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Cerebral palsy—The current outlook
CEREBRAL
PALSY--THE
CURRENT
OUTLOOK
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well to become more knowledgeable in at least one of the handicaps which children are likely to develop, and none is more interesting than the opthalmological handicaps.
4.
The Need for Attractive Frames Finally, in any ideal ophthalmic service for children, consideration must be given to the provision of attractive frames for glasses where necessary. Up to the age of 10 the present free range of glasses is adequate if not attractive. The National Health Service Act, 1961, which came into force on 16th May, 1961, makes it possible to supply free lenses in certain frames bought by parents to children aged I0 or over, and either under 16 years or aged 16 and over, who are still receiving full-time school education. This regulation will do little to encourage adolescent children to wear their glasses if parents are unable or refuse to buy special frames for them. Furthermore, these new regulations only apply to older children receiving full-time school education at an establishment which is a school within the meaning of the Education Act, 1944. This interpretation excludes children over 16 years who are in fulltime attendance at a Technical College. Every effort should be made to provide fashionable frames, so that our young people will wear the spectacles prescribed. (1 wish to thank Dr. C. B..Crane, Medical Officer of Health and Principal School Medical Officer of York, for her help and guidance in writing this article and for permission to publish it.)
CEREBRAL PALSY--THE CURRENT OUTLOOK By
R. W. K I N D ,
M.R.C.S., L.R.C.P., D.P.H.
M.O.H. Wigston, Oadby and Market Harborough ; Assistant Medical Officer, Leicestershire C.C. A QUARTER Of a century ago the demonstration of a case of cerebral palsy was an interesting and unusual event which evoked no more emotional response than any other case of gross paediatric pathology. To-day this situation has altered. Cerebral palsy is the subject of its own pressure groups. The emotions of those personally involved with the problem are no longer concealed, are the subject of publicity and even exploitation. Two national bodies have come into being to concern themselves with the problem and the balance sheet of one of them testifies to the energies of its organisers on the one hand and the response of the public on the other. We work therefore in a climate of opinion in which it is increasingly
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necessary and increasingly difficult to maintain a global perspective of the problem. CLASSIFICATION Cases of cerebral palsy are classified on a basis of clinical manifestations° The following categories are widely accepted : - 1. Spasticity, characterised by an exaggerated stretch reflex, increased tendon jerks and an extensor plantar response, and further differentiated according to the three common forms of presentation into:-(i) Quadriplegia in which all four limbs are involved. (ii) Paraplegia in which the lower limbs only are affected. (iii) Hemiplegia where the involvement is of the arm and leg on one side. 2. Athetosis. 3. Ataxia. 4. Tremor, characterised by involvement of the whole body in a rapidly repeated and rhythmic tremor. 5. Rigidity, characterised by resistance of muscles to movement over their full range but associated with no exaggerated stretch reflex. INCIDENCE
Published figures suggest that the incidence of cerebral palsy varies between one and seven per 1,000 according to the country studied. The overall incidence is reported between one and two per 1,000 in Great Britain. The established incidence in Leicestershire where some attempt is being made to include all minimal cases lies between one and two per 1,000 and details are shown in the appendix. The reported incidence of the different categories shows a similar variation over a wide range. It is generally agreed that tremor and rigidity are the least common variants at under 1 ~ . Spasticity predominates, accounting for some 70 to 80~o of cases. Published analyses are commonly weighted by the inclusion of an excess of cases presenting features to which the investigator was directing particular attention. The appendix is no exception. For reasons which are not apparent cerebral palsy manifests itself in males more frequently than in females. DIAGNOSIS
Early recognition of cerebral palsy depends upon the early detection of departures from the normal sequence of developmental milestones and the acceptable age limits within which they occur. The problem bristles with diagnostic pitfalls, not merely because cerebral palsy can range from a minimal handicap which passes undetected until school age to a disability so gross as to be immediately apparent, but because disturbed maturation can be a manifestation of other conditions.
