Shapiro Syndrome Secondary to a Vein of Galen Malformation Responding to Cyproheptadine: A Case Report and Review

Mansour H, Hmaimess G, Tohme R, Alam W, Ezzeddine L and Adem C

Published on: 2019-09-10


Though extremely rare, Shapiro syndrome classically presents with a triad of hypothermia, hyperhidrosis, and agenesis of corpus callosum. Around 50 cases of Shapiro syndrome have been reported to date. Interestingly, while Shapiro Syndrome is idiopathic, it is postulated that Vein of Gaven Malformation (VAGM) can lead to compression of the hypothalamus and corpus callosum, leading to underdevelopment of the corpus callosum and manifestations of Shapiro Syndrome. Here we report the case of a 5-month-old baby boy, with history of VAGM, and controlled epilepsy presenting with symptoms mimicking Shapiro Syndrome, with a successful response to Cyproheptadine.