Download PDF
PSYCH up2date 2022; 16(06): 473-489
DOI: 10.1055/a-1851-3700
Organische psychische Störungen

Neuropsychiatrische Störungen bei Chorea Huntington

Detlef Wietelmann
› Further Information
Also available at
    Permissions and Reprints All articles of this category

Die Chorea Huntington ist die häufigste neurodegenerative Erkrankung, die monogenetisch autosomal dominant vererbt wird und deren Diagnose anhand der CAG-Trinukleotidzahl meistens eindeutig präklinisch gestellt werden kann. Bislang ist der Verlauf trotz sehr spannender Therapieansätze, die eine Reduktion des Genprodukts anstreben, nicht aufzuhalten. Antidepressiva und hochpotente Neuroleptika sind die am häufigsten eingesetzten Medikamente.
Kernaussagen

  • Neuropsychiatrische Störungen bei der HD sind sehr häufig, treten im gesamten Krankheitsverlauf auf, betreffen viele Domänen (z.B. Kognition, Stimmung, psychotische Symptome und Verhaltensauffälligkeiten) und beginnen oft Jahre vor den ersten motorischen Symptomen.

  • Neuropsychiatrische Syndrome lassen sich klinisch nicht ausreichend sicher von den primären psychiatrischen Störungen unterscheiden.

  • Sie treten gehäuft komorbide auf und sind mit einer geringeren Funktionalität der Betroffenen und höheren Belastung von Angehörigen und Pflegenden assoziiert.

  • Suizidalität ist im Vergleich zur Allgemeinbevölkerung deutlich häufiger und sollte im gesamten Krankheitsverlauf und besonders zeitnah zur Diagnosestellung exploriert werden.

  • Neuropsychiatrische Syndrome können pharmakogen, z.B. durch die gegen die Chorea eingesetzten Medikamente, ausgelöst werden.

  • Sehr typisch sind die fehlende Krankheitseinsicht und geringe Absprachefähigkeit, die den Umgang mit den Betroffenen erheblich erschweren.

  • Die psychopharmakologische Behandlung entspricht aufgrund der unzureichenden Datenlage den Empfehlungen bei primären psychiatrischen Störungen.

  • Die Indikation und Dosis dopamin-antagonistischer Medikamente, z.B. bei Psychose oder ausgeprägter Irritabilität, sollte regelmäßig überprüft werden, da im Krankheitsverlauf die Wahrscheinlichkeit extrapyramidalmotorischer Nebenwirkungen zunimmt und spätere Stadien durch ein zunehmend akinetisch-rigides Syndrom mit Immobilität gekennzeichnet sind.

  • Nichtpharmakologische Interventionen werden durch Experten empfohlen, aber bislang nicht ausreichend durch Daten gestützt.

Schlüsselwörter

Motorik - Neuropsychiatrie - Komorbidität - Suizidalität - Anosognosie - Psychopharmakotherapie


Publication History

Article published online:
11 November 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • Literatur

