Primary diffuse leptomeningeal gliomatosis

Abstract

Primary diffuse leptomeningeal gliomatosis is a rare central nervous system neoplasm in which focal or diffuse evidence of gliomatous tissue is identified in the subarachnoid space with no evidence of a primary tumour. A case is presented and the differential diagnosis and management are discussed.

Primary Diffuse Leptomeningeal Gliomatosis

“…As expected in a polyradiculopathy, patients are more likely to present with signs of lower motor neuron dysfunction (areflexia, atrophy, fasciculations) than to show signs of upper motor neuron involvement (hyperreflexia, Babinski responses, and other pathologic signs), although both can be seen in PDLG. The prominent involvement of the cauda equina seen in 2 of our patients has been reported in other cases in the literature [6, 17, 19]. …”

“…Less than one sixth of all reported cases even demonstrate cellular atypia [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 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].…”

“…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].…”