A comprehensive cognitive analysis of cervical dystonia: A single centre study.

Introduction: Cervical dystonia (CD) presents as a motor disorder but has a number of non-motor features. Studies have demonstrated diverse changes in cognition in patients with CD. The rarity of this disorder, phenotypic heterogeneity, and, in particular, a lack of consistency in cognitive testing measures limits clear definition of cognitive changes in this disorder. The relationship between cognition, motor symptoms and quality of life has not been well defined. We undertook a comprehensive analysis of cognition in CD. Methods: Patients with adult onset idiopathic isolated CD (AOICD) who had completed a battery of cognitive assessments- general intellectual functioning, verbal and visual memory, executive functions and social cognition measures, were included. Participants were assessed for mood symptoms, motor severity and quality of life. Results: 13 patients (8 women) with AOICD were included covering 40 cognitive subtests. Mean age was 59.9 years and mean TWSTRS-2 severity was 11. Mean estimated premorbid function was in the normal range. Overall performance on most measures were within normal limits. The lowest mean z-score was observed in Florida Affect Battery (social cognition) subtests, z = -1.75 and -0.81. and in verbal recall, z = -0.82. The majority of patients (75%) scored below population mean on spatial working memory and (62%) performed below population mean on word retrieval and working memory. Conclusion: We provide detailed cognitive results across a wide range of measures. Although patients tended towards average outcomes on the majority of tests, poorer performance than expected averages were noted in measures of social cognition, word retrieval, spatial working memory and, processing speed.

Interpreting the clinical importance of the relationship between subjective fatigue and cognitive impairment in multiple sclerosis (MS): How BICAMS performance is affected by MS-related fatigue.

BACKGROUND: There is evidence that subjective fatigue can influence cognitive functioning in multiple sclerosis (MS). DeLuca et al.'s (2004) Relative Consequence Model proposes that impairments to other high-level cognitive functions, such as memory, result from the disease's effect on information processing speed. OBJECTIVE: The primary aims of the study were to investigate both 1) the relationship between subjective fatigue and cognitive functioning, as measured by the widely used Brief International Cognitive Assessment for Multiple Sclerosis (BICAMS) in MS; and 2) the consequential effect of fatigue on information processing speed as predicted by the Relative Consequence Model. METHODS: 192 participants with MS attending tertiary referral MS centre completed the Modified Fatigue Impact Scale and BICAMS. RESULTS: Multiple correlation analyses determined that there were statistically significant relationships between all domains assessed by the BICAMS and levels of fatigue, such that higher levels of self-reported fatigue were associated with lower performance on information-processing, and visual and verbal learning. After controlling for information processing speed, the strength of correlation between fatigue and learning performance weakened. Linear regression analysis showed that fatigue predicted the most variance in verbal learning and 11.7% of the overall variance in BICAMS performance. CONCLUSION: Subjective fatigue and objective cognitive performance in MS are related. Caution is advised in the interpretation of BICAMS scores in cases where high levels of fatigue are present, and more detailed neuropsychological assessments may be required in order to accurately identify objective cognitive impairment independent of subjective fatigue.

Investigating the association of mood and fatigue with objective and subjective cognitive impairment in multiple sclerosis.

Discrepancies between subjective cognitive difficulties and objective measures of cognitive function in people with MS have been identified and may be related to mood and fatigue. The aim of the present study was to examine associations of depression and fatigue with discrepancies between subjective and objective cognitive functioning in pwMS. 177 participants with MS attending a University Hospital Department of Neurology MS Outpatient clinic completed the Brief International Cognitive Assessment for MS (BICAMS), MS Neuropsychological Questionnaire (MSNQ), Hospital Anxiety and Depression Scale (HADS) and Modified Fatigue Impact Scale (MFIS). To quantify the discrepancy between objective (BICAMS) and subjective (MSNQ) cognitive functioning, discrepancy scores were calculated by subtracting MSNQ z-score from composite BICAMS z-score. Based on their discrepancy score, participants were grouped as 'Underestimated', 'Overestimated' and 'Non-discrepant'. 39% of the total sample demonstrated poorer subjective cognitive functioning than their objective cognitive performance suggested ('Underestimated'). 23% of the total sample indicated lower objective scores than their subjective report suggests ('Overestimated'). 38% participants indicated relatively no discrepancy between objective and subjective cognitive measures ('Non-discrepant'). Significant differences were observed between the discrepancy groups in terms of depression and fatigue, with the 'Underestimated' group demonstrating greater levels of depression and fatigue (ps < .01). Regression analysis indicated that cognitive fatigue and depression significantly contributed to variance in subjective cognitive functioning. Our findings suggest that subjective reports of cognitive function may be influenced by depression and fatigue, emphasising the importance of cognitive, mood and fatigue screening as part of routine clinical care.

