A 4-GENERATION DUTCH FAMILY WITH CEREBRAL AUTOSOMAL-DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY (CADASIL), LINKED TO CHROMOSOME 19P13

We describe a four-generation Dutch family suffering from cerebral aut osomal dominant arteriopathy with subcortical infarcts and leukoenceph alopathy (CADASIL). Of twenty affected family members, ten are still a live. Age at onset of the strokes was between 29 and 52 years, with a mean of 41.8 years. This family has the typical clinical and radiologi cal features of CADASIL (except for the occurrence of ischemic heart d isease at a relatively young age in some subjects), and is linked to c hromosome 19p13. This disease has so far been described in families fr om Finland, France, Germany, Italy, Japan, Spain and the United Kingdo m, and there is a remarkable clinical and genetical homogeneity among all families reported, including this Dutch family.