This study describes 200 consecutive individuals with CADASIL who were seen at a specialised CADASIL service in London, UK. It provides a good description about which features are most common in the disease. The average age of assessment was 48 years and the average age at which symptoms first occurred was 34 years, although there was a wide age range. Migraine was the most common symptom affecting 75% of individuals. More than half of individuals had suffered a stroke and the average age of onset for this was 46 years. Importantly, this study demonstrated that common risk factors for stroke seemed to make CADASIL worse. For example, both high blood pressure and smoking were associated with an increased risk of stroke. This emphasizes how important it is to treat common cardiovascular risk factors in patients with CADASIL.
Reference: Adib-Samii P, Brice G, Martin RJ, Markus HS. Clinical spectrum of CADASIL and the effect of cardiovascular risk factors on phenotype; study in 200 consecutively recruited individuals. Stroke. 2010;41: 630-4.