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Degenerative Kleinhirnerkrankungen und Differenzialdiagnosen

Degenerative cerebellar diseases and differential diagnoses

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Zusammenfassung

Klinisches/methodisches Problem

Klinisch imponieren Kleinhirnsyndrome durch Ataxie, Dysarthrie, Dysmetrie, Intentionstremor und Augenbewegungsstörungen.

Radiologische Standardverfahren

Neben der Anamnese und klinischen Untersuchung ist die Bildgebung v. a. wichtig um andere Erkrankungen wie Hydrozephalus und Multiinfarktdemenz von degenerativen Kleinhirnerkrankungen zu differenzieren. Zu den degenerativen Erkrankungen mit Kleinhirnbeteiligung gehören der Morbus Parkinson, die Multisystematrophie sowie weitere Erkrankungen einschließlich der spinozerebellären Ataxien.

Bewertung

Neben der MRT sind auch nuklearmedizinische Untersuchungen zur Differenzierung hilfreich.

Empfehlung für die Praxis

Axiale Fluid-attenuated-inversion-recovery(FLAIR)- und T2-gewichtete Sequenzen können mitunter eine Signalsteigerung im Pons als Ausdruck einer Degeneration der pontinen Neuronen und transversalen Bahnen im Brückenfuß zeigen. Die Bildgebung ist aber v. a. notwendig, um andere Erkrankungen wie Normaldruckhydrozephalus, Multiinfarktdemenz und zerebelläre Läsionen auszuschließen.

Abstract

Clinical/methodical issue

Cerebellar syndromes result in distinct clinical symptoms, such as ataxia, dysarthria, dysmetria, intention tremor and eye movement disorders.

Standard radiological methods

In addition to the medical history and clinical examination, imaging is particularly important to differentiate other diseases, such as hydrocephalus and multi-infarct dementia from degenerative cerebellar diseases. Degenerative diseases with cerebellar involvement include Parkinson’s disease, multiple system atrophy as well as other diseases including spinocerebellar ataxia.

Achievements

In addition to magnetic resonance imaging (MRI), nuclear medicine imaging investigations are also helpful for the differentiation.

Practical recommendations

Axial fluid-attenuated inversion recovery (FLAIR) and T2-weighted sequences can sometimes show a signal increase in the pons as a sign of degeneration of pontine neurons and transverse fibers in the basilar part of the pons. The imaging is particularly necessary to exclude other diseases, such as normal pressure hydrocephalus (NPH), multi-infarct dementia and cerebellar lesions.

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Correspondence to W. Reith.

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Interessenkonflikt

W. Reith, S. Roumia und P. Dietrich geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Reith, W., Roumia, S. & Dietrich, P. Degenerative Kleinhirnerkrankungen und Differenzialdiagnosen. Radiologe 56, 976–982 (2016). https://doi.org/10.1007/s00117-016-0180-0

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  • DOI: https://doi.org/10.1007/s00117-016-0180-0

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