washington workshop

From :Dr.G.Shashikala , India Comments on the new definition of Cerebral Palsy both on original definition and correspondence Dear Sir I am following the publication of the proposed new definition and classification of Cerebral Palsy (1) in DMCN with great interest. Subsequent commentaries (2) and communications (3) also have thrown up lot of interesting perspectives. At the outset this effort of the special group really needs to be complimented because it has considered the many controversies in the definition of Cerebral Palsy rationally and has widened the scope of a newer definition contributing to a better understanding of this disorder. I particularly compliment the inclusion of seizures and behavior as a part of the definition itself so that the conflicting management options after a dual developmental diagnosis are minimized. This should help better communication to families. However I feel a few of the wordings probably need to be changed in the definition itself rather than explain their usage in the annotation: 1.There is a great scope for misunderstanding the term “Non progressive” as many of the critics have pointed out. Why not clarify as “Non progressive Cerebral disturbances with often changing and dynamic clinical manifestations”.(4) 2.Clarifying the term development, the authors seem to suggest that a diagnosis of cerebral palsy should be done before 18 months of age but do not specify the earliest age for diagnosis or the upper age limit. This is one of the problems many of us face in the developmental surveillance of high risk infants. How early do we start using the term Cerebral Palsy ?. The prevalent terms like Static Encephalopathy with gross motor developmental delay do not really appear meaningful to parents. Should we use terms like ”Suspect Cerebral Palsy / Impending Cerebral Palsy / Established Cerebral Palsy / Non disabling Cerebral Palsy” which have different connotations to different professionals. 3.The etiology of Cerebral Palsy in developing countries would substantiate the requirement for specifically mentioning the upper age limit (2). We find the natural history of motor impairment practically being the same in pre term cerebral diplegia and the ones acquired due to infections in the early childhood. 4.Progressive musculo – skeletal pathology should be mentioned as one of the accompaniments (3) not only because it causes the greatest activity limitation but also because recent research seems to suggest that the spastic muscle of Cerebral Palsy is structurally quite different fro the ones due to chronic denervation changes (5). It is also necessary that the definition specifies and retains Gross Motor Impairment as the dominant and earliest marker as in the Mutch definition to differentiate from other developmental disabilities. 5.It would be highly discriminative if we exclude children without activity limitation from the definition. What term should we use for children belonging to the GMFCS- I class with normal intelligence, who may not have any limitations in ADL excepting slow walking speed and decreased ability for sports (2) 6.I personally feel that the term behavior should also include specific learning disabilities like spelling mistakes and slow hand writing which are far more common than Autism as these are direct consequences of motor impairment. 7.In the end, I am just suggesting an approach which I have followed for the past two decades as a developmentalist, which was also presented during my lecture to the International Committee at the LA meeting. Why not we practice an axial system of diagnosis instead of calling the conglomeration of findings as classification as follows ? Axis 1- Etiology Axis 2- Timing of Cerebral Insult Axis 3- Tone and Anatomical Distribution Axis 4- Radiology and Imaging Axis 5- Electrical- Seizure, EMG studies etc Axis 6- Comorbidities Axis 7- Psycho Social and Functional Such an approach will immensely help in comprehensive communication to families to help their coping with the problem and to prioritize multi disciplinary management strategies, which are the goal of services with a family centered approach. If all the changes suggested by the critics are incorporated, obviously the revised definition will be a long one. A problem I foresee is how do we popularize this definition among the therapists and pediatricians ?. Can the group suggest some follow up action that can be adhered to by all countries ?. References : 1.Rosen Baum. P, Dan B, Leviton A, Paneth N, Jacobsson B, Goldstein M, M Bax (2005)- Proposed definition and classification of Cerebral Palsy, DMCN 47 No.8, Pg 571-576 2.Sunanda Kolli Reddy, Definition and classification of Cerebral Palsy- Commentary, DMCN 47 No.8 Pg 508-509 3.The Australian Cerebral Palsy Register – Presented by Nadia Badawi et al, Keith Edwards et al, Michael deLacy et al, Peter Flett et al, Matthew Sealy et al, Dinah Reddihough et al, Eve Blair et al- Letters to the editors Proposed new definition of Cerebral Palsy does not solve any of the problems of existing definitions – DMCN 2006 48 No. 1 Pg 78 4.H. Kerr Graham – Absence of reference to progressive musculo skeletal Pathology in the definition of Cerebral Palsy- Letters to the editor DMCN – 2006 48 No.1 Pg 78-79 5.Jared R H Foran, Suzanne Steinman, Iiona Barash, Henry G Chambers, Richard L Lieber- Structural and mechanical alterations in spastic muscle – DMCN reviews 47 No.10 Pg-713-71 Senders Address Dr(Mrs)G.Shashikala, MD Developmental Neurologist Member, Executive Committee, Indian Academy Of Cerebral Palsy Nagpur, India E-Mail Id : drgshashikala@rediffmail.com

