Abstract

WE have recently seen an 11-year-old boy who insidiously developed dysarthria, dysphagia, drooling, and a change in temperament characterized by nervousness and emotional lability. A steady deterioration continued both mentally and physically which was reflected in his poor school performance. About 12 months after the onset of symptoms he began carrying his left arm in a constant flexed posture and experienced oculogyric crises lasting from minutes to an hour. The boy was first seen and admitted to Vanderbilt University Hospital 18 months after the onset of symptoms. Examination revealed a lack of facial expression and an odd sardonic smile. The glabellar tap sign (Meyerson) was positive. Dysarthria, dysphagia, and weakness in chewing were present. Though a gag reflex was elicited on stimulation of both sides of the soft palate, no movement was noted on phonation. At rest there was a tremor and cogwheel rigidity confined to the left arm.

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