Title: European Academy for Childhood Disability (EACD): Recommendations on the definition, diagnosis and intervention of developmental coordination disorder (long version)*
Abstract: These recommendations were approved at two consensus conferences in Maulbronn (Germany) (26/27 March 2010 and 15/16 July 2010) with representatives from the the German and Swiss medical and therapeutic societies listed below and supervised by the Association of the Scientific Medical Societies in Germany (AWMF, members comprising 154 specialty societies). The AWMF represents Germany in the Council for International Organizations of Medical Sciences (for further information see http://www.awmf.de). The key recommendations of the clinical practice guideline on developmental coordination disorder (DCD) for Germany and Switzerland are identical to the recommendations on DCD agreed upon by an expert panel initiated by the EACD. The recommendations have been discussed with the European Academy of Childhood Disability (EACD). The EACD considers the present Swiss–German guideline as recommendations for the definition, diagnosis, assessment, and intervention of DCD in other countries. The participants were as follows. Rainer Blank (Chair of the Scientific Committee of the EACD) Hans Forssberg (Chair of the EACD) The recommendations were approved by a European panel of experts at the EACD meeting in Brussels, 26 May 2010 and through further Delphi rounds. (in alphabetical order) J M Albaret (France), A Barnett (UK), R Geuze (the Netherlands), D Green (Israel/UK), M Hadders-Algra (the Netherlands), S Henderson (UK), M L Kaiser (Switzerland), A Kirby (UK), R P Lingam (UK), H Polatajko (Canada), M Schoemaker (the Netherlands), B Smits-Engelsman (the Netherlands), H van Waelvelde (Belgium), P Wilson (Australia) S Zoia (Italy). ‘Underlying mechanisms’: P Wilson (Australia); ‘Consequences’, ‘Comorbidity’, ‘Definition and assessment’: R Blank (Germany); ‘Treatment’: B Smits-Engelsman (the Netherlands) H Becker (Germany), R Blank (Germany), O Jenni (Switzerland), M Linder-Lucht (Germany), H Polatajko (Canada), F Steiner (Switzerland), R Geuze (the Netherlands), B Smits-Engelsman (the Netherlands), P Wilson (Australia) The full guideline process was consistently advised by international experts in the field: B Smits-Engelsman (Physiotherapist, the Netherlands); H Polatajko (Occupational therapist, Canada); P Wilson (Neuropsychologist, Australia); R Geuze (Clinical physicist/neuropsychologist, the Netherlands); The clinical practice guideline on DCD for Germany and Switzerland has been approved by representatives of the following professional societies (not yet confirmed by the boards of the associations). Neuropaediatric Society for German-speaking countries (lead society); German Society of Child and Adolescent Medicine; German Society of Social Paediatrics and Adolescent Medicine; German Society of Child Psychiatry and Psychotherapy; Swiss Society for Developmental Paediatrics; Forum Praxispädiatrie, Switzerland German Association of Occupational Therapists; Swiss Association of Occupational Therapists; Zentralverband Physiotherapie (Germany); Physiotherapia Paediatrice, Schweizerische Vereinigung der Kinderphysiotherapeutinnen; Motopädenverband (Germany) A Mundt (patient group representative from Selbständigkeits-Hilfe bei Teilleistungsschwächen eV [SEHT eV]) R Blank (Neuropaediatric Society for German-speaking countries) S Akhbari-Ziegler (Physiotherapia Paediatrice, Schweizerische Vereinigung der Kinderphysiotherapeutinnen) J Buchmann (German Society of Child Psychiatry and Psychotherapy) A Jagusch-Espei (German Association of Occupational Therapists) O Jenni (Swiss Society for Developmental Paediatrics, SGEP, Swiss Society of Paediatrics SGP) M Linder-Lucht, V Mall (German Society of Child and Adolescent Medicine) A Oberle (German Society of Social Paediatrics and Adolescent Medicine) R Schmid (Resident Paediatricians’ Association of Germany) J Seeländer (German Association of Physiotherapists) F Steiner (Forum Praxispädiatrie, Switzerland) H Trillen-Krayenbühl (Ergotherapieverband, Switzerland) R Werthmann (Deutscher Motopaedenverband) R Blank (Germany) M Haag (Germany) M Haag; Prof Dr Med Rainer Blank, Kinderzentrum Maulbronn, Knittlinger Steige 21, D-75433 Maulbronn, Germany. E-mail: [email protected] and [email protected] The clinical practice guideline was agreed on and written in March 2011. It is valid until the next revision, at the latest until March 2016. A revision is planned about every 3 years by the representative group and the international advisory board. In case of new knowledge or experience that have considerable influence on the recommendations of this clinical practice guideline, the representative group and, if necessary, the international advisory board will rapidly produce the latest information. This clinical practice guideline on developmental coordination disorder (CPG–DCD) for German-speaking countries, particularly Germany and