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Myasthenia Gravis

Myasthenia Gravis. (Samter’s Ch. 51). Neuromuscular disorder characterized by weakness and fatigability of skeletal muscles decrease in the number of available nicotinic acetylcholine receptors (AChRs) at neuromuscular junctions due to an antibody-mediated autoimmune attack

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Myasthenia Gravis

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  1. Myasthenia Gravis (Samter’s Ch. 51)

  2. Neuromuscular disorder characterized by weakness and fatigability of skeletal muscles • decrease in the number of available nicotinic acetylcholine receptors (AChRs) at neuromuscular junctions due to an antibody-mediated autoimmune attack • 200–400 cases per million (one of the less common autoimmune disorders) • Current treatment highly effective; a specific cure remains elusive • USA: prevalence = 14.2 cases/1 million • Appear at any age • women: onset 20 - 40 years of age • men, at 40-60 • (women : men = 3:2) • Familial occurrence is rare

  3. Annual incidence: 0.25-2/100,000 • Spontaneous remission: 20% • Without treatment, 20-30% die in 10 years • Heterogeneous disorder • – 90% no specific cause • Genetic predisposing factor: HLA association; HLA-BW46 in chinese ocular MG • 75%: thymus abnormal • – 65%: hyperplasia • – 10%: thymoma, rarely in children; often (20%) in patients aged 30-40 years

  4. NEJM 1994, 1997 Neurologic Clinics 1997; BJA 2002 JOAO 2004

  5. Lambert-Eaton Myasthenic Syndrome • Drug-induced Myasthenic Syndromes • Congenital Myasthenic Sybdromes • Myasthenic Syndromes associated with denervation

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