Meilensteine im Verlauf des Idiopathischen Parkinson-Syndroms

Zusammenfassung

Die Parkinson-Krankheit ist eine progrediente neurodegenerative Erkrankung. Es gibt mehrere Skalen, die den Schweregrad der Erkrankung beschreiben. Klinisch führt immer noch die Einteilung nach Hoehn und Yahr, wohingegen sich von Seiten der Neuropathologie die Braak Stadien durchgesetzt haben. Mittels neuroanatomischer Untersuchungen, klinischer Verlaufsbeobachtungen und Zusatzdiagnostik wie SPECT und PET Untersuchungen des dopaminergen Systems haben sich einige Meilensteine der Erkrankung heraus kristallisiert. Initial ist der Untergang der dopaminergen Neurone relativ rasch progredient, wobei dies für die Patienten nicht offensichtlich ist, da sie klinische Symptome erst bei einem Untergang dopaminerger Neurone von mehr als 60% bemerken. Während dieses Zellunterganges in der substantia nigra, d. h. in der Prodromalphase, treten aber schon prämotorische Symptome wie Hyposmie, Obstipation, REM-Schlaf Verhaltensstörungen und Depression auf. Dies legt nahe, dass die Erkrankung nicht in der substantia nigra beginnt. Diese klinische Beobachtung wird durch die Arbeiten von Braak und anderen unterstützt, die erste Lewy-Körperchen und α-Synuklein Aggregation im Bulbus olfactorius und in der Medulla oblongata beschreiben. Im Rahmen einer aufsteigenden Pathologie lässt sich dann auch die Entstehung einer Demenz verstehen, die durch das Auftreten von cortikalen Lewy-Körper charakterisiert ist. Meilensteine wie visuelle Halluzinationen, Demenz, Heimunterbringung und häufige Stürze läuten die Endphase der Erkrankung ein und gehen dem Tod der Patienten 3–5 Jahre voran, unabhängig davon, ob die Patienten jung oder alt an Parkinson erkrankt sind. Dies wiederum lässt eine ­exponentielle Endphase der Erkrankung vermuten.

Abstract

Parkinson’s disease is a progressive neurodegenerative disorder. There are various ways to des­cribe the severity and progresion of the disease. For clincal purposes the Hoehn and Yahr staging is still the mostly used, whereas the Braak stag­ing is the best neuropathological staging. Using neuroanatomic analyses, clinical follow-up and technical investigations with SPECT and PET some typical milestones of Parkinson’s disease have been established. At onset the cell death of dopaminergic neurons is high which is not noted by the patients due to the fact that motor symp­toms do not occur until the patients have lost more than 60% of their nigral dopaminergic cells. In this prodromal phase non-motor symptoms such as hyposmia, REM sleep behaviour disorder, constipation and depression occur. This implies that the disease does not start in the substantia nigra which is supported by the findings of Braak and colleagues who detected Lewy pathology first in the olfactory bulb and medulla oblongata. Thereafter, Lewy bodies spread out to the cortex which explains dementia. Milestones such as visual hallucinations, dementia, admittance to a nursing home and frequent falls indicate that death will occur within the next 3–5 years independent of the age of the patient at the onset of Parkinson’s disease. This suggests that the end stage is related to an exponential cell death.

Schlüsselwörter

Parkinson-Syndrom - nicht motorische Symptome - Demenz - Braak

Keywords

Parkinson’s disease - non-motor symptoms - dementia - Braak staging

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