02/02/2006 11:14:44

Alas, your first attempt at producing a new definition for CP – as published in DMCN (Issue 08 - august 2005) – missed the target that is if the aim was to provide a tool for intervention strategies, which seek to improve the possibilities for participation and activity for children with CP. Up-to-date knowledge on the nature of a brain damage indicates that a congenital brain damage will not be sufficiently understood if only looked upon from the angle of motor symptoms. Outside the rather small world of CP scientists seem to have a consensus of opinion that a brain damage, which is rooted in the early development of the brain, will involve and affect the overall functioning profile of the person - with individual traits and features of course. Furthermore it seems evident that cognition – here understood as the processes that control memory, perception and attention – is the basis of human behaviour including movement. The ability of walking should be grasped as a cognitive skill. The brain is involved – even in automated movements like walking – as it has been shown that there are no automated movements on spinal level in humans. Equally abilities like imaging a scent or recalling a sound are known to involve the motor area of the brain. In other words the motor behaviour is not a stand-alone function. Therefore when you diagnose a motor disability you automatically also diagnose cognitive deficits. The task is to assess and document the affect on the overall individual functioning profile. These considerations should be reflected in a modern definition of CP. As Brian Neville so shrewdly phrased it in an introduction lecture in Bled, 2001: “It seems crazy to throw out evidence just because we seem to have fastened on a motor disorder”. I propose that the next step in developing a new definition of CP takes another direction. In stead of trying to narrow down the definition with the risk of being caught in the trap of prioritizing I suggest that you open up the definition to cover the whole range of the overall individual functioning profile. This would give the neuro-paediatrician the basis for making use of a multi disciplinary assessment in order to document the specific deficits in the child diagnosed with CP and even of more importance it would provide the parents with the background and argumentation for demanding such an interdisciplinary documentation on behalf of their child. In this way the definition would work as a backbone for intervention strategies and not just be a tool of elimination or differential diagnosis within the neuro-paediatric clinic. I take the opportunity to propose a rough draft for a definition, which aims at opening up the comprehension of CP: Cerebral palsy signifies a complex syndrome caused by a brain damage predominantly occurring prior to birth during early development of the brain. The symptoms appear multi faceted and will affect the overall individual functioning profile of the child. The diagnosis is by tradition established on the motor symptoms of the condition, but it is important to assess and document the affects on the overall individual functioning profile of the child. This definition places the child with CP in focus and provides the basis for intervention strategies that aim at participation and activity, which must be the highest priority for the “abilitation” of children with cerebral palsy.

02/01/2006 13:17:00

Thanks for your endeavors in developing a new and comprehensive definition for CP. I think considering the following points would be essential in definition of CP. First, adding non-progressive to this definition because: 1) such as the term CP (that is explained in the definition), clinicians have known CP with its nature of being non-progressive; 2) from therapists point of view, managing and consulting non-progressive persons and their families are required different methods than progressive; and 3) even in epidemiological studies it is required to distinguish between these two disorders that their consequences and therefore their management planning, and policies are different. It is also imperative to distinguish between the non-progressive brain disturbances and probable progression limitations in movement and posture secondary to disorder of movement and posture. From my point of view, as a clinician, disorders of movement and posture resulting from brain disturbances in clients with CP are only a part of their problems in movement and posture. Because of the persons’ activity limitations, in a long term, further limitations in active and passive movements can develop in clients with CP especially when there is no or limited interventions. This point is required to be included in the definition to observe the life long influences of CP on the persons. This is also important for the insurance companies to cover the cost of interventions. Finally to have a comprehensive definition, it seems necessary to distinguish between problems directly resulting from brain disturbances and those indirectly caused by disorders in movement and posture. For example, sensory impairments can either caused directly by brain disturbances or indirectly by the limited experiences of hand movements, and incomplete employing of upper limbs in activities of daily living. Thanks again for developing this challenging subject.

