{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T03:22:36Z","timestamp":1776914556361,"version":"3.51.2"},"reference-count":12,"publisher":"Georg Thieme Verlag KG","issue":"01","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropediatrics"],"published-print":{"date-parts":[[2021,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Subacute sclerosing panencephalitis (SSPE) is a rare and progressive inflammatory disease of central nervous system due to aberrant measles virus with an outcome that is nearly always fatal. In acute fulminant SSPE, the disease rapidly evolves leading to death within 3 months of the diagnosis. We report here four cases of fulminant SSPE with atypical presentations, two of them presented at very early age with history of congenital measles infection in first case and gait abnormality as initial symptom in second case; acute disseminated encephalomyelitis (ADEM) with refractory seizures in third case, unilateral myoclonus with hemiparesis in fourth case at the onset of disease, respectively. The typical periodic electroencephalographic (EEG) complexes, elevated cerebrospinal fluid (CSF), and serum antimeasles antibodies in our patients led to the diagnosis of SSPE. A high index of clinical suspicion in fulminant type with awareness of atypical features, EEG, and CSF studies are of paramount importance in establishing its diagnosis.<\/jats:p>","DOI":"10.1055\/s-0040-1715623","type":"journal-article","created":{"date-parts":[[2020,10,16]],"date-time":"2020-10-16T23:04:51Z","timestamp":1602889491000},"page":"052-055","source":"Crossref","is-referenced-by-count":11,"title":["Atypical Presentation of Fulminating Subacute Sclerosing Panencephalitis: A Case Series"],"prefix":"10.1055","volume":"52","author":[{"given":"Pranab K.","family":"Dey","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, R. G. Kar Medical College and Hospital, Kolkata, West Bengal, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arindam","family":"Ghosh","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Midnapore Medical College, Midnapore, West Bengal, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2020,10,16]]},"reference":[{"issue":"916","key":"ref1","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1136\/pmj.78.916.63","article-title":"Subacute sclerosing panencephalitis","volume":"78","author":"R K Garg","year":"2002","journal-title":"Postgrad Med J"},{"issue":"04","key":"ref2","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1007\/BF02724111","article-title":"Diagnostic dilemmas in fulminant subacute sclerosing panencephalitis (SSPE)","volume":"71","author":"N Sarkar","year":"2004","journal-title":"Indian J Pediatr"},{"issue":"10","key":"ref3","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1001\/archneur.1979.00500460044004","article-title":"The variable natural history of subacute sclerosing panencephalitis: a study of 118 cases from the Middle East","volume":"36","author":"W S Risk","year":"1979","journal-title":"Arch Neurol"},{"key":"ref4","first-page":"638","article-title":"Subacutesclerosingpanencephalitis (SSPE): an insight into the diagnostic errors from a tertiary care university hospital","volume":"22","author":"L K Prasanth","year":"2007","journal-title":"J Child Neurol"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"1124","DOI":"10.1016\/S0140-6736(95)90858-7","article-title":"Rapid progressive subacute sclerosing panencephalitis after perinatally acquired measles virus infection","volume":"345","author":"K Zwiauer","year":"1995","journal-title":"Lancet"},{"issue":"01","key":"ref6","first-page":"58","article-title":"Subacute sclerosing panencephalitis (SSPE) associated with congenital measles infection","volume":"47","author":"E Sim\u015fek","year":"2005","journal-title":"Turk J Pediatr"},{"issue":"11","key":"ref7","first-page":"1067","article-title":"Subacute sclerosing panencephalitis with tics as first symptom","volume":"50","author":"P K Dey","year":"2013","journal-title":"Indian Pediatr"},{"issue":"12","key":"ref8","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1007\/BF02763061","article-title":"Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis","volume":"73","author":"S Comert","year":"2006","journal-title":"Indian J Pediatr"},{"issue":"07","key":"ref9","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1177\/0883073808330184","article-title":"Subacute sclerosing panencephalitis (SSPE) presenting as acute disseminated encephalomyelitis in a child","volume":"24","author":"J Goraya","year":"2009","journal-title":"J Child Neurol"},{"issue":"09","key":"ref10","doi-asserted-by":"crossref","first-page":"1947","DOI":"10.1016\/j.clinph.2007.06.008","article-title":"Electroencephalographic and imaging profile in a subacute sclerosing panencephalitis (SSPE) cohort: a correlative study","volume":"118","author":"S Praveen-kumar","year":"2007","journal-title":"Clin Neurophysiol"},{"issue":"01","key":"ref11","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1111\/j.1600-0404.1981.tb00751.x","article-title":"Unilateral subacute sclerosing panencephalitis complexes with contralateral myoclonus","volume":"63","author":"Q Ghanem","year":"1981","journal-title":"Acta Neurol Scand"},{"issue":"04","key":"ref12","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1017\/S0317167100003127","article-title":"Subacute sclerosing panencephalitis presenting with unilateral periodic myoclonic jerks","volume":"30","author":"Z M Shivji","year":"2003","journal-title":"Can J Neurol Sci"}],"container-title":["Neuropediatrics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/s-0040-1715623.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,1,28]],"date-time":"2021-01-28T00:56:24Z","timestamp":1611795384000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/s-0040-1715623"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,10,16]]},"references-count":12,"journal-issue":{"issue":"01","published-online":{"date-parts":[[2021,1,27]]},"published-print":{"date-parts":[[2021,2]]}},"URL":"https:\/\/doi.org\/10.1055\/s-0040-1715623","relation":{},"ISSN":["0174-304X","1439-1899"],"issn-type":[{"value":"0174-304X","type":"print"},{"value":"1439-1899","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,10,16]]}}}