A case of Shapiro’s Syndrome in an African young man

Abstract

Shapiro’s syndrome is a rare congenital neurological disease characterized by episodes of excessive sweating and hypothermia, and by complete/partial agenesis of the corpus callosum. We report an 18 year-old male who was referred to Princess Marina Hopsital with a longstanding history of episodic shaking chills, excessive sweating, fatigue, and unsteady gait. During the episodes, he was bradycardic (pulse rate of less than 36 beats/minute), with blood pressure of less than 80/45 mmHg, and his axillary body temperatures were unrecordably low. Neurologic examination showed cerebellar signs. The MRI of the brain revealed agenesis of the corpus callosum with no other abnormalities. The patient responded to clonidine therapy. Shapiro’s syndrome is an important consideration when evaluating a patient with episodic hyperhidrosis and hypothermia. The syndrome is rare worldwide and to the best of our knowledge, this report constitutes the first documentation of a case from Africa.