15/02/2005 03:14:57

I presented this draft definition at a conference of physical therapists and care providers recently and received an interesting, and I think valid, comment. The comment voiced concern over the inclusion of the term "developmental" in the definition because third party payors (in the United States)would not pay for services if a condition is "developmental." While the refusal to pay such services because a condition is conceptualized as "developmental" is wrong (in my view), it is a reality in the United States that is unlikely to change anytime soon. So, including the term "developmental" in the definition could be a great disservice to patients and families in the United States.

14/12/2004 14:55:11

As a group of professionals working together in the field of CP in Europe since 1998, with one of the major aims to reach agreement on definition and classification of CP (available in print DMCN 2000;42:816-24 and on CDRoM as a reference and training Manual), we were very interested to read this proposition, and would like to react on the proposed document. 1. We are in favour of excluding the progressive disorders from the CP group for the following two reasons: – the pathogenesis of progressive disorders is quite different from the pathogenesis of brain lesions leading to CP - for a long time now progressive disorders are excluded from the CP definition, thus including them will not allow comparisons over time. It would be better to have in addition another group of disorders, entitled “progressive brain disorders” for example, rather than to enlarge the CP definition 2. Although we still recommend to ascertain children at 5 years of age, we recognise that some progressive disorders might be registered wrongly as CP, due to the delay required in some circumstances to confirm a diagnosis of progressive disorder. This constitutes a real difficulty when ascertaining CP cases. Within the SCPE common database we decided to flag such dubious cases. So we were able to quantify the cases associated with a named syndrome, according to the Smith’s book (5th edition). For some of them, these cases can be misdiagnosed “CP”, but overall they do not represent more than 2.5 % of all the CP cases. 3. We would like to insist that when offering a “new” definition of CP, it should absolutely be accompanied by a guideline describing the inclusion/exclusion criteria which have to be used for satisfying this definition. For example there must be recommendation about the peculiar situation of children who die early with clinical patterns similar to CP. And it must be much more precisely explained if progressive disorders have to be included or not, rather than “progressive disorders may be consistent with the proposed definition”. 4. We totally agree that the CP definition must be simple and rely on phenomenology criteria and not on aetiology criteria, since there are great variations in ability of performing diagnosis in different places, and also the techniques for these diagnosis are improving over time. In addition the CP definition must be valuable and logical for both epidemiologists and clinicians and, by implication, independent of the country in which the child lives. SCPE collaborative Group C. Cans, P Guillem, J Fauconnier (Grenoble, FR); C Arnaud, H Grandjean (Toulouse, FR); A Meberg, G Andersen (Tonsberg, NO); V McManus, D Coghlan (Cork, IE);H. Dolk, J Parkes (Belfast, UK); P Uvebrant, E Beckung, B Hagberg, G Hagberg (Göteborg, SW); O. Hensey, V Dowding (Dublin, IE); S. Jarvis, A Colver (Newcastle, UK); J Kurinzcuk, G. Surman, M Gainsborough, A Johnson (Oxford, UK); I. Krägeloh-Mann, U Petruch, R Michaelis (Tübingen, DE); MJ. Platt (Liverpool, UK), M. Topp, P Udall (Copenhagen, DK); MG. Torrioli, Giorgio Schirripa (Roma, IT); C Pallas (Madrid, SP), A Prasauskiene (Kaunas, LT), JW Gorter (Utrecht, NL), M Wichers (Tilburg, NL).

06/12/2004 17:06:14

Thank you for working on a revised definition of cerebral palsy. I have a couple of comments about the ICF terminology used. I am puzzled why you would refer to the 1980 definition of 'disability' rather than adopting the newer 2001 definition that encompasses challenges at the components of Body and Activty/Participation. Using old terminology only serves to confuse readers. If the new definition of 'disability' is used, then you do not need the term 'activity restriction'. This term also does not reflect the new ICF terms. A deficit at the component of activity is referred to as an 'activity limitation', not restriction. I am a bit concerned about the list of co-morbidities associated with the motor impairment - can a list ever include all the possible co-morbities that may be present? Finally, I wonder if the definition should mention that, although the brain lesion is not progressive, secondary problems can alter the motor presentation of cerebral palsy. It's always easy to find the 'holes' in a draft - I appreciate the time and discussion that must have gone into this endeavor. Thank you again for initiating this challenging task.

