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Med. Sci. 2017, 5(4), 26;

Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity

Department of Neurological Surgery, University of California, San Diego, 200 West Arbor Drive #8893, La Jolla, CA 92103, USA
Department of Neurological Surgery, University of California, San Francisco, 400 Parnassus Avenue, San Francisco, CA 94122, USA
These authors contributed equally to this work.
Author to whom correspondence should be addressed.
Received: 18 September 2017 / Revised: 30 October 2017 / Accepted: 3 November 2017 / Published: 7 November 2017
Full-Text   |   PDF [197 KB, uploaded 8 November 2017]   |   Review Reports


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. View Full-Text
Keywords: brainstem; measles; neurodegeneration; neuroimaging; subacute sclerosing panencephalitis brainstem; measles; neurodegeneration; neuroimaging; subacute sclerosing panencephalitis
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).

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Upadhyayula, P.S.; Yang, J.; Yue, J.K.; Ciacci, J.D. Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity. Med. Sci. 2017, 5, 26.

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