A Report: the Definition and Classification of Cerebral Palsy April 2006
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Executive Committee used this information and additional A report: the definition comments from the international community to generate a report on the Definition and Classification of Cerebral Palsy, and classification April 2006. The Executive Committee presents this report with the intent of providing a common conceptualization of CP for of cerebral palsy use by a broad international audience. Cerebral palsy (CP) is a well-recognized neurodevelopmental April 2006 condition beginning in early childhood and persisting through the lifespan. Originally reported by Little in 1861 (and origi- nally called ‘cerebral paresis’), CP has been the subject ofbooks Report Executive Committee: and papers by some of the most eminent medical minds of Peter Rosenbaum (Definition Panel Chair) MD, CanChfld the past one hundred years. At the end of the 19th century, Centre for Childhood Disability Research, Hamilton, Ontario, Sigmund Freud and Sir William Osler both began to contribute Canada. important perspectiveson the condition. From the mid-l940s, Nigel Paneth (Classification Panel Chair) MD, Department of the founding fathers of the American Academy for Cerebral Epidemiology, Michigan State University, East Lansing, MI, USA. Palsy and Developmental Medicine (Carlson, Crothers, Deaver, Alan Leviton MD, Neuroepidemiology Unit, Children’s Fay, Perlstein, and Phelps) in the United States, and Mac Keith, Hospital, Boston, MA, USA. Polani, Bax and Ingram of the Little Club in the United Kingdom, Murray Goldstein* (Co-Chair) DO, MPH, United Cerebral Palsy were among the leaders who moved the concepts and descrip- Research & Educational Foundation, Washington DC, USA. tions of CP forward and caused this condition to become the Martin Bax (Co-Chair) DM, FRCP, Division of Paediatrics, focus of treatment services, advocacy, and research efforts. Obstetrics and Gynaecology,Imperial College, London, UK. It has always been a challenge to define ‘cerebral palsy’, as documented by the number of attempts that have been made Panel Consultants: over the years. For example, Mac Keith and Polani (1959) Diane Damiano PhD PT,Washington University Department of defined CP as ‘a persisting but not unchanging disorder of Neurology, St. Louis, MO, USA. movement and posture, appearing in the early years of life Bernard Dan MD, PhD, H6pital Universitaire des Enfants, and due to a non-progressive disorder of the brain, the result Reine Fabiola,Universite Libre de Bruxelles,Brussels, Belgium. of interference during its development.’ In 1964, Bax report- Bo Jacobsson MD, PhD, Perinatal Center, Sahlgrenska ed and annotated a definition of CP suggested by an interna- University Hospital East, Goteborg, Sweden. tional working group that has become a classic and is still used. It stated that CP is ‘a disorder of movement and pos- *Correspondence to Murray Goldstein, UCP Research and ture due to a defect or lesion of the immature brain.’ Though Educational Foundation; Suite 700,1660 L Street Nvz: this brief sentence is usually all that is cited by authors, addi- Washington, DC, USA 20036. tional comments were added by Bax: ‘For practical purposes it is usual to exclude from cerebral palsy those disorders of posture and movement which are (1) ofshort duration, (2) due to progressive disease, or (3) due solely to mental deficiency.’ The group for which Bax was the reporter felt that this simple For a variety of reasons, the definition and the clawification of sentence could be readily translated into other languages cerebral palsy (CP) need to be reconsidered. Modern brain and hoped that it might be universally accepted. At that time, imaging techniques have shed new light on the nature of the it was felt that it was wiser not to define precisely what they underlying brain injury and studies on the neurobiology of and meant by ‘immature brain’, as any such definition might limit pathology associated with brain development have further services to those in need. Like its predecessors, this formula- explored etiologic mechanisms. It is now recognized that tion of the CP concept placed an exclusive focus on motor assessing the extent of activity restriction is part of CP aspects, and also stressed the specific consequences of early as evaluation and that people without activity restriction should opposed to late-acquiredbrain damage. Not formally included not be included in the CP rubric. Also, previous definitions have in the concept were sensory, cognitive, behavioral and other not given sufficient prominence to the non-motor associated impairments very prevalent in people with ‘disor- neurodevelopmental disabilities of performance and behaviour dered movement and posture due to a defect or lesion of