Keywords
Reverse Shapiro syndrome; West syndrome; Corpus callosum agenesis; Hyperthermia
Abstract
Reverse Shapiro syndrome consists of the association of unexplained hyperthermia and corpus callosum agenesis. The genetic basis is still unidentified; yet, neurochemical abnormalities as well as hypothalamic dysfunction have been proposed as possible etiologies. Only four cases of reverse Shapiro Syndrome have been reported to date and many therapeutic managements have been proposed without any radical success. Here we report a new case of a 6 months old male patient, with controlled cryptogenic epilepsy who is also presenting with a reverse Shapiro syndrome. The patient presented with recurrent episodes of hyperthermia that responded completely to a treatment by cyproheptadine.
Citation
Mansour H, Sabouneh M and Hmaimess G. A Case of Reverse Shapiro Syndrome Responding to Cyprohepatadine. SM J Pediatr. 2017; 2(2): 1010