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Neuroradiologie Scan 2018; 08(02): 129-158
DOI: 10.1055/a-0578-3683
CME-Fortbildung
© Georg Thieme Verlag KG Stuttgart · New York

Erkrankungen der weißen Substanz in der Bildgebung

White matter diseases with radiologic-pathologic correlation
Nicolae Sarbu
,
Robert Y. Shih
,
Robert V. Jones
,
Iren Horkayne-Szakaly
,
Laura Oleaga
,
James G. Smirniotopoulos
Subject Editor: Wissenschaftlich verantwortlich gemäß Zertifizierungsbestimmungen für diesen Beitrag ist Professor Dr. med. Michael Forsting, Universitätsklinikum Essen.
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Publication History

Publication Date:
16 April 2018 (online)

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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.

Kernaussagen

  • Erkrankungen der weißen Substanz zeichnen sich durch ein verändertes Muster der Myelinisierung aus und umfassen ein breites Spektrum kongenitaler und erworbener Prozesse. Sie lassen sich in demyelinisierende und dysmyelinisierende Prozesse unterteilen. Demyelinisierende Erkrankungen können wiederum verschiedenen Kategorien zugeordnet werden.

  • Bei multipler Sklerose handelt es sich um eine primäre demyelinisierende Erkrankung unbekannter Ätiologie, die durch perivenuläre Entzündung bzw. Demyelinisierung bei relativem Axonerhalt gekennzeichnet ist. In der MRT-Bildgebung stellt sie sich in Form von periventrikulären, juxtakortikalen, infratentoriellen und im Rückenmark lokalisierten Läsionen dar.

  • TDL können mit Neoplasien mit hochgradiger Kontrastmittelanreicherung verwechselt werden. Mögliche Anhaltspunkte für die Diagnose bilden eine unvollständige ringförmige Anreicherung und/oder Diffusionsrestriktion, ein schwach ausgebildetes periläsionales Ödem, ein relativ geringer raumfordernder Effekt sowie ein geringes zerebrales Blutvolumen in der Perfusionsbildgebung.

  • Die HIV-Enzephalopathie weist einige Gemeinsamkeiten mit der PML auf: Beide verursachen konfluierende Läsionen der weißen Substanz ohne signifikanten raumfordernden Effekt oder Kontrastmittelanreicherung. Jedoch sind die Läsionen bei PML eher peripher lokalisiert und asymmetrisch angeordnet, bei der HIV-Enzephalopathie hingegen eher zentral lokalisiert und symmetrisch verteilt.

  • Mikroangiopathien stellen bei Weitem die häufigste Ursache von Erkrankungen der weißen Substanz dar und lassen sich in 6 verschiedene Typen unterteilen: Arteriolosklerose, zerebrale Amyloidangiopathie, hereditäre Vaskulopathien, inflammatorische Vaskulitiden, venöse Kollagenose und Sonstige.

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
 

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