13/11/2004 17:30:14

Always great to experience new attempts defining cerebral palsy. From the workshop programme one find multifaceted perspectives represented - except maybe the patient / sufferer perspective. The core of cerebral palsy – from a sufferer’s perspective – is the fact that the brain damage underlying cerebral palsy involves and affects the global profile of functionality in the person. That seems to be the major reason for the fact that 70% of adults with cerebral palsy are not able to support themselves by competitive employment. The definition of cerebral palsy must recognize that the brain damage per se will involve some degree of perceptual sensory processing deficit and hence cognitive impairment - which by the way modern neuroscience also has shown to be the cause and effect relationship regarding the motor impairment. The far greater majority of brain damages in cerebral palsy have their roots in events taking place in the early development of the brain – often involving deep layer white matter lesions. The damages occur before the actual formation of the higher brain centres - not regarding the physiological aspect, but certainly the functional one in as much as the main synaptogenesis takes place from birth to approx. 5 y. Furthermore recent neuroscience results suggest that white matter lesions have impact on the function of higher brain centres regardless of their location. In addition the brain damage within cerebral palsy can be diagnosed by a psychological test battery showing the presence of a perceptual / cognitive aspect of the brain damage which must be acknowledged. Holding persons with cerebral palsy in respect one has to yield to the common knowledge of modern neuroscience and psychology and in so doing include the perceptual sensory processing impairments in the definition of cerebral palsy. Otherwise people with cerebral palsy (especially the children) will continue being misunderstood as intellectual impaired and moreover find it difficult to obtain self knowledge and self acceptance which are necessary to live an active participating life.

02/11/2004 12:58:25

Dear workshop participants, you have done a great job and we all hope that we will come to better definition of CP, especially with inclusion also perceptual problems which are maybe the biggest problem of persons with CP. Unfortunately in the description of history of attempts to find an adequate definition there is a mistake (look at the text on the bottom). Mrs. L.Mutch as an English native speaker made a report of the International workshop on island Brioni in the year 1990(16-20 September). 48 participants from 12 countries discussed about cerebral palsy and put together definition which is still the most adequate one. It was published in Dev Med Child Dev.(1992,34:547-55). Names of authors of the definition are in alphabetic order. Initiator, organiser and chair person of the Workshop was Milivoj Velickovic Perat, who has added to definition "early" term ("...anomalies of the brain arising in the early stages of its development"). Namely our brain is developing thru all of our life. **************************************** The heterogeneity of disorders covered by the term CP, as well as advances in understanding of development in infants with early brain damage, led Mutch and colleagues to modify the definition of CP in 1992 as follows: ‘an umbrella term covering a group of non-progressive, but often changing, motor impairment syndromes secondary to lesions or anomalies of the brain arising in the early stages of development.’

30/10/2004 23:23:46

October 28, 2004 Definition of Cerebral Palsy I would add the term non-progressive to the definition. “Cerebral palsy describes a group of developmental disorders of movement and posture causing activity restriction or disability that attributes to disturbances of the central nervous system that are non progressive. The motor impairment may be accompanied by other disorders such as epilepsy, deficits of sensations, cognition deficits, communication difficulties and behavior problems”. I do not feel that progressive disorders should be included in the definition. It might be appropriate to include in the discussion of the definition something as follows: The diagnosis of cerebral palsy is established by a history and physical. Commonly, one should perform serial history and physical examinations to insure that the lesion of the central nervous system is static. The patient’s clinical condition will change over time but these changes should be due to of problems such as contractures development or to changes in the muscle tone. It is unclear as to whether these changes in tone are manifestations of a progressive disorder or occurs as a result of the static encephalopathy. Research is obviously necessary to better understand the changes in muscle tone such as spasticity or dystonia. Changes in muscle tone as the pts age has never been explained to my knowledge. It seems to me that once a consensus definition has been developed, then it should be subjected to careful scrutiny at a future conference. Individual could bring their cases that do not fit the definition, yet carry the dx of CP. This was successfully done for Spinal Muscular Atrophy prior to the discovery of the Gene and was very helpful to better understand the definition of the SMA vs. other entities. Barry S Russman, MD

28/10/2004 16:45:19

We are concerned that reference to the “permanence” of cerebral palsy and that the primary lesion in the brain is “non-progressive” have been omitted from the new definition. We are also unconvinced by the inclusion of “activity limitations and disability” in the first sentence as this may not be judged consistently between clinicians and lead to the exclusion and under-ascertainment of children in GMFCS Level I. Functional limitations are a consequence not only of the primary impairment but also the co-morbidities already listed and the musculoskeletal deformities suggested by our Australian colleagues. We unequivocally endorse the inclusion of activity limitations in the definition but suggest this would be more appropriately included in the second sentence. Lastly, if activity limitations and disability describe the same construct then are both necessary?

