[Demyelinating disorders]

Abstract

Leukoencephalopathies in adults are frequent and exhibit highly variable aetiology, including multiple acquired causes such as inflammatory, vascular or toxic diseases and neoplasias. In contrast leukodystrophies are genetically determined, chronic progressive myelin disorders with a variable pathogenetic background and a great diversity of clinical and paraclinical findings. Some diseases, namely those with an additional inborn error of metabolism, are treatable. Genetic counselling appears to be of major importance for patients and their families. In the light of numerous acquired adulthood leukoencephalopathies a clear delineation of late-onset genetic leukodystrophies is necessary. Clinical symptoms and MRI patterns of some of the major leukodystrophies are reported, including possibilities of biochemical and genetic testing.