Switzerland, is strongly in accordance with the European recommendations of the European Academy of Childhood Disability (EACD) from May 2010 (Brussels) and an international consensus, the International Leeds Consensus (2006).1 It was formed by a nominal group-consensus process chaired by an independent representative from the Association of the Scientific Medical Societies in Germany (AWMF). The AWMF represents Germany in the Council for International Organizations of Medical Sciences. The CPG–DCD was initiated by the Neuropaediatric Society for German-speaking countries. It funded the second and third consensus conference in Germany. The first consensus conference was connected with an international symposium in Maulbronn, Germany and funded by the Child Centre Maulbronn. The financial responsibilities were not undertaken by any other party. The development of the CPG–DCD took place between spring 2008 and autumn 2010. The systematic review of the literature related to the key questions was first performed in autumn 2008 and then updated in January 2010 (reviewing all relevant literature from 1995 to January 2010). The following panels were involved in the development of the CPG–DCD: (1) national experts in the field; (2) international experts and an advisory board; (3) national representatives of professional groups; (4) a patients’ representative from a parent organization. Because of a lack of research and recognized experts on DCD in German-speaking countries, it was considered necessary to involve a board of international experts. As DCD is variously defined in different countries, it was also necessary to initiate an international consensus to confirm and/or modify the Leeds Consensus. The CPG–DCD contains the essential elements of systematic guideline development published by the AWMF. The consensus was obtained in a formal nominative group process. This was based, wherever possible, on an evidence-based literature search. The recommendations were made in relation to expected costs and benefits, for example intervention methods using more sessions with the same outcome received lower recommendation levels than methods requiring fewer sessions. The goals of assessment and interventions were carefully analysed with respect to the International Classification of Functioning (ICF). The methodological process was in accordance with a previous report on an S3-guideline (an S3-guideline is the highest quality standard of evidence-based practice recommendations approved by the AWMF).2 The present document is the long version of the CPG–DCD. Further documents are a short version (German), a version for Parents and Teachers (English/German) and a pocket version (algorithm; English and German). As a large proportion of the target group are children below the age of 8 years, the intention to write a child version has not been implemented. The general goals of this guideline are the following: (1) to determine and prioritize key questions on aetiology, diagnosis and intervention; (2) to raise high-priority practice questions; (3) to provide knowledge on the best evidence-based practice; (4) to point out research gaps; (5) to define individual diagnostic and intervention strategies based on clinical decision rules and evidence-based knowledge; (6) to make recommendations for a variety of different disciplines and to define their roles within clinical practice; (7) to recognize an interdisciplinary approach with physicians of different disciplines and therapists; (8) to identify specific national aspects, for example concerning the use of the International Classification of Diseases, 10th revision (ICD-10) compared with the Diagnostic and Statistical Manual of Mental Disorders, 4th edition (DSM-IV); (9) to provide an effective implementation strategy of the guideline by involving all medical and paramedical organizations relevant in assessment and treatment; (10) to identify possible barriers for implementation; (11) to provide a basis for clinical training and for implementation in quality management systems. In addition, specific goals of the CPG–DCD are the following: to improve the identification of children with DCD; to increase the use of effective treatments and reduce the use of ineffective treatments; to decrease the burden of the disorder and increase quality of life; to improve performance of everyday activities and participation at home, school, and at leisure; to improve personal and environmental resources; to improve access to services, in particular healthcare services; to help clarify responsibilities and propose models of cooperation among the various relevant professionals, for example by defining clinical pathways; to help prevent long-term consequences of DCD, for example by timely, effective intervention; to raise community awareness of DCD. As with every CPG, the CPG–DCD is not a rule of what to do or how to do in a legal sense. It cannot be a basis for legal sanctions.3,4 The CPG–DCD has been developed on the basis of the