Cerebral amyloid angiopathy-related inflammation presenting with steroid-responsive higher brain dysfunction: case report and review of the literature

Journal of Neuroinflammation

Makoto Uchino¹  Teruyuki Hirano¹  Yasushi Maeda¹  Taro Yamashita¹  En Kimura¹  Satoshi Yamashita¹  Takayuki Kosaka¹  Akihiko Ueda¹  Hideya Sakaguchi¹ 

[1]Department of Neurology, Faculty of Life Sciences, Kumamoto University 1-1-1 Honjo, Kumamoto 860-0811, Japan

关键词: higher brain dysfunction;    CAA-related inflammation;    cerebral amyloid angiopathy;   

Others  :  1213126
DOI  :  10.1186/1742-2094-8-116

 received in 2011-05-20, accepted in 2011-09-14,  发布年份 2011

【 摘 要 】

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.

【 授权许可】

   
2011 Sakaguchi et al; licensee BioMed Central Ltd.

【 图 表 】

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