  • 1 Frank W, Lindenberg KS, Mühlbäck A. et al. Krankheitsmodifizierende Therapieansätze bei der Huntington-Krankheit, Blicke zurück und Blicke voraus. Nervenarzt 2022; 93: 179-190
    CrossrefPubMedSearch in Google Scholar
  • 2 Dorsey ER, Beck CA, Darwin K. et al. Natural history of Huntington disease. JAMA Neurol 2013; 70: 1520-1530
    CrossrefPubMedSearch in Google Scholar
  • 3 Wexler A, Wild EJ, Tabrizi SJ. George Huntington: a legacy of inquiry, empathy and hope. Brain 2016; 139: 2326-2333
    CrossrefPubMedSearch in Google Scholar
  • 4 Cui S-S, Ren R-J, Wang Y. et al. Tics as an initial manifestation of juvenile Huntington’s disease: case report and literature review. BMC Neurology 2017; 17: 152
    CrossrefPubMedSearch in Google Scholar
  • 5 Zarotti N, Dale M, Eccles F. et al. Psychological interventions for people with Huntington’s disease: A call to arms. J Huntingtons Dis 2020; 9: 231-243
    CrossrefPubMedSearch in Google Scholar
  • 6 Rawlins MD, Wexler NS, Wexler AR. et al. The prevalence of Huntington’s disease. Neuroepidemiol 2016; 46: 144-153
    CrossrefPubMedSearch in Google Scholar
  • 7 Ohlmeier C, Saum K-U, Galetzka W. et al. Epidemiology and health care utilization of patients suffering from Huntington’s disease in Germany: real world evidence based on German claims data. BMC Neurology 2019; 19: 318
    CrossrefPubMedSearch in Google Scholar
  • 8 Solberg OK, Filkuková P, Frich JC. et al. Age at death and causes of death in patients with Huntington disease in Norway in 1986–2015. J Huntingtons Dis 2018; 7: 77-86
    CrossrefPubMedSearch in Google Scholar
  • 9 Saft C. et al. S2k-Leitlinie Chorea/Morbus Huntington. 2017. In: Deutsche Gesellschaft für Neurologie, Hrsg. Leitlinien für Diagnostik und Therapie in der Neurologie. Accessed August 01, 2022 at: www.dgn.org/leitlinien
  • 10 Fusilli C, Migliore S, Mazza T. et al. Biological and clinical manifestations of juvenile Huntington’s disease: a retrospective analysis. Lancet Neurol 2018; 17: 986-993
    CrossrefPubMedSearch in Google Scholar
  • 11 McAllister B, Gusella JF, Landwehrmeyer GB. et al. Timing and impact of psychiatric, cognitive, and motor abnormalities in Huntington disease. Neurol 2021; 96
    CrossrefPubMedSearch in Google Scholar
  • 12 Julayanont P, Heilmann KM, McFarland NR. et al. Early-motor phenotype relates to neuropsychiatric and cognitve disorders in Huntington’s disease. Mov Disord 2020; 35: 781-788
    CrossrefSearch in Google Scholar
  • 13 Bachoud-Lévi AC, Ferreira J, Massart R. et al. International Guidelines for the Treatment of Huntington’s disease. Front Neurol 2019; 10: 710
    CrossrefPubMedSearch in Google Scholar
  • 14 Teixeira AL, Cruz de Souza L, Pessoa Rocha N. et al. Revisiting the neuropsychiatry of Huntington’s disease. Dement Neuropsychol 2016; 10: 261-266
    CrossrefPubMedSearch in Google Scholar
  • 15 Coppen EM, Roos RAC. Current pharmacological approaches to reduce Chorea in Huntington’s disease. Drugs 2017; 77: 29-46
    CrossrefPubMedSearch in Google Scholar
  • 16 Orth M, Handley OJ, Schwenke C. et al. Observing Huntington’s Disease: the European Huntington’s Disease Network´s REGISTRY. PLOS Curr 2010; 2: RRN1184
    CrossrefPubMedSearch in Google Scholar
  • 17 Thompson JC, Harris J, Sollom AC. et al. Longitudinal evaluation of neuropsychiatric symptoms in Huntington’s disease. J Neuropsychiatry Clin Neurosci 2012; 24: 53-60
    CrossrefPubMedSearch in Google Scholar
  • 18 Nguyen HHP, Weydt P. Huntington Erkrankung. Medizinische genetik 2018; 30: 246-251
    CrossrefSearch in Google Scholar
  • 19 Julien CL, Thompson JC, Wild S. et al. Psychiatric disorders in preclinical Huntington’s disease. J Neurol Neurosurg Psychiatry 2007; 78: 939-943
    CrossrefPubMedSearch in Google Scholar
  • 20 Jensen P, Fenger K, Bolwig TG. et al. Crime in Huntington’s disease: a study of registered offences among patients, relatives, and controls. J Neurol Neurosurg Psychiatry 1998; 65: 467-471
    CrossrefPubMedSearch in Google Scholar
  • 21 Exuzides A, Matos JE, Patel AM. et al. Understanding the burden associated with Huntingtonʼs disease in manifest patients and care partners – Comparing to Parkinsonʼs Disease and the general population. Brain Sci 2022; 12: 161
