Yoshimura M, Yamanouchi H, Kuzuhara S, Mori H, Sugiura S, Mizutani T, Shimada H, Tomonaga M, Toyokura Y
Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology, Japan.
J Neurol. 1992 Oct;239(8):441-50. doi: 10.1007/BF00856809.
Dementia is in addition to cerebral haemorrhage major symptom of cerebral amyloid angiopathy (CAa). In order to explore the pathological basis for dementia in CAa-related conditions, we made a clinicopathological analysis of CAa, with special attention to dementia. Among 150 patients (mean age 78.6 years) with autopsy-proven intracranial haemorrhage in Tokyo Metropolitan Geriatric Medical Center, CAa with cerebral haemorrhage accounted for 8.0% (12 cases), associated with hypertension and metastatic brain tumour. Among 38 patients with lobar haemorrhage, CAa represented the second most common cause (21.1%) of intracranial haemorrhage after hypertension. A total of 20 patients with CAa (mean age 82.5 years) were studies clinically and pathologically. Hypertension was present in 50%. Thirteen had a history of stroke and others had either ill-defined or no strokes. The average number of strokes 2.9. Fifteen patients (75%) had dementia. Based on the clinicopathological grounds for dementia, CAa-related conditions could be divided into three subtypes: "haemorrhagic", "dementia-haemorrhagic" and "dementia" type. Haemorrhagic type (30%, 6 cases) showed multiple recurrent lobar haemorrhages caused by CAa. Hypertension was present in only 1 patient. The incidence of senile plaques and neurofibrillary tangles was generally correlated with age. Only 1 patient had dementia. The dementia-haemorrhagic type (40%, 8 patients) had recurrent strokes with cerebral haemorrhage after preceding dementia. There were two different neuropathological subsets: CAa with atypical senile dementia of Alzheimer type (SDAT) and CAa with diffuse leucoencephalopathy. Patients with CAa with atypical SDAT had multiple cerebral haemorrhages caused by CAa combined with atypical Alzheimer-type pathology. Patients with CAa with diffuse leucoencephalopathy had cerebral haemorrhages in combination with diffuse white matter damage like Binswanger's subcortical vascular encephalopathy (BSVE). The incidence of senile changes correlated with age. Patients with the dementia type (30%, 6 patients) showed progressive dementia with or without haemorrhage. All had hypertension. They had a combined condition of Alzheimer-type pathology with conspicuous CAa with BSVE. Dementia in CAa-related conditions may be responsible for multiple factors including not Alzheimer-type degeneration, but also diffuse leucoencephalopathy like Binswanger's disease. We also found an asymptomatic type, an ischaemic type, a vasculitis type and an hereditary type in this condition.
除脑出血外，痴呆是脑淀粉样血管病（CAa）的主要症状。为探究CAa相关病症中痴呆的病理基础，我们对CAa进行了临床病理分析，特别关注痴呆情况。在东京都老人综合医院150例经尸检证实有颅内出血的患者（平均年龄78.6岁）中，伴有脑出血的CAa占8.0%（12例），与高血压和脑转移瘤相关。在38例脑叶出血患者中，CAa是仅次于高血压的第二常见颅内出血原因（21.1%）。共有20例CAa患者（平均年龄82.5岁）接受了临床和病理研究。50%的患者有高血压。13例有中风病史，其他患者中风情况不明确或无中风史。平均中风次数为2.9次。15例患者（75%）有痴呆。基于痴呆的临床病理依据，CAa相关病症可分为三种亚型：“出血型”、“痴呆 - 出血型”和“痴呆型”。出血型（30%，6例）表现为由CAa引起的多次复发性脑叶出血。仅1例患者有高血压。老年斑和神经原纤维缠结的发生率一般与年龄相关。仅1例患者有痴呆。痴呆 - 出血型（40%，8例）在先前痴呆后出现伴有脑出血的复发性中风。有两种不同的神经病理亚组：伴有非典型阿尔茨海默型老年性痴呆（SDAT）的CAa和伴有弥漫性白质脑病的CAa。伴有非典型SDAT的CAa患者有由CAa引起的多次脑出血并伴有非典型阿尔茨海默型病理改变。伴有弥漫性白质脑病的CAa患者有脑出血并伴有类似宾斯旺格皮质下血管性脑病（BSVE）的弥漫性白质损害。老年变化的发生率与年龄相关。痴呆型（30%，6例）患者表现为有或无出血的进行性痴呆。所有患者都有高血压。他们患有阿尔茨海默型病理改变合并明显的CAa和BSVE。CAa相关病症中的痴呆可能由多种因素导致，包括并非阿尔茨海默型退变，还包括类似宾斯旺格病的弥漫性白质脑病。我们还发现了这种病症的无症状型、缺血型、血管炎型和遗传型。
