Frontale Dysfunktion bei Chorea Huntington

 

 Buy Article Permissions and Reprints

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.

Literatur

• 1 Hofmann N. Understanding the neuropsychiatric symptoms of Huntington's disease.  J Neurosci Nurs. 1999;  31 309-313
  PubMedGoogle Scholar
• 2 Caine E D, Hunt R D, Weingartner H, Ebert M H. Huntington's dementia. Clinical and neuropsychological features.  Arch Gen Psychiatry. 1978;  35 377-384
  PubMedGoogle Scholar
• 3 Foroud T, Siemers E, Kleindorfer D. et al . Cognitive scores in carriers of Huntington's disease gene compared to noncarriers.  Ann Neurol. 1995;  37 657-664
  CrossrefPubMedGoogle Scholar
• 4 Dose M, Lange H W. The benzamide tiapride: treatment of extrapyramidal motor and other clinical syndroms.  Pharmacopsychiatry. 2000;  33 19-27
  Article in Thieme ConnectPubMedGoogle Scholar
• 5 van Vugt J P, Siesling S, Vergeer M, van der Velde E A, Roos R A. Clozapine versus placebo in Huntington's disease: a double blind randomised comparative study.  J Neurol Neurosurg Psychiatry. 1997;  63 35-39
  PubMedGoogle Scholar
• 6 Leroi I, Michalon M. Treatment of the psychiatric manifestations of Huntington's disease: a review of the literature.  Can J Psychiatry. 1998;  43 933-940
  PubMedGoogle Scholar
• 7 Madhusoodanan S, Brenner R, Moise D, Sindagi J, Brafman I. Psychiatric and neuropsychological abnormalities in Huntington's disease: a case study.  Ann Clin Psychiatry. 1998;  10 117-120
  CrossrefPubMedGoogle Scholar
• 8 Aylward E H, Codori A M, Rosenblatt A. et al . Rate of caudate atrophy in presymptomatic and symptomatic stages of Huntington's disease.  Mov Disord. 2000;  15 552-560
  CrossrefPubMedGoogle Scholar
• 9 Aylward E H, Anderson N B, Bylsma F W. et al . Frontal lobe volume in patients with Huntington's disease.  Neurology. 1998;  50 252-258
  CrossrefPubMedGoogle Scholar
• 10 Mendez M F. Huntington's disease: update and review of neuropsychiatric aspects.  Int J Psychiatry Med. 1994;  24 189-208
  PubMedGoogle Scholar
• 11 Kirkwood S C, Siemers E, Stout J C. et al . Longitudinal cognitive and motor changes among presymptomatic Huntington disease gene carriers.  Arch Neurol. 1999;  56 563-568
  CrossrefPubMedGoogle Scholar
• 12 Walker E, Lewis N, Loewy R, Palyo S. Motor dysfunction and risk for schizophrenia.  Dev Psychopathol. 1999;  11 509-523
  CrossrefPubMedGoogle Scholar
• 13 Lawrence A D, Hodges J R, Rosser A E. et al . Evidence for specific cognitive deficits in preclinical Huntington's disease.  Brain. 1998;  121 1329-1341
  CrossrefPubMedGoogle Scholar
• 14 Manschreck T C, Maher B A, Candela S F, Redmond D, Yurgelun-Todd D, Tsuang M. Impaired verbal memory is associated with impaired motor performance in schizophrenia: relationship to brain structure.  Schizophr Res. 2000;  43 21-32
  CrossrefPubMedGoogle Scholar
• 15 Sprengelmeyer R, Lange H, Homberg V. The pattern of attentional deficits in Huntington's disease.  Brain. 1995;  118 145-152
  PubMedGoogle Scholar
• 16 Jason G W, Suchowersky O, Pajurkova E M. et al . Cognitive manifestations of Huntington disease in relation to genetic structure and clinical onset.  Arch Neurol. 1997;  54 1081-1088
  PubMedGoogle Scholar
• 17 Hanes K R, Andrewes D G, Pantelis C, Chiu E. Subcortical dysfunction in schizophrenia: a comparison with Parkinson's disease and Huntington's disease.  Schizophr Res. 1996;  19 121-128
  CrossrefPubMedGoogle Scholar
• 18 Hanes K R, Andrewes D G, Pantelis C. Cognitive flexibility and complex integration in Parkinson's disease, Huntington's disease, and schizophrenia.  J Int Neuropsychol Soc. 1995;  1 545-553
  PubMedGoogle Scholar

Priv.-Doz. Dr. Dieter F. Braus

NMR-Forschung am Zentralinstitut für Seelische Gesundheit

Postfach 122120

68072 Mannheim

Email: dfbraus@zi-mannheim.de