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Published on: July 4, 2007
Posterior Fossa Progressive Multifocal Leukoencephalopathy Secondary to Rituximab
Mounika Guduru1, Venkata Sunil Bendi2, Mariana S Bebawy3
1Radiology, Creighton University Medical Center, Omaha, USA.
Abstract:
Progressive multifocal leukoencephalopathy (PML) is a rare fatal central nervous system disorder characterized by infection-induced demyelination of white matter due to the opportunistic reactivation of John Cunningham virus in an immunocompromised patient. PML is associated with many immune-mediated diseases, lymphoproliferative conditions, and immunosuppressive agents. In this case report, we present a 79-year-old female patient diagnosed with rheumatoid arthritis who developed posterior fossa PML while on rituximab. She presented with subacute cerebellar ataxia, dysarthria, and nystagmus, and her brain MRI showed right pontine and pontocerebellar lesion with diffusion restriction and heterogenous enhancement highly characteristic of PML. Though many cases of PML with rituximab were reported in the literature, our case describes a rare type of PML affecting the posterior fossa in an HIV-negative patient on rituximab.
Insights
Progressive multifocal leukoencephalopathy (PML) is a rare brain disorder. This case highlights a rare posterior fossa PML in an HIV-negative patient with rheumatoid arthritis on rituximab.
Area of Science:
- Neuroimmunology
- Infectious Diseases
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, fatal demyelinating CNS disorder.
- PML results from John Cunningham virus reactivation in immunocompromised individuals.
- It is linked to immune-mediated diseases, lymphoproliferative conditions, and immunosuppressive therapies.
Observation:
- A 79-year-old female with rheumatoid arthritis developed PML while receiving rituximab.
- The patient presented with subacute cerebellar ataxia, dysarthria, and nystagmus.
- Brain MRI revealed characteristic PML lesions in the posterior fossa.
Findings:
- The case presents a rare instance of posterior fossa PML.
- This occurred in an HIV-negative patient treated with rituximab.
- The imaging findings were highly indicative of PML.
Implications:
- This case expands the understanding of PML presentation and localization.
- It underscores the risk of PML in patients on rituximab, even without HIV.
- Highlights the importance of considering PML in immunocompromised patients with neurological symptoms.
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