Journal article
Brainstem progressive multifocal leukoencephalopathy.
- Breville G Division of Neurology, Department of Neurosciences, Geneva University Hospital, Geneva, Switzerland.
- Koralnik IJ Division of Neuro-Infectious Diseases and Global Neurology, Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
- Lalive PH Division of Neurology, Department of Neurosciences, Geneva University Hospital, Geneva, Switzerland.
- 2020-10-31
Published in:
- European journal of neurology. - 2020
English
BACKGROUND AND PURPOSE
Progressive multifocal leukoencephalopathy (PML) is a severe infection caused by the polyomavirus JC that develops in the central nervous system (CNS) of immunosuppressed patients. The infection frequently starts in the brain hemispheres and can spread into other CNS regions such as the brainstem. Initial isolated PML brainstem lesions are exceptional. We aimed to describe the challenging diagnosis of PML with isolated brainstem lesions at the time of disease onset.
METHODS
We describe a case of PML starting with an isolated brainstem lesion and reviewed clinical, radiological, and biological features of all the reported cases of isolated brainstem PML.
RESULTS
Isolated brainstem PML at disease onset is extremely rare. In addition to our case, only nine PML cases presenting with strictly isolated radioanatomical brainstem location at the time of disease onset were retrieved through the literature. All patients share similar brain magnetic resonance imaging features, without contrast enhancement. Eight patients presented with initial clinical worsening, but full recovery occurred in three patients, partial recovery in two, and death in three. Even if prognosis is reserved because of the surrounding vital structures in the brainstem, clinical recovery may occur.
CONCLUSIONS
This case report and literature review emphasize that isolated brainstem lesion is an atypical presentation of PML at disease onset.
Progressive multifocal leukoencephalopathy (PML) is a severe infection caused by the polyomavirus JC that develops in the central nervous system (CNS) of immunosuppressed patients. The infection frequently starts in the brain hemispheres and can spread into other CNS regions such as the brainstem. Initial isolated PML brainstem lesions are exceptional. We aimed to describe the challenging diagnosis of PML with isolated brainstem lesions at the time of disease onset.
METHODS
We describe a case of PML starting with an isolated brainstem lesion and reviewed clinical, radiological, and biological features of all the reported cases of isolated brainstem PML.
RESULTS
Isolated brainstem PML at disease onset is extremely rare. In addition to our case, only nine PML cases presenting with strictly isolated radioanatomical brainstem location at the time of disease onset were retrieved through the literature. All patients share similar brain magnetic resonance imaging features, without contrast enhancement. Eight patients presented with initial clinical worsening, but full recovery occurred in three patients, partial recovery in two, and death in three. Even if prognosis is reserved because of the surrounding vital structures in the brainstem, clinical recovery may occur.
CONCLUSIONS
This case report and literature review emphasize that isolated brainstem lesion is an atypical presentation of PML at disease onset.
- Language
-
- English
- Open access status
- closed
- Identifiers
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- DOI 10.1111/ene.14617
- PMID 33128290
- Persistent URL
- https://sonar.ch/global/documents/23042
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