Psychiatric and neurodevelopmental aspects of Becker muscular dystrophy

Abstract

Little is known about the relationship between Becker Muscular Dystrophy (BMD) and mental disorders. This study aimed to clarify whether BMD is a risk factor for psychiatric diseases. We asked genetically or immunohistochemically confirmed BMD patients to participate in the study interview. Participants who consented to psychiatric tests underwent further assessments of intellectual, psychological, and neurodevelopmental disorders. In total, 76 (73%) of 105 BMD patients (median age, 37 years) completed the interview. Of these, 6 had developmental disorders (mental retardation, pervasive developmental disorder), 33/76 (43%) experienced bullying in school, 11 exhibited problematic behaviors such as cutting class and violent incidents, and 16 had psychiatric disorders (schizophrenia spectrum, 5; depressive spectrum, 4; stress-related disorders, 3; obsessive-compulsive and related disorders, 2; somatic symptom and related disorders, 2; bipolar and related disorders, 1). Mean IQ was normal, whereas 13/40 (32.5%) of participants were in a depressive state. High trait anxiety was found in 20/40 (50%) of patients, while 15/40 (38.5%) were in an anxious state. Review of MRI data from 14 participants revealed brain atrophy caused solely by BMD and unrelated to any other complication. Our findings suggest that BMD patients are at risk of developing psychiatric disorders. Physical handicap or bullying may influence their mental state, as many of them have high trait anxiety. Parents, teachers, and supporters should be mindful of the daily environment of BMD patients and provide support to help them cope with stress.

Decision-Making And Selection Bias in Four Observational Studies on Duchenne and Becker Muscular Dystrophy

“…An important example of protocol burden was the amount of time that participation would cost patients or parents, needing to take time off from school or work. This was also underscored by a previous observational study in BMD patients that reported lack of time as main reason for non-participation [5]. The four studies at our institute mainly took place on week days, mostly outside of school hours.…”

“…These neuromuscular diseases form a spectrum in which DMD patients lose ambulation around their early teens, while BMD patients have a milder but more variable disease course [2,3]. In both diseases, a higher prevalence of learning and behavioral disabilities has been reported [4][5][6]. In DMD, this is associated with absence of different dystrophin isoforms in the brain [7,8].…”

“…Such detailed and high quality natural history data would enable their use for placebo arms, and for determination of primary and secondary outcome measures in interventional trials. While considerations for not participating have been described for interventional trials [12][13][14], only one observational study reported on this topic [5].…”

The adult multidisciplinary respiratory neuromuscular clinic

“…The presence of psychological disturbance in patients with NMD is well established across many conditions [113][114][115][116][117][118]. Given that mental health conditions are associated with poorer health outcomes [119], it is likely that they have an important impact on outcomes in NMD patients too.…”

The Neurocognitive and Behavioral Profiles of 3 Brothers With Becker Muscular Dystrophy

“… 1 In addition to skeletal muscle and heart pathology, patients with BMD may have neurodevelopmental-, behavioral comorbidities, learning disabilities and epilepsy. 1 , 4 – 7 For instance, autism features, inattention/hyperactivity features, language–speech delays and difficulties in reading, spelling and arithmetic are found in BMD. 1 , 4 , 6 , 7 It has been suggested that these comorbidities are related to a disturbed production of brain isoforms (i.e.…”