Abstract
Amyloid-beta related angiitis (ABRA) of the central nervous system (CNS) is a rare disorder with overlapping features of primary angiitis of the CNS and cerebral amyloid angiopathy. We evaluated a 74-year-old man with intermittent left sided weakness and MRI findings of leptomeningeal enhancement, vasogenic edema, and subcortical white matter disease proven to have ABRA. We discuss clinicopathological features and review the topic of ABRA.
Highlights
CASE REPORT A 74-year-old man with a past medical history of chronic lymphocytic leukemia (CLL) stage 0, renal cell carcinoma, hypertension, and hyperlipidemia awoke one morning with numbness of the thumb and first two fingers of the left hand “marching” up to the left arm over the course of few seconds
He received Levetiracetam 500 mg twice daily that was subsequently increased to 750 mg twice daily. He was discharged home without a definite diagnosis. The day, he developed severe pain over the right temple and presented to another institution where a second MRI showed similar findings, and a right temporal leptomeningeal biopsy showed intramural as well as perivascular inflammatory infiltrates with granulomatous features in the leptomeninges and penetrating blood vessels associated with vasculopathic changes, as well as positive labeling for beta-amyloid on immunohistochemical studies consistent with amyloid-beta related angiitis (ABRA)
Amyloid-beta related angiitis is a predominantly granulomatous angio-destructive inflammatory mediated disease affecting leptomeningeal and cortical vessels characterized by meningeal lymphocytosis and abundant amyloid-beta deposition within the vessel walls (1, 2)
Summary
CASE REPORT A 74-year-old man with a past medical history of chronic lymphocytic leukemia (CLL) stage 0 (not receiving treatment), renal cell carcinoma (clear cell type, nuclear grade II-untreated), hypertension, and hyperlipidemia awoke one morning with numbness of the thumb and first two fingers of the left hand “marching” up to the left arm over the course of few seconds. The day, he developed severe pain over the right temple and presented to another institution where a second MRI showed similar findings, and a right temporal leptomeningeal biopsy showed intramural as well as perivascular inflammatory infiltrates with granulomatous features in the leptomeninges and penetrating blood vessels associated with vasculopathic changes, as well as positive labeling for beta-amyloid on immunohistochemical studies consistent with amyloid-beta related angiitis (ABRA). Sharing characteristics of both PACNS and cerebral amyloid angiopathy (CAA), ABRA has some differentiating clinical and pathological features. A neuropathological variant of ABRA characterized by non-destructive perivascular inflammatory infiltration and amyloid deposition is known as CAA-related inflammation (CAA-RI) (5, 6).
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