Brain-related comorbidities in boys and men with Duchenne Muscular Dystrophy

Autor: Jos G.M. Hendriksen, Marlien W. Aalbers, Richard F.M. Chin, Johan S.H. Vles, Ruben G. F. Hendriksen
Přispěvatelé: Klinische Neurowetenschappen, RS: MHeNs - R1 - Cognitive Neuropsychiatry and Clinical Neuroscience, MUMC+: MA Med Staf Spec Neurologie (9), Psychiatrie & Neuropsychologie
Jazyk: angličtina
Rok vydání: 2018
Předmět:
Male
0301 basic medicine
Obsessive-Compulsive Disorder
Pediatrics
Duchenne muscular dystrophy
Comorbidity
Duchenne Muscular Dystrophy
Epilepsy
0302 clinical medicine
Surveys and Questionnaires
Epidemiology
Prevalence
EPIDEMIOLOGY
Child
Incidence (epidemiology)
Neurodevelopmental disorders
Neuropsychology
DP140
General Medicine
Sleep disorders
ASSOCIATION
Autism spectrum disorder
MALES
Anxiety
medicine.symptom
Adult
musculoskeletal diseases
medicine.medical_specialty
congenital
hereditary
and neonatal diseases and abnormalities
DEFICIT HYPERACTIVITY DISORDER
Clinical Neurology
ORGANIZATION
03 medical and health sciences
Seizures
medicine
Humans
Attention deficit hyperactivity disorder
Pediatrics
Perinatology
and Child Health
business.industry
AUTISM SPECTRUM DISORDER
medicine.disease
COGNITIVE IMPAIRMENT
Muscular Dystrophy
Duchenne
030104 developmental biology
Attention Deficit Disorder with Hyperactivity
Pediatrics
Perinatology and Child Health
Questionnaire study
Neurology (clinical)
business
RETARDATION
030217 neurology & neurosurgery
Zdroj: European Journal of Paediatric Neurology, 22(3), 488-497. ELSEVIER SCI LTD
Hendriksen, R G F, Vles, J S H, Aalbers, M W, Chin, R F M & Hendriksen, J G M 2017, ' Brain-related comorbidities in boys and men with Duchenne Muscular Dystrophy : A descriptive study ', European Journal of Paediatric Neurology . https://doi.org/10.1016/j.ejpn.2017.12.004
ISSN: 1532-2130
1090-3798
DOI: 10.1016/j.ejpn.2017.12.004
Popis: Aim: Duchenne Muscular Dystrophy (DMD) is more than a muscle disease since there is a higher prevalence of neuropsychological comorbidities. Similarly, the prevalence of epilepsy is increased. Given the nowadays-increasing interest in brain-related comorbidities in DMD, this study aimed to evaluate the relationship between DMD, epilepsy, and associated neurodevelopmental disorders in an international sample of DMD patients.Method: Using a questionnaire-based study we investigated the occurrence of self/by-proxy reported brain-related comorbidities in a group of 228 DMD patients. We evaluated the presence of epilepsy and other brain-related comorbidities, but also the specific mutation in the dystrophin gene. With respect to epilepsy, all individually reported epilepsy cases as based on the questionnaire results including information provided on epilepsy treatment, EEG abnormalities, and a description of how a typical seizure would look like, were independently and blindly re-assessed by two external paediatric neurologists (Cohen's kappa of 0.85).Results: Based on the latter, 18 (7.9%) DMD patients were considered to have epilepsy. In patients with both DMD and epilepsy, certain other brain-related comorbidities (i.e. attention deficit hyperactivity disorder, obsessive compulsive disorder, anxiety disorders and sleep disorders) were significantly more prevalent.Conclusion: This study is supportive of a high occurrence of epilepsy and other brain-related comorbidities in DMD. Furthermore this study shows for the first time that the frequency of some of these disorders appear to be further increased when epilepsy is present next to DMD. As this study is limited by the self/by proxy setup and the lack of response rates, future studies should elucidate the true incidence of the (triangular) cooccurrence between epilepsy, neurodevelopmental deficits, and DMD. (C) 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
Databáze: OpenAIRE
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