Mesh : Action Potentials / physiology Adult Electric Stimulation Electromyography Electrophysiology Fluoxetine / therapeutic use Humans Male Myasthenic Syndromes, Congenital / diagnosis drug therapy physiopathology Neural Conduction / physiology Neuromuscular Junction / physiopathology Serotonin Uptake Inhibitors / therapeutic use

来  源:   DOI:10.1590/s0004-282x2006000200028   PDF(Sci-hub)

Abstract:
The slow-channel syndrome is one of the congenital myasthenic syndromes attributed to inherited kinetic disorders of the ion channel of the acetylcholine receptor of the neuromuscular junction. This is a case report of 25-years-old man with progressive ptosis and limitation of ocular movements since infancy, presented a 6-years history of worse of the external ophthalmoparesis and muscular weakness in the shoulders and hands. The motor nerve conduction studies after a supramaximal single stimulus disclosed a double compound muscle action potential (CMAP) that disappeared after a voluntary contraction of 30 seconds. Repetitive stimulation of facial and spinal accessory nerves showed a CMAP decrement greater than 10% with disappeared of the second potential. The patient received fluoxetine with mild improvement of muscular weakness, but persisted with: ptosis, limitation of ocular movements and repetitive CMAP in the motor nerve conduction study. The characteristic of disease are discussed.
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