Related Experiment Video
Updated: Jun 27, 2026

Flow Cytometric Analysis of Lymphocyte Infiltration in Central Nervous System during Experimental Autoimmune Encephalomyelitis
Published on: November 17, 2020
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
Abstract:
A 64-year-old Japanese man who was diagnosed as having cerebral infarcts at an early clinical stage was found to have progressive multifocal leukoencephalopathy (PML). A decrease of leukocytes and lymphocytes had been detected in the previous year. During a total clinical course of 11 months, he showed marked depletion of lymphocytes ranging from 264/microL to 459/microL. Autopsy disclosed no underlying diseases such as malignancies or tuberculosis. Extensive PML lesions were seen in the cerebral white matter. Small perivascular cuffs comprising many CD8+ T lymphocytes and a few CD4+ T cells were scattered in the PML lesions. CD20+ B cells were rarely evident. The subsets of the infiltrating lymphocytes differed from those of primary or spontaneous PML. Similar extensive PML lesions were observed not only in the cerebellum and brainstem but also in the spinal cord. All 26 segments of the spinal cord, especially the cervical, lumbar and sacral cord, showed extensive lesions involving the lateral and anterior columns. To our knowledge, only three cases of PML with such extensive spinal cord lesions have been reported previously. These three cases, and the present one, may represent a group of PML that shows extensive lesions in the spinal cord as well as the cerebrum, cerebellum and brainstem. The underlying disease in the present case was unclear. Because lymphocytopenia is not observed in primary or spontaneous PML, and the immunohistochemical findings of the infiltrating lymphocytes in the present case are different from primary or spontaneous PML, the decrease in his total blood lymphocytes may have played a significant role in his immunosuppressed condition as the underlying disease.
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.
Related Concept Videos
Multiple Sclerosis l: Introduction
Encephalitis ll: Pathophysiology
Encephalitis l: Introduction
Disorders of Leukocytes
Leukopenia may result from bone marrow disorders, autoimmune diseases, and infectious diseases. For example, conditions such as multiple myeloma and aplastic anemia can impair the bone marrow's ability to produce adequate leukocytes. Similarly, autoimmune diseases like lupus and viral infections such as HIV can prompt the immune system...
Primary Lymphoid Organs
The red bone marrow is a soft, spongy tissue nestled in the interior of long bones such as the humerus and femur. It is the site...
Cryptococcal Meningitis
