Background Although considered a motor disorder, adult onset isolated focal dystonia has many non-motor symptoms. There is a paucity of neuropsychological research on cognitive processing in adult onset focal dystonia. Methods We employed a battery of clinical and cognitive assessments, including basic and complex social cognition, and assessed 46 patients with adult-onset cervical dystonia, compared to 46 age-, sex–, education-, and premorbid IQ-matched healthy controls. Results Significant between-group differences were observed in relation to measures of memory encoding, recall and recognition, as well as multimodal measures of basic Social Cognition (emotion recognition: face and prosody), but not complex Social Cognition (mentalising). There were no deficits observed in multimodal measures of executive function. Controlling for mood did not affect performance. Conclusion In this multi-dimensional assessment of cognition in cervical dystonia, we report deficits in memory encoding, and in social cognition. Further investigation of social cognitive processes, memory, and sustained attention are required. Longitudinal studies are also needed to further delineate the role of psychological distress on cognitive outcomes and document the cognitive profile over time.
Objective.To investigate the incidence and nature of language change and its relationship to executive dysfunction in a population-based incident ALS sample, with the hypothesis that patterns of frontotemporal involvement in early ALS extend beyond areas of executive control to regions associated with language processing.Methods.One hundred and seventeen population-based incident ALS cases without dementia and 100 controls matched by age, sex and education were included in the study. A detailed assessment of language processing including lexical processing, word spelling, word reading, word naming, semantic processing and syntactic/grammatical processing was undertaken. Executive domains of phonemic verbal fluency, working memory, problem-solving, cognitive flexibility and social cognition were also evaluated.Results.Language processing was impaired in this incident cohort of individuals with ALS, with deficits in the domains of word naming, orthographic processing and syntactic/grammatical processing. Conversely, phonological lexical processing and semantic processing were spared. While executive dysfunction accounted in part for impairments in grammatical and orthographic lexical processing, word spelling, reading and naming, primary language deficits were also present.Conclusions.Language impairment is characteristic of ALS at early stages of the disease, and can develop independently of executive dysfunction, reflecting selective patterns of frontotemporal involvement at disease onset. Language change is therefore an important component of the frontotemporal syndrome associated with ALS.
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