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Neurological Surgery 2020-Jul

[Cerebral Amyloid Angiopathy-related Inflammation Demonstrating Early Venous Filling on Digital Subtraction Angiography:A Case Report]

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Daisuke Yamamoto
Daisuke Ishima
Madoka Inukai
Jun Niki
Ryo Usui
Hiroyuki Koizumi
Makoto Saegusa
Kazutoshi Nishiyama
Toshihiro Kumabe

キーワード

概要

Cerebral amyloid angiopathy-related inflammation(CAA-RI)is a rare condition thought to be caused by an inflammatory response to amyloid beta(Aβ)protein in the walls of the small arteries and capillaries of the cerebral cortex. A 73-year-old female presented with left hemiparesis and dysarthria. Fluid-attenuated inversion recovery(FLAIR)imaging disclosed progressive enlargement of infiltrative white matter abnormalities in the right temporo-occipito-parietal lobes. Interestingly, digital subtraction angiography(DSA)demonstrated early venous filling. Pathological examination of the biopsy specimen demonstrated lymphocytes infiltration surrounding the blood vessels and in the thickened walls with amyloid-beta deposition. The diagnosis given was CAA-RI. The patient was successfully treated with high dose corticosteroids and clinical improvement was associated with shrinkage of the high intensity lesion on FLAIR imaging. Early venous filling resolved on the follow-up DSA. Most patients with CAA-RI can be treated with corticosteroids. However, the clinical condition will worsen without appropriate treatment. Early diagnosis is the key. If an expanding disease of the white matter appears in an elderly patient, we should exclude other cerebrovascular diseases by DSA, followed by biopsy without delay. The present case demonstrated that early venous filling on DSA may appear until inflammation is resolved by the treatment of CAA-RI.

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