Empathy and aversion: the neural signature of mentalizing in Tourette syndrome.

BACKGROUND: Previous studies suggest that adults with Tourette syndrome (TS) can respond unconventionally on tasks involving social cognition. We therefore hypothesized that these patients would exhibit different neural responses to healthy controls in response to emotionally salient expressions of human eyes. METHOD: Twenty-five adults with TS and 25 matched healthy controls were scanned using fMRI during the standard version of the Reading the Mind in the Eyes Task which requires mental state judgements, and a novel comparison version requiring judgements about age. RESULTS: During prompted mental state recognition, greater activity was apparent in TS within left orbitofrontal cortex, posterior cingulate, right amygdala and right temporo-parietal junction (TPJ), while reduced activity was apparent in regions including left inferior parietal cortex. Age judgement elicited greater activity in TS within precuneus, medial prefrontal and temporal regions involved in mentalizing. The interaction between group and task revealed differential activity in areas including right inferior frontal gyrus. Task-related activity in the TPJ covaried with global ratings of the urge to tic. CONCLUSIONS: While recognizing mental states, adults with TS exhibit greater activity than controls in brain areas involved in the processing of negative emotion, in addition to reduced activity in regions associated with the attribution of agency. In addition, increased recruitment of areas involved in mental state reasoning is apparent in these patients when mentalizing is not a task requirement. Our findings highlight differential neural reactivity in response to emotive social cues in TS, which may interact with tic expression.

'It's a curse!': coprolalia in Tourette syndrome.

BACKGROUND AND PURPOSE: Coprolalia is a complex socially inappropriate vocal tic most frequently reported in the context of Tourette syndrome (TS) and widely portrayed as a cardinal characteristic of this condition throughout popular culture. This study investigated which clinical factors may predispose some patients with TS to experience coprolalia and the impact of this symptom on quality of life. METHODS: Participants were 60 patients with TS (39 males, mean age 32.15, SD 14.1 years) of whom 50% reported mental coprolalia (urges) and 33% reported actual involuntary swearing as a tic. Relationships between the presence of coprolalia and a range of clinical variables including severity of tics, obsessive-compulsive symptoms, attention problems, anxiety, depression, premonitory urges for tics and quality of life were investigated. RESULTS: The presence of urges to utter obscene language was significantly related to non-obscene socially inappropriate symptoms and self-reported tic severity. Although experiencing socially inappropriate urges in general was correlated with the presence of mental coprolalia, only the presence of more severe tics was a good indicator of outbursts of obscene vocal tics. Having coprolalia was related to significantly poorer quality of life in TS. CONCLUSIONS: As outbursts of coprolalia exert a specific negative impact on quality of life clinicians should consider improvement in this symptom during evaluation of treatment efficacy.

Is Huntington's disease associated with deficits in theory of mind?

OBJECTIVES: People with Huntington's disease (HD) can exhibit interpersonal difficulties and deficits in recognizing emotional facial expressions. We investigated whether individuals with HD exhibit impairments in the understanding of other people's mental states, an aspect of Theory of Mind (ToM). MATERIALS AND METHODS: Sixteen patients with HD and sixteen healthy controls completed two ToM tasks. One task involved recognising socially inappropriate behaviour and the other task required participants to judge complex mental states from photographs of people's eyes alone. To assess relationships between executive function and ToM, participants completed measures of verbal fluency, working memory and inhibition. The Problem Behaviours Assessment-short form (Neuropsychiatry Neuropsychol Behav Neurol, 14, 2001and 219) was completed twice using information from patients and their close relatives (where possible) to identify relationships between ToM impairment and behavioural problems. RESULTS: Patients with HD made significantly more errors on ToM tasks than controls, exhibiting difficulties in judging the social appropriateness of story character's behaviour and problems inferring complex mental states from photographs of people's eyes. Patients with HD also exhibited executive dysfunction. However, there was little evidence that executive impairments were related to ToM deficits. No correlations were apparent between problem behaviours and ToM errors. CONCLUSIONS: HD is associated with deficits in ToM. Furthermore, some of patients' ToM difficulties appear independent of executive dysfunction.

Quality of life in young people with Tourette syndrome: a controlled study.

Quality of life (QoL) may be adversely affected by Tourette syndrome (TS). Although the core symptoms of this complex neurodevelopmental disorder are tics, patients often present with an array of behavioural difficulties, such as co-morbid obsessive compulsive disorder (OCD) or attention deficit hyperactivity disorder (ADHD). In this study we investigated whether young people with TS exhibited poorer QoL in comparison to healthy individuals and an epilepsy control group. We also analysed whether greater tic severity or co-morbid OCD and\or ADHD led to greater differences in perceived QoL. The Youth Quality of Life Instrument-Research Version (Edwards et al. in J Adolesc 25:275-286, 2002) was used to assess QoL and a range of clinical scales were administered to assess anxiety, depression and other behavioural symptoms. TS was associated with significant differences in aspects of QoL related to home and social activities, involving peer and family interactions. Patients with more severe tics reported a greater negative impact on QoL. Patients with TS and no associated diagnoses (pure TS) presented with lower QoL scores in the environment domain, poorer perceived QoL in general, and depressive features. Co-morbid OCD appeared to exert a greater impact on self and relationship QoL domains. The presence of both OCD and ADHD as co-morbidities led to more widespread problems. In conclusion, TS can be associated with poorer perceived QoL. Although social aspects of QoL may be more vulnerable to TS in general, co-morbid conditions make an important contribution in determining which aspects of QoL are most affected in the individual.

Quality of life in young people with treatment-responsive epilepsy: A controlled study.

OBJECTIVE: Quality of life (QoL) has been shown to be lower in individuals with epilepsy than the general public. However, few studies have investigated the QoL of individuals with well-controlled epilepsy. This study investigated the effects of epilepsy on QoL in persons with treatment-responsive seizures, beyond factors directly related to the presence of seizures. METHODS: Fifty young patients with controlled epilepsy and 102 healthy controls completed a generic, multidimensional, self-report QoL instrument, along with standardized scales assessing anxiety, depression, and other emotional or behavioral difficulties. RESULTS: Young people with epilepsy reported increased anxiety (P=0.037) and more emotional and behavioral difficulties (P<0.001). Though there were was no difference between the groups in Total QoL score, treatment-responsive epilepsy was associated with lower QoL within the Self domain (P=0.016). CONCLUSIONS: Epilepsy may exert a negative influence on QoL in relation to thoughts and feelings about the self in the context of complete seizure remission. Future research should investigate the therapeutic value of interventions targeting detrimental changes to self-perception in young people living with controlled epilepsy.