Frontale Dysfunktion bei Chorea Huntington

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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