28/10/2004 14:27:23

This is my suggested definition for CP. “Cerebral palsy describes a group of developmental disorders of movement and posture, causing activity restriction or disability, that are attributed to permanent disturbances occurring in the fetal or infant brain. The motor impairment may be accompanied by progressive musculoskeletal disorders and by epilepsy, hearing and/or visual disorders, and by impairment of sensation, cognition, communication and/or behaviour”. Whatever we might say as clinicians about the non-progressive nature of CP, and then which of the increasing number of genetic and metabolic disorders we may or may not be comfortable to include, the disturbances centrally are definitely permanent. However, it is important to add in the progressive musculoskeletal component if you are going to mention most of the other co-morbidities. For awhile, I disagreed with Kerr Graham on this issue, but I now think that he is right and that previously I was adopting too much of a neurological perspective and not enough of what confronts us in everyday clinical practice. Also, why give epilepsy such a prominent feature when really it is the musculoskeletal disorder that is more closely aligned to the motor impairment, and epilepsy is really no more or less important than visual disorders etc. in my experience.

25/10/2004 01:47:10

I will try to digest the several interesting comments that have been added, and comment further. For now I simply want to suggest here that the word 'infant' in the Oxford dictiopnary is far less specific in terms of age than the one that Dr. Goldstein has found in Stedman's.

22/10/2004 14:41:33

Annotation n°8. To my knowledge the terms “limitation of activity” and the “restriction of participation” are encountered more often in the ICF 2001 than the term “activity restriction”. Would it not bring confusion to use “activity restriction” instead of “activity limitation” ? the latter seems to me more appropriate to amplify the previous concept of “disability”.

13/10/2004 13:59:24

Beside consensual definition of CP we do need also consensual guidelines for inclusion/exclusion criteria. What about a child with severe hypotonia within the context of severe mental retardation, and/or what about a clumsy child ?

13/10/2004 13:55:24

I agree that, in order to afford properly, and “equally” in different countries or states, all the needs of children with motor deficiency, it can be useful to work on a larger group of disabled children with motor impairment, of which CP can be a “sub-group”. As an epidemiologist, and despite the difficulties in being sure that it is a non-progressive disorder at 5 years old, I rather like to maintain the exclusion criteria of progressive disorders from the CP group, this might help a lot when monitoring prevalence rates, and when classifying CP subtypes. The suggestion to include children with neuro-progressive disorders, based on common needs for these children, can apply also to children with spinal or neuro-muscular disorder or brain tumour…

13/10/2004 13:53:18

Whilst I understand that clinically a slowly progressive condition may be indistinguishable from a non progressive condition, the proposed new definition would include the severe neurodegenerative diseases and indeed brain tumours. Is this what we want?

07/10/2004 09:52:07

In the explanatory annotation sections 2 and 6, the word impairments should replace disabilities

07/10/2004 09:31:25

Epilepsy A question has been raised about the implied prominence of the placing of "epilepsy" in the second sentence of the Definition. Would it be better to revise the second sentence to read "--- accompanied by impairment of sensation, cognition, communication and/or behavior and/or by a seizure disorder".

04/10/2004 15:24:04

Stedman's Medical Dictionary (25th Edition)defines infant as "a child under 1 year of age; more specifically, a newborn." The present proposal would thus exclude an 18 month old who is recovering from a recent cerebral infarction. Athough a bit clumsy, I believe we need to expand the first sentence to be"----in the fetal, infant and early childhood brain". In the explanation, the point needs to be made that early childhood is meant to include the period 1-5 years of age, more specifically, 1-3 yerars of age.

04/10/2004 15:14:55

Stedman's Medical Dictionary (25th Edition)defines infant as "a child under 1nyear of age; more specifically, a newborn." The present proposal would thus exclude an 18 month old who is recovering from a recent cerebral infarction. Athough a bit clumsy, I believe we need to expand the first sentence to be"----in the fetal, infant and early childhood brain". In the explanation, the point needs to be made that early childhood is meant to include the period 1-5 years of age, more specifically, 1-3 yerars of age.

04/10/2004 15:14:22