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Twenty-two patients suffering from diffuse leptomeningeal gliomatosis (LG) were identified at the Salpêtrière Hospital between January 1989 and January 1994: 20 patients had a known primary supratentorial glioma when LG was diagnosed (8 glioblastomas, 5 anaplastic astrocytomas, 3 anaplasic oligodendrogliomas, 2 astrocytomas and 2 oligodendrogliomas); 2 patients had primary LG. The delay between the discovery of the primary tumour and the development of LG was 5 +/- 4 months in glioblastomas and 22 +/- 16 months for others gliomas. In 1/2 primary LG, autopsy demonstrated an hippocampic astrocytoma which was undetected pre-mortem even on MRI; in the second patient with primary LG no autopsy was obtained and the diagnosis was based on meningeal biopsy. An epidemiological study was made by comparing 13 LG with 275 supratentorial gliomas without LG who were seen during the same period. The incidence of symptomatic LG was 4.7% and was more frequent in anaplasic astrocytomas (6.5%) and anaplasic olidendrogliomas (11.4%) than in glioblastomas (3%) but the difference was not statistically significant. The age at diagnosis of LG was 56 +/- 11 years in glioblastomas and 41 +/- 7 in others gliomas, related to tumour histology. Among the 22 studied cases, only 27% had a meningeal syndrome; multifocal neurological involvement was present in 74% of patients combining at various degree signs of cerebral, cranial nerves and roots or spinal cord dysfunction. Contrast enhancing lesions in the meninges or ventricules on CT/MRI were found in 82% of cases and 45% had an hydrocephalus. In the CSF 94% of patients had a protein level over 1 milligram and 47% of them had a low glucose level < 2.7 mmol/l; malignant cells were found in 47% of cases. Among 11 patients who received radiotherapy and/or chemotherapy, 3 improved and 2 were stable and their survival ranged from 6 to 34+ months. Criteria allowing diagnosis of LG are defined and the therapeutic option are reviewed.