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Der Radiologe

, Volume 50, Issue 9, pp 775–783 | Cite as

Stoffwechselstörungen mit typischen Veränderungen im MRT

  • M. Warmuth-MetzEmail author
Leitthema
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Zusammenfassung

Die Einteilung von Stoffwechselstörungen nach ihrer Ätiologie ist für den diagnostischen Neuroradiologen nicht sinnvoll, da sich aus der zugrunde liegenden Störung keine Rückschlüsse auf die zu erwartende MR-Morphologie ziehen lassen. Deshalb sollen anhand typischer bildmorphologischer Veränderungen in Zusammenschau mit den jeweiligen klinischen Charakteristika einige leicht einzuordnende Stoffwechselstörungen dargestellt werden. Es handelt sich um den Morbus Canavan, Morbus Pelizaeus-Merzbacher, Morbus Alexander, die X-chromosomal vererbte Adrenoleukodystrophie und Adrenomyeloneuropathie, die mitochondrialen Störungen MELAS (mitochondriale Enzephalomyopathie, Laktazidose und Stroke-like-Episoden) und Leigh-Syndrom sowie die L-2-Hydroxyglutarazidurie.

Schlüsselwörter

Stoffwechselstörung Mitochondriale Störungen Morbus Alexander Morbus Pelizaeus-Merzbacher Morbus Canavan L-2-Hydroxyglutarazidurie X-chromosomale Adrenoleukodystrophie (XALD) 

Metabolic disorders with typical alterations in MRI

Abstract

The classification of metabolic disorders according to the etiology is not practical for neuroradiological purposes because the underlying defect does not uniformly transform into morphological characteristics. Therefore typical MR and clinical features of some easily identifiable metabolic disorders are presented. Canavan disease, Pelizaeus-Merzbacher disease, Alexander disease, X-chromosomal adrenoleukodystrophy and adrenomyeloneuropathy, mitochondrial disorders, such as MELAS (mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes) and Leigh syndrome as well as L-2-hydroxyglutaric aciduria are presented.

Keywords

Metabolic disorders Mitochondrial disorders Alexander disease Pelizaeus-Merzbacher disease Canavan disease L-2-hydroxyglutaric aciduria X-chromosomal adrenoleukodystrophy (XALD) 

Notes

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  1. 1.Abteilung für NeuroradiologieKlinikum der Universität WürzburgWürzburgDeutschland

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