{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T21:21:20Z","timestamp":1792099280628,"version":"4.4.0"},"reference-count":70,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2022,5,17]],"date-time":"2022-05-17T00:00:00Z","timestamp":1652745600000},"content-version":"vor","delay-in-days":15,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,5,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Myotonic dystrophy type 1 is an autosomal dominant multisystemic disorder affecting muscular and extra muscular systems, including the central nervous system. Cerebral involvement in myotonic dystrophy type 1 is associated with subtle cognitive and behavioural disorders, of major impact on socio-professional adaptation. The social dysfunction and its potential relation to frontal lobe neuropsychology remain under-evaluated in this pathology. The neuroanatomical network underpinning that disorder is yet to disentangle. Twenty-eight myotonic dystrophy type 1 adult patients (mean age: 46 years old) and 18 age and sex-matched healthy controls were included in the study. All patients performed an exhaustive neuropsychological assessment with a specific focus on frontal lobe neuropsychology (motivation, social cognition and executive functions). Among them, 18 myotonic dystrophy type 1 patients and 18 healthy controls had a brain MRI with T1 and T2 Flair sequences. Grey matter segmentation, Voxel-based morphometry and cortical thickness estimation were performed with Statistical Parametric Mapping Software SPM12 and Freesurfer software. Furthermore, T2 white matter lesions and subcortical structures were segmented with Automated Volumetry Software. Most patients showed significant impairment in executive frontal functions (auditory working memory, inhibition, contextualization and mental flexibility). Patients showed only minor difficulties in social cognition tests mostly in cognitive Theory of Mind, but with relative sparing of affective Theory of Mind and emotion recognition. Neuroimaging analysis revealed atrophy mostly in the parahippocampal and hippocampal regions and to a lesser extent in basal ganglia, regions involved in social navigation and mental flexibility, respectively. Social cognition scores were correlated with right parahippocampal gyrus atrophy. Social dysfunction in myotonic dystrophy type 1 might be a consequence of cognitive impairment regarding mental flexibility and social contextualization rather than a specific social cognition deficit such as emotion recognition. We suggest that both white matter lesions and grey matter disease could account for this social dysfunction, involving, in particular, the frontal-subcortical network and the hippocampal\/arahippocampal regions, brain regions known, respectively, to integrate contextualization and social navigation.<\/jats:p>","DOI":"10.1093\/braincomms\/fcac111","type":"journal-article","created":{"date-parts":[[2022,5,17]],"date-time":"2022-05-17T10:36:17Z","timestamp":1652783777000},"source":"Crossref","is-referenced-by-count":13,"title":["Unravelling the impact of frontal lobe impairment for social dysfunction in myotonic dystrophy type 1"],"prefix":"10.1093","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5869-5456","authenticated-orcid":false,"given":"Alexandre","family":"Morin","sequence":"first","affiliation":[{"name":"Institut du Cerveau et de la Moelle \u00e9pini\u00e8re (ICM), UMRS 975, ICM-INSERM 1127 , 75013 Paris, France"},{"name":"Service de Neurologie, CHU Rouen, Centre 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France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Dubois","sequence":"additional","affiliation":[{"name":"Institut du Cerveau et de la Moelle \u00e9pini\u00e8re (ICM), UMRS 975, ICM-INSERM 1127 , 75013 Paris, France"},{"name":"D\u00e9partement de Neurologie, Institut de la M\u00e9moire et de la Maladie d\u2019Alzheimer, Centre National D\u00e9mences Rares, H\u00f4pital Piti\u00e9-Salp\u00eatri\u00e8re, APHP , 75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Eymard","sequence":"additional","affiliation":[{"name":"Centre de r\u00e9f\u00e9rence des maladies neuromusculaires Nord\/Est\/Ile de France, Institut de Myologie, Hospital Piti\u00e9-Salp\u00eatri\u00e8re, APHP , 75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire-Cecile","family":"Michon","sequence":"additional","affiliation":[{"name":"Centre de r\u00e9f\u00e9rence des maladies neuromusculaires Nord\/Est\/Ile de France, Institut de Myologie, Hospital Piti\u00e9-Salp\u00eatri\u00e8re, APHP , 75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Angeard","sequence":"additional","affiliation":[{"name":"U1129, Paris Descartes University, Sorbonne Paris Cit\u00e9 , Paris, France"},{"name":"Institut de Myologie, Groupe Hospitalier Piti\u00e9-Salp\u00eatri\u00e8re, APHP , Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony","family":"Behin","sequence":"additional","affiliation":[{"name":"Centre de r\u00e9f\u00e9rence des maladies neuromusculaires Nord\/Est\/Ile de France, Institut de Myologie, Hospital Piti\u00e9-Salp\u00eatri\u00e8re, APHP , 75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"Laforet","sequence":"additional","affiliation":[{"name":"Centre de r\u00e9f\u00e9rence des maladies neuromusculaires Nord\/Est\/Ile de France, Institut de 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