Maladie de Huntington : données actuelles sur la prise en charge thérapeutique des troubles psychiatriques

Abstract

ObjectiveHuntington's disease is a neurodegenerative genetic, autosomal dominant disease. George Huntington describes this trouble in 1872. In 1993, scientist founded mutation on IT15 gene on 4th chromosome. Prevalence in France is 1/10 000 without sex predominance or race. The main symptoms are: choreic movements, axial impairment, psychiatric disorders and the presence of cognitive impairment. The diagnostic is clinic, genetic and radiologic. Despite the absence of curative treatment, symptomatic care supports deficit symptoms of the pathology, thus improves the quality of life of patients. In the context of a comprehensive and optimal therapy, health professionals provide recommendations for guidelines like Huntington's disease national reference center in August 2015. We will define diagnostic and clinical aspects of Huntington's disease, and then we will focus our analysis on the psychiatric aspect. Material and methodFirst, we will illustrate management provided by our teams. Patient A had positive diagnostic of Huntington's disease and personal psychiatric antecedent of depression. She was hospitalized in neurological unit for a panic attack in August 2015. They give tetrabenazine and to prevent depressive effect we prescribed antidepressant first (serotonin–norepinephrine reuptake inhibitor), then antipsychotic (risperidone, then haloperidol) and benzodiazepine. Patient B had Huntington's disease in family and personal diagnostic of Huntington's disease and personal psychiatric antecedent of depression. She was admitted in psychiatric unit for psychotic symptom. She received antipsychotic (risperidone) and antidepressant (venlafaxine) with benzodiazepine. Both of them had clinical improvement. Patient A, went to reeducation care, patient B went home with nurse. When we analyzed literature on the psychiatric therapeutic in Huntington's disease with review, we found that apathy is the most psychiatric symptom in Huntington's disease (50–80% Inserm). Benzodiazepine had good result for anxiety. We must search and treat psychiatric disorder like depression and mood disorder. Irritability and anxiety are symptoms of mood disorder and can be treated with antidepressive and mood stabilizer. Suicide is an emergency because of the mortality rate. The guideline is the same of suicide in psychiatric emergency. Antipsychotics are not avoided and used for choreic and psychotic symptoms (delirium, hallucination…) but second generation antipsychotics are generally preferable because of the lesser presence of adverse event. ResultsThe use of selective serotonin reuptake inhibitors (SSRIs) and serotonin–norepinephrine reuptake inhibitor (SNRI) for moods sign are the most frequently used therapy reference. Electroconvulsive therapy (ECT) and mood stabilizer have similar results to those obtained in mood disorders without neurodegenerative pathology. Antipsychotic like risperidone or olanzapine are good requirements. Guidelines of Huntington's disease national reference center and literature give these therapeutic choices. With our two patients, we can see clinical improvement and the main objective was return home. Literature explain important role of psychotherapy, social, diet and hygiene habits. We must have global care like other psychiatric patient and more because of neurological reach. ConclusionThe implementation of classical specific therapeutic in psychiatry improves these symptoms and optimizes the quality of life for our patients. A comprehensive and multidisciplinary support involving medical and paramedical rehabilitation is a necessity to take into account.