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13 Cards in this Set
- Front
- Back
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Son will you please just read the slide and tell me the components of a motor unit
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motor axon, synapse, muscle fiber, myofibrils
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Myasthenia Gravis
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ONLY MOTOR SX, NO SENSORY SX
autoimmune attach of postsynaptic NMJ Ach receptors presents: fatigueability (improves w/ rest), ptosis, EOM weak (diploplia), trouble eating --> ventilator Ach Ab and muscle specific kinase Ab Dx w/ EMG or injection of short acting AchEi (Tensilon test), ice pack improves sx (ice pack test) Thymoma or thymic hyperplasiais often present and thymectomy is indicated Other autoimmune dz may be present Tx: thymectomy, AchEi, steroids, IVIg, plasma exchange |
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Neonatal MG
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babies of myasthenic mothers
passive transfer of Ab transient disease |
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Congenital MG
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present similarly to neonatal MG
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Lambert Eaton Myasthenic Syndrome
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acquired autoimmune (often paraneoplastic)
Ab against voltage gated Ca channels strength improves w/ continuing effort |
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Myopathies
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Dz that affects primarily muscle
i.e. muscular dystrophy, metabolic, congenital, mitochondrial proximal>distal muscle weakness elevated CK or CPK, myoglobinuria cramps or exercise induces pain check AST and ALT if CK elevated |
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Duchenne Muscular Dystrophy
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reduced or absent expression of dystrophin gene (X-chromosome)
tx: steroids and supportive care Becker = mild Duchenne |
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Myotonic Dystrophy
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delayed relaxation from handshake
temporal and masseter atrophy (myotonic facies), heart arrhythmias, sleep apnea, EDS, gastroporesis, impaired cognition, personality disorder common repeat dz showing anticipation |
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Inclusion Body Myositis
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3 males: 1female
painless progressive muscle weakness distal>proximal *hallmark* beta amyloid deposition in muscle |
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Dermatomyositis
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perifasicular atrophy, capillary changes
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Toxic Myopathy
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STATINS (HMG CoA Reductase Inhibitors), fibrates, EtOH, antiretrovirals, amiodarone, procainamide, chloroquine, D-penicillamine
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Metabolic Myopathies
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exercise induced
cramps, pain, weak, stiff, myoglobin in urine |
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Mitochondrial Myopathies
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progressive or static weak
EOM often involved ragged red fibers = accumulation of abnormal mitochondria |
