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Published on: September 19, 2010
Inflammatory progressive multifocal leukoencephalopathy with human T-cell lymphotropic virus-1 coinfection
Sachiko Hasebe1, Kota Maekawa2, Yukiko Shishido-Hara3
1Department of Neurology, Kyoto University Graduate School of Medicine Faculty of Medicine, Kyoto, Japan sachiko_h@kuhp.kyoto-u.ac.jp.
Abstract:
A middle-aged man with progressive multifocal leukoencephalopathy (PML) in a human T-cell lymphotropic virus type-1 (HTLV-1) carrier on haemodialysis presented with mild dysarthria and ataxia. Brain MRI revealed asymmetric T2-hyperintense lesions in the cerebral white matter, cerebellum and brainstem. A small amount of JC virus (JCV) genome in cerebrospinal fluid was detected by PCR and cerebellar biopsy demonstrated JCV-DNA presence. Pathological findings showed demyelinating lesions and glial cells with mildly enlarged nuclei, accompanied by T-lymphocytes, neutrophils and plasma cell infiltration. The CD4+/CD8+ratio was 0.83. High-dose corticosteroid therapy was effective for inflammatory PML lesions, and the administration of mefloquine combined with mirtazapine led to favourable outcome. The encephalitis in this case is considered to have occurred secondarily to JCV infection in the presence of HTLV-1 infection. Therefore, it is crucial to investigate the presence of HTLV-1 in order to understand the aetiology of this brain inflammation.
Insights
This case highlights progressive multifocal leukoencephalopathy (PML) in an HTLV-1 carrier. Early detection of JC virus (JCV) and HTLV-1 is crucial for managing this rare brain inflammation.
Area of Science:
- Neurovirology
- Immunology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease.
- Human T-cell lymphotropic virus type-1 (HTLV-1) carriers are susceptible to opportunistic infections.
Observation:
- A middle-aged man on haemodialysis with HTLV-1 presented with dysarthria and ataxia.
- Brain MRI showed asymmetric white matter lesions; JC virus (JCV) DNA confirmed in CSF and cerebellar biopsy.
- Pathology revealed demyelination, glial changes, and inflammatory cell infiltration with a CD4+/CD8+ ratio of 0.83.
Findings:
- High-dose corticosteroids reduced inflammation.
- Mefloquine and mirtazapine treatment led to a favorable outcome.
- Encephalitis was secondary to JCV infection in the context of HTLV-1.
Implications:
- Investigating HTLV-1 is vital for diagnosing the cause of brain inflammation.
- This case underscores the importance of considering co-infections in neurological conditions.
- Effective management strategies involve addressing both the viral infection and inflammation.
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