title: [A rare case of thermoregulation disorder: Shapiro syndrome].
abstract: Anomalies of the corpus callosum are the most frequent malformations of the central nervous system. The triad of spontaneous periodic hypothermia and hyperhydrosis with the agenesis of corpus callosum is described as Shapiro syndrome. Shapiro syndrome is a very rare condition and it can occur in every age group. The presence of agenesis of corpus callosum is not a strict criteria of the syndrome; the most important presenting symptom is paroxysmal hypothermia. Although the definite cause of recurrent hypothermia is unknown, dysfunction of the hypothalamus is suspected. From therapeutic aspects, only supportive therapy is available. In this report the authors present the first Shapiro syndrome case diagnosed in Hungary. The main symptoms of the 21-year-old male patient were recurrent hyperhydrosis with hypothermia resulting in severe general malaise. The skull magnetic resonance imaging demonstrated agenesis of corpus callosum. The patient was treated with clonidine resulting in significant improvement of symptoms.
authors: Neurológiai Osztály, Jász-Nagykun-Szolnok Megyei Hetényi Géza Kórház-Rendelőintézet Szolnok.
authors: Júlia
authors: J
authors: Szabó
authors: Neurológiai Klinika, Szegedi Tudományegyetem, Általános Orvostudományi Kar, Szent-Györgyi Albert Klinikai Központ Szeged, Semmelweis u. 6., V. emelet, 6725.
authors: Dénes
authors: D
authors: Zádori
authors: Neurológiai Klinika, Szegedi Tudományegyetem, Általános Orvostudományi Kar, Szent-Györgyi Albert Klinikai Központ Szeged, Semmelweis u. 6., V. emelet, 6725.
authors: Edina Tímea
authors: ET
authors: Varga
authors: Neurológiai Klinika, Szegedi Tudományegyetem, Általános Orvostudományi Kar, Szent-Györgyi Albert Klinikai Központ Szeged, Semmelweis u. 6., V. emelet, 6725.|MTA-SZTE Idegtudományi Kutatócsoport Szeged.
authors: László
authors: L
authors: Vécsei
mesh_terms: D061085:Agenesis of Corpus Callosum / Q000150:complications* / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D001833:Body Temperature Regulation / Q000187:drug effects; D003000:Clonidine / Q000627:therapeutic use; D003337:Corpus Callosum / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D006814:Hungary; D006945:Hyperhidrosis / Q000150:complications / Q000209:etiology* / Q000473:pathology; D007035:Hypothermia / Q000150:complications / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D055815:Young Adult
chemical_list: D003000:Clonidine
keywords: Shapiro syndrome; Shapiro-szindróma; corpus callosum agenesia; corpus callosum agenesis; termoregulációs zavar; thermoregulation disorder