4.2 Article

Progressive Multifocal Leukoencephalopathy Localized in the Cerebellum and Brainstem Associated with Idiopathic CD4+ T Lymphocytopenia

Journal

INTERNAL MEDICINE
Volume 55, Issue 12, Pages 1645-1647

Publisher

JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.55.6649

Keywords

brainstem; cerebellum; crescent-shaped; idiopathic CD4(+) T lymphocytopenia; progressive multifocal leukoencephalopathy

Funding

  1. JSPS KAKENHI Grant [26461286]
  2. Research Committee of Prion Disease and Slow Virus Infection, Research on Policy Planning and Evaluation for Rare and Intractable Diseases from the Ministry of Health, Labour and Welfare of Japan
  3. Research Committee of Molecular Pathogenesis and Therapies for Prion Disease and Slow Virus Infection
  4. Practical Research Project for Rare/Intractable Disease from Japan Agency for Medical Research and Development, AMED
  5. [H24-AIDS-Wakate-002]
  6. Grants-in-Aid for Scientific Research [26461286] Funding Source: KAKEN

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Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease that favors the cerebrum and typically occurs in immunosuppressed patients. We herein report the case of a 66-year-old man with PML, idiopathic CD4(+) T lymphocytopenia (ICL), and chronic renal failure. Cranial magnetic resonance imaging (MRI) showed a crescent-shaped lesion in the left cerebellum, brainstem, and middle cerebellar peduncle. Although the patient did not present with HIV infection, collagen diseases, or tumors, JC virus DNA was detected in the cerebrospinal fluid. Clinicians should consider PML and ICL in the differential diagnosis if the patient develops progressive ataxia and a crescent-shaped cerebellar lesion on MRI.

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