Instruments for the Assessment of Behavioral and Psychosocial Functioning in Duchenne and Becker Muscular Dystrophy; a Systematic Review of the Literature.

OBJECTIVE: This systematic review aims to provide an overview of instruments used to assess behavioral and psychosocial functioning of patients with Duchenne and Becker muscular dystrophy, as well as to review the psychometric properties and applicability of these instruments. METHODS: Five databases (Embase, Psyc.info, ERIC, Pubmed/Medline, and Cochrane) were searched from inception to June, 2018. Potential articles were rated by two independent reviewers. A predefined PROSPERO form (CRD42017074518) was used to extract data from included articles. RESULTS: Sixty-one instruments were used in 54 studies. The Child Behavior Checklist is commonly used, but it lacks disease specific psychometric information. Sixteen instruments that contained disease specific psychometric information were included for final evaluation. The results displayed three instruments that are potentially valid for screening of psychosocial problems: The Psychosocial Adjustment and Role Skills Scale 3rd edition, the Pediatric Quality of Life Inventory Generic module, and the Life Satisfaction Index for Adolescents with Duchenne muscular dystrophy. Appropriate instruments for screening of behavioral problems may be: the Strengths and Difficulties Questionnaire, the Generalized Anxiety Disorder-7 item questionnaire, and the Patient Health Questionnaire-9 item questionnaire. CONCLUSIONS: Further research on psychometric properties of screening instruments is crucial to ascertain a gold standard for clinical and research purposes. Meanwhile, for definite diagnostics purposes we recommend a multimethod, multisource, multisetting assessment in this high-risk population.

Clinical evaluation of language fundamentals in Rolandic epilepsy, an assessment with CELF-4.

BACKGROUND: In clinical practice, Rolandic epilepsy is in many cases associated with developmental language impairment. However, from the literature it is unclear exactly which domains are affected; A wide variety of investigations are reported that each provide a different representation of language performance in these patients. AIMS: The aim of this study is to compare performance on the language domains between children with Rolandic epilepsy and healthy controls. METHODS: Prospective study of children with Rolandic epilepsy compared to healthy controls. 25 children (mean age 136.6 months, SD 23.0) with Rolandic epilepsy and 25 age-matched healthy controls were tested on their language function using the CELF-4 (Clinical evaluation of Language Fundamentals, Dutch edition). The healthy control were not matched regard to other important factors, particularly educational attainment and co-morbidity. Expressive language, receptive language, language content, language structure and language working memory were tested. RESULTS: In children with Rolandic epilepsy, the core language score was significant lower compared with healthy controls. They scored specifically lower on the receptive language index and language content index (both p = 0.002). A trend towards decreased expressive language index was observed (p = 0.054). Language structure and language working memory were in the normal range. CONCLUSION: Language was found to be impaired in children with typical Rolandic epilepsy. Especially semantic language processing including receptive language and language content was significantly impaired. The common denominator of these functions is semantic language processing.

Subtypes of learning disabilities: neuropsychological and behavioural functioning of 495 children referred for multidisciplinary assessment.

Aim of the present study was two fold: (1) to evaluate the course of referring and diagnosing Learning Disabilities (LD) and the contribution of multidisciplinary assessment and (2) to describe characteristics of three LD subtypes: Attention with or without Motor function Disabilities (AMD), Verbal Learning Disabilities (VLD) and Non-Verbal Learning Disabilities (NVLD). Diagnostics, behavioural and neuropsychological data from 495 children aged 6-17 years were described. First, AMD and VLD was the most frequent LD. Multidisciplinary assessment could contribute to the diagnostic process of LD, especially in diagnosing uncommon LD and comorbidities. Secondly, behavioural ratings, information processing, attention regularity and visual-motor integration proved to be most sensitive in discriminating between the three LD subtypes. However, diagnosing NVLD requires additional developmental information. Multiple discriminant function analysis correctly classified 61.7% of a selection of the present sample into LD subtypes as diagnosed by the multidisciplinary team. It is believed that the three subtypes are clinically relevant and suggestions are made to test the present classification functions in an independent sample, preferably diagnosed using a structured diagnostic interview.