Um Erkrankungen der weißen Substanz korrekt zu diagnostizieren, ist ein systematisches Vorgehen unter Berücksichtigung der Bildgebungsbefunde, der klinischen Merkmale und der Labordaten von entscheidender Bedeutung. Die genaue Kenntnis des pathologischen Substrats ist für das Verständnis der radiologischen Manifestationen fundamental. Die entsprechenden Fakten werden deshalb in diesem Beitrag strukturiert und nachvollziehbar aufbereitet.
Abstract
White matter diseases include a wide spectrum of disorders that have in common impairment of normal myelination, either by secondary destruction of previously myelinated structures (demyelinating processes) or by primary abnormalities of myelin formation (dysmyelinating processes). The pathogenesis of many white matter diseases remains poorly understood. Demyelinating disorders are the object of this review and will be further divided into autoimmune, infectious, vascular, and toxic-metabolic processes. Autoimmune processes include multiple sclerosis and related diseases: tumefactive demyelinating lesions, Balo concentric sclerosis, Marburg and Schilder variants, neuromyelitis optica (Devic disease), acute disseminated encephalomyelitis, and acute hemorrhagic leukoencephalopathy (Hurst disease). Infectious processes include Lyme disease (neuroborreliosis), progressive multifocal leukoencephalopathy, and human immunodeficiency virus (HIV) encephalopathy. Vascular processes include different types of small-vessel disease: arteriolosclerosis, cerebral amyloid angiopathy, cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), primary angiitis of the central nervous system, Susac syndrome, and neurolupus. Toxic-metabolic processes include osmotic myelinolysis, methotrexate leukoencephalopathy, and posterior reversible encephalopathy syndrome. The imaging spectrum can vary widely from small multifocal white matter lesions to confluent or extensive white matter involvement. Understanding the pathologic substrate is fundamental for understanding the radiologic manifestations, and a systematic approach to the radiologic findings, in correlation with clinical and laboratory data, is crucial for narrowing the differential diagnosis.
Schlüsselwörter autoimmunologisch vermittelte Erkrankungen der weißen Substanz - infektiöse Erkrankungen der weißen Substanz - vaskuläre Erkrankungen der weißen Substanz - toxisch-metabolische Erkrankungen der weißen Substanz - radiologisch-pathologische Korrelate
Keywords autoimmune white matter diseases - infectious white matter diseases - vascular white matter diseases - toxic-metabolic white matter diseases - radiologic-pathologic correlation
