Diffuse form of primary leptomeningeal gliomatosis

Abstract

Primary leptomeningeal gliomatosis is rare, and the diffuse form is even rarer with only three cases reported in the literature. A fourth case is described in this report. Computerized tomography (CT) findings showed hydrocephalus with enhancement of the cerebral cisterns, and analysis of cerebrospinal fluid obtained by lumbar puncture showed many atypical cells. Based on these findings, a diagnosis of leptomeningeal tumor was made. There was some improvement in neurological and CT findings following radiotherapy and chemotherapy.

A case of primary diffuse leptomeningeal gliomatosis predominantly involving the cervical spinal cord and mimicking chronic meningitis

“…The most common presenting symptom is headache followed by vomiting, seizures, and neck stiffness (1,4,5,7). The signs are also quite different including cranial nerve palsies, papilledema, altered level of consciousness, and those depending on spinal cord involvement (1,2,4,8,9,16,18). CSF evaluation is usually consistent with elevated protein levels, absence of atypical cells in almost all cases.…”

Primary Diffuse Leptomeningeal Gliomatosis

“…The CSF leukocyte count is abnormal but only slightly elevated (mean: 25 cells/mm 3 ) given the severity of disease in these patients [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27]. In contrast, the opening pressure is typically markedly elevated (mean: 448 mm H 2 O) and the protein concentration is often extremely high (mean: 873 mg/dl) [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27]. While there are scattered reports of hypoglycorrhachia in PDLG, often the CSF glucose concentrations are normal or only minimally decreased [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27].…”

“…The development and use of MRI has increased this figure to 91% [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27]. Additionally, MRI of the spine has revealed abnormalities in 100% of the reported cases studied in this fashion [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27]. Clearly, the number of reported cases of PDLG has increased parallel to the development and use of MRI as a clinical adjuvant.…”

“…Almost universally, the diagnosis of PDLG is made only by meningeal biopsy or autopsy [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27]. Only a single case in the literature documents a definitive diagnosis of glioma from CSF cytologic studies [23].…”

“…Prior to high resolution imaging, the definitive diagnosis of PDLG required autopsy to rule out a primary parenchymal source for the leptomeningeal process [1,2,3,4,5,6,7,8]. Many of the cases reported as representing PDLG are complicated by focal parenchymal involvement, which has been interpreted as a secondary parenchymal infiltration of a primary leptomeningeal glioma [29,30,31,32,33,34,35,36].…”