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 Diane Damiano 16 Olaf Dammann and Karl Kuban 17 Olof Flodmark 18 Floyd Gilles 19 H Kerr Graham 21 Deborah Hirtz 23 Sarah Love 24 John Mantovani 26 John McLaughlin 27 Greg O’Brien 28 T Michael O’Shea 29 Terence Sanger, Barry Russman, and Donna Ferriero 30 Other Current Definitions and Classifications Eve Alberman and Lesley Mutch 32 Eve Blair, Nadia Badawi, and Linda Watson 33 Christine Cans et al. 35 Future Directions Martin Bax, Olof Flodmark, and Clare Tydeman 39 John Mantovani 42 Lewis Rosenbloom 43 1 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. Peter Baxter 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 CP Cerebral palsy (CP) is now familiar to most health and social Definition and service professionals, as well as to many members of the gener- al public, as a physically disabling condition. In fact, although classification of CP only affects between 2 and 3 per 1000 live births, it is thought to be the most common cause of serious physical dis- cerebral palsy: a 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 historical perspective 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- Christopher Morris MSc DPhil, Department of Public Health, lications. However, what exactly the term ‘cerebral palsy’ University of Oxford, UK. describes has been debated for more than 150 years, and dis- cussions about how the different manifestations of CP can be Correspondence to Christopher Morris, MRC Special Training best classified continue to the present day. Fellow in Health Services Research, Department of Public Health, Old Road Campus, University of Oxford, OX3 7LF. Before 1900 E-mail: [email protected] 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 definition of a diagnosis identifies explicitly which cases the seminal work describing cerebral paralysis, and particu- are to be recorded under that term and, by implication, which larly the related musculoskeletal issues, was elucidated by an are to be specifically excluded. The definition is the basis for English orthopaedic surgeon named William Little in one of a planning treatment and for counting cases in a population. series of lectures in 1843 entitled ‘Deformities of the Human Classification within a diagnosis categorizes those cases with Frame’. Whilst his lectures focused on joint contractures and similar characteristics together and distinguishes those cases deformities resulting from long-standing spasticity and with diverse features apart. The design of a classification paralysis, Little clearly indicated that the cause of the spasticity system, for instance whether it is organized into nominal or and paralysis was often damage to the brain during infancy, ordinal categories, will vary depending on the concept being and specifically preterm birth and perinatal asphyxia (Little classified and intended purpose for which classification is 1843). Little also noted that behavioural disorders and being made. The most frequently cited definition of cerebral epilepsy were only occasional complications and not central palsy was published by Bax (1964) as ‘a disorder of posture to the condition. and movement due to a defect or lesion in the immature At about the same time, a German orthopaedic surgeon, von brain’. The label does however encompass a variety of Heine, was reporting similar clinical syndromes as a result of syndromes and some, therefore, prefer the term cerebral infections such as scarlet fever and vaccinations (von Heine palsies. 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 published discussion by conceding to the President of the causes. He noted that it was difficult to know whether later Obstetrical Society of London that for every ‘one (case) that problems resulted from birth trauma, as described by Little, depended on abnormal or premature labour there were or whether in fact there were predisposing factors that may twenty or more from other causes incidental to later life’. have caused these infants to have difficult births. He thought Sarah McNutt, an American physician, continued to raise the the task of separating congenital from acquired cases impos- profile of the risks of long-term disability arising from birth sible in some cases and generally unhelpful. Freud was aware trauma (McNutt 1885). Notably, the American Neurological that children with ataxic symptoms might require a separate Association admitted her as their first female member; but group, as became the case after the work of Batten (1903), the content of her lectures apparently made her unpopular but at the time of his writing he had not seen enough cases of with some eminent obstetricians whilst she was on a tour in non-progressive ataxia to be sure.