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Aktuelle Neurologie 2017; 44(04): 260-266
DOI: 10.1055/s-0043-103081
Übersicht
© Georg Thieme Verlag KG Stuttgart · New York

Neudefinition der Parkinson-Erkrankung

Redefinition of Parkinson’s Disease
Eva Schäffer
Neurologische Klinik, Medizinische Fakultät, Christian-Albrechts-Universität zu Kiel
,
Daniela Berg
Neurologische Klinik, Medizinische Fakultät, Christian-Albrechts-Universität zu Kiel
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Publication History

Publication Date:
19 May 2017 (online)

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Zusammenfassung

2015 stellte eine Arbeitsgruppe der International Parkinson and Movement Disorders Society (MDS) neue klinische Diagnosekriterien für die Parkinson-Erkrankung vor. Diese Übersichtsarbeit fasst die Erkenntnisse der Pathophysiologie, unterschiedlicher klinischer Symptommanifestationen und des Krankheitsverlaufs zusammen, die Grundlage für eine Neudefinition und Basis für die neuen, zusammenfassend dargestellten Diagnosekriterien der Parkinson-Erkrankung sind. Wesentliche Erkenntnisse, die in die neuen Diagnosekriterien einfließen, sind u. a. (i) das Erkennen der Relevanz von nicht-motorischen Symptomen, die die Lebensqualität des Patienten entscheidend beeinflussen können und eine zunehmende Relevanz im Rahmen der Früh- und Differenzialdiagnostik des Parkinson-Syndroms einnehmen. (ii) Die Einordnung einer Demenz im Rahmen der Parkinson-Erkrankung. Während in den vergangenen Jahren eine klare Trennung zwischen Parkinson-Erkrankung und Lewy-Körperchen-Demenz stattfand, wird nun ein Kontinuum postuliert, das unter dem Oberbegriff der Lewy-Körperchen-Erkrankungen die Parkinson-Erkrankung ohne, mit später und mit früher (bereits im ersten Jahr des Auftretens motorischer Symptome) Demenz beinhaltet. (iii) Das Verständnis eines sehr langsam sich über verschiedene Teile des Nervensystems ausbreitenden Prozesses der Neurodegeneration. Dies führte zur Definition verschiedener Phasen der Parkinson-Erkrankung, der präklinischen, prodromalen und klinischen Phase. Insbesondere die Charakterisierung der prodromalen Phase, durch Verwendung klinischer Parameter und Etablierung von noch weiter zu entwickelnden Biomarkern, eröffnet die Möglichkeit der Frühdiagnose und langfristig frühen Therapie des Parkinson-Syndroms. (iv) Die Erkenntnis, dass die klinische Phase durch unterschiedliche Verlaufsformen gekennzeichnet ist. Für genetische Varianten (z. B. LRRK2 oder GBA) wird eine separate klinisch-genetische Einteilung empfohlen, während für die Subtypen-Klassifizierung des idiopathischen Parkinson-Syndroms prognostische und/oder therapeutische Konsequenz bei der Einteilung von Subtypen gefordert wird. Für die Diagnosestellung der Parkinson-Erkrankung postuliert die MDS-Arbeitsgruppe, den bisherigen Goldstandard aus typischer klinischer Präsentation und post-mortem Nachweis einer α-Synucleinopathie beizubehalten. Für die klinischen Diagnosekriterien wurde ein Konzept erarbeitet, welches den diagnostischen Prozess eines erfahrenen Klinikers nachzeichnen soll. Die Diagnosekriterien beinhalten daher neben den klassischen motorischen Kardinalsymptomen neue absolute Ausschlusskriterien, unterstützende Kriterien und sogenannte „Red Flags“. Miteinbezogen werden können ferner spezifische Zusatzuntersuchungen (z. B. bildgebende Verfahren), darüber hinaus wird der zeitliche Verlauf von Symptomen und deren Ausprägungsgrad mitberücksichtigt.

Abstract

In 2015, a working group of the International Parkinsonʼs and Movement Disorders Society (MDS) presented new clinical diagnostic criteria for Parkinsonʼs disease (PD). Key findings: (i) Non-motor symptoms are relevant in the early and differential diagnosis of Parkinsonʼs syndrome (PS). (ii) A continuum of Lewy body disease is postulated that includes PD without, with later and earlier (already in the first year with motor symptoms) dementia. (iii) A preclinical, prodromal and clinical phase of PD is defined. Characterization of the prodromal phase with clinical parameters enables early diagnosis and in the long term early therapy of PS; (iv) The clinical phase is characterized by different progression forms. A separate clinical-genetic classification is recommended for genetic variants (eg LRRK2 or GBA); prognostic and/or therapeutic consequences are required for subtype classification of idiopathic PS. Gold standard for diagnosis of PD, according to MDS, continue to be typical clinical presentation and post-mortem detection of α-synucleinopathy. Diagnostic criteria include, besides classical motor cardinal symptoms, new absolute exclusion criteria, supporting criteria and so-called “red flags”. Additional examinations (eg, imaging methods) can also be included, besides symptom chronology and their degree of expression.

Schlüsselwörter

Parkinson-Erkrankung, Neudefinition, klinische Diagnosekriterien

Key words

Parkinson’s disease, redefinition, clinical diagnostic criteria
 

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