Abstract Details

Title Meningioangiomatosis: A Rare Presentation with Progressive Cortical Blindness and Encephalopathy

Topic Neuro-oncology

Presentation(s) P01 - (-)

Poster/Presentation
Number 111

Objective

Background Meningioangiomatosis is extremely rare entity involving the cerebral cortex and overlying leptomeninges. MA often causes seizures, headaches, or transient neurologic deficits. We present a unique case report of MA with permanent and progressive cortical deficits.

Design/Methods A 58-year-old, previously healthy Hispanic man presented with 6-month history of headaches, progressive left sided visual defects and right-sided paresthesias. He was seen in the inpatient and followed up in an outpatient setting for about a year-and-a-half. During that period, he became cortically blind though his headaches and paresthesias resolved.

Results Initial computed tomography (CT) scan of the head showed contrast enhancing hypodensities in the bilateral parietal and occipital lobes. Magnetic resonance imaging (MRI) of the brain showed an area of restricted diffusion that enhanced with gadolinium in the cortex of the left occipital lobe. FLAIR sequences showed hyperintensities in both occipital and parietal lobes extending to the posterior frontal lobes bilaterally. His serum and CSF showed no evidence of most of the antibodies or mutations. MRI of the brain, eighteen months later revealed increased hyperintensities, restricted diffusion and contrast enhancement extending to the posterior temporal lobe, occipital lobes and frontoparietal lobes. An occipital lobe biopsy revealed plaque-like proliferation of small capillaries throughout the cortex replacing the parenchyma. These vessels were ensheathed by spindle-shaped mesenchymal cells that appeared meningothelial or fibroblastic. Many entrapped cortical neurons displayed neurofibrillary change and granulovacuolar degeneration. This was diagnostic for meningioangiomatosis.

Conclusions MA is an extremely rare entity which is generally associated with neurofibromatosis and resolves after resection. This case is unique for four reasons; the patient does not have NF-2, never had seizures, symptoms are permanent and the disease is progressive.

Authors/Disclosures
Akshay M. Shah, MD (San Jose Pacific Neurology) PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Myrka R. Torres-Melendez, MD (Banner-University of Arizona)	Dr. Torres-Melendez has nothing to disclose.
Kendra Drake, MD, FAAN (Western Neuro)	Dr. Drake has nothing to disclose.
	No disclosure on file
Ludwig Kappos, MD, FAAN (RC2NB, University Hospital Basel)	Dr. Kappos has nothing to disclose.

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