Progressive multifocal leukoencephalopathy showing extensive spinal cord involvement in a patient with

Shigeki Takeda1, Kazunori Yamazaki, Teruo Miyakawa

  • 1Department of Pathology, Niigata Neurosurgical Hospital, Nishi-ku, Niigata, Japan. s-takeda@deluxe.ocn.ne.jp

Insights

This study reports a rare case of progressive multifocal leukoencephalopathy (PML) with extensive spinal cord lesions in a Japanese man. The patient

Area of Science:

  • Neurology
  • Immunology
  • Pathology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system.
  • PML is typically associated with severe immunosuppression.
  • The typical presentation involves cerebral white matter lesions.

Observation:

  • A 64-year-old Japanese man presented with cerebral infarcts and was diagnosed with PML.
  • The patient exhibited significant lymphocytopenia (low lymphocyte count) prior to and during the illness.
  • Autopsy revealed extensive PML lesions in the brain, brainstem, cerebellum, and spinal cord.

Findings:

  • Immunohistochemistry of PML lesions showed a unique infiltrate of CD8+ T lymphocytes, differing from typical PML.
  • Extensive spinal cord involvement was noted across all segments, particularly in the cervical, lumbar, and sacral regions.
  • The underlying cause of immunosuppression remained unclear, but lymphocytopenia was a prominent feature.

Implications:

  • This case highlights a potential subtype of PML characterized by extensive spinal cord lesions and distinct lymphocyte infiltration patterns.
  • The findings suggest that unexplained lymphocytopenia may be a significant underlying factor in some PML cases.
  • Further research is needed to understand the pathogenesis and clinical spectrum of PML, especially concerning spinal cord involvement.

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