Objective: To describe the characteristics and phenomenology of psychotic symptoms in a Spanish cohort of HD patients.

Background: Neuropsychiatric features are characteristic symptoms in Huntington’s disease (HD). Compelling evidence proved high prevalence of alterations on mood and affect. However, little is known about psychotic symptoms in HD along the course of the disease from premanifested to advanced stages.

Methods: From the Spanish Registry cohort, genetically positive patients with psychiatric, cognitive and motor assessment were included. We analyzed demographic, genetic, clinical data and family history. Cognitive, motor and functionality were assessed with the Unified Huntington Disease Rating Scale (UHDRS), psychiatric disturbances with the Problem Behavior Assessment Scale (PBA-S). Those patients with psychosis were interviewed by a psychiatrist and the phenomenology of the psychosis was recorded. Individuals were divided in premanifested (UHDRS <5) and manifested (UHDRS≥5).

Results: 264 patients were included (46.2% males; mean age of 45.2±12.5 years; mean CAG 44.1±4.7; 22% premanifested). Psychotic symptoms were present in 12.1% of the sample. Delusions were present in the 10.2% with paranoid delusion being the most frequent followed by jealousy. Hallucinations were present in 4.5% being the most frequent the visual hallucinations. The 28% of the psychotic patients had family history of HD and psychosis. UHDRS cognitive score (p=0.05) and TFC (p=0.01) were more impaired in patients with psychotic symptoms. The psychotic group had higher scores in other neuropsychiatric symptoms as depression (p=0.003) apathy (p=0.0003), irritability (p=0.0005) or executive dysfunction (p=0.000). We found lineal correlations of psychosis and apathy (r=0.5, p=0.016), depression (r=0.42, p=0.004) and executive dysfunction (r=0.47, p=0.006). We could identify two types of psychotic individuals. Patients with a younger disease onset and higher number of CAG had a schizophrenia-like phenotype with psychosis preceding motor and cognitive symptoms. However, patients with older disease onset and less number of CAG developed motor and cognitive decline before psychosis symptoms.

Conclusions: Psychotic symptoms are infrequent in HD and can be present before the disease motor onset. There is a family aggregation of psychosis in HD. Psychosis is associated with other neuropsychiatric symptoms as apathy, depression or executive dysfunction and confers a worse functional capacity.

« Back to 2016 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/psychosis-in-huntingtons-disease/