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Published on: May 7, 2020
Primary diffuse leptomeningeal gliomatosis: report of a case presenting with chronic meningitis
Sung-Hun Kim1, Dong-Chul Jun, Jin Se Park
1Department of Neurology, Seoul National University College of Medicine, Seoul, Korea.
Abstract:
Neoplastic meningitis occurs in approximately 5% of patients with cancer. Primary diffuse leptomeningeal gliomatosis is a rare condition whereby a glioma arises from heterotopic cell nests in the leptomeninges. We report here a case presenting with clinical features similar to those of chronic infectious meningitis without positive cerebrospinal fluid cytology. Neurological signs in our patient deteriorated progressively without responding to antitubercular, antiviral, or antibiotic therapy. Leptomeningeal biopsy sampling revealed the condition to be primary diffuse leptomeningeal gliomatosis.
Insights
Primary diffuse leptomeningeal gliomatosis is a rare brain tumor. This case highlights its presentation mimicking infectious meningitis, confirmed by leptomeningeal biopsy.
Area of Science:
- Neuro-oncology
- Neuropathology
Background:
- Neoplastic meningitis affects ~5% of cancer patients.
- Primary diffuse leptomeningeal gliomatosis is a rare glioma arising from heterotopic leptomeningeal cells.
Purpose of the Study:
- To report a rare case of primary diffuse leptomeningeal gliomatosis.
- To highlight diagnostic challenges and confirm the diagnosis via biopsy.
Main Methods:
- Clinical case presentation.
- Cerebrospinal fluid analysis (cytology).
- Leptomeningeal biopsy and histopathological examination.
Main Results:
- The patient presented with symptoms mimicking chronic infectious meningitis.
- Cerebrospinal fluid cytology was negative for malignant cells.
- Leptomeningeal biopsy confirmed primary diffuse leptomeningeal gliomatosis.
Conclusions:
- Primary diffuse leptomeningeal gliomatosis can present insidiously with symptoms mimicking infectious meningitis.
- Diagnosis requires a high index of suspicion and confirmation through leptomeningeal biopsy.
- This rare condition poses significant diagnostic and therapeutic challenges.
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