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Die diffuse Lewy-Körperchen-Erkrankung oder besser das Parkinson-Syndrom mit früher Demenz — eine neue Krankheitsentität?

  • R. Benecke
Conference paper
  • 67 Downloads

Zusammenfassung

Während noch vor wenigen Jahren die klinische Differenzialdiagnose von degenerativen Erkrankungen mit Parkinson-Syndromen und Demenzen iibersichtlich erschien, sind in den letzten Jahren eine Reihe von kliniko-pathologischen Studien veröffentlicht worden, die auf eine wesentlich größere Vielfalt von degenerativen ZNS-Erkrankungen mit diesen klinischen Leitsymptomen hinweisen. Die meisten Neurowissenschaftler akzeptieren nach wie vor den Morbus Parkinson als Kränkheitsentitat, obwohl die Identifikation verschiedener genetischer Aberrationen bereits auf die klinische Heterogenität dieser Erkrankung hinweisen. Bis jetzt sind spezifische Mutationen in 3 verschiedenen Genen [22, 30, 45] und fünf weitere Genloci beschrieben worden [7, 9, 11, 48, 49], die kausal mit familiären Parkinson-Syndromen verbunden sind. Es ist offensichtlich, dass die genetisch determinierten Fälle mit Parkinson-Syndrom nicht ohne weiteres unter der Rubrik Morbus Parkinson eingruppiert werden können, da eine Vielzahl von betroffenen Familienmitgliedern ungewöhnliche klinische und/oder neuropathologische Besonderheiten zeigen wie frühes Manifestationsalter oder Fehlen von intraneuralen Lewy-Körperchen in der Substantia nigra. Es ist noch völlig offen, ob letztendlich für alle Patienten mit Morbus Parkinson eine genetische Grundlage gefunden wird und wir die entsprechenden Erkrankungen dann als PARK1 bis -X bezeichnen oder ob eine Restgruppe von Patienten mit typischem Morbus Parkinson verbleibt, die vielleicht neben einer multigenetisch bedingten Suszeptibilität eine Reihe von toxischen Umwelteinflüssen im Sinne einer multifaktoriellen Genese erkennen lassen. In einem kürzlich verfassten Artikel war bei der jetzt schon erkennbaren Heterogenität der pathophysiologischen Grundlagen vorgeschlagen worden, den Begriff Morbus Parkinson nicht mehr zu verwenden, sondern besser von Dopa-responsiven Parkinson-Syndromen zu sprechen [3]. Innerhalb der Dopa-responsiven Parkinson-Syndrome könnten zum jetzigen Zeitpunkt idiopathische Parkinson-Syndrome (iPS), für die bisher keine genetische Grundlage zu beschreiben ist, von den genetisch determinierten Parkinson-Syndromen unterschieden werden.

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© Springer-Verlag Berlin Heidelberg 2003

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  • R. Benecke

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