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Progressive multifocal leukoencephalopathy limited to the brain stem
O Kastrup1, M Maschke, H C Diener
1Neurologische Universitätsklinik, University of Essen, Germany. oliver.kastrup@uni-essen.de
Abstract:
Progressive multifocal leukoencephalopathy (PML) is a subacute demyelinating slow-virus encephalitis caused by the JC polyomavirus in 2-5% of patients with AIDS. MRI typically shows multiple lesions in the cerebral hemispheres. We present a rare case of rapidly evolving and lethal PML with a severe bulbar syndrome and spastic tetraparesis in a patient with AIDS. MRI showed high-signal lesions on T2-weighted images confined to the brain stem, extending from the medulla oblongata to the midbrain. JC virus polymerase chain reaction in cerebrospinal fluid was positive, and neuropathology showed the findings of PML. This case was also notable because of the rapid progression despite improved immune status with antiretroviral therapy.
Insights
Progressive multifocal leukoencephalopathy (PML) is a rare, rapidly fatal brain infection in AIDS patients. This case showed brainstem-confined lesions and rapid progression despite treatment, highlighting PML
Area of Science:
- Neuroscience
- Virology
- Immunology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by the JC polyomavirus, typically affecting immunocompromised individuals, particularly those with AIDS.
- PML commonly presents with cerebral hemisphere lesions on MRI, but rare presentations can occur.
Observation:
- A rare case of rapidly progressing and lethal PML in an AIDS patient with severe bulbar syndrome and spastic tetraparesis is presented.
- Magnetic resonance imaging (MRI) revealed high-signal lesions restricted to the brainstem, from the medulla oblongata to the midbrain.
Findings:
- JC virus polymerase chain reaction in cerebrospinal fluid was positive, confirming the diagnosis.
- Neuropathology confirmed the findings consistent with PML.
- The patient experienced rapid disease progression despite an improved immune status due to antiretroviral therapy.
Implications:
- This case underscores the potential for severe, brainstem-confined PML presentations in AIDS patients.
- The rapid progression despite antiretroviral therapy suggests potential limitations of current treatments in certain PML cases.
- Further research into understanding and managing aggressive PML variants is warranted.