Py. Dietrich et al., PRIMARY DIFFUSE LEPTOMENINGEAL GLIOMATOSIS (PDLG) - A NEOPLASTIC CAUSE OF CHRONIC MENINGITIS, Journal of neuro-oncology, 15(3), 1993, pp. 275-283
Cancerous 'chronic meningitis' may be related to subarachnoid space in
volvement by solid tumors, hematologic malignancies or rarely intrapar
enchymatous gliomas. Primary leptomeningeal gliomatosis is a rare cond
ition that is attributed to malignant transformation of heterotopic ne
uroglial tissue. We discuss the clinical and biological features of a
patient who died with the diffuse form of primary leptomeningeal gliom
atosis (PDLG). A literature search shows that a one to two months long
non-specific prodromal phase followed by a fluctuating neurologic dow
nhill course is suggestive of this disease. Cerebro-spinal fluid (CSF)
cytology has been diagnostic in only 1 of 8 reported cases. Recent te
chnical progress, including the use of GFAP (glial fibrillary acidic p
rotein) directed antibody, may enhance the sensitivity of CSF cytologi
es. Diagnosis may require repeated cerebral biopsies, because the hemi
spheric lesions are often separated by normal tissue. PDLG must be add
ed to the large differential diagnosis of 'chronic meningitis'.