Social Cognition Abilities in Patients With Different Multiple Sclerosis Subtypes.

OBJECTIVES: Multiple sclerosis (MS) can be associated with impaired social cognition. However, social cognition skills have never been explored in the different subtypes of MS. The first aim of this study was to examine whether MS subtypes differ on the course of social cognition. The second aim was to explore the relationship between social cognition performances and executive abilities and mood variables. METHODS: Sixty-two patients with MS and 33 healthy matched controls performed experimental tasks assessing facial emotion recognition, theory of mind (ToM), and cognitive functioning, in particular executive functions. RESULTS: Patients differed from controls on both measures of social cognition. On the ToM measures, patients with progressive MS scored significantly lower than healthy participants. Patients with primary progressive MS performed worse than both healthy participants and patients with relapsing-remitting MS on the recognition of fearful facial expressions, while patients with secondary progressive MS performed worse on the recognition of facial expressions of sadness. Patients' social cognition difficulties were correlated with processing speed, working memory, and verbal fluency. CONCLUSIONS: These preliminary results suggested that there may be qualitative differences in social cognition difficulties among the phenotypes. Furthermore, these impairments were related to executive functions, but unrelated to patients' disability or level of depression. These data highlight the need for research to determine the real impact of these deficits on interpersonal relationships among patients and to confirm these disorders in a larger population with progressive forms of MS. (JINS, 2017, 23, 653-664).

Social cognition and executive functioning in multiple sclerosis: A cluster-analytic approach.

Multiple sclerosis (MS) is associated with deficits in social cognition, the process underlying social interaction and cognitive function. However, the relationships between executive impairment and social cognition remain unclear in MS. Previous studies exclusively focused on group comparisons between healthy controls and patients with MS, treating the latter as a homogeneous population. The variability of socio- and neurocognitive profiles in this pathology therefore remains underexplored. In the present study, we used a cluster analytic approach to explore the heterogeneity of executive and social cognition skills in MS. A total of 106 patients with MS were compared with 53 healthy matched controls on executive (e.g., working memory) and social cognition (facial emotion recognition and theory of mind) performances. A cluster analysis was then performed, focusing on the MS sample, to explore the presence of differential patterns of interaction between executive and social cognition difficulties and their links to sociodemographic, clinical and cognitive variables. We identified three distinct functional profiles: patients with no executive or social cognition deficits (Cluster 1); patients with difficulties in facial emotion recognition and theory of mind and, to a lesser extent, executive functioning (Cluster 2); and patients with executive functioning difficulties only (Cluster 3). Clinical characteristics (disease duration, disability, fatigue) did not differ between clusters. CONCLUSIONS: These results suggest that there are qualitative differences in the social cognition and executive difficulties that are commonly found among patients with MS. If replicated, the identification of these profiles in clinical practice could allow for more individualized rehabilitation.

Social cognition impairments in relapsing-remitting multiple sclerosis.

Theory of Mind (ToM) is the ability to attribute independent mental states to self and others to explain and predict behavior. Impairment of ToM is well established in developmental pathologies. In neurological populations, investigation of ToM is still rare but data suggest that ToM impairment could contribute to behavioral and social disturbances. In addition to neurological signs, multiple sclerosis (MS) presents with disorders of cognition and behavior directly related to brain damage. The aim of this study was to assess ToM abilities and recognition of facial emotional expression in adults with MS. We compared 64 patients with relapsing MS and 30 matched healthy controls on three levels of ToM tasks, a facial emotion recognition task, and a neuropsychological assessment. MS patients performed significantly worse than controls in emotion recognition and all ToM tasks (p < .02). These deficits were not correlated with demographic variables or neuropsychological test performance. These findings underscore the importance of assessing ToM and facial recognition in MS, as dysfunction in these areas may impact upon social interaction and, thus, impair quality of life for both patients with MS and their families.

Juvenile frontotemporal dementia with parkinsonism associated with tau mutation G389R.

Frontotemporal lobe degeneration includes a large spectrum of neurodegenerative disorders. Patients with frontotemporal dementia with parkinsonism linked to chromosome 17 exhibit heterogeneity in both clinical and neuropathological features. Here, we report the case of a young patient with a G389R mutation. This teenager girl was 17 years old when she progressively developed severe behavioral disturbances. First, she was considered to be suffering from atypical depression. After 2 years, she was referred to the department of neurology. By this time, the patient exhibited typical frontotemporal dementia with mild extrapyramidal disorders. The main behavioral features included apathy and reduced speech output. MRI and SPECT showed a frontotemporal atrophy and hypofixation, respectively. She died 7 years after onset. Three relatives on her father side had also died after early onset dementia. Genetic testing revealed a heterozygous guanine to cytosine mutation at the first base of codon 389 (Exon 13) of MAPT, the tau gene, resulting in a glycine to arginine substitution, in the patient and her non-affected father. Postmortem neuropathological and biochemical data indicate a Pick-like tau pathology but with phosphoserine 262-positive immunoreactivity. This case is remarkable because of the extremely early onset of the disease.