Neuromuscular transmission disorder characterized by fluctuating weakness and fatigability of bulbar and other voluntary muscles without loss

of reflexes or impairment of sensation or other neurologic function. Diagnostic criteria: A. Characteristic signs and symptoms One or more of the following: 1. Diplopia, ptosis, dysarthria, weakness in chewing, difficulty in swallowing, muscle weakness with preserved deep tendon reflexes, and, less commonly, weakness of neck extension and flexion, and weakness of trunk muscles 2. Increased weakness during exercise and repetitive use with at least partially restored strength after periods of rest 3. Dramatic improvement in strength following administration of anticholinesterase drug (edrophonium (Tensiln) and neostigmine); and one or more of the following: B. EMG and repetitive stimulation of a peripheral nerve: In myasthenia gravis repetitive stimulation at a rate of 2 per second shows characteristic decremental response which is reversed by edrophonium or neostigmine. Single fiber studies show increased jitter. C. Antibodies to Acetylcholine Receptors Exclusions: Congenital myasthenic syndrome, progressive restricted myopathies, steroid and inflammatory myopathies, motor neuron disease Multiple sclerosis, variants of Guillain-Barr syndrome (e.g., Miller-Fisher syndrome) Organophosphate toxicity, botulism, black widow spider venom Eaton-Lambert syndrome Stroke Medications: neuromuscular blocking agents, aminoglycosides, penicillamine, antimalarial drugs, colistin, streptomycin, polymyxin B, tetracycline Hypokalemia; hypophosphatemia Severity: (Osserman classification):

I: Ocular myasthenia IIA: Mild generalized myasthenia with slow progression: no crises, responsive to drugs IIB. : Moderately severe generalized myasthenia : severe skeletal and bulbar involvement but no crises; drug response less than satisfactory III: Acute fulminating myasthenia, rapid progression of severe symptoms, with respiratory crises and poor drug response IV: Late severe myasthenia, same as III but progression over 2 years from class I to II

References: 1. Ossermann KE. Myasthenia gravis. New York: Grune and Stratton; 1958. 2. Engel AG. Myasthenic syndromes. In: Engel AG, Franzini Armstrong C, editors. Myology. 2nd ed. New York: McGraw Hill; 1994. p. 1798-835. Created: October 11, 2006