's muscular dystrophy
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What is in the pseudohypertrophied calf muscle of Duchene kids?
fibrous, inflammatory, adipose tissue
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Role of Dystrophin?
links actin to plasma membrane
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What muscles are involved in Duchene's mUscular dystrophy?
skeletal and cardiac
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