{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T02:32:24Z","timestamp":1790994744741,"version":"4.1.0"},"reference-count":28,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2011,11,16]],"date-time":"2011-11-16T00:00:00Z","timestamp":1321401600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2012,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p><jats:bold>Purpose:\u2002<\/jats:bold> The occurrence of acute encephalopathy in children with Dravet syndrome has been reported sporadically. This study clarified the features of acute encephalopathy in children with Dravet syndrome.<\/jats:p><jats:p><jats:bold>Methods:\u2002<\/jats:bold> Through the mailing list of the Annual Zao Conference on Pediatric Neurology, we collected 15 patients with clinically diagnosed Dravet syndrome, who had acute encephalopathy, defined as a condition with decreased consciousness with or without other neurologic symptoms, such as seizures, lasting for &gt;24\u2003h in association with infectious symptoms.<\/jats:p><jats:p><jats:bold>Key Findings:\u2002<\/jats:bold> There were seven boys and eight girls. A mutation of the <jats:italic>SCN1A<\/jats:italic> gene was present in nine (truncation in six and missense in three). The frequency of seizures during the 3\u2003months before the onset of acute encephalopathy was monthly in seven children and none in three. The median age at the onset of acute encephalopathy was 44\u2003months (range 8\u2013184 months). All children had status epilepticus followed by coma as the initial manifestation. Two different distributions of brain lesions were observed on diffusion\u2010weighted images during the acute phase: cerebral cortex\u2013dominant lesions with or without deep gray matter involvement and subcortical\u2010dominant lesions. Four children died; nine survived with severe sequelae, and two had moderate sequelae.<\/jats:p><jats:p><jats:bold>Significance:\u2002<\/jats:bold> We must be aware that acute encephalopathy is an important complication in children with Dravet syndrome, and associated with fulminant clinical manifestations and a poor outcome.<\/jats:p>","DOI":"10.1111\/j.1528-1167.2011.03311.x","type":"journal-article","created":{"date-parts":[[2011,11,17]],"date-time":"2011-11-17T08:06:53Z","timestamp":1321517213000},"page":"79-86","source":"Crossref","is-referenced-by-count":58,"title":["Acute encephalopathy in children with Dravet 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