Lammie G A, Rakshi J, Rossor M N, Harding A E, Scaravilli F
Department of Neuropathology, National Hospital for Neurology and Neurosurgery, London, UK.
Clin Neuropathol. 1995 Jul-Aug;14(4):201-6.
CADASIL is a recently recognized familial form of subcortical multi-infarct dementia. The pathogenesis of the disease is unknown, but it is characterized pathologically by a novel vasculopathy affecting leptomeningeal and subcortical arteries. We describe 2 cases in which the diagnosis could be made from the cerebral biopsy appearances of affected leptomeningeal vessels. The ultrastructural appearances of the vessel wall deposit are illustrated, and their similarity to those of immune complex deposits discussed.
大脑常染色体显性动脉病伴皮质下梗死和白质脑病（CADASIL）是一种最近才被认识的家族性皮质下多发性梗死性痴呆。该病的发病机制尚不清楚，但其病理特征是一种影响软脑膜和皮质下动脉的新型血管病。我们描述了2例可根据受影响软脑膜血管的脑活检表现做出诊断的病例。文中展示了血管壁沉积物的超微结构表现，并讨论了它们与免疫复合物沉积物的相似性。
