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| 1 The Definition and Classification of Cerebral Palsy Contents
Foreword Peter Baxter 2 Historical Perspective Christopher Morris 3 Definition and Classification Document Peter Rosenbaum, Nigel Paneth, Alan Leviton, Murray Goldstein, and Martin Bax 8 Workshop Presentations Allan Colver 15
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30 Other Current Definitions and Classifications Eve Alberman and Lesley Mutch 32
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35 Future Directions Martin Bax, Olof Flodmark, and Clare Tydeman 39
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Foreword This supplement is centred on the final version of the Report on the Definition and Classification of Cerebral Palsy from the group chaired by Murray Goldstein and Martin Bax. We have devoted a Supplement to it for several reasons, including the importance of the topic and the advantage of having a separate stand-alone section to use for reference. It also allows the Report to be seen in its context. This final version of the Report is based on the discussion paper published last year, which was accompanied by commentaries, 1–3 and followed by an extensive discussion on the Castang website (www.castangfoundation.net/workshops_washington_ pub- lic.asp) as well as correspondence in the Journal. 4 These comments have been taken into account in the revised version. It is fol- lowed by a section summarizing most of the presentations at the workshop in Bethesda in 2004 which provided the background to the present Report. At that meeting selected international experts discussed specific aspects. These are very informative and reflect a wide range of considerations and perspectives, both on the difficulties involved and on the value and use of classifica- tion in terms of diagnosis, prognosis, management, and clinical trials. The presentation by Krägeloh-Mann has since been expanded into a review of the role of neuroimaging in cerebral palsy (CP), which is published separately in the accompanying issue of the journal (DMCN 2007; 49: p 144–151). The Report is preceded by a paper giving a brief history of the concept of CP, which is later also covered by Gilles from a patho- logical perspective. In a subsequent section are three papers describing the definitions and classifications currently in use by the European (SCPE) and the Australian research groups, and those of Mutch et al., as it is instructive to compare the different meth- ods used in formulating these. The final section has brief articles looking forward to the implications of the report on clinical practice and the provision of health care. I hope that this Supplement will be useful. It illustrates the difficulties inherent in trying to agree what we mean by the terms we use and that a classification that suits one purpose, such as a diagnostic approach, may not always be ideal for others, such as therapy issues. Defining and classifying CP is far from easy, so the group who have produced the Report deserve applause. We do need a consensus that can be used in all aspects of day-to-day care and for future research on CP.
References 1. Bax M, Goldstein M, Rosenbaum P, Leviton A, Paneth N, Dan B, Jacobsson B, Damiano D; Executive Committee for the Definition of Cerebral Palsy. (2005) Proposed definition and classification of cerebral palsy, April 2005. Dev Med Child Neurol 47: 571–576. 2. Carr LJ, Reddy SK, Stevens S, Blair E, Love S. (2005) Definition and classification of cerebral palsy. Dev Med Child Neurol 47: 508–510. 3. Baxter P, Rosenbloom L. (2005) CP or not CP? Dev Med Child Neurol 47: 507. 4. Badawi N, Novak I, McIntyre S, Edwards K, Raye S, deLacy M, Bevis E, Flett P, van Essen P, Scott H, et al. (2006) Proposed new definition of cerebral palsy does not solve any of the problems of existing definitions. Dev Med Child Neurol 48: 78–79 (Letter). 2
Definition and classification of cerebral palsy: a historical perspective Christopher Morris MSc DPhil, Department of Public Health, University of Oxford, UK. Correspondence to Christopher Morris, MRC Special Training Fellow in Health Services Research, Department of Public Health, Old Road Campus, University of Oxford, OX3 7LF. E-mail: christopher.morris@dphpc.ox.ac.uk The definition of a diagnosis identifies explicitly which cases are to be recorded under that term and, by implication, which are to be specifically excluded. The definition is the basis for planning treatment and for counting cases in a population. Classification within a diagnosis categorizes those cases with similar characteristics together and distinguishes those cases with diverse features apart. The design of a classification system, for instance whether it is organized into nominal or ordinal categories, will vary depending on the concept being classified and intended purpose for which classification is being made. The most frequently cited definition of cerebral palsy was published by Bax (1964) as ‘a disorder of posture and movement due to a defect or lesion in the immature brain’. The label does however encompass a variety of syndromes and some, therefore, prefer the term
Cerebral palsy (CP) is now familiar to most health and social service professionals, as well as to many members of the gener- al public, as a physically disabling condition. In fact, although CP only affects between 2 and 3 per 1000 live births, it is thought to be the most common cause of serious physical dis- ability in childhood (Surveillance of Cerebral Palsy in Europe 2000). Historically, CP was predominantly studied in relation to the pathology and aetiology of the impairment. Discussion regarding the definition and classification of CP was first recorded in medical literature during the nineteenth century, predominately in French, German, and English language pub- lications. However, what exactly the term ‘cerebral palsy’ describes has been debated for more than 150 years, and dis- cussions about how the different manifestations of CP can be best classified continue to the present day. Before 1900 The quest to correlate brain lesions with their clinical mani- festation began with early French publications by patholo- gists debating the association of hemiplegia of the body with hemiatrophy of the brain identified by post-mortem (Lallemand 1820, Cazauvieilh 1827 [as cited in Ingram 1984]). However, the seminal work describing cerebral paralysis, and particu- larly the related musculoskeletal issues, was elucidated by an English orthopaedic surgeon named William Little in one of a series of lectures in 1843 entitled ‘Deformities of the Human Frame’. Whilst his lectures focused on joint contractures and deformities resulting from long-standing spasticity and paralysis, Little clearly indicated that the cause of the spasticity and paralysis was often damage to the brain during infancy, and specifically preterm birth and perinatal asphyxia (Little 1843). Little also noted that behavioural disorders and epilepsy were only occasional complications and not central to the condition. At about the same time, a German orthopaedic surgeon, von Heine, was reporting similar clinical syndromes as a result of infections such as scarlet fever and vaccinations (von Heine 1860). He cited the work of his compatriot Henoch, who had written his dissertation several years earlier, describing hemiple- gia in children (Henoch 1842). It has been suggested that it was actually von Heine, rather than Little, who first distinguished CP from the flaccid paralysis caused by poliomyelitis (Osler 1889, Bishop 1958). However, Little was known to have spent some years studying in Germany during the 1830s and it is possible that there was some cross-fertilization of ideas, although this is not formally recorded. Regardless, CP was known for many years after as ‘Little’s Disease’. In his best known work, published in 1862, Little expands on the association between a large number of his patients’ clinical presentation and their birth history as recalled by the family (Little 1862). Little differentiated between the congeni- tal deformities observed at the time of birth, such as falipes equinovarus, and the limb deformities that developed subse- quent to preterm, difficult, or traumatic births, due to what he termed spastic rigidity. He demonstrated his familiarity with the work of French, German, and Irish pathologists in constructing his theory. Little grouped the clinical presenta- tion of 47 cases as either: (1) hemiplegic rigidity affecting one side only, although lesser impairment of the apparently unin- volved limb was frequently observed; (2) paraplegia affect- ing both legs more than arms; and (3) generalized rigidity. Little showed careful consideration for his audience in the Historical Perspective Christopher Morris 3
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