methodological recommendations of the AWMF and the German Instrument for Methological Guideline Appraisal. The clinical practice guideline may be used by healthcare professionals involved in the care of children with confirmed or suspected DCD (physicians, therapists), and by parents and nursery nurses, teachers, or other educational professionals (the adapted version). To support the application of the CPG in practice, a short version of the guideline, a table of all recommendations with levels, a flowchart with links to the recommendations, and a version for parents, teachers, and nursery nurses will be provided (available from: http://www.awmf.org/leitlinien/detail/ll/022-017.html). The CPG–DCD should apply to children with long-standing, non-progressive problems of specific motor skill performance, not attributable to any other known medical or psychosocial condition. Children may suffer from motor problems for which the guideline does not apply such as cerebral palsy, neurodegenerative disorders, traumatic brain injuries, inflammatory brain diseases, toxic and teratogenic disorders, malignancies, any motor problem due to other diagnosed medical conditions that may explain the poor motor performance. Children with mental retardation are generally not identified as having DCD because of assessment difficulties (pragmatic reasons). These children, however, may also have symptoms of poor motor coordination. Therefore, general recommendations for treatment indications and specific intervention methods may also be applied to the group of children with mental retardation, though the research so far has excluded these children from evaluation. DCD is a frequently occurring disorder with estimates of 5 to 6% being the most frequently quoted percentage in the literature.5,6 It is a chronic disorder with considerable consequences in daily life. At least 2% of all children with normal intelligence suffer severe consequences in everyday living, and a further 3% have a degree of functional impairment in activities of daily living or school work.7 Nonetheless, DCD is largely underrecognized by healthcare and educational professionals.8,9 On the other hand, there are considerable costs for long-term treatment, with questionable efficacy. According to the ‘Heilmittel-Report 2008’, the treatment of ‘sensorimotor disorders’ ranked number one within occupational therapy interventions with 2.5 million therapy sessions (costing almost €125 million) in 2006 reported by the AOK, the largest health insurance company in Germany,10 alone. About €400 million are spent for sensorimotor therapy in occupational therapy.10 This is almost 50% of all occupational therapy interventions and over 90% of all occupational therapy sessions with children and adolescents under 15 years. There are several questions and issues concerning DCD. Major problems arise from the current lack of consensus on the following: definition and terminology (how to define, best name for the disorder); diagnosis and assessment (how to assess for diagnosis, how to monitor during development and treatment); epidemiology (how many diagnosed, undiagnosed cases); outcome and prognosis (what consequences, in which areas of everyday living and participation); underlying mechanisms (developmental and/or learning disorder, poor information processing, etc.); comorbidities (what to treat, barriers to treatment); treatment indication (when and what to treat); intervention methods (which, how long, how intensive). These questions were the reason for the development of this CPG. The authors of the guideline hope to achieve improvements in the definition (national and international), diagnosis, and assessment of DCD as well as in the treatment indication and specific intervention. Further, the CPG–DCD should help to increase professional attention to this area which is, so far, widely neglected in German-speaking countries. The research on DCD is extremely underdeveloped in these countries: for example, there have been almost no original papers in international journals in the past 10 years coming out of Germany. To ensure that the guideline is responsive to the expectations of the children and their parents, a parent organization for children with learning disorders took part in the entire guideline process (Annette Mundt, Parent support group: Selbstaendigkeitshilfe bei Teilleistungsschwaechen). The following expectations were identified: (1) more awareness and recognition of the problem by the community, healthcare professionals, nursery nurses, and parents; (2) improved access to services, particularly healthcare services; (3) establish a clear diagnosis (transparency of diagnostic criteria, explaining the diagnosis, and initiating the necessary examinations); (4) better information about therapeutic options and types of therapy for parents; (5) information about effectiveness of intervention with respect to (a) improvement of motor function, (b) improvement of performance in daily