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