Primary Diffuse Leptomeningeal Gliomatosis

• Published in: European neurology Vol. 62; no. 1; pp. 16 - 22
• Main Authors: Jicha, Gregory A., Glantz, Janel, Clarke, Michelle J., Lehwald, Lenora M., Russo, David P., Giannini, Caterina, Wald, John T., Uhm, Joon, Kumar, Neeraj, Aksamit, Allen J., Wetmore, Cynthia J.
• Format: Journal Article
• Language: English
• Published: Basel, Switzerland S. Karger AG 01.01.2009
• Subjects: Adolescent; Antineoplastic Agents, Alkylating - therapeutic use; Brain - pathology; Dacarbazine - analogs & derivatives; Dacarbazine - therapeutic use; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Male; Meningeal Neoplasms - diagnosis; Meningeal Neoplasms - drug therapy; Meningeal Neoplasms - pathology; Meningeal Neoplasms - radiotherapy; Meninges - pathology; Meningitis - diagnosis; Neoplasms, Neuroepithelial - diagnosis; Neoplasms, Neuroepithelial - drug therapy; Neoplasms, Neuroepithelial - pathology; Neoplasms, Neuroepithelial - radiotherapy; Original Paper; Perfusion Imaging; Spinal Cord - pathology; Treatment Outcome; Young Adult; Primary diffuse leptomeningeal gliomatosis; Chronic meningitis; Temozolomide; Glioma; Meningeal neoplasms
• ISSN: 0014-3022; 1421-9913
• DOI: 10.1159/000216838

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.