Impaired social cognition in relation to behavioral problems in manifest and premanifest Huntington’s disease

Background: Social cognition impairments are potential markers of Huntington’s disease (HD) onset and progression. Behavioral problems, such as apathy, irritability, aggression and perseveration, are also symptoms of HD and frequently occur before the onset of motor symptoms. Social cognition can be associated with behavioral problems, but data on this association in HD are scare. <br/>Aims: Investigate the association between social cognition impairments and behavioral problems in premanifest and manifest HD. <br/>Methods/Techniques: HD patients underwent a neurological examination and tests of social cognition (emotion recognition, theory of mind (ToM), and empathy). Social cognition data were compared to a healthy control group. For patients who participated in Enroll-HD, behavioral data were available 4 years after the social cognition tests. <br/>Results/Outcome: Fifty patients (n=15 premanifest) participated. Impairments in emotion recognition and ToM were found in the manifest group only. Behavioral data were available for 31 patients (n=7 premanifest). While the premanifest HD patients had mild behavioral problems, manifest HD patients had more severe and diverse behavioral problems. ToM performance in the whole group was significantly associated with the severity of apathy 4 years later. <br/>Conclusions: Social cognition is impaired in manifest HD and worse ToM performance was related to more apathy 4 years later. This confirms that social cognition impairments can serve as a marker of disease onset and points out possible approaches for neuropsychological treatment. Future longitudinal studies need to show whether social cognition can also be used as (one of several) variables to predict disease progression. <br/>