Stroke Research Group, Department of Brain Repair and Rehabilitation, UCL Institute of Neurology, Queen Square, London, UK.
J Neurol Neurosurg Psychiatry. 2012 Feb;83(2):124-37. doi: 10.1136/jnnp-2011-301308. Epub 2011 Nov 5.
Sporadic cerebral amyloid angiopathy (CAA) is a common age related cerebral small vessel disease, characterised by progressive deposition of amyloid-β (Aβ) in the wall of small to medium sized arteries, arterioles and capillaries of the cerebral cortex and overlying leptomeninges. Previously considered to be a rare neurological curiosity, CAA is now recognised as an important cause of spontaneous intracerebral haemorrhage and cognitive impairment in the elderly, two fundamental challenges in the field of cerebrovascular disease. Our understanding of the pathophysiology and clinical manifestations of CAA continues to evolve rapidly, with the use of transgenic mouse models and advanced structural and/or molecular neuroimaging techniques. Yet, despite remarkable recent interest, CAA remains under-recognised by neurologists and stroke physicians. In this review, a fresh look at key developments in understanding the complex pathophysiology, important clinical and radiological features, diagnostic approaches and prospects for rational therapies for this enigmatic small vessel disorder is provided.
散发型脑淀粉样血管病(Cerebral Amyloid Angiopathy,CAA)是一种常见的与年龄相关的脑小血管疾病,其特征是淀粉样β(Amyloid-β,Aβ)在大脑皮质的小至中等大小动脉、小动脉和毛细血管壁以及软脑膜上逐渐沉积。CAA 以前被认为是一种罕见的神经学奇事,但现在已被认为是老年人自发性脑出血和认知障碍的重要原因,这是脑血管疾病领域的两个基本挑战。随着转基因小鼠模型和先进的结构和/或分子神经影像学技术的应用,我们对 CAA 的病理生理学和临床表现的理解正在迅速发展。然而,尽管最近引起了极大的关注,但神经病学家和中风医生对 CAA 的认识仍然不足。在这篇综述中,我们对理解这种复杂的病理生理学、重要的临床和影像学特征、诊断方法以及合理治疗这种神秘的小血管疾病的前景的关键进展进行了新的探讨。