Psychiatr Prax 2003; 30(1): 33-36
DOI: 10.1055/s-2003-36624
Originalarbeit
© Georg Thieme Verlag Stuttgart · New York

Frontale Dysfunktion bei Chorea Huntington

Neuropsychologische und therapeutische AspekteFrontal Dysfunctions in Huntington's DiseaseNeurospsychology and TherapyErik  Weimer1 , Stefan  Ries2 , Heike  Tost1 , Dieter  F.  Braus1
  • 1NMR-Forschung am Zentralinstitut für Seelische Gesundheit, Universität Heidelberg
  • 2Gemeinschaftspraxis für Neurologie und Psychiatrie Reifschneider/Unsorg/Ries, Erbach/Odenwald
Further Information

Publication History

Publication Date:
13 January 2003 (online)

Zusammenfassung

Die Chorea Huntington (HD) ist eine autosomal-dominant vererbte und progressiv-degenerative Erkrankung des Zentralnervensystems. Klinisch imponieren vor allem einschießende, irreguläre (choreiforme) Hyperkinesen am ganzen Körper, Grimassieren und Artikulationsstörungen. Psychopathologische Veränderungen treten sehr häufig auf und gehen den typischen motorischen Symptomen teilweise um Jahre voraus. Wir schildern hier die Behandlung einer Patientin, bei welcher die psychopathologischen und kognitiven Auffälligkeiten ganz im Vordergrund standen, mit dem atypischen Antipsychotikum Amisulprid. Der klinische Verlauf wird beschrieben und die Frühdiagnostik unter besonderer Berücksichtigung neuropsychologischer Aspekte diskutiert.

Abstract

Huntington's disease is a hereditary and progressive disease of the central nervous system. Sudden and irregular hyperkinesias are prominent. Psychopathological findings are very frequent and often proceed the decline of motor functions for years. Almost all the patients develop a dementia in later stages of the illness. This case study shows the treatment of a patient with Huntington's disease, verified by genetic analysis, with the atypical antipsychotic drug amisulpride. In this case the psychiatric disturbances and cognitive impairments were the only signs of the disease. The treatment showed an impressive improvement. Neuropsychological aspects of the disease are discussed.

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Priv.-Doz. Dr. Dieter F. Braus

NMR-Forschung am Zentralinstitut für Seelische Gesundheit

Postfach 122120

68072 Mannheim

Email: dfbraus@zi-mannheim.de