Haan J, Roos R A, Algra P R, Lanser J B, Bots G T, Vegter-Van der Vlis M
Department of Neurology, University Hospital, Leiden, The Netherlands.
Brain. 1990 Oct;113 ( Pt 5):1251-67. doi: 10.1093/brain/113.5.1251.
The clinical history and magnetic resonance imaging (MRI) findings are presented of 7 patients with hereditary cerebral haemorrhage with amyloidosis--Dutch type (HCHWA-D). The diagnosis was based on clinical and genealogical data, was confirmed in 3 patients at autopsy and in 2 others by biopsy. Focal neurological signs, and at least some degree of global cognitive deterioration, were observed in all patients, with unequivocal dementia in 4. MRI showed haemorrhages and areas of gliosis and, to a variable extent, hyperintensity of the white matter in T2-weighted images. Neuropathological examination revealed a large recent haemorrhage together with residual lesions from previous haemorrhages or infarcts in all patients examined. The white matter lesions, present on MRI, turned out to be areas of 'incomplete infarction' with demyelination. It is concluded that (hereditary) amyloid angiopathy can lead to strokes, but also to subcortical ischaemic encephalopathy. Amyloid angiopathy should therefore be considered in the differential diagnosis of white matter lesions, found on CT or MRI, especially when patients present with a cerebral haemorrhage. The relationship between HCHWA-D and Alzheimer's disease, another disease with cerebral amyloid deposition and diffuse white matter involvement, is discussed.
本文呈现了7例荷兰型遗传性脑出血伴淀粉样变性（HCHWA-D）患者的临床病史及磁共振成像（MRI）检查结果。诊断依据临床和系谱数据，3例经尸检确诊，2例经活检确诊。所有患者均出现局灶性神经体征，且至少有一定程度的整体认知功能减退，4例患者出现明确的痴呆症状。MRI显示出血、胶质增生区域，以及在T2加权图像上不同程度的白质高信号。神经病理学检查发现，所有接受检查的患者均有近期大量出血，以及既往出血或梗死的残留病灶。MRI上出现的白质病变，结果显示为伴有脱髓鞘的“不完全梗死”区域。得出的结论是，（遗传性）淀粉样血管病可导致中风，也可导致皮质下缺血性脑病。因此，在CT或MRI上发现白质病变进行鉴别诊断时，应考虑淀粉样血管病，尤其是当患者出现脑出血时。本文还讨论了HCHWA-D与阿尔茨海默病之间的关系，阿尔茨海默病是另一种伴有脑淀粉样沉积和弥漫性白质受累的疾病。
