{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T01:10:34Z","timestamp":1777857034508,"version":"3.51.4"},"reference-count":30,"publisher":"SAGE Publications","issue":"4","license":[{"start":{"date-parts":[[2022,7,1]],"date-time":"2022-07-01T00:00:00Z","timestamp":1656633600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JND"],"published-print":{"date-parts":[[2022,7,1]]},"abstract":"<jats:p>Background: In Becker muscular dystrophy evidence for neurocognitive and behavioral comorbidity is evolving. More insight into the extend of these problems is of great importance for early detection and remediation in clinical practice. Objective: In this study we aimed to describe the neurocognitive and behavioral features of a Dutch adult cohort of BMD patients, and to evaluate correlations to motor function outcomes. Methods: 28 adult BMD patients were included. Intelligence, executive functioning, verbal memory and reaction times were assessed cross-sectionally. Additionally, patients completed questionnaires on behavioral and emotional symptoms, psychosocial and executive functions. Results were compared to normative data and correlated with disease severity as measured by the 10-meter run\/walk test and Performance of the Upper Limb version 1.2. Results: 15 patients (53.6%) had a high educational level despite frequent grade repeating (48.3%) during primary or secondary school. Neuropsychological testing revealed that intellectual abilities, verbal memory, processing speed and executive functioning were statistically significant below average, but still within normal range. Regarding outcomes of the behavioral questionnaires, no significant differences were reported compared to the norm population. No relevant correlations with disease severity were found. Conclusions: This cohort of adult BMD patients exhibits minor cognitive impairments and no significant behavioral problems. The lower outcomes on processing speed and verbal memory, combined with the relatively high prevalence of grade repeating during primary and secondary school, implies that these minor impairments played a role in childhood. However, the on average high educational levels suggests that they grow out of their cognitive impairments with ageing.<\/jats:p>","DOI":"10.3233\/jnd-210770","type":"journal-article","created":{"date-parts":[[2022,6,14]],"date-time":"2022-06-14T13:24:55Z","timestamp":1655213095000},"page":"543-553","source":"Crossref","is-referenced-by-count":7,"title":["The neurocognitive profile of adults with Becker muscular dystrophy in the Netherlands"],"prefix":"10.1177","volume":"9","author":[{"given":"Za\u0131da","family":"Koeks","sequence":"first","affiliation":[{"name":"Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Danique M.J.","family":"Hellebrekers","sequence":"additional","affiliation":[{"name":"Centre for Neurological Learning Disabilities, Kempenhaeghe, Heeze, the Netherlands"},{"name":"School for Mental Health and Neuroscience>, Maastricht University, Maastricht, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nienke M.","family":"van de Velde","sequence":"additional","affiliation":[{"name":"Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands"},{"name":"Duchenne Center Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Iris","family":"Alleman","sequence":"additional","affiliation":[{"name":"Department of Orthopaedics, Rehabilitation and Physical Therapy, Leiden University Medical Center, Leiden, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pietro","family":"Spitali","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, Leiden University Medical Center, Leiden, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hermine A.","family":"van Duyvenvoorde","sequence":"additional","affiliation":[{"name":"Department of Clinical Genetics, Leiden University Medical Center, Leiden the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jan J.G.M.","family":"Verschuuren","sequence":"additional","affiliation":[{"name":"Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands"},{"name":"Duchenne Center Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jos G.M.","family":"Hendriksen","sequence":"additional","affiliation":[{"name":"Centre for Neurological Learning Disabilities, Kempenhaeghe, Heeze, the Netherlands"},{"name":"School for Mental Health and Neuroscience>, Maastricht University, Maastricht, The Netherlands"},{"name":"Duchenne Center Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erik H.","family":"Niks","sequence":"additional","affiliation":[{"name":"Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands"},{"name":"Duchenne Center Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","reference":[{"issue":"6","key":"10.3233\/JND-210770_ref1","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1016\/0092-8674(87)90579-4","article-title":"Dystrophin: The protein product of the Duchenne muscular dystrophy locus","volume":"51","author":"Hoffman","year":"1987","journal-title":"Cell"},{"issue":"9307","key":"10.3233\/JND-210770_ref2","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1016\/S0140-6736(02)07815-7","article-title":"The muscular