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PALSY--THE
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Frequent reappraisal of cases is essential. Frequent minimal cases can be found where the perinatal history is suggestive, where a transient spasticity is manifest in the early stages, where milestones are delayed but where by the age of two or three years no apparent disability remains and the child's development and intelligence are within normal limits for his family. On the other hand in some minimal cases the full appearance of athetosis is delayed. The following case histories illustrate the need for continued observation. Case o n e was first seen at the age of one month because her mother thought that 'she was stiffer than her other children'. The perinatal history was highly suggestive and it was evident that she had a minimal spasticity of the right arm and leg. She was kept under observation. Her milestones were delayed but she appeared to improve. At four years of age function was unimpaired, no spasticity was evident and her intelligence quotient (Terman-Merrill Scale) was 110. She was subsequently admitted t6 school where her progress has been normal. Case t w o was first seen at the age of two months at his general practitioner's request. The perinatal history contained nothing suggestive. Examination showed a well developed child with some adductor spasticity and generalised hypolonia of the trunk muscles. He was kept under observation. His milestones were delayed. He sat up at 10 months, walked with support at two years and walked alone at two years and six months. No further spasticity had been detected. At three years athetoid movements supervened to immobilise him. Case t h r e e was admitted to hospital at the age of nine days with an intra-cranial haemorrhage following a difficult instrumental delivery. No active treatment was called for and at one month he was discharged fit and well. He was observed at very frequent intervals up to two years and six months when all milestones had been passed early and he was alert and active. He was next seen at four years when he developed major epilepsy and had an obvious, if slight, right hemiplegia. Vision and hearing were unaffected, his spatial sense was disordered, his intelligence quotient was 100. It is hard to resist the observation that closer scrutiny should have brought the condition to light earlier. Case o n e is not exceptional. Among 1,200 consecutive babies presen~d for examination at infant welfare centres over a period of four years five similar cases came to light as did three other cases with a grosser disability. ASSOCIATED DISABILITIES Cerebral palsied children commonly exhibit additional disabilities arising out of their brain damage. These can be conveniently grouped under the following headings of which a numerical illustration appears in the appendix. I. Epilepsy. Convulsive episodes are a relatively common feature associ-
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ated with cerebral palsy. The proportion of cases reported to be so affected ranges from 14 ~o 40 %. 2. Sensory disabilities. (i) Defective vision. Serious defects of vision other than blindness include squint and abnormalities of muscle balance, nystagmus and herrdanopia. Hemianopia is seen more often in hemiplegics. The oiher ocular defects do not appear to correlate closely with the type of cerebral palsy. Refractive errors appear some two to five times commo~er than among normal children. (ii) Defective hearing. Defects of hearing are variously reported as affecting between 3 and 20 % of cases of cerebral palsy. Defective hearing for high frequencies is relatively commoner among athetoids particularly where a history of kernicterus or severe anoxia is established. A unilateral hearing loss is not uncommon and presents a greater handicap to the cerebral palsied than the normal child. (iii) Defective touch and position sense. Defective discrimination of touch, position sense and stereognosis are among the additional burdens borne by the hemiplegic. Athetosis and ataxia are not usually associated with discriminative failure of this kind. (iv) Laterality. In a world peopled with and planned by a preponderance of the dominant right hand the left-handed individual is at a slight disadvantage. To the cerebral palsied child this is disproportionately so. Crossed laterality is twice as common among hemiplegics as normal children and whilst it may present little ultimate problem to the normal child it is one more burden to those already handicapped. 3. Intelligence. The intelligence of cerebral palsied children varies over the range encountered in physically normal children. Only the mean is different and this deviated in a downward direction. Psychological handicaps. The cerebral palsied child responds to the climate of emotional feeling surrounding him as does any normal child. The parent of the afflicted child however cannot be regarded as an average parent. Parental attitude is characteristically anxious, apprehensive, shocked, demanding reassurance, failing to secure it, looking elsewhere for it, feeling guilt and ultimately reaching acceptance. In all ef this there may be increasing demands on the child of false expectations for his future. Expectations may be frustrated often and rejection of the child by the parent may be a sequel, commonly masked by overprotection. .
