Subacute sclerosing panencephalitis: A clinical appraisal

Introduction: Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. The aim of the present study is to describe the clinical profile and natural history...

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Main Authors: Sujit Abajirao Abajirao, M D Nair, Harsha J Kambale
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2013-01-01
Series:Annals of Indian Academy of Neurology
Subjects:
Online Access:http://www.annalsofian.org/article.asp?issn=0972-2327;year=2013;volume=16;issue=4;spage=631;epage=633;aulast=Abajirao
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author Sujit Abajirao Abajirao
M D Nair
Harsha J Kambale
author_facet Sujit Abajirao Abajirao
M D Nair
Harsha J Kambale
author_sort Sujit Abajirao Abajirao
collection DOAJ
description Introduction: Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. The aim of the present study is to describe the clinical profile and natural history of patients with SSPE. Methods: We collected data of patients with SSPE during 2004-2010 who fulfilled Dyken′s criteria. We analyzed demographical, clinical, electrophysiological, and imaging features. Results: Study included 34 patients, 26 (76.5%) males with age of onset from 3 to 31 years. Twenty one patients were below 15 years of age formed childhood SSPE and 13 above 15 years of age constituted adult onset group. 85.3% had low-socioeconomic status. Eleven received measles vaccination and seven were unvaccinated. 59.9% patients had measles history. Most common presenting symptom was scholastic backwardness (52.5%) followed by seizures (23.5%). Three patients each had cortical blindness, macular degeneration, decreased visual acuity, and optic atrophy. Electroencephalographic (EEG) showed long interval periodic complexes and cerebrospinal fluid anti-measles antibody was positive in all. Magnetic resonance imaging was done in 70.5% with was abnormal in 52.5%. Mean incubation period of SSPE after measles was 9.6 years. The follow-up duration was 1-10 years, (average of 2 years). Only one patient died from available data of follow-up, 9 were stable and 10 deteriorated in the form of progression of staging. Conclusion: SSPE is common in low-socioeconomic status. The profile of adult onset did not differ from childhood onset SSPE, except for a longer interval between measles infection and presence of the ophthalmic symptom as presenting feature in adult onset group.
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spelling doaj.art-12eb25549b4f41e3baa0ca2c6894c98d2022-12-21T19:04:21ZengWolters Kluwer Medknow PublicationsAnnals of Indian Academy of Neurology0972-23271998-35492013-01-0116463163310.4103/0972-2327.120497Subacute sclerosing panencephalitis: A clinical appraisalSujit Abajirao AbajiraoM D NairHarsha J KambaleIntroduction: Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. The aim of the present study is to describe the clinical profile and natural history of patients with SSPE. Methods: We collected data of patients with SSPE during 2004-2010 who fulfilled Dyken′s criteria. We analyzed demographical, clinical, electrophysiological, and imaging features. Results: Study included 34 patients, 26 (76.5%) males with age of onset from 3 to 31 years. Twenty one patients were below 15 years of age formed childhood SSPE and 13 above 15 years of age constituted adult onset group. 85.3% had low-socioeconomic status. Eleven received measles vaccination and seven were unvaccinated. 59.9% patients had measles history. Most common presenting symptom was scholastic backwardness (52.5%) followed by seizures (23.5%). Three patients each had cortical blindness, macular degeneration, decreased visual acuity, and optic atrophy. Electroencephalographic (EEG) showed long interval periodic complexes and cerebrospinal fluid anti-measles antibody was positive in all. Magnetic resonance imaging was done in 70.5% with was abnormal in 52.5%. Mean incubation period of SSPE after measles was 9.6 years. The follow-up duration was 1-10 years, (average of 2 years). Only one patient died from available data of follow-up, 9 were stable and 10 deteriorated in the form of progression of staging. Conclusion: SSPE is common in low-socioeconomic status. The profile of adult onset did not differ from childhood onset SSPE, except for a longer interval between measles infection and presence of the ophthalmic symptom as presenting feature in adult onset group.http://www.annalsofian.org/article.asp?issn=0972-2327;year=2013;volume=16;issue=4;spage=631;epage=633;aulast=AbajiraoMeaslesmyoclonussubacute sclerosing panencephalitis
spellingShingle Sujit Abajirao Abajirao
M D Nair
Harsha J Kambale
Subacute sclerosing panencephalitis: A clinical appraisal
Annals of Indian Academy of Neurology
Measles
myoclonus
subacute sclerosing panencephalitis
title Subacute sclerosing panencephalitis: A clinical appraisal
title_full Subacute sclerosing panencephalitis: A clinical appraisal
title_fullStr Subacute sclerosing panencephalitis: A clinical appraisal
title_full_unstemmed Subacute sclerosing panencephalitis: A clinical appraisal
title_short Subacute sclerosing panencephalitis: A clinical appraisal
title_sort subacute sclerosing panencephalitis a clinical appraisal
topic Measles
myoclonus
subacute sclerosing panencephalitis
url http://www.annalsofian.org/article.asp?issn=0972-2327;year=2013;volume=16;issue=4;spage=631;epage=633;aulast=Abajirao
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