the that commonly accompany CP, nor to the progression of immature brain’, and often significantly disabling. musculoskeletal difficulties that often occurs with advancing The heterogeneity of disorders covered by the term CP, as age. In order to explore this information, pertinent material was well as advances in understanding of development in infants reviewed on July 11-13,2004 at an international workshop in with early brain damage, led Mutch and colleagues to modify Bethesda, MD (USA) organized by an Executive Committee and the definition of CP in 1992 as follows: ‘an umbrella term participated in by selected leaders in the preclinical and clinical covering a group of non-progressive, but often changing, sciences. At the workshop, it was agreed that the concept motor impairment syndromes secondary to lesions or anom- ‘cerebral palsy’ should be retained. Suggestions were made alies of the brain arising in the early stages of development.’ about the content of a revised definition and classification of CP This definition continued to emphasize the motor impairment that would meet the needs of clinicians, investigators, health and acknowledged its variability, previously underscored in the officials, families and the public and would provide a common MacKeith and Polani definition; it also excluded progressive language for improved communication. Panels organized by the disease, a point introduced in Bax’s annotation. 8 Definition and Classification of CP In response to the emerging need to evaluate the status of more significant in different persons or at different life periods, information about cerebral palsy and revisit the language e.g. some aspects of the motor impairment, sensory loss, intel- presently used to describe it, an International Workshop on lectual disability, attentional difficulty, epilepsy, musculoskeletal Definition and Classification of Cerebral Palsy was held in dysfunction and many others maybe more prominent or more Bethesda, Maryland (USA), on July 11-13 2004, co-sponsored problematic at different stages of the life of a person with CP by United Cerebral Palsy Research and Educational Foundation in the USA and the Castang Foundation in the United Kingdom: References support was provided by the National Institutes of Health/ 1. Freud S. (1897) Die infantile Cerebrallahmung. In: Nothnagel H, editor. SpecieIIePathoIogie und Therapie,Bd IX,Teil 111. Vienna: National Institute of Neurological Disorders and Stroke and Holder. p 1-327. the Dana Foundation. The task of the participants (listing fol- 2. Osler W (1899) 7&eCerebral Palsies of Children.A CIinicaI Study lows) was to revisit and update the definition and classifica- for thelnffrmaryfor Nervous Diseases. Philadelphia: Blakiston. tion of cerebral palsy in light of emerging understanding of 3. Little Club. (1959) Memorandum on terminology and classification of ‘cerebral palsy’. (Mac Keith R, et al., editors) developmental neurobiology and changing concepts about Cereb Palsy Bull 1: 27-35. impairments, functional status and personal ‘participation’. 4. Bax MCO. (1964) Terminology and classification of cerebral palsy. Reassessment of the definition of CP was prompted by a host Dev Med ChiIdNeurol6:295-307. of factors: changes in delivery of care to children with disabil- 5. Mutch LW Alberman E. Hagberg B, Kodama K, Velickovic MV ities; recognition that children with slowly progressive inborn (1992) Cerebral palsy epidemiology: where are we now and where are we going?Dev Med Child NeuroI 34: 547-555. errors of metabolism can present with motor difficulties at times indistinguishable from those of children with nonpro- gressive disease; increased availability of high-quality brain What follows is: The Defrnition and C1 assifiwion of imaging to identify impairments in brain structure; acknowl- Cerebral Palsv. Aod 2006, an annotated explanation of edgment that developmental motor impairment is almost the terms used, and the thinking behind the choice of invariably associated with a range of other disabilities; and those words. This material was authored by the members of increased understanding about associated antecedents and the Executive Committee functioning in panels enriched with correlates of CP expertise from consultants and by comments and suggestions The Workshop participants agreed that CP as conceptual- from many reviewers responding to drafts provided to the ized previously had proved to be a useful nosologic construct, international community. The Definition and Classification but that previous definitions had become unsatisfactory. They of Cerebral Palsy, April 2006 document is offered for interna- underlined that CP is not an etiologic diagnosis, but a clinical tional consensus and adoption, with the intent of providing a descriptive term. Reservations were expressed about the exclu-