[Concurrence of acute autonomic and sensory neuropathy and myasthenia gravis--a case report and pathogenetic considerations].
A 22-year-old woman admitted to the hospital on Mar. 19, 1985 because of syncopal attacks and numbness on the limbs. Examination revealed peripheral sympathetic as well as parasympathetic dysfunctions and loss of myelinated as well as unmyelinated fibers in a biopsied sural nerve. A diagnosis of acute autonomic and sensory neuropathy (AASN) was made. She was treated with prednisolone but did not respond to the therapy. Only L-DOPS was effective on orthostatic hypotension. Recovery of the neurological conditions was insufficient till May 1987, when she complained of easy fatigability when speaking and chewing. Pharmacological and electrodiagnostic tests showed typical myasthenic phenomenon. At this time, serum anti-ACh receptor antibody was 741nmol/l and a diagnosis of myasthenia gravis was confirmed. Anti-ACh receptor antibody was proven to be elevated in the serum collected on April 1985 when the initial symptom of AASN had appeared. A thymectomy resulted in prompt and complete remission of semiologies of both myasthenia gravis and AASN. These suggest that immunological abnormality might play an important role in the pathogenesis of AASN in this case.
Senda
Y
Y
Sugimura
K
K
Koike
Y
Y
Matsuoka
Y
Y
Takahashi
A
A
D000328:Adult; D001323:Autoantibodies / Q000032:analysis; D001342:Autonomic Nervous System Diseases / Q000175:diagnosis / Q000209:etiology*; D004576:Electromyography; D005260:Female; D006801:Humans; D009157:Myasthenia Gravis / Q000175:diagnosis / Q000209:etiology*; D011950:Receptors, Cholinergic / Q000276:immunology
D001323:Autoantibodies; D011950:Receptors, Cholinergic