Tang Sung-Chun, Jeng Jiann-Shing, Liu Hon-Man, Yip Ping-Keung
Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan.
Cerebrovasc Dis. 2003;15(1-2):140-2. doi: 10.1159/000067136.
Brain capillary telangiectasia (BCT) are usually small, solitary, benign in clinical manifestation, or found incidentally at autopsy. However, diffuse BCT are rarely reported. A 39-year-old woman had the first generalized seizure 10 years previously. Thereafter, she had sustained progressive spastic paraparesis and blurred vision. Computed tomography (CT) showed diffuse brain atrophy with numerous calcified spots. Contrast T1-weighted magnetic resonance images showed diffuse faintly enhancing lesions with stippled appearance in the whole brain. Cerebral angiography revealed multiple small nets of dilated capillaries and Xenon CT showed diffuse low cerebral blood flow in the resting and good cerebral reserve capacity in the acetazolamide challenge test. Therefore, diffuse BCT was diagnosed clinically. Although BCT are not rare, diffuse BCT with slowly progressive neurological symptoms had never been reported before. It causes global cerebral ischemia that lead to brain atrophy and a degenerative course.
脑毛细血管扩张症（BCT）通常较小，为单发，临床表现良性，或在尸检时偶然发现。然而，弥漫性BCT鲜有报道。一名39岁女性在10年前首次出现全身性癫痫发作。此后，她持续出现进行性痉挛性截瘫和视力模糊。计算机断层扫描（CT）显示弥漫性脑萎缩并伴有大量钙化点。对比增强T1加权磁共振成像显示全脑弥漫性轻度强化病灶，呈斑点状外观。脑血管造影显示多个扩张毛细血管的小网，氙CT显示静息状态下脑血流弥漫性降低，乙酰唑胺激发试验显示脑储备能力良好。因此，临床诊断为弥漫性BCT。虽然BCT并不罕见，但此前从未报道过伴有缓慢进展性神经症状的弥漫性BCT。它会导致全脑缺血，进而导致脑萎缩和病程进展。