Five-year follow up of the original Irish BICAMS validation cohort.

INTRODUCTION: Cognitive impairment is common in multiple sclerosis at all stages of the condition. The natural history of cognition in multiple sclerosis has been considered to be deterioration of cognitive functioning over time. The development of the Brief International Cognitive Assessment for Multiple Sclerosis(BICAMS) has allowed standardization of a screening tool for cognitive impairment which can be easily performed in the neurology clinic. Cross-sectional and validation studies using BICAMS have been widely reported, however minimal longitudinal assessment of cognition using BICAMS has taken place to date. OBJECTIVES: The objective of this study was to evaluate the prevalence of cognitive impairment at a five-year interval in participants of an original BICAMS validation study. We will also evaluate change of the BICAMS subtests over time. MATERIALS AND METHODS: Participants of the original BICAMS validation study were invited to participate in the study. Demographic and clinical details were collected. BICAMS subtests, anxiety, depression and fatigue questionnaires were completed. RESULTS: Fifty out of the original 67 participants completed BICAMS five years post original assessment. The prevalence of cognitive impairment in this cohort with a mean age of 49 and a median EDSS of 2.5 (EDSS of 2.0 at initial BICAMS testing) remained stable five years following initial BICAMS screening assessment, X2(1)=0.36, p=.548. There was no significant difference in SDMT scores between 2014 and 2019 t(48) = 1.08, p=.15. There was an improvement in CVLT-II, t(49)=-3.03; p=.004 and BVMT-R, t(49)=-3.38; p=.001. CONCLUSIONS: This study demonstrates overall stability in the prevalence of cognitive impairment as assessed by the BICAMS. The interval of five years between assessment reduces the possibility of practice effects, although familiarity with the testing protocol may exert an influence.

Non-motor features of cervical dystonia: Cognition, social cognition, psychological distress and quality of life.

INTRODUCTION: Non-motor features of cervical dystonia (CD) have been identified, including depression, anxiety, and neuropsychological deficits. The aims were: to provide a clinical neuropsychological profile of CD patients with specific focus on social cognition; assess levels of psychological distress; and investigate the relationship between non-motor features of CD, including cognitive functioning, psychological distress, CD severity, pain, and health-related quality of life (HR-QoL). METHODS: A multi-domain neuropsychological assessment battery was administered to 46 participants with CD, examining cognitive and social cognitive domains. Clinical data on dystonia severity, pain, psychological distress and HR-QoL were collected. RESULTS: The majority of participants with CD performed within the average range across most tests of cognition. Scores were significantly lower than standardized norms in social cognition, processing speed, and aspects of memory. High levels of anxiety (Hospital Anxiety and Depression Scale [HADS-A] ≥ 11, 30%) and depression (HADS-D ≥ 11; 29%) were observed. Psychological distress, CD severity, pain and HR-QoL were not significantly associated with neuropsychological functioning after controlling for multiple comparisons. Low HR-QoL was associated with higher levels of pain and psychological distress, but not severity of motor symptoms. CONCLUSION: Results indicate that psychological distress and deficits in cognitive and social cognitive functioning are likely distinct features of CD. While motor symptoms do not appear to impact HR-QoL, pain and psychological distress were associated with low HR-QoL. Findings highlight the importance of addressing non-motor symptoms in the treatment of CD.

Social cognition in cervical dystonia: A case-control study.

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.