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DOI: 10.1055/s-0032-1331773
Atypische Parkinsonsyndrome – Neues aus Diagnostik und Therapie
Atypical Parkinsonian Disorders – New Aspects in Diagnosis and TherapyPublication History
Publication Date:
03 January 2013 (online)

Zusammenfassung
Die meisten Patienten mit Parkinsonismus leiden an einem idiopathischen Parkinsonsyndrom (idiopathic Parkinson`s disease=IPD), doch präsentiert sich eine beträchtliche Minderzahl mit zusätzlichen atypischen Symptomen. Diese als atypische Parkinsonsyndrome (atypical parkinsonian disorders=APDs) bezeichneten Erkrankungen, können (differenzial-) diagnostische Schwierigkeiten bereiten. Allen APDs gemeinsam sind die rasche Progression und der häufig schlecht oder gar nicht auf Levodopa ansprechende Parkinsonismus. Neben der Multisystematrophie (MSA) und der Demenz mit Lewy-Körperchen (Dementia with Lewy bodies=DLB), die zusammen mit dem IPD zu den α-Synucleinopathien gezählt werden, werden die progressive supranukleäre Blickparese (PSP) und kortikobasale Degeneration (CBD; beides Tauopathien) dem Komplex der APDs zugeordnet. In den letzten Jahrzehnten lieferten eine Fülle von wissenschaftlichen Studien zahlreiche neue Erkenntnisse und steigerten damit auch das Bewusstsein für die APDs in der Klinik. Durch die Anwendung moderner Untersuchungsverfahren in Kombination mit internationalen Diagnosekriterien kann verlässlicher denn je die Diagnose eines APD gestellt werden.
Dieser Artikel gibt einen Überblick über die aktuellen Entwicklungen in den Bereichen Diagnostik und Therapie auf dem Gebiet der APDs.
Abstract
Most patients presenting with parkinsonism suffer from idiopathic Parkinson’s disease (IPD). However some patients develop additional atypical features that can cause problems in the differential diagnosis. The atypical parkinsonian disorders (APDs) include synucleinopathies such as multiple system atrophy (MSA) and dementia with Lewy bodies (DLB), as well as progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) which belong to the tauopathies. All APDs share a rapid progression and a poor or absent response to Levodopa treatment. In the past few years new scientific approaches increased the knowledge about these syndromes dramatically and with it also the awareness of the clinicians. Improved international diagnostic criteria and advances in neuroimaging have significantly facilitated the diagnosis of this group of diseases.
This article provides a comprehensive summary of novel developments in the diagnostic and therapeutic fields of APDs.
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