{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T18:14:26Z","timestamp":1759342466914,"version":"3.41.2"},"reference-count":12,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2019,5,20]],"date-time":"2019-05-20T00:00:00Z","timestamp":1558310400000},"content-version":"vor","delay-in-days":2,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,6,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Shapiro syndrome is extremely rare and is characterized by the triad of spontaneous periodic hypothermia, hyperhidrosis and agenesis of the corpus callosum, resulting in neurological and psychological disorders. The exact mechanism of this syndrome is unknown and treatment consists of controlling the periodic attacks. This case report describes a case of Shapiro syndrome presenting with ventricular fibrillation (VF) who was treated with dual chamber implantable cardioverter defibrillator (ICD) therapy.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>A 45-year-old man, suffering from Shapiro syndrome with frequent hypothermic attacks, was admitted to the emergency department with an out of hospital cardiac arrest caused by VF due to hypothermia. To prevent cardiac death during future hypothermic attacks with VF, the patient was treated with a dual chamber ICD. Within 1 month after ICD implantation the patient had two events of ventricular tachycardia\/VF during hypothermia, which were both successfully terminated by an ICD shock. One year after ICD implantation the patient suffered from an uncontrolled urinary tract infection and the patient passed away. Post-mortem interrogation of the ICD did not reveal further episodes of VF and showed a higher supraventricular heartrate in the last days before his death, probably due to a sinus tachycardia driven by the infection. It was concluded that the most likely cause of death was an uncontrolled sepsis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>The current case showed that ICD therapy can be successful in treating VF episodes in patients with unexpected periods of hypothermia.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytz074","type":"journal-article","created":{"date-parts":[[2019,5,20]],"date-time":"2019-05-20T05:00:30Z","timestamp":1558328430000},"source":"Crossref","is-referenced-by-count":4,"title":["Mode of death in Shapiro syndrome: a case report"],"prefix":"10.1093","volume":"3","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8541-6088","authenticated-orcid":false,"given":"Natalia Joanna","family":"Braams","sequence":"first","affiliation":[{"name":"Department of Cardiology, Amsterdam UMC, Vrije Universiteit Amsterdam, De Boelelaan 1117, Amsterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthijs L","family":"Hendriks","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Amsterdam UMC, Vrije Universiteit Amsterdam, De Boelelaan 1117, Amsterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vokko P","family":"van Halm","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Amsterdam UMC, Vrije Universiteit Amsterdam, De Boelelaan 1117, Amsterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2019,5,18]]},"reference":[{"key":"2019091012201158000_ytz074-B1","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1093\/brain\/92.2.423","article-title":"Spontaneous recurrent hypothermia accompanying agenesis of the corpus callosum","volume":"92","author":"Shapiro","year":"1969","journal-title":"Brain"},{"key":"2019091012201158000_ytz074-B2","doi-asserted-by":"crossref","first-page":"405","DOI":"10.5152\/npa.2014.6996","article-title":"Remission of episodic sweating attacks and comorbid depression in Shapiro syndrome: case report","volume":"51","author":"Bozkurt Zincir","year":"2014","journal-title":"Noro Psikiyatr Ars"},{"key":"2019091012201158000_ytz074-B3","doi-asserted-by":"crossref","first-page":"130","DOI":"10.4103\/0028-3886.76868","article-title":"Episodic hyperhydrosis with corpus callosum agenesis: a rare case of Shapiro syndrome","volume":"59","author":"Darku","year":"2011","journal-title":"Neurol India"},{"key":"2019091012201158000_ytz074-B4","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1007\/s10072-015-2438-0","article-title":"A Chinese case of Shapiro syndrome: responsive to carbamazepine","volume":"37","author":"Zhao","year":"2016","journal-title":"Neurol Sci"},{"key":"2019091012201158000_ytz074-B5","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1152\/ajplegacy.1953.175.3.389","article-title":"Experimental hypothermia; respiratory and blood pH changes in relation to cardiac function","volume":"175","author":"Osborn","year":"1953","journal-title":"Am J Physiol"},{"key":"2019091012201158000_ytz074-B6","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1136\/hrt.19.1.59","article-title":"Electrocardiographic changes in induced hypothermia in man","volume":"19","author":"Fleming","year":"1957","journal-title":"Br Heart J"},{"key":"2019091012201158000_ytz074-B7","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1253\/circj.CJ-13-0704","article-title":"J waves in accidental hypothermia","volume":"78","author":"Higuchi","year":"2014","journal-title":"Circ J"},{"key":"2019091012201158000_ytz074-B8","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1136\/hrt.21.3.343","article-title":"The significance of changes in the electrocardiogram in hypothermia","volume":"21","author":"Emslie-Smith","year":"1959","journal-title":"Br Heart J"},{"key":"2019091012201158000_ytz074-B9","doi-asserted-by":"crossref","first-page":"874","DOI":"10.1136\/bmj.289.6449.874","article-title":"Cardiac arrhythmias during rewarming of patients with accidental hypothermia","volume":"289","author":"Rankin","year":"1984","journal-title":"Br Med J (Clin Res Ed)"},{"key":"2019091012201158000_ytz074-B10","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1197\/j.aem.2006.05.022","article-title":"Rewarming rates in urban patients with hypothermia: prediction of underlying infection","volume":"13","author":"Delaney","year":"2006","journal-title":"Acad Emerg Med"},{"key":"2019091012201158000_ytz074-B11","doi-asserted-by":"crossref","first-page":"1998","DOI":"10.1378\/chest.120.6.1998","article-title":"Severe accidental hypothermia treated in an ICU: prognosis and outcome","volume":"120","author":"Vassal","year":"2001","journal-title":"Chest"},{"key":"2019091012201158000_ytz074-B12","doi-asserted-by":"crossref","first-page":"1558","DOI":"10.1136\/heartjnl-2015-308821","article-title":"ECG features and proarrhythmic potentials of therapeutic hypothermia","volume":"102","author":"Lee","year":"2016","journal-title":"Heart"}],"container-title":["European Heart Journal - Case Reports"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/ehjcr\/advance-article-pdf\/doi\/10.1093\/ehjcr\/ytz074\/28671035\/ytz074.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/ehjcr\/article-pdf\/3\/2\/ytz074\/28888742\/ytz074.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,9,10]],"date-time":"2019-09-10T17:25:12Z","timestamp":1568136312000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ehjcr\/article\/doi\/10.1093\/ehjcr\/ytz074\/5491553"}},"subtitle":[],"editor":[{"given":"Borislav","family":"Dinov","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]},{"given":"Habib","family":"Khan","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]},{"given":"Jelena","family":"Kornej","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]},{"given":"Amir","family":"Aziz","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]},{"given":"Peregrine","family":"Green","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2019,5,18]]},"references-count":12,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2019,5,18]]},"published-print":{"date-parts":[[2019,6,1]]}},"URL":"https:\/\/doi.org\/10.1093\/ehjcr\/ytz074","relation":{},"ISSN":["2514-2119"],"issn-type":[{"type":"electronic","value":"2514-2119"}],"subject":[],"published-other":{"date-parts":[[2019,6]]},"published":{"date-parts":[[2019,5,18]]},"article-number":"ytz074"}}