Abstract
We present the case of a 43-year-old lady who presented with headaches, visual impairment, and seizures, progressing rapidly over the course of a few weeks. Extensive workup excluded an inflammatory or infectious cause. Imaging studies revealed diffuse thickening of the leptomeninges and serial CSF analysis showed raised opening pressures and increased protein levels. A diagnostic biopsy of the lower thoracic dura confirmed the diagnosis of primary diffuse leptomeningeal gliomatosis (PDGL). She was managed supportively for her symptoms and unfortunately she passed away a few weeks later.
Highlights
Primary diffuse leptomeningeal gliomatosis is a rare cause of raised intracranial pressure and can affect any patients of any age
Primary diffuse leptomeningeal gliomatosis is a rare condition characterised by infiltration of the meninges by neoplastic glial cells without evidence of primary tumour in the brain or the spinal cord [1]
It is an fatal condition which is characterised by infiltration of meninges by tumour cells composed of nests of heterotopic glial cells
Summary
Primary diffuse leptomeningeal gliomatosis is a rare cause of raised intracranial pressure and can affect any patients of any age. It is a challenging diagnosis requiring expert opinion and other differentials must be ruled out before reaching the final diagnosis which usually can only be confirmed with a biopsy
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