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Updated: Jun 23, 2026
A Protocol for Rapid Post-mortem Cell Culture of Diffuse Intrinsic Pontine Glioma (DIPG)
Published on: March 7, 2017
Primary diffuse leptomeningeal gliomatosis
Gregory A Jicha1, Janel Glantz, Michelle J Clarke
1Department of Neurology and Sanders-Brown Center on Aging, University of Kentucky, Lexington, KY, USA.
Background:
Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare presentation of a primary central nervous system glial tumor.
Methods:
Four case reports of PDLG in young males aged 14-24 years are presented. These reports are discussed in the context of the existing literature.
Results:
The clinical presentation of 4 new cases of PDLG resembled chronic meningitis with and without polyradiculopathy. Spinal fluid studies are typically nondiagnostic, but characteristically show elevated opening pressure, an elevated protein level, and a relative paucity of cellular reaction. An accurate antemortem diagnosis required contrast-enhanced imaging and meningeal biopsy in all 4 of our cases. Treatment strategies including craniospinal radiation and chemotherapeutic approaches, alone or in combination, have not been proven to alter the course of the disease. Initial responses to temozolomide and radiation treatments in all 4 of our cases were promising, resulting in temporary stabilization of the disease and prolonging life expectancy over what was previously reported in the literature.
Conclusion:
Total neuroaxis contrast-enhanced MRI scanning is required for directing biopsy confirmation and detecting the extent of the disease. More effective therapeutic strategies are needed, but the combination of temozolomide and radiation therapy may slow disease progression.
Insights
Primary diffuse leptomeningeal gliomatosis (PDLG) in young males presents like meningitis. Temozolomide and radiation showed promise in slowing disease progression, but better therapies are needed.
Area of Science:
- Neuro-oncology
- Neurosurgery
- Neurology
Background:
- Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare central nervous system glial tumor presentation.
- This study focuses on PDLG in young males, aged 14-24 years.
Observation:
- PDLG clinical presentation mimics chronic meningitis, potentially with polyradiculopathy.
- Cerebrospinal fluid analysis often shows elevated pressure and protein with minimal cellularity, making it nondiagnostic.
- Accurate diagnosis requires contrast-enhanced imaging and meningeal biopsy.
Findings:
- Temozolomide and radiation therapy demonstrated initial promise, stabilizing disease and extending survival in four young male patients.
- Current treatment strategies like radiation and chemotherapy have not definitively altered the disease course.
- Contrast-enhanced MRI of the entire neuroaxis is crucial for diagnosis and staging.
Implications:
- Effective therapeutic strategies for PDLG remain elusive.
- Combination therapy with temozolomide and radiation may offer a means to slow disease progression.
- Further research is needed to develop more effective treatments for this rare condition.
