Shapiro syndrome with hypothalamic hypothyroidism.

Arq Neuropsiquiatr

Department of Pediatric Intensive Care, Hospital Sabara, Sao Paulo, Brazil.

Published: June 2008

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http://dx.doi.org/10.1590/s0004-282x2008000300029DOI Listing

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SummaryShapiro's syndrome is a rare neurological disease. The triad of Shapiro's syndrome includes episodes of hyperhidrosis, hypothermia and complete/partial agenesis of the corpus callosum. We report a case of a young male who had episodic chills, increased sweating and fatigue.

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Objectives: Shapiro's syndrome (SS) is a rare condition characterized by spontaneous periodic hypothermia. The underlying pathophysiological mechanisms and etiology of this syndrome remain controversial, and fewer than 100 cases have been reported to date. The objective of this case report is to present a unique iatrogenic case of SS and contribute additional insights into the underlying etiology of this rare disorder.

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Unlabelled: Spontaneous periodic hypothermia (SPH) is an exceptionally rare condition characterised by paroxysmal episodes of spontaneous hypothermia. While commonly associated with Shapiro syndrome, which includes SPH, hyperhidrosis and agenesis of the corpus callosum, there are also cases of SPH that do not exhibit these defining characteristics, known as 'Shapiro syndrome variant'. These variants may present with diverse brain imaging findings but no agenesis of the corpus callosum, suggesting different potential aetiologies.

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