activities, (c) improvement of participation, particularly at school; (6) finally, parents expect information on how the guideline is implemented (knowledge translation). The guideline group decided to focus on three basic key questions. The definition of DCD was subject to expert consensus. For communication between experts, health professionals, and parents, it was regarded as important to develop a generally recognized definition of DCD based on the ICD-10 (DSM-IV, Text Revision [-TR] in countries where it is the legal basis11,12). The findings of impaired functions or underlying mechanisms were extracted from a systematic literature search. The impairment should reflect the levels of the ICF such as body function and structure (motor, sensory, cognitive function, emotional/affective function), activities of daily living (basic and instrumental), participation (home, school, and community), and personal and environmental factors. The question on impairment does not aim at specific clinical practice recommendations but to increase understanding of the disorder, its severity, and its natural course. Applicability and test criteria of assessment instruments were subject to a systematic literature search and, where not possible, were addressed by experts’ opinions and a consensus conference. The question of how DCD can be identified should be answered by examining the role of medical history and interview, questionnaires, clinical examination, and motor tests. Further, assessment instruments should be discussed with respect to daily living, school/leisure and the role of clinical compared with natural settings. The answer to how and when to measure progress should reflect levels of body function and structure (e.g. motor functions, sensory, cognitive functions, emotional/affective functions, language functions), and activities of daily living (e.g. self-care, academic performance) and participation (at home, school and community), acknowledging personal and environmental factors. The treatment efficacy should be answered by systematic evaluation of the literature and, where not possible, answered by a nominative group process during a consensus conference. As in the key question on assessment, the levels of the ICF should be considered as body function and structure (motor, sensory, cognitive function, emotional/affective function), activities of daily living (basic and instrumental) and participation (home, school, and community), and personal and environmental factors. Effectiveness should also be discussed with respect to efficiency (cost–benefit). Several further questions were of great interest but could only be addressed to some extent in this guideline. Which interactions do occur by treating comorbid conditions (e.g. pharmacological treatment with stimulants of children with attention-deficit–hyperactivity disorder [ADHD])? Are there barriers to access healthcare services or treatment services for DCD (e.g. parental education, language, cultural, geographic, socio-economic status, health services policies)? What are the views and opinions about DCD of parents, patients, and teachers? Based on the key questions, the identified main areas of interest for clinical recommendations are identification/diagnosis, treatment indication, and treatment outcome. Using a democratic group process (blind voting) the guideline group decided on the relevance (priorization) of target variables with respect to the systematic literature search (1, very important: critical for making a decision; 9, not important at all [e.g. surrogate, no evidence for correlation with hard endpoint]). Relevant target variables are shown in Tables I and II. Original papers addressing of key questions 2 (assessment) and 3 (treatment) were categorized according to the level of evidence using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) and Oxford systems. In contrast to intervention studies, an established grading system for the different types of diagnostic study does not exist. Therefore, the GRADE system and the Oxford definition had to be modified and adapted (see Table VII in Appendix I). In some studies the level of evidence (LOE) had to be adjusted according to specific criteria. The level of evidence was decreased in cases of serious (−1) or very serious (−2) limitations to study quality, important inconsistency (−1), imprecise or sparse data (−1), high probability of reporting bias (−1). The level of evidence was increased in case of consistent evidence from two or more observational studies with no probable confounders (+1), evidence of a dose response gradient (+1), all probable confounders would have reduced the effect (+1). The levels and strength of recommendations used is directly related to the level of evidence (Tables III and IV). Several recommendations are based on a formal consensus within a nominative group process, particularly those dealing with definition. Recommendations based on group consensus (good clinical practice [GCP]) are included in