M. Mayer

TL;DR: The phenotypic spectrum and natural history of the disease in 102 affected individuals from 29 families with biopsy‐proven CADASIL were delineated, and the extent of disability in different age groups was studied.

TL;DR: Electron microscopy has revealed characteristic electron-dense granular deposits in the basal lamina of vessels of patients with CADASIL, confirming that general microangiopathy is a typical feature of this syndrome and is present in the early phase of the disease with or without clinical manifestation.

TL;DR: In CADASIL patients, a common pattern of cerebral lesion distribution is found and the total T1 lesion volume is an important parameter to correlate with disability, as it may be helpful in predicting the natural history of the disease.

TL;DR: In this article, the authors measured changes of regional cerebral blood volume (rCBV) with dynamic contrastenhanced MR imaging and correlated the changes to disability and cognitive performance, and found that rCBV measured in the hyperintense subcortical white matter in individuals with CADASIL was decreased and inversely correlated with disability.

TL;DR: The data showed no evidence for a different type of movement preparation in MM subjects as compared to normals, and it is proposed that the additional ipsilateral cortical activation around movement onset may be the cortical mechanism, which compensates for abnormal ipsilaterally corticospinal pathways in subjects with persistent MM.