title: [Amyloid beta-related angiitis: brain lesions showing leptomeningeal gadolinium enhancement on MRI and characteristic surgical pathologic features].
abstract: Amyloid-β-related angiitis (ABRA) of the CNS occurs in association with vasculitis of small-and medium-sized leptomeningeal arteries. Here, we describe the clinicopathological features of a 76-year-old man with ABRA. The patient suffered progressive truncal oscillation, aphasia, and recent memory disturbance with a subacute disease onset. His cerebrospinal fluid showed a mild increase in protein levels (101 mg/dL) and pleocytosis (8/mm(3)). High-intensity brain lesion were detected on T(2)-weighted and FLAIR MRI scans, and prominent spread of gadolinium enhancement spreading was observed through the sulci of the left occipital and temporal lobes and left cerebellar hemisphere. A biopsy of the left temporal lesion showed a granulomatous and angiodestructive inflammation with infiltration of many CD4(+) T-lymphocytes and multinucleated giant cells and with fibrinoid necrosis of the arterial walls in the subarachnoid space. Immunolabeling for Aβ(1-40) revealed the abundant deposition of this protein in the affected arteries. On the basic of the diagnosis of ABRA, immunosuppressive therapy was conducted, and it ameliorated the clinical course.
authors.affiliation: Department of Neurology, Niigata City General Hospital, Japan.
authors.forename: Yuka
authors.initials: Y
authors.lastname: Koike
authors.forename: Haruka
authors.initials: H
authors.lastname: Ouchi
authors.forename: Tomoe
authors.initials: T
authors.lastname: Sato
authors.forename: Junsuke
authors.initials: J
authors.lastname: Shimbo
authors.forename: Aki
authors.initials: A
authors.lastname: Sato
authors.forename: Osamu
authors.initials: O
authors.lastname: Sasaki
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Shibuya
authors.forename: Kouichirou
authors.initials: K
authors.lastname: Okamoto
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Kakita
authors.forename: Shuichi
authors.initials: S
authors.lastname: Igarashi
mesh_terms: D000368:Aged; D016229:Amyloid beta-Peptides / Q000378:metabolism*; D001706:Biopsy; D001921:Brain / Q000098:blood supply / Q000378:metabolism / Q000473:pathology*; D002555:Cerebrospinal Fluid / Q000737:chemistry; D005682:Gadolinium; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D014657:Vasculitis / Q000175:diagnosis / Q000473:pathology*
chemical_list: D016229:Amyloid beta-Peptides; D005682:Gadolinium