Meningothelial hyperplasia and superficial cortical vein attenuation

Case contributed by Dr Angel Donato


Presented to ER with hallucinations and delirium. Physical exam revealed decreased sensation to the left face and palm of the hand. Three lumbar punctures were completed with normal cell counts and elevated protein levels. A twenty-four hour EEG (on and off valproate) found no evidence of seizure activity.

Patient Data

Age: 40 years
Gender: Male

Gadolinium-enhanced images demonstrate diffuse engorgement of multiple vascular structures extending from hemispheric cortex through subcortical white matter, deep white matter and subependymal margins on both cerebral hemispheres as well as throughout cerebellum and brainstem.

Susceptibility weighted images demonstrate remarkable accentuation of the parenchymal vessels.

There is no recognizable venous obstruction to account for vascular engorgement. There is not a dural fistula.

Cortical biopsy reports meningothelial hyperplasia.

Case Discussion

Due to the MRI findings, a stereotactic biopsy was performed by the treating neurosurgical team.

Meningothelial hyperplasia is a poorly characterized entity and occasionally causes diagnostic difficulties due to its overlapping histologic features with meningioma. It is a reactive process characterized by a proliferation of meningothelial cells that reaches a thickness of ten or more cell layers with PR positivity, which is not seen in normal cells.

In this case, the brain MRI shows remarkable accentuation of the parenchymal vessels throughout the full thickness of the cortex and white matter. 

The relationship between the vascular attenuation and the histological finding of meningothelial hyperplasia is unclear and a causal relationship has not been firmly established. 

This case was presented in Eurorad.

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Case information

rID: 51068
Published: 3rd Jan 2018
Last edited: 24th Jun 2021
Inclusion in quiz mode: Included

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