dystrophies","volume":"359","author":"Emery","year":"2002","journal-title":"Lancet"},{"issue":"2","key":"10.3233\/JND-210770_ref3","first-page":"98","article-title":"The clinical, genetic and dystrophin characteristics of Becker muscular dystrophy","volume":"240","author":"Bushby","year":"1993","journal-title":"I. Natural history. Journal of Neurology"},{"issue":"4","key":"10.3233\/JND-210770_ref4","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1016\/j.jpeds.2012.03.020","article-title":"Attention deficit hyperactivity disorder and cognitive function in Duchenne muscular dystrophy: Phenotype-genotype correlation","volume":"161","author":"Pane","year":"2012","journal-title":"J Pediatr"},{"issue":"1","key":"10.3233\/JND-210770_ref5","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1111\/dmcn.12922","article-title":"Neurodevelopmental, emotional, and behavioural problems in Duchenne muscular dystrophy in relation to underlying dystrophin gene mutations","volume":"58","author":"Ricotti","year":"2016","journal-title":"Dev Med Child Neurol"},{"issue":"3","key":"10.3233\/JND-210770_ref6","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1016\/S0960-8966(99)00108-X","article-title":"Loss of Dp140 regulatory sequences is associated with cognitive impairment in dystrophinopathies","volume":"10","author":"Bardoni","year":"2000","journal-title":"Neuromuscul Disord"},{"issue":"3","key":"10.3233\/JND-210770_ref7","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1002\/ana.24222","article-title":"Reduced cerebral gray matter and altered white matter in boys with Duchenne muscular dystrophy","volume":"76","author":"Doorenweerd","year":"2014","journal-title":"Ann Neurol"},{"issue":"1","key":"10.3233\/JND-210770_ref8","doi-asserted-by":"crossref","first-page":"21","DOI":"10.2478\/bjmg-2013-0014","article-title":"Association between loss of dp140 and cognitive impairment in duchenne and becker dystrophies","volume":"16","author":"Chamova","year":"2013","journal-title":"Balkan J Med Genet"},{"issue":"7","key":"10.3233\/JND-210770_ref9","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1017\/S0012162201000913","article-title":"Intelligence and Duchenne muscular dystrophy: Full-scale, verbal, and performance intelligence quotients","volume":"43","author":"Cotton","year":"2001","journal-title":"Dev Med Child Neurol"},{"issue":"5","key":"10.3233\/JND-210770_ref10","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1177\/0883073807309775","article-title":"Neuropsychiatric disorders in males with duchenne muscular dystrophy: Frequency rate of attention-deficit hyperactivity disorder (ADHD), autism spectrum disorder, and obsessive\u2013compulsive disorder","volume":"23","author":"Hendriksen","year":"2008","journal-title":"J\u00a0Child Neurol"},{"issue":"11","key":"10.3233\/JND-210770_ref11","doi-asserted-by":"crossref","first-page":"1472","DOI":"10.1177\/0883073815570154","article-title":"Cognitive and neurobehavioral profile in boys with duchenne muscular dystrophy","volume":"30","author":"Banihani","year":"2015","journal-title":"J Child Neurol"},{"issue":"4","key":"10.3233\/JND-210770_ref12","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1016\/j.ejpn.2017.02.007","article-title":"Assessing mental health in boys with Duchenne muscular dystrophy: Emotional, behavioural and neurodevelopmental profile in an Italian clinical sample","volume":"21","author":"Colombo","year":"2017","journal-title":"Eur J Paediatr Neurol"},{"issue":"2","key":"10.3233\/JND-210770_ref14","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1177\/0883073807307975","article-title":"Cognitive and psychological profile of males with Becker muscular dystrophy","volume":"23","author":"Young","year":"2008","journal-title":"Journal of Child Neurology"},{"issue":"3","key":"10.3233\/JND-210770_ref15","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1016\/S0960-8966(99)00108-X","article-title":"Loss of Dp140 regulatory sequences is associated with cognitive impairment in dystrophinopathies","volume":"10","author":"Bardoni","year":"2000","journal-title":"Neuromuscular Disorders"},{"issue":"2","key":"10.3233\/JND-210770_ref17","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1212\/WNL.46.2.461","article-title":"Cognitive dysfunction as the major presenting feature of Becker\u2019s muscular dystrophy","volume":"46","author":"North","year":"1996","journal-title":"Neurology"},{"issue":"1","key":"10.3233\/JND-210770_ref19","doi-asserted-by":"crossref","first-page":"99","DOI":"10.3233\/JND-140005","article-title":"Forty-five years of duchenne muscular dystrophy in The Netherlands","volume":"1","author":"van den Bergen","year":"2014","journal-title":"J Neuromuscul Dis"},{"issue":"5","key":"10.3233\/JND-210770_ref26","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1080\/09297049.2011.613816","article-title":"Verbal learning and memory as measured by the Rey-Auditory Verbal Learning Test: ADHD with and without learning disabilities","volume":"18","author":"Vakil","year":"2012","journal-title":"Child Neuropsychol"},{"issue":"1","key":"10.3233\/JND-210770_ref28","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s00787-003-0298-2","article-title":"The