Psychopathology in Verified Huntington’s Disease Gene Carriers

Abstract

Huntington's disease is characterized by motor, cognitive, and neuropsychiatric symptoms. This study reviews original research on psychopathology in Huntington's disease that uses standardized instruments in verified gene carriers. Frequently reported neuropsychiatric symptoms are depressed mood, anxiety, irritability, and apathy, with prevalences of 33% to 76%. Obsessive-compulsive symptoms and psychosis occur less often with prevalences of 10% to 52% and 3% to 11%, respectively. Available research provides little insight into the true prevalences of psychopathology in Huntington's disease due to small sample sizes, use of different methodologies, and lack of comparison groups. Future research requires larger cohorts stratified to disease stage, consistent methodologies, and adequate comparison groups.

Longitudinal changes in the fronto-striatal network are associated with executive dysfunction and behavioral dysregulation in Huntington's disease: 30 months IMAGE-HD data

“…A growing number of functional neuroimaging studies have characterized the neural correlates of motor and cognitive decline in HD both before and after clinical diagnosis (Wolf et al, 2007, Klöppel et al, 2009, Paulsen, 2009, Wolf et al, 2009, Wolf et al, 2011, Georgiou-Karistianis et al, 2013, Georgiou-Karistianis et al, 2013, Poudel et al, 2014; however, studies investigating changes in brain function linked with progression of neuropsychiatric symptoms remain sparse (Wolf and Kloppel, 2013). Psychiatric disturbance is often cited as the most debilitating feature of HD symptomatology with direct implications for functional capacity and quality of life (Hamilton et al, 2003, Duijn et al, 2007. Establishing the underlying functional brain changes associated with the development of psychiatric symptoms is of great importance in the context of identifying target brain regions with functional relevance that could be used for drug development (Katz, 2004, Kozauer andKatz, 2013), new alternative treaments such as stem cell therapy (Maucksch, Vazey, Gordon, & Connor, 2013), multidisciplinary rehabilitation (Cruickshank, et al, 2015), and transcranial magnetic stimulation (Berardelli & Suppa, 2013;Medina & Tunez, 2010).…”

Cognition in Huntington's Disease

“…They sometimes dominate the clinical picture. Around 72-98% of HD individuals develop significant neuropsychiatric problems, including both affective psychoses and nonwww.intechopen.com affective psychoses (Mendez, 1994;Paulsen et al, 2001;van Duijn et al, 2007). Major depression (Larsson et al, 2006) and manic episodes also occur (van Duijn et al, 2008).…”

“…Apathy and irritability (33% to 76%) are also amongst the first symptoms (van Duijn et al, 2007). When depression occurs it is often characterized by hopelessness, guilt and shame (Baudic et al, 2006;Kessler, 1987).…”

“…In almost half of HD cases clinical onset is indicated with psychiatric symptoms such as depression, anxiety and aggressive outbursts (Close Kirkwood et al, 2002a;Julien et al, 2007;van Duijn et al, 2007). Sometimes onset of the disease presents with schizophrenic or manic-like symptoms (Julien et al, 2007;Shiwach, 1994).…”

Exploring the Validity of the Short Version of the Problem Behaviours Assessment (PBA-s) for Huntington’s disease: A Rasch Analysis

“…A wide range of behavioural symptoms have been recognised in HD, most commonly depression, irritability, apathy and anxiety which occur in up to seventy-six percent of patients [9]. Obsessive-compulsive behaviours, suicidal ideation and psychosis (hallucinations and delusions) occur less frequently.…”