ASSESSMENT
By assessment is implied a procedure which views the child from all aspects
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and attempts some definition of prognosis in order to fit the available facilities for treatment and education to him in the best possible way. Assessment is continuous over a long period of time and is an exercise in co-operation. Paediatrician and physical medicine specialist may combine to establish the diagnosis and initiate treatment, defective hearing or vision may involve the audio!ogist or ophthalmologist. Establishing the extent of the intellectual handicap and emotional imbalance of the child calls for a psychologist more clinical than educational, a field in which an experienced school medical c,fficer might work to good effect. The occasional child presents features calling for consultation with the neurologist whilst the orthopaedic surgeon fulfils an important role in treatm,mt. Behind all of these stands the family's general practitioner who must be kept informed of every stage of progress. His vital task must be supportive to the parents for it is he who will be first involved when the parents become frustrated and dissatisfied with progress and wish for 'another opinion'. One individual should co-ordinate the work of all of these agents. It matters little who the co-ordinator is so long as he has an appreciation of the work of all specialities involved in the case and can ensure that they are subordinated to the needs of the child as a person and do not become ends in themselves. TREATMENT
It is not possible to consider the physical, educational, social and emotional aspects of treatment separately without obscuring the situation by over simplification. All of the~;e aspects are necessarily coincident in time and overlap in practice. Any failure to integrate them will result in a less satisfactory end result for the child. The physical disability may vary from the imperceptible to complete immobility. The associated disabilities may be negligible or maximal with defects of sight and hearing and epilepsy coexisting. The intellect may be relatively unimpaired or grossly defective. The home may be emotionally warm and the parents co-ope~ative and understanding or the child may be rejected. The personality of the child may vary as does that of normal children, drive and motivation will show their usual variety. The possible combinations ensure that no two cerebral palsied children are the same and that no one pattern of treatment will serve more than a few children. Physical treatment, including speech therapy, must recognise that nothing can be done to replace nerve ceils that have been destroyed and that no therapy can fill the functional gap which remains. Physical treatment therefore aims at developing the residual potentiality and concentrates on the achievement of as near normal function as is possible. The procedures which different schools adopt to further these common aims show a wide variation of apparent technique and principle. That all progressive authorities achieve much the same levels of progress reflects on the
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multi-factorial nature of cerebral palsy and suggests that the enthusiasm and personality of the therapist is not the least important of many variables. Defects of hearing and vision are so common among the cerebral palsied as to warrant routine consultation with the audiologist and ophthalmologist. The importance of even small departures from normality is such as to introduce another handicap. The following case illustrates this. Case four, an athetoid boy aged nine years with good mobility, moderately dysarthric speech, intelligence quotient 75, had been screened by pure tone audiometry on several occasions. He was variously reported as having no hearing loss or having no loss greater than 10 db. His teacher regarded him as behaving in school as a partially deaf child and reported that his attainments lagged behind his ability level by one year. He was provided with a hearing aid. His behaviour improved rapidly and in the following six months his progress in reading and number was equivalent to two years. On retesting his intelligence quotient had appreciated to 95. Squint is common among hemiplegics and quadriplegics. There is commonly an associated cerebral defect and stereoscopic vision fails to develop. Nystagmus may restrict visual efficiency whilst athetoids frequently fail in their ability to move the line of sight progressively in a controlled direction. Refractive errors occur more frequently than among normal children and correction is relatively more important. Particularly is this so in ataxic children where normal vision may be the principal pathway open to allow balance and muscular co-ordination to be improved. Defects of peripheral vision occasionally have the bizarre effect of making the score on a performance test critically dependent upon the relative positions of the child and the test material. Ascertainment and frequent reappraisal of the child's intelligence is very important. Firstly, because low intelligence levels exert a profound effect on physical development and can constitute a serious diagnostic dit~culty. Secondly, because the benefits of physical treatment are substantially related to the child's ability to co-operate. Low intelligence is a limiting factor. Thirdly, because the appropriate educational treatment depends upon it. The Terman-Merrill Scale is still the most reliable and most widely applicable. A proper understanding of the aims and principles of each test item allows for slight detail modifications of administration. Since no deviation of the required standard of response is involved the validity of the resultant score is not significantly reduced. It is of the greatest importance that the administrator of the test should be familiar with the responses of normal children to the many situations which can arise. Only in this way can the handicapped child's response be kept in perspective and discriminated. The mutual adjustment of the child and his parents has to be kept in mind at every stage of treatment. The intelligence of the parents may place a limit on the extent to which they can co-operate in therapy whilst their emotional
