Abstract Details

Title A Case of Progressive Left Leg Weakness and Vision Changes: Focal Inflammatory Cerebral Amyloid Angiopathy Masquerading as a Glioma

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P12 - Poster Session 12 (5:30 PM-6:30 PM)

Poster/Presentation
Number 6-003

Objective To emphasize the need to consider tumefactive focal inflammatory cerebral amyloid angiopathy (CAA) in the differential diagnosis of CNS lesions.

Background Although classically considered an etiology of cognitive impairment in the elderly, CAA represents a spectrum of disease. CAA-related inflammation (CAA-ri), a distinct CAA phenotype, is characterized by vascular inflammation from amyloid deposition. CAA-ri may rarely appear as a tumefactive lesion, mimicking CNS malignancy. We present a case of biopsy-proven focal CAA-ri initially misidentified as a glioma, with the goal of increasing awareness of this entity.

Design/Methods

Not applicable

Results A 68-year-old man with Parkinson’s disease presented with progressive left lower extremity weakness, word finding difficulty and vision changes.

Neurological examination revealed Parkinsonian features, left homonymous superior quadrantanopia and left leg weakness. Brain MRI revealed a non-enhancing FLAIR hyperintensity in the subcortical right parieto-occipital lobe, with numerous susceptibility-weighted foci within this area, consistent with microhemorrhages versus vascular inflammation. Flash frozen pathology was initially suggestive of glioma, prompting lesionectomy.

Final pathology revealed inflammation of small and medium sized blood vessels, multinucleated giant cells and positive Congo-red staining, consistent with necrotizing vasculitis and amyloid angiopathy. Treatment with steroids was deferred following risk-benefit analysis.

Conclusions We report a unique case of tumefactive CAA-ri, a clinicopathologic variant of CAA, manifesting as a mass-like lesion with distinguishing radiographic features. Our literature review identified only three prior reports of this entity. Clinical presentation depends on lesion size, location, and presence of edema and inflammation. While our patient lacked systemic co-pathologies, uterine involvement and carpal tunnel syndrome have been reported. Management relies on vascular risk modification, and steroids or immunosuppressants for active inflammation.

Our case underscores the need to recognize CAA-ri in the differential of mass-like lesions. Recognizing the spectrum of CAA-related disease is key in understanding the natural history of the disease, and potential benefit of future anti-amyloid therapies.

Authors/Disclosures
Cameron Mohammadi, MD (MedStar Georgetown University Hospital) PRESENTER	Dr. Mohammadi has nothing to disclose.
Sigmund Lilian, MD	Dr. Lilian has nothing to disclose.
Huanwen Chen, MD (MedStar Georgetown University Hospital)	Dr. Chen has nothing to disclose.
Michelle Offit, MD, MS (MedStar Georgetown University Hospital)	No disclosure on file
Mark M. Lin, MD, PhD, FAAN (Medstar Washington Hospital Center)	Dr. Lin has nothing to disclose.
Nicholas S. Streicher, MD	Dr. Streicher has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion . Dr. Streicher has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Immunovent. Dr. Streicher has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. Dr. Streicher has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Amgen. Dr. Streicher has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Alexion.
Priyanka Sabharwal, MD, MBBS, FAAN (Medstar Washington Hospital Center)	Dr. Sabharwal has nothing to disclose.