Demenzen mit Lewy-Körperchen

C. J. G. Lang; M. Bergmann

doi:10.1055/s-2002-33858

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Fortschr Neurol Psychiatr 2002; 70(9): 476-494
DOI: 10.1055/s-2002-33858

Originalarbeit

Demenzen mit Lewy-Körperchen

Dementias with Lewy BodiesC. J. G. Lang¹ , M. Bergmann²

¹Neurologische Klinik der Friedrich-Alexander-Universität Erlangen (Direktor: Prof. Dr. B. Neundörfer)
²Institut für klinische Neuropathologie, Zentralkrankenhaus Bremen-Ost (Leiter: PD Dr. M. Bergmann)

Die Klinik für Psychiatrie und Psychotherapie des Bezirkskrankenhauses Bayreuth (Chefarzt: Prof. Dr. M. Wolfersdorf) übermittelte freundlicherweise klinische Daten und Computertomogramme. Herrn Prof. Dr. K. H. Plate und Dr. T. Acker von der Neuropathologischen Abteilung des Pathologischen Instituts und Herrn Dr. S. Probst-Cousin von der Neurologischen Klinik der Friedrich-Alexander-Universität Erlangen danken wir für überlassene Hirnschnitte, Herrn Prof. Dr. T. Kuwert und Dr. A. Schwarz von der Nuklearmedizinischen Klinik der Friedrich-Alexander-Universität Erlangen für SPECT- und Herrn Prof. Dr. P. Bartenstein von der Johannes-Gutenberg-Universität Mainz für PET-Abbildungen.

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Publication Date:
05 September 2002 (online)

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Zusammenfassung

Demenzen mit Lewy-Körperchen sind eine nicht seltene Ursache kognitiver und motorischer Beeinträchtigungen im höheren Lebensalter. Sie müssen je nach Ausbreitung und Begleitpathologie in diffuse Lewy-Körperchen-Erkrankung bzw. Demenz mit Lewy-Körperchen, Morbus Parkinson mit begleitender Alzheimer-Pathologie und die Lewy-Körperchen-Variante des Morbus Alzheimer differenziert werden. Als bisher zuverlässigste diagnostische Merkmale der Demenzen mit Lewy-Körperchen haben sich fluktuierende kognitive und Bewusstseinsbeeinträchtigungen, visuelle Störungen (Halluzinationen, visuell-perzeptive und visuokonstruktive Auffälligkeiten) und frühzeitig einsetzende extrapyramidalmotorische Symptome vom hypokinetisch-rigiden Typ mit einer Neigung zu häufigen Stürzen erwiesen. Maßgeblich sind derzeit die Konsensus-Kriterien nach McKeith u. Mitarb. [48]. Zusätzlich scheinen nach jüngsten Untersuchungen in erster Linie morphologisch und funktionell bildgebende Verfahren (SPECT, PET), in zweiter Linie Liquoruntersuchungen (Homovanillinsäure) einen Beitrag leisten zu können. Selbst unter Ausschöpfung aller Möglichkeiten ist aber eine diagnostische Treffsicherheit von 85 % derzeit kaum zu erzielen. Die Diagnose der Lewy-Körperchen-Variante des Morbus Alzheimer ist, wie an einem Fallbeispiel gezeigt wird, ante mortem oft nicht verlässlich möglich. Klinisch ist diese Gruppe von Demenzen vor allem dadurch bedeutsam, dass eine erhebliche Neuroleptikaüberempfindlichkeit besteht und moderne zentralcholinerg wirksame Medikamente eine viel versprechende Therapieform darstellen.

Abstract

Dementias with Lewy bodies are no rare cause of cognitive and motor impairments in old age. Neuropathologically, they must be distinguished into diffuse Lewy body disease resp. dementia with Lewy bodies, Parkinson's disease with concomitant Alzheimer's pathology, and the Lewy body variant of Alzheimer's disease according to extent and concomitant pathology. The most reliable diagnostic features of dementia with Lewy bodies are fluctuating disturbances of cognition and consciousness, visual disorders (hallucinations, visuoperceptive and visuoconstructive impairments), and early extrapyramidal signs of the hypokinetic-rigid type with a propensity to frequent falls. The pertinent diagnostic criteria are the consensus criteria according to McKeith et al. [48]. Additional contributions are to be expected by functional neuroimaging (SPECT, PET) and CSF examination (homovanillic acid). However, even assuming the most favorable conditions a diagnostic accuracy of 85 % is presently hard to achieve. Particularly, as is demonstrated using a case example, reliable antemortem diagnosis of Lewy body variant of Alzheimer's disease is hardly possible. Clinically, this group of diseases is important, since increased neuroleptic sensitivity must be taken into account and modern central cholinergic agents seem to be a promising therapeutic option.

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Prof. Dr. med. Dipl.-Psych. C. J. G. Lang

Neurologische Universitätsklinik

Schwabachanlage 6

91054 Erlangen

Email: christoph.lang@neuro.imed.uni-erlangen.de