Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of inflammatory cerebral amyloid angiopathy with white matter lesions appearing after brain biopsy

Yosuke Takeuchi, M.D.1), Shuei Murahashi, M.D.1), Yasuyuki Hara, M.D., Ph.D.1), Makoto Nakajima, M.D., Ph.D.2) and Mitsuharu Ueda, M.D., Ph.D.2)

1) Department of Neurology, Kumamoto Rosai Hospital
2) Department of Neurology, Graduate School of Medical Sciences, Kumamoto University

A 76-year-old woman with a 7-year history of dementia presented to our hospital with generalized convulsive seizure for the first time. Contrast-enhanced brain magnetic resonance imaging revealed leptomeningeal enhancement mainly in the right occipital lobe and multiple lobar microbleeds in the bilateral cerebral and cerebellar subcortex. No white matter lesions were observed. A brain biopsy of the right parieto-occipital lobe revealed cerebral amyloid angiopathy (CAA). White matter lesions appeared in the right parieto-occipital lobe three days after the biopsy, and we considered inflammatory CAA. Three courses of methylprednisolone pulse followed by oral prednisolone therapy gradually reduced leptomeningeal and white matter lesions. An apolipoprotein E genotype investigation identified the ε2/ε3 genotype. In patients with inflammatory CAA, a risk of exacerbation should be considered after brain biopsy, in which the ε2 allele might play a role.
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(CLINICA NEUROL, 61: 188|193, 2021)
key words: inflammatory cerebral amyloid angiopathy, brain biopsy, apolipoprotein E, ε2 allele

(Received: 21-Aug-20)