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Primary diffuse leptomeningeal gliomatosis
Abstract:
A review of the literature on primary diffuse meningeal gliomatosis (DMG) yielded three cases and we report a fourth. DMG is a syndrome characterized by extensive basal and spinal chronic meningitis with mental confusion, headaches, diplopia, papilledema and cranial nerve palsies. The cerebrospinal fluid (CSF) has a markedly elevated protein content, moderate mononuclear pleocytosis and a normal or low glucose. This picture invariably leads to the diagnosis and treatment of tuberculous or fungal meningitis despite persistently negative cerebrospinal fluid (CSF) cultures. Reaction of exfoliated CSF cells with glial fibrillary acidic protein (GFAP) immunoperoxidase labelled antibody is suggested as a diagnostic tool. A basal meningeal biopsy appears to be the only alternative diagnostic approach.
Insights
Diffuse meningeal gliomatosis (DMG) is a rare syndrome mimicking meningitis. Early diagnosis using GFAP immunoperoxidase staining of CSF cells is crucial for effective treatment.
Area of Science:
- Neurology
- Oncology
- Pathology
Background:
- Diffuse meningeal gliomatosis (DMG) presents as extensive basal and spinal meningitis.
- Clinical symptoms include confusion, headaches, diplopia, papilledema, and cranial nerve palsies.
Observation:
- Cerebrospinal fluid (CSF) shows elevated protein, mononuclear pleocytosis, and normal/low glucose.
- DMG is often misdiagnosed as tuberculous or fungal meningitis due to negative CSF cultures.
Findings:
- A review identified three cases of DMG, with a fourth case reported here.
- Glial fibrillary acidic protein (GFAP) immunoperoxidase staining of exfoliated CSF cells is proposed as a diagnostic method.
Implications:
- Accurate diagnosis of DMG is critical for appropriate patient management.
- Basal meningeal biopsy is suggested as an alternative diagnostic approach when CSF analysis is inconclusive.