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Progressive Multifocal Leukoencephalopathy with Balanced CD4/CD8 T-Cell Infiltration and Good Response to Mefloquine
Nobuo Sanjo1, Satoko Kina, Yukiko Shishido-Hara
1Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Japan.
Abstract:
A 53-year-old man was admitted for sub-acute progressive dementia and Gerstmann syndrome. MRI demonstrated lesions in the white matter involving the left parietal lobe, accompanied by speckled or faint linear peripheral enhancement. Brain biopsy revealed JC virus infection in oligodendrocytes and balanced infiltration of CD8+ and CD4+ T lymphocytes. We diagnosed progressive multifocal leukoencephalopathy (PML) with controlled inflammation. The finding of CD4/CD8 T cells in the infected PML brain suggested therapeutically valuable immune system involvement, which we decided to preserve by withholding corticosteroids. We treated the patient with risperidone, cytarabine and mefloquine to suppress virus replication, but not with the corticosteroid that is conventionally used in inflammatory PML cases. The patient was discharged three months after admission, and one year later, his score on the Mini-Mental State Examination had recovered to 26/30, from 5/30 on admission.
Insights
This study details a progressive multifocal leukoencephalopathy (PML) case in a 53-year-old man. Treatment focused on viral suppression and preserving immune response, leading to significant cognitive recovery.
Area of Science:
- Neuroscience
- Virology
- Immunology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system caused by the JC virus.
- PML typically affects immunocompromised individuals and can lead to severe neurological deficits.
Observation:
- A 53-year-old man presented with sub-acute dementia and Gerstmann syndrome.
- MRI revealed white matter lesions in the left parietal lobe with peripheral enhancement.
- Brain biopsy confirmed JC virus infection in oligodendrocytes and a balanced infiltration of CD8+ and CD4+ T lymphocytes.
Findings:
- The patient was diagnosed with PML with controlled inflammation, indicated by the presence of CD4/CD8 T cells.
- Treatment involved risperidone, cytarabine, and mefloquine to suppress viral replication, deliberately withholding corticosteroids.
- This approach aimed to preserve the immune system's involvement in controlling the infection.
Implications:
- The patient showed significant cognitive recovery, with Mini-Mental State Examination scores improving from 5/30 to 26/30 within a year.
- This case suggests that preserving immune response by avoiding corticosteroids may be a valuable therapeutic strategy in PML.
- Further research into immunomodulatory therapies for PML is warranted.
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