Neuroimaging in myotonic dystrophy type 1.

摘要

A 56-year-old woman with a 10-year history of myotonic dystrophy type 1 (MD) presented with progressive lower extremity weakness. MRI showed multiple discrete and confluent areas of abnormal signal intensity diroughout the subcortical white matter with predominant involvement of the frontal and anterior temporal lobes (figure 1).The confluent white matter lesions in the anterior temporal lobes seen in MD can be radiologically confused with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Unlike CADASIL, MD also displays skull abnormalities (figure 2).12 Both syndromes should be considered in patients with confluent temporal lobe lesions on MRI.