the guideline. A strong agreement (strong consensus ≥95%; if only 10 or fewer participants were present, ≥90% agreement) is marked as GCP++; a moderate agreement (consensus ≥75–95%; if only 10 or fewer participants were present, ≥90% agreement) is marked as GCP+. Current prevalence estimates for DCD range from 5 to 20%, with 5 to 6% being the most frequently quoted percentage in the literature.13 It is generally recognized that these children have problems with motor skills that are significant enough to interfere with both social and academic functioning.6 Kadesjo et al.6 found a prevalence rate of 4.9% for severe DCD and of 8.6% for moderate DCD in a population-based study of 7-year-old children in Sweden. The Avon Longitudinal Study of Parents and Children study found 1.8% of children aged 7 years had severe DCD, with another 3% defined as having probable DCD with consequences for everyday life.7 We note that epidemiological information is largely dependent on how strictly selection criteria are applied. DCD is more common in males than in females, with male:female ratios varying from 2:1 to 7:1.6,7 Although DCD is relatively common, it is still largely unrecognized by healthcare professionals and nursery nurses.8,9 Motor performance difficulties of children with DCD are often viewed as ‘mild’ and, thus, not warranting attention compared with the needs of children with more severe impairments such as cerebral palsy. DCD occurs across cultures, races, and socio-economic conditions. The disorder is idiopathic in nature, although several hypotheses for the cause of DCD have been recently proposed (see section 7.2). In the clinical practice and the scientific community, there are still many ambiguities in the definition and the diagnosis of DCD. Evidence suggests that DCD is a unique and separate neurodevelopmental disorder which can, and often does, co-occur with one or more other neurodevelopmental and neurobehavioural disorders. Commonly, these disorders include ADHD, specific language impairment, specific learning disabilities, autistic spectrum disorder (ASD) and developmental dyslexia or reading disability. Some of these comorbidities are so strongly associated with DCD that DCD has even been regarded as a part of these disorders (e.g. ASD and DCD is not allowed according to DSM-IV classification; furthermore, the concept of deficits in attention, motor control and perception14,15 includes aspects of ADHD and DCD). Because key question 1 relates to this topic, definitional recommendations are made based on a nominative group process. According to the ICD-10 (revised version 2007), DCD, called SDDMF, is defined as a ‘disorder in which the main feature is a serious impairment in the development of motor coordination that is not solely explicable in terms of general intellectual disability or of any specific congenital or acquired neurological disorder. Nevertheless, in most cases a careful clinical examination shows marked neurodevelopmental immaturities such as choreiform movements of unsupported limbs or mirror movements and other associated motor features, as well as signs of impaired fine and gross motor coordination.’ The definition excludes abnormalities of gait and mobility (R26–), isolated lack of coordination (R27–), and motor impairment secondary to mental retardation (F70–F79) or to other medical and psychosocial disorders. The definition of DCD according to ICD-10 requires that the diagnosis is not solely explicable by mental retardation or any specific congenital or acquired neurological disorder. DCD is included in the section ‘Learning disorders’ and the section ‘Motor skills disorders’ (315.4 Developmental coordination disorder). The term was endorsed in the International Consensus Meeting in London/Ontario, Canada, in 1994. DCD according to DSM-IV is defined by the following four criteria. Performance in daily activities that require motor coordination is substantially below that expected given the person’s chronological age and measured intelligence. The disorder may be manifested by marked delays in motor milestones (e.g. walking, crawling, sitting), dropping things, by ‘clumsiness’ and by poor performance in sports or poor handwriting. The disturbance described in criterion A significantly interferes with academic achievement or activities of daily living. The disturbance is not due to a general medical condition (e.g. cerebral palsy, hemiplegia, or muscular dystrophy) and does not meet criteria for a pervasive developmental disorder.