Strengths and Difficulties Questionnaire (SDQ)\u2013further evidence for its reliability and validity in a community sample of Dutch children and adolescents","volume":"12","author":"Muris","year":"2003","journal-title":"Eur Child Adolesc Psychiatry"},{"issue":"2","key":"10.3233\/JND-210770_ref29","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/j.jpsychores.2011.01.005","article-title":"Normative values for the hospital anxiety and depression scale (HADS) in the general German population","volume":"71","author":"Hinz","year":"2011","journal-title":"J Psychosom Res"},{"issue":"2","key":"10.3233\/JND-210770_ref30","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1080\/13803395.2013.875519","article-title":"Symptom Checklist 90-Revised in neurological outpatients","volume":"36","author":"Ruis","year":"2014","journal-title":"J Clin Exp Neuropsychol"},{"issue":"11","key":"10.3233\/JND-210770_ref31","doi-asserted-by":"crossref","first-page":"712","DOI":"10.1016\/j.nmd.2010.06.014","article-title":"North Star Ambulatory Assessment, 6-minute walk test and timed items in ambulant boys with Duchenne muscular dystrophy","volume":"20","author":"Mazzone","year":"2010","journal-title":"Neuromuscul Disord"},{"issue":"9","key":"10.3233\/JND-210770_ref32","doi-asserted-by":"crossref","first-page":"e0239064","DOI":"10.1371\/journal.pone.0239064","article-title":"Test-retest reliability of the Performance of Upper Limb (PUL) module for muscular dystrophy patients","volume":"15","author":"Gandolla","year":"2020","journal-title":"PLoS One"},{"issue":"4","key":"10.3233\/JND-210770_ref33","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1017\/S0012162205000496","article-title":"Association between intellectual functioning and age in children and young adults with Duchenne muscular dystrophy: Further results from a meta-analysis","volume":"47","author":"Cotton","year":"2005","journal-title":"Dev Med Child Neurol"},{"issue":"2","key":"10.3233\/JND-210770_ref35","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1016\/j.nmd.2017.11.018","article-title":"Cognitive profile in Duchenne muscular dystrophy boys without intellectual disability: The role of executive functions","volume":"28","author":"Battini","year":"2018","journal-title":"Neuromuscular Disorders"},{"issue":"1","key":"10.3233\/JND-210770_ref36","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/j.jocn.2010.07.118","article-title":"Neuropsychological impairments and the impact of dystrophin mutations on general cognitive functioning of patients with Duchenne muscular dystrophy","volume":"18","author":"Wingeier","year":"2011","journal-title":"Journal of clinical neuroscience"},{"issue":"2","key":"10.3233\/JND-210770_ref37","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1111\/j.1469-8749.2007.00123.x","article-title":"Verbal and memory skills in males with Duchenne muscular dystrophy","volume":"49","author":"Hinton","year":"2007","journal-title":"Developmental Medicine & Child Neurology"},{"issue":"1","key":"10.3233\/JND-210770_ref38","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1017\/S1355617701711058","article-title":"Selective deficits in verbal working memory associated with a known genetic etiology: The neuropsychological profile of Duchenne muscular dystrophy","volume":"7","author":"Hinton","year":"2001","journal-title":"Journal of the International Neuropsychological Society: JINS"},{"issue":"7","key":"10.3233\/JND-210770_ref39","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1017\/S1355617716000461","article-title":"Digit span performance in children with dystrophinopathy: A verbal span or working memory contribution","volume":"22","author":"Leaffer","year":"2016","journal-title":"Journal of the International Neuropsychological Society: JINS"},{"issue":"8","key":"10.3233\/JND-210770_ref40","doi-asserted-by":"crossref","first-page":"1359","DOI":"10.1080\/13854046.2011.617782","article-title":"The neuropsychological profile of infantile Duchenne muscular dystrophy","volume":"25","author":"Mento","year":"2011","journal-title":"The Clinical Neuropsychologist"},{"issue":"1","key":"10.3233\/JND-210770_ref41","doi-asserted-by":"crossref","first-page":"12575","DOI":"10.1038\/s41598-017-12981-5","article-title":"Timing and localization of human dystrophin isoform expression provide insights into the cognitive phenotype of Duchenne muscular dystrophy","volume":"7","author":"Doorenweerd","year":"2017","journal-title":"Sci Rep"}],"container-title":["Journal of Neuromuscular Diseases"],"original-title":[],"link":[{"URL":"https:\/\/content.iospress.com\/download?id=10.3233\/JND-210770","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T23:49:59Z","timestamp":1777506599000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/full\/10.3233\/JND-210770"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,7,1]]},"references-count":30,"journal-issue":{"issue":"4"},"URL":"https:\/\/doi.org\/10.3233\/jnd-210770","relation":{},"ISSN":["2214-3599","2214-3602"],"issn-type":[{"value":"2214-3599","type":"print"},{"value":"2214-3602","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,7,1]]}}}