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DOI: 10.1055/s-0042-123730
Neudefinition atypischer Parkinson-Syndrome
Redefinition of Atypical Parkinsonian SyndromesPublikationsverlauf
Publikationsdatum:
11. April 2017 (online)
![](https://www.thieme-connect.de/media/aktneu/201702/lookinside/thumbnails/0097_10-1055-s-0042-123730-1.jpg)
Zusammenfassung
Die Definition der atypischen Parkinson-Syndrome befindet sich im Wandel. Der Oberbegriff atypische Parkinson-Syndrome umfasst Erkrankungen mit unterschiedlichen zugrundeliegenden Pathologien. Dies sind zum einen die Multisystematrophie (MSA) und die Demenz mit Lewy-Körpern (DLK), die beide durch intrazelluläre Aggregate des Proteins alpha-Synuklein gekennzeichnet sind; zum anderen die Kortikobasale Degeneration (CBD) und die Progressive Supranukleäre Blickparese (PSP), die sich durch Aggregate des Proteins Tau auszeichnen. Die aktuelle Syndrom-basierte Klassifikation dieser Erkrankungen entspricht nicht mehr den Anforderungen der Zeit, da 1) zahlreiche klinisch-pathologische Studien gezeigt haben, dass das klinische Syndrom oft nicht mit der molekular-pathologischen Diagnose übereinstimmt, 2) ein klinisches Parkinson Syndrom mitunter in Patienten mit pathologischer Diagnose eines atypischen Parkinson Syndroms sogar fehlt und 3) aktuelle Therapie-Studien, die in die molekularen Krankheitsmechanismen eingreifen, keine Symptom-orientierte, sondern eine pathogenetisch orientierte Diagnose erfordern. Die frühe und korrekte Vorhersage der zugrundeliegenden molekularen Pathologie als Voraussetzung für eine ursächliche Therapie stellt daher eine Herausforderung dar, der das alte Krankheitskonzept nicht mehr gewachsen ist.
Abstract
The definition of atypical parkinsonian syndromes is changing. The umbrella term of atypical parkinsonian syndrome includes diseases with different underlying pathologies. These are, on the one hand, multiple system atrophy (MSA) and dementia with Lewy bodies (DLB), both characterized by intracellular aggregates of the protein alpha-synuclein and on the other hand, corticobasal degeneration (CBD) and progressive supranuclear gaze visualization (PSP), which are characterized by aggregates of the protein tau. The current syndrome-based classification of these diseases no longer meets current requirements since 1) numerous clinical pathological studies have shown that the clinical syndrome often does not coincide with the molecular-pathological diagnosis, 2) sometimes in patients with a pathological diagnosis of an typical parkinsonian syndrome, a clinical parkinsonian syndrome is even missing and 3) current therapeutical trials based on the molecular disease mechanisms require not a symptom-oriented, but a pathogenetically oriented diagnosis. The early and correct identification of the underlying molecular pathology as a prerequisite for a causative therapy therefore presents a challenge that the old disease concept is no longer capable of meeting.
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