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Related Experiment Video

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Mouse Models of Periventricular Leukomalacia

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Published on: May 18, 2010

Brainstem progressive multifocal leukoencephalopathy.

Gautier Breville¹, Igor J Koralnik², Patrice H Lalive^1,3,4

¹Division of Neurology, Department of Neurosciences, Geneva University Hospital, Geneva, Switzerland.

European Journal of Neurology

|October 31, 2020

Summary

Progressive multifocal leukoencephalopathy (PML) with isolated brainstem lesions is a rare CNS infection. This study highlights this atypical presentation and its diagnostic challenges.

Keywords:

JC virus brainstem progressive multifocal leukoencephalopathy

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Area of Science:

Neuroscience
Infectious Diseases
Radiology

Background:

Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system (CNS).
PML is caused by the John Cunningham (JC) virus in immunosuppressed individuals.
Typically, PML lesions originate in the cerebral hemispheres, with brainstem involvement being less common.

Observation:

This report details a rare case of PML presenting with an isolated brainstem lesion.
A literature review identified nine additional cases of PML with strictly isolated brainstem lesions at onset.
Affected patients exhibited similar magnetic resonance imaging (MRI) features without contrast enhancement.

Findings:

Isolated brainstem PML at disease onset is exceptionally rare.
Clinical outcomes varied, with three patients experiencing full recovery, two partial recovery, and three fatalities.
Despite the critical location, some patients with brainstem PML can achieve clinical recovery.

Implications:

This emphasizes that isolated brainstem lesions represent an atypical presentation of PML.
Recognizing this rare manifestation is crucial for timely diagnosis and management of PML.
Further research into the specific factors influencing PML localization and prognosis is warranted.