title: Cerebral amyloid angiopathy-related inflammation presenting with steroid-responsive higher brain dysfunction: case report and review of the literature.
abstract: A 56-year-old man noticed discomfort in his left lower limb, followed by convulsion and numbness in the same area. Magnetic resonance imaging (MRI) showed white matter lesions in the right parietal lobe accompanied by leptomeningeal or leptomeningeal and cortical post-contrast enhancement along the parietal sulci. The patient also exhibited higher brain dysfunction corresponding with the lesions on MRI. Histological pathology disclosed β-amyloid in the blood vessels and perivascular inflammation, which highlights the diagnosis of cerebral amyloid angiopathy (CAA)-related inflammation. Pulse steroid therapy was so effective that clinical and radiological findings immediately improved.CAA-related inflammation is a rare disease, defined by the deposition of amyloid proteins within the leptomeningeal and cortical arteries associated with vasculitis or perivasculitis. Here we report a patient with CAA-related inflammation who showed higher brain dysfunction that improved with steroid therapy. In cases with atypical radiological lesions like our case, cerebral biopsy with histological confirmation remains necessary for an accurate diagnosis.
authors.affiliation: Department of Neurology, Faculty of Life Sciences, Kumamoto University 1-1-1 Honjo, Kumamoto 860-0811, Japan. hideya5783@fc.kuh.kumamoto-u.ac.jp
authors.forename: Hideya
authors.initials: H
authors.lastname: Sakaguchi
authors.forename: Akihiko
authors.initials: A
authors.lastname: Ueda
authors.forename: Takayuki
authors.initials: T
authors.lastname: Kosaka
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yamashita
authors.forename: En
authors.initials: E
authors.lastname: Kimura
authors.forename: Taro
authors.initials: T
authors.lastname: Yamashita
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Maeda
authors.forename: Teruyuki
authors.initials: T
authors.lastname: Hirano
authors.forename: Makoto
authors.initials: M
authors.lastname: Uchino
mesh_terms: D016657:Cerebral Amyloid Angiopathy / Q000150:complications / Q000188:drug therapy* / Q000473:pathology* / Q000503:physiopathology*; D004660:Encephalitis / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D013256:Steroids / Q000627:therapeutic use*
chemical_list: D013256:Steroids