* If mental retardation is present, motor difficulties are in excess of those usually associated with mental retardation. If a general medical (e.g. neurological) condition or sensory deficit is present, code the condition on axis III (DSM-IV). Looking at original papers, the term ‘DCD’ was used in 52.7%, ‘clumsy children’ in 7.2%, and ‘developmental dyspraxia’ in 3.5% of articles (see systematic review from January 1995 to December 2005 by Magalhaes et al.16). In 23.5% of the articles other terms were used. In the Leeds Consensus,1 the term DCD was favoured. The existence of subtypes of DCD is likely, but could not be consistently confirmed by research evidence (see, for example, review by Visser17). The Dyspraxia Foundation (UK) recommends the use of the term ‘developmental dyspraxia’.18 This term defines dyspraxia as ‘an impairment or immaturity of the organization of movement’ and in many patients there are associated problems with language, perception and reasoning. A distinction between developmental dyspraxia and DCD has been postulated.19 Indeed, a dysfunction in the process of forming ideas, motor planning, and execution can be found in DCD. However, the term ‘dyspraxia’ has not become recognized as separate entity or subgroup of DCD (see section 7.2).20,21 Another definition comes from Sweden. Gillberg et al.15 have argued for the presence of a syndrome called deficits in attention, motor control, and perception (DAMP). However, this concept has not become recognized outside Sweden. Non-verbal learning disability is believed by some to be a neuropsychological disability.22 Although it has been studied for the past 30 years,22 it has not yet been included as a diagnostic category in the DSM-IV-TR. Many characteristics associated with non-verbal learning disability are similar to those that describe other, more ‘established’ disorders, such as Asperger syndrome, specific learning disabilities, and DCD. At present, the DSM-IV criteria are better defined than the ICD-10 criteria. The Leeds Consensus group (2006) agreed to re-confirm the London consensus and accept the DSM-IV-TR11,12 as the most suitable set of diagnostic criteria that are currently available. The consensus of the guideline group also decided to use the DSM name DCD and their criteria. In Table V the official terminology for DCD is given as it applies to other languages. The term developmental coordination disorder (DCD) should be used to refer to children with developmental motor problems in countries which adhere to the DSM-IV-TR classification. In countries where ICD-10 has legal status, the term specific developmental disorder of motor functions (SDDMF) (F82, ICD-10) should be used. The term DCD is used because this wording is well recognized in the English literature. It is taken from the DSM classification. However, in several European countries, the ICD-10 has legal status. Thus, the terminology of the ICD-10 must be used in those countries. Accordingly, the term SDDMF is added in brackets throughout this document (for the purposes of countries using ICD-10 terminology). Moreover, the following recommendations were also related to the ICD-10. Where concepts differ between DSM and ICD-10, specific comments are provided (specific recommendations 2a and 6a, see Supporting Information, section 13.7). Motor performance that is substantially below expected levels given the child’s chronological age and appropriate opportunities for skill acquisition. The poor motor performance may manifest as (1) poor balance, clumsiness, dropping or bumping into things, or (2) persistent difficulty in the acquisition of basic motor skills (e.g. catching, throwing, kicking, running, jumping, hopping, cutting, colouring, printing, handwriting). Marked delays in achieving developmental motor milestones (e.g. walking, crawling, sitting) may be reported. The disturbance in criterion I significantly interferes with activities of daily living or academic achievement (e.g. self-care and self-maintenance, handwriting, academic/school productivity, pre-vocational and vocational activities, and leisure and play). An impairment of motor coordination that is not solely explainable by mental retardation. The disturbance cannot be explained by any specific congenital or acquired neurological disorder or any severe psychosocial problem (e.g. severe attentional deficits or severe psychosocial problems, e.g. deprivation). This CPG–DCD aims to minimize differences in interpretation and classification between ICD-10 and DSM-IV, because the disorders are considered to represent similar conditions. Criterion III is largely consistent with criteria C and D in the DSM-IV (the exception is the exclusion of ASD see recommendation 6). DCD (SDDMF) should not be diagnosed if (1) motor performance cannot be assessed by a motor test (e.g. because of mental retardation or a medical disorder) or (2) if, after a comprehensive assessment including clinical history, examination and consideration of teacher and parent reports, the motor dysfunction can be explained by another condition including a neurological or psychosocial disorder or severe mental retardation. In the comments of F82 (ICD-10), it is mentioned that some children with DCD (SDDMF) may show marked ‘neurodevelopmental immaturities’ such as choreiform movements of unsupported limbs or mirror movements and other associated motor features. According to the current literature and clinical practice experience, the roles of th