engleski

Primary diffuse leptomeningeal gliomatosis - a case report

The primary diffuse leptomeningeal gliomatosis PDLG) is a rare malignant condition of the meninges caused by glial cells infiltration, without the evidence of a primary brain or spinal cord tumour. It often manifests the symptoms and physical findings of the chronic aseptic meningitis, raised intracranial pressure and epileptic seizures. 37-year-old woman was admitted with non-specific symptoms of a tension-like retrobulbar headache, nausea and vomiting, neck pain, visual blurring and flashing lights. Initial neuroimaging of the brain and the spinal cord was normal with signs of papilloedema. As the symptoms progressed, a high-resolution MRI was performed which showed signs of elevated intracranial pressure and hydrocephalus. Cerebrospinal fluid (CSF) showed proteinorachia (2, 97 g/L). A ventriculo-peritoneal shunt was implanted but without any clinical improvement. She developed a cognitive decline, ataxia, bulbar nerve palsy and started getting generalized tonic-clonic seizures. The control MRI showed diffuse leptomeningeal and pachymeningeal gadolinium imbibition and CSF levels of protein continued to rise (21.70 g/l). The open brain biopsy was performed, but unfortunately followed by rapid clinical deterioration and death. PDLG is a rare disease and only 50 cases are reported in the literature so far. The disease has a rapid and progressive course (4-15 months) and the diagnosis is usually made during the autopsy. It is necessary to define early diagnostic signs in order to start early oncological treatment with the aim of slowing the progression of the disease. The paper will therefore point out early neuroradiological signs of this disease, which in this case were not recognized.

primary diffuse leptomeningeal gliomatosis; magnetic resonance imaging; proteinorachia

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