Jellinger KA | ||||
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Lewy-Körper-Demenz und Parkinson-Demenz: aktuelle Konzepte und Perspektiven // Dementia with Lewy bodies and Parkinson disease-dementia: current concepts and perspectives Journal für Neurologie, Neurochirurgie und Psychiatrie 2021; 22 (4): 167-174 Volltext (PDF) Summary Praxisrelevanz Abbildungen
Keywords: Diagnose, diagnostische Biomarker, klinische Symptomatik, Lewy-Körper-Demenz, Morphologie, Neurologie, Parkinson-Demenz, Synukleinopathie, Therapie, Therapieoption, clinical aspects, dementia with Lewy bodies, diagnostic biomarkers, morphology, Parkinson disease-dementia, synucleinopathies, treatment options Dementia with Lewy bodies (DLB) and Parkinson disease-dementia (PDD) are two closely related major neurocognitive disorders with Lewy bodies of unknown etiology, showing notable overlap of clinical, genetic, neurochemical, pathophysiological and morphological features. According to international consensus, their diagnosis is based on an arbitrary distinction between the time of onset of motor and cognitive symptoms; in DLB cognitive deterioration precedes parkinsonism for at least 12 months, while in PDD, dementia develops in the course of well-established parkinsonism (the one-year rule). Clinically, both syndromes show disorders of cognitive and executive functions/dementia, parkinsonism, visual hallucinations and fluctuating attention, REM sleep behavioral disorder, parkinsonism and memory disorders being more severe in PDD. Both are morphologically characterized by widespread α-synuclein/Lewy body lesions associated with β-amyloid and tau pathologies, the latter being responsible for earlier onset and more severe cognitive deficits in DLB. Clinico-pathological and animal model studies have provided some insight into the pathophysiology of both DLB/PDD showing some differences in the molecular biology and spreading pattern of α-synuclein strains leading to the heterogeneity of both syndromes that are currently considered either as the same illness in the spectrum of synucleinopathies, as phenotypes of a continuum of disorders or as two separate neurodegenerative disorders. Despite validated criteria the clinical diagnosis is difficult, but the diagnostic accuracy could be increased by use of biological markers. To date, no disease-modifying therapies are available. Limited treatment options include cholesterinase inhibitors, levodopa, atypical antipsychotics, and non-pharmacological methods, however with limited success. Active and passive anti-synuclein or tau immunotherapies may open new aspects for future treatments.
Kurzfassung: Lewy-Körper-Demenz (DLB) und Parkinson-Demenz (PDD) sind zwei verwandte kognitive Störungen ungeklärter Ätiologie aus dem Spektrum der Synukleinopathien, die zahlreiche gemeinsame klinische, genetische,
neurochemische, pathophysiologische und morphologische Merkmale aufweisen. Ihre Diagnose beruht nach internationalem
Konsens auf dem Zeitintervall zwischen dem Beginn motorischer und kognitiver Symptome: Bei DLB gehen letztere der Parkinsonsymptomatik mindestens 12 Monate voraus, während sich bei PDD die Demenz erst im Verlauf der ausgeprägten Parkinsonsymptome entwickelt (sog. Ein-Jahresregel). |