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.