    CrossrefPubMedSearch in Google Scholar
  • 22 European Huntington’s disease Network. Accessed September 01, 2022 at: www.ehdn.org
  • 23 Tampi RR, Weber M, Masterson GA. et al. Early warnings: neuropsychiatric manifestations of Huntington’s disease. Psychiatric Times 2021; 38: 25-27
    Search in Google Scholar
  • 24 Adrissi J, Nadkarni NA, Gausche E. et al. Electroconvulsive therapy (ECT) for refractory psychiatric symptoms in Huntington’s disease: A case series and review of the literature. J Huntingtons Dis 2019; 8: 291-300
    CrossrefPubMedSearch in Google Scholar
  • 25 Mowafi W, Millard J. Electroconvulsive therapy for severe depression, psychosis and chorea in a patient with Huntington’s disease: case report and review of the literature. B J Psych Bull 2021; 45: 97-104
    CrossrefSearch in Google Scholar
  • 26 Anderson KE, van Duijn E, Craufurd D. et al. Clinical Management of neuropsychiatric symptoms of Huntington Disease: Expert-based consensus guidelines on agitation, anxiety, apathy, psychosis, and sleep disorders. J Huntingtons Dis 2018; 7: 355-366
    CrossrefPubMedSearch in Google Scholar
  • 27 Paulsen JS. Cognitive Impairment in Huntington’s Disease: Diagnosis and Treatment. Curr Neurol Neurosci Rep 2011; 11: 474-483
    CrossrefPubMedSearch in Google Scholar
  • 28 Zhang Y, Zhou J, Gehl CR. et al. Mild cognitive impairment as an early landmark in Huntington’s disease. Front Neurol 2021;
    CrossrefPubMedSearch in Google Scholar
  • 29 Peavy GM, Jacobson MW, Goldstein JL. et al. Cognitive and functional decline in Huntington’s disease: Dementia criteria revisited. Mov Disord 2010; 25: 1163-1169
    CrossrefPubMedSearch in Google Scholar
  • 30 Davis MY, Keene CD, Jayadev S. et al. The co-occurence of Alzheimer´s disease and Huntington’s disease: A neuropathological study of 15 elderly Huntington’s disease subjects. J Huntingtons Dis 2014; 3: 209-217
    CrossrefPubMedSearch in Google Scholar
  • 31 Julayanont P, McFarland NR, Heilmann KM. Mild cognitive impairment and dementia in motor manifest Huntington’s disease: Classification and prevalence. J Neurol Sci 2020; 408: 116523
    CrossrefPubMedSearch in Google Scholar
  • 32 Dorsey ER, Beck CA, Darwin K. et al. Natural history of Huntington’s disease. JAMA Neurol 2013; 70: 1520-1530
    CrossrefPubMedSearch in Google Scholar
  • 33 Stanley K, Whitfield T, Kuchenbaecker K. et al. Rate of cognitive decline in Alzheimer´s disease stratified by age. J Alzheimers Dis 2019; 69: 1153-1160
    CrossrefPubMedSearch in Google Scholar
  • 34 Vattakatuchery JJ, Kurien R. Acetylcholinesterase inhibitors in cognitve impairment in Huntington’s disease: A brief review. World J Psychiatr 2013; 22: 62-64
    CrossrefPubMedSearch in Google Scholar
  • 35 Li Y, Hai S, Zhou Y. et al. Cholinesterase inhibitors for rarer dementias associated with neurological conditions (review). Cochrane Database Syst Rev 2015; 3: CD009444
    CrossrefPubMedSearch in Google Scholar
  • 36 EppingEAPaulsen JS. Depression in early stages of Huntington’s disease. Neurodegener Dis Manag 2011; 1: 407-414
    CrossrefPubMedSearch in Google Scholar
  • 37 Julien CL, Thompson JC, Wild S. Psychiatric disorders in preclinical Huntington’s disease. Neurol Neurosurg Psychiatry 2007; 78: 939-943
    CrossrefPubMedSearch in Google Scholar
  • 38 Scheuing L, Chiu C-T, Liao H-M. et al. Preclinical and clinical investigations of mood stabilizers for Huntington’s disease: What have we learned?. Int J Biol Sci 2014; 10: 1024-1038
    CrossrefPubMedSearch in Google Scholar
  • 39 Greenberg M, Paddu N, Bhivandkar S. et al. Treating depression and suicidality in Huntington’s disease. Psychiatric Ann 2020; 50
    CrossrefSearch in Google Scholar
  • 40 Honrath P, Dogan I, Wudarczyk O. et al. Risk factors of suicidal ideation in Huntington’s disease: literature review and data from enroll-HD. J Neurol 2018; 265: 2548-2561
    CrossrefPubMedSearch in Google Scholar
  • 41 Van Duijn E, Fernandes AR, Abreu D. et al. Incidence of completed suicide and suicide attempts in a global prospective study of Huntington’s disease. BJ Psych Open 2021; 31: e158
    CrossrefPubMedSearch in Google Scholar
