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Primary diffuse leptomeningeal gliomatosis mimicking a chronic inflammatory meningitis

Melissa W Ko1, Peter E Turkeltaub, Edward B Lee

  • 1Department of Neurology, State University of New York Upstate Medical University, Syracuse, NY 13202, USA. kom@upstate.edu

Journal of the Neurological Sciences
|January 13, 2009
PubMed

Insights

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare brain cancer that mimics meningitis. This case highlights diagnostic challenges, emphasizing repeat biopsies for progressive neurological symptoms.

Area of Science:

  • Neurology
  • Oncology
  • Pathology

Background:

  • Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare neoplastic condition.
  • PDLG involves glial cell infiltration of the meninges without a primary brain tumor.
  • It often presents with symptoms mimicking chronic meningitis and increased intracranial pressure.

Purpose of the Study:

  • To report a challenging case of PDLG diagnosed post-mortem.
  • To highlight the diagnostic difficulties in identifying PDLG.
  • To emphasize the importance of considering repeat leptomeningeal biopsies.

Main Methods:

  • Case report of PDLG diagnosed post-mortem.
  • Review of clinical, radiological, and diagnostic findings.
  • Analysis of diagnostic procedures including cytology and leptomeningeal biopsy.

Main Results:

  • PDLG was diagnosed post-mortem, despite initial suspicion of neoplastic etiology.
  • Multiple ante-mortem diagnostic attempts, including cytologies and a leptomeningeal biopsy, were inconclusive.
  • The case underscores the difficulty in diagnosing PDLG ante-mortem.

Conclusions:

  • PDLG presents diagnostic challenges due to its non-specific symptoms and lack of definitive criteria.
  • Persistent inflammatory infiltrates with progressive neurological decline warrant consideration of multi-site or repeat leptomeningeal biopsies.
  • Early and accurate diagnosis of PDLG remains difficult, often requiring post-mortem confirmation.

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