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Article type: Research Article
Authors: Chu, Shuguanga; 1 | Xu, Feijiad; 1 | Su, Yab | Chen, Hongc | Cheng, Xinb; *
Affiliations: [a] Department of Radiology, Huashan Hospital, Fudan University, Shanghai, China | [b] Department of Neurology, Huashan Hospital, State Key Laboratory of Medical Neurobiology, Fudan University, Shanghai, China | [c] Department of Neuropathology, Huashan Hospital, Fudan University, Shanghai, China | [d] Department of Radiology, Shanghai Tenth People’s Hospital, Tongji University, Shanghai, China
Correspondence: [*] Correspondence to: Xin Cheng, MD, PhD, Department of Neurology, Huashan Hospital, State Key Laboratory of Medical Neurobiology, Fudan University, 12 Wulumuqi Zhong Road,Shanghai 200040, China. Tel.: +86 21 5288 7145; Fax: +86 21 6248 1088; E-mail: chengxin@fudan.edu.cn.
Note: [1] These authors contributed equally to this work.
Abstract: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a relatively rare syndrome of reversible encephalopathy and could be divided into two subtypes of inflammatory CAA (ICAA) and amyloid-β-related angiitis (ABRA) according to histopathology. We present a case of pathologically proved ABRA with partial seizures and headache, and a focal lesion in the right temporal lobes on magnetic resonance imaging. Summarized from previous 139 ABRA and ICAA cases, ABRA is preferred when the lesion is enhanced on MRI and requires combination drug therapy, while ICAA is highly suspected with ApoE genotype of ɛ4/ɛ4. More clinical markers for diagnosis of CAA-ri warrant further researches.
Keywords: Amyloid-β-related angiitis, cerebral amyloid angiopathy, cerebral amyloid angiopathy-related inflammation, inflammatory cerebral amyloid angiopathy
DOI: 10.3233/JAD-151036
Journal: Journal of Alzheimer's Disease, vol. 51, no. 2, pp. 525-532, 2016
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