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Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity

Subacute sclerosing panencephalitis (SSPE) is a rare progressive neurological disorder of early adolescence caused by persistent infection of the measles virus, which remains prevalent worldwide despite an effective vaccine. SSPE is a devastating disease with a characteristic clinical course in subc...

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Autores principales: Upadhyayula, Pavan S., Yang, Jason, Yue, John K., Ciacci, Joseph D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5753655/
https://www.ncbi.nlm.nih.gov/pubmed/29112137
http://dx.doi.org/10.3390/medsci5040026
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author Upadhyayula, Pavan S.
Yang, Jason
Yue, John K.
Ciacci, Joseph D.
author_facet Upadhyayula, Pavan S.
Yang, Jason
Yue, John K.
Ciacci, Joseph D.
author_sort Upadhyayula, Pavan S.
collection PubMed
description Subacute sclerosing panencephalitis (SSPE) is a rare progressive neurological disorder of early adolescence caused by persistent infection of the measles virus, which remains prevalent worldwide despite an effective vaccine. SSPE is a devastating disease with a characteristic clinical course in subcortical white matter; however, atypical presentations of brainstem involvement may be seen in rare cases. This review summarizes reports to date on brainstem involvement in SSPE, including the clinical course of disease, neuroimaging presentations, and guidelines for treatment. A comprehensive literature search was performed for English-language publications with keywords “subacute sclerosing panencephalitis” and “brainstem” using the National Library of Medicine PubMed database (March 1981–September 2017). Eleven articles focusing on SSPE of the brainstem were included. Predominant brainstem involvement remains uncharacteristic of SSPE, which may lead to misdiagnosis and poor outcome. A number of case reports have demonstrated brainstem involvement associated with other intracranial lesions commonly presenting in later SSPE stages (III and IV). However, brainstem lesions can appear in all stages, independent of higher cortical structures. The varied clinical presentations complicate diagnosis from a neuroimaging perspective. SSPE of the brainstem is a rare but important clinical entity. It may present like canonical SSPE or with unique clinical features such as absence seizures and pronounced ataxia. While SSPE generally progresses to the brainstem, it can also begin with a primary focus of infection in the brainstem. Awareness of varied SSPE presentations can aid in early diagnosis as well as guide management and treatment.
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spelling pubmed-57536552018-01-08 Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity Upadhyayula, Pavan S. Yang, Jason Yue, John K. Ciacci, Joseph D. Med Sci (Basel) Review Subacute sclerosing panencephalitis (SSPE) is a rare progressive neurological disorder of early adolescence caused by persistent infection of the measles virus, which remains prevalent worldwide despite an effective vaccine. SSPE is a devastating disease with a characteristic clinical course in subcortical white matter; however, atypical presentations of brainstem involvement may be seen in rare cases. This review summarizes reports to date on brainstem involvement in SSPE, including the clinical course of disease, neuroimaging presentations, and guidelines for treatment. A comprehensive literature search was performed for English-language publications with keywords “subacute sclerosing panencephalitis” and “brainstem” using the National Library of Medicine PubMed database (March 1981–September 2017). Eleven articles focusing on SSPE of the brainstem were included. Predominant brainstem involvement remains uncharacteristic of SSPE, which may lead to misdiagnosis and poor outcome. A number of case reports have demonstrated brainstem involvement associated with other intracranial lesions commonly presenting in later SSPE stages (III and IV). However, brainstem lesions can appear in all stages, independent of higher cortical structures. The varied clinical presentations complicate diagnosis from a neuroimaging perspective. SSPE of the brainstem is a rare but important clinical entity. It may present like canonical SSPE or with unique clinical features such as absence seizures and pronounced ataxia. While SSPE generally progresses to the brainstem, it can also begin with a primary focus of infection in the brainstem. Awareness of varied SSPE presentations can aid in early diagnosis as well as guide management and treatment. MDPI 2017-11-07 /pmc/articles/PMC5753655/ /pubmed/29112137 http://dx.doi.org/10.3390/medsci5040026 Text en © 2017 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Upadhyayula, Pavan S.
Yang, Jason
Yue, John K.
Ciacci, Joseph D.
Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity
title Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity
title_full Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity
title_fullStr Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity
title_full_unstemmed Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity
title_short Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity
title_sort subacute sclerosing panencephalitis of the brainstem as a clinical entity
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5753655/
https://www.ncbi.nlm.nih.gov/pubmed/29112137
http://dx.doi.org/10.3390/medsci5040026
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