  • 42 World Health Organization (WHO). Suicide worldwide in 2019, global health estimates. Geneva: WHO; 2019. Accessed August 02, 2022 at: https://www.who.int/publications/i/item/9789240026643
  • 43 Kachian ZR, Cohen-Zimerman S, Bega D. et al. Suicidal ideation and behaviour in Huntington’s disease: Systematic review and recommendations. J Affect Disord 2019; 250: 319-329
    CrossrefPubMedSearch in Google Scholar
  • 44 Paoli RA, Botturi A, Ciammola A. et al. Neuropsychiatric burden in Huntington’s disease. Brain Sci 2017; 7: 67
    CrossrefPubMedSearch in Google Scholar
  • 45 McGarry A, McDermott MP, Kieburtz K. et al. Risk factors for suicidality in Huntington disease An analysis of the 2CARE clinical trial. Neurology 2019; 92: e1643-e1651
    CrossrefPubMedSearch in Google Scholar
  • 46 Mulin E, Leone E, Dujardin K. et al. Diagnostic criteria for apathy in clinical practice. Int J Geriatr Psychiatry 2011; 26: 158-165
    CrossrefPubMedSearch in Google Scholar
  • 47 Gelderblom H, Wüstenberg T, McLean T. et al. Bupropion for the treatment of apathy in Huntington’s disease: A multicenter, randomised, double-blind, placebocontrolled, prospective crossover trial. PLoS ONE 2017; 12: e0173872
    CrossrefPubMedSearch in Google Scholar
  • 48 Karagas NE, Pessoa Rocha N, Stimming EF. Irritability in Huntington’s disease. J Huntingtons Dis 2020; 9: 107-113
    CrossrefPubMedSearch in Google Scholar
  • 49 Dale M, van Duijn E. Anxiety in Huntington’s disease. J Neuropsych Clin Neurosci 2015; 27: 262-271
    CrossrefPubMedSearch in Google Scholar
  • 50 Oosterloo M, Craufurd D, Nijsten H. et al. Obsessive-compulsive and perseverative behaviors in Huntington’s Disease. J Huntingtons Dis 2019; 8: 1-7
    CrossrefPubMedSearch in Google Scholar
  • 51 Anderson KE, Gehl CR, Marder KS. et al. Comorbidities of obsessive and compulsive symptoms in Huntington disease. Nerv Ment Dis 2010; 198: 334-338
    CrossrefPubMedSearch in Google Scholar
  • 52 Roman OC, Stovall J, Claassen DO. Perseveration and suicide in Huntington’s disease. J Huntingtons Dis 2018; 7: 185-187
    CrossrefPubMedSearch in Google Scholar
  • 53 Rasgon A, Lee WH, Leibu E. et al. Neural correlates of affective and non-affective cognition in obsessive compulsive disorder: A meta-analysis of functional imaging studies. Eur Psychiatry 2017; 46: 25-32
    CrossrefPubMedSearch in Google Scholar
  • 54 Hazari N, Narayanaswamy JC, Venkatasubramanian G. Neuroimaging findings in obsessive-compulsive disorder: A narrative review to elucidate neurobiological underpinnings. Indian J Psychiatry 2019; 61: S9-S29
    CrossrefPubMedSearch in Google Scholar
  • 55 Rocha NP, Mwnagi B, Gutierrez Candano CA. et al. The clinical picture of psychosis in manifest Huntington’s disease: A comprehensive analysis of the Enroll-HD Database. Front Neurol 2018; 9: 930
    CrossrefPubMedSearch in Google Scholar
  • 56 Ding J, Gadit AM. Psychosis with Huntington’s disease: role of antipsychotic medications. BMJ Case Rep 2014: 2013– 2015;
    CrossrefPubMedSearch in Google Scholar
  • 57 Majothi N, Lee HY, Nagarajan P. et al. Treatment of psychosis in Huntington’s disease with clozapine. Prog Neurol Psychiatry 2020; 24: 14-16
    CrossrefSearch in Google Scholar
  • 58 Wibawa P, Zombor R, Dragovic M. et al. Anosognosia is associated with greater caregiver burden and poorer executive function in Huntington disease. J Geriatric Psychiatry Neurol 2020; 33: 52-58
    CrossrefPubMedSearch in Google Scholar
  • 59 Sitek EJ, Thompson JC, Craufurd D. et al. Unawareness of deficits in Huntington’s disease. Jf Huntingtons Dis 2014; 3: 125-135
    CrossrefPubMedSearch in Google Scholar
  • 60 Duff K, Paulsen JS, Beglinger LJ. et al. „Frontal“ behaviors before the diagnosis of Huntington’s disease and their relationship to markers of disease progression: Evidence of early lack of awareness. J Neuropsychiatry Clin Neurosci 2010; 22: 196-207
    CrossrefPubMedSearch in Google Scholar
  • 61 Herzog-Krzywoszanska R, Krzywoszanski L. Sleep disorders in Huntington’s disease. Front Psychiatry 2019; 10: 221
    CrossrefPubMedSearch in Google Scholar
  • 62 Voysey Z, Fazal SV, Lazar AS. et al. The sleep and circadian problems of Huntington’s disease: when, why and their importance. J Neurol 2021; 268: 2275-2283
    CrossrefPubMedSearch in Google Scholar