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involvement with the child may restrict his progress. These factors can be decisive when the question of day or residential school arises. Case five was a very attractive girl aged four years. She had a limited quadriplegia. Intellectually handicapped (I.Q. 60) she is covertly rejected by her parents in favour of her normal twin. At home there is little co-operation either in therapy or in the provision of social stimulation. Admitted for intensive therapy she progresses well during the week only to show regression on Monday mornings following a week-end at home. A fully residential unit suited her needs much better. Case six is an athetoid boy aged nine years. He is over-protected by his mother whose husband has recently deserted her. She confides all her troubles to him. When he was first admitted his maladjustment was as great a handicap as his athetosis. His intelligence quotient when first seen was 45. As he has become better adjusted this has appreciated to 90. He shows a slight setback after each week-end at home and a pronounced regression after each holiday. Case seven is a paraplegic girl aged nine years. She has an intelligence quotient of 120, no additional handicaps and is socially and emotionally well adjusted. Her parents are highly co-operative and accept her disability. She has been admitted to a local junior school where her self-propelled chair allows her full mobility in single-story buildings. Educationally and administratively she presents little difficulty. Case eight is a mobile hemiplegic boy aged 10 years having an intelligence quotient of 65. He has very understanding parents who accept his limitations. His admission to school was delayed until the age of six years when he joined the five-year-old stream at his local infant school. At the age of eight .years he transferred to the junior school where, because classes are not strictly streamed by ability, he was able to fit into a suitable group. Socially and emotionally his development is commensurate with his normal coevals. From an educational point of view the disposal of the cerebral palsied follows initially much the same path as that of normal children. The first question to be decided is that of educability. The grossly defective leave no doubts. Only with regard to the doubtful and the definitely educable do arguments arise. Should they be admitted to day or residential schools? To schools exclusively for cerebral palsied or with normal children ? Or given home tuition ? Underlying all these questions is the more im~portant one . . . educated to what purpose ? The ideal purpose must be to become self-supporting, non-dependent, members of society. However, a majority of the cerebral palsied will never become self-sufficient. This majority will include many who are technically educable. Consequently a more limited objective must be aimed at, that of becoming as nearly normal socially, emotionally and culturally as limited iablities will permit.
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Cerebral palsied children irrespective of intellectual ability seem to resemble educationally subnormal children in displaying relative social and emotional backwardness. Many of them, for example, have no idea of how to play. The skills and abilities acquired by the normal child in playing with his fellows are commonly denied the handicapped child by lack of opportunity, lack of ability to participate and lack of ability to profit. It is necessary in the education of the cerebral palsied to recognise this and to allow time for much social education before learning readiness for more formal subjects is reached. The educational treatment received should be appropriate to the child's abilities, intellectual, sensory and motor. Ideally no cerebral palsied child should be educated alongside normal children unless it can be shown that no serious sensory loss is present, that motor function is adequate for the performance of work and that no significant maladjustment exists. All children require some individual attention, require to be recognised and accepted as individuals. Handicapped children require even more. This is plainly impossible in classes of 40. Hence the ordinary day school is useless for more than a very small percentage of cerebral palsied childrer~. The child of average or below average intelligence associated with sensory disabilities but reasonable motor function is better off in a school for educationally subnormal children where teaching techniques specifically applicable to such disabilities are more likely to be found and classes are smaller. A difficult disposal problem is posed by the child with a gross sensory disability and superior intelligence. He needs highly individual attention involving specialised techniques but carried out in a framework which allows him social contact with normal children. Under present arrangements he is likely to be found in an ordinary day school where remedial teaching is not available. Case nine was referred to a school medical officer at the age of 12 years. He had been admitted to his secondary modern school one term previously and on a basis of his junior school record had been placed in the D stream. His work was such that he remained at the bottom of this class. His form master and headmaster however expressed the view that in practical situations he showed superior intelligence but that his inability to write and his difficulty with reading were keeping him back. Inquiry revealed a suggestive perinatal history and examination showed an ataxic cerebral palsy with severe disturbance of spatial sense. His intelligence quotient was 125. Further inquiry showed that he had been absent from school on the dates of routine medical examinations and that neither his parents nor previous infant or junior school teachers had noticed anything wrong. The child with motor handicaps which render him substantially immobile will require continuous physical treatment. His progress will be determined to a large extent by his will to co-operate. Motivation can best be secured in a
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social or work setting. Therapy detached from real life situations is apt to be unproductive. Treatment should therefore be fully integrated with school work and social life. This presupposes a specialist establishment with provision for therapy within it. The suggestion that the cerebral palsied child can well share the provisions for physiotb.erapy in schools for the physically handicapped is less realistic than at first appears. Few of the more severely affected cases of cerebral palsy escape sensory losses. It is well recognised that such sensory losses impose limitations on learning and demand teaching techniques which are not necessarily to be found in schools catering for acquired physical defects. There is also the case of the child being cared for in an environment including many more severely disabled. The situation may then develop in a dull child where lack of motivation brings progress to a standstill. Case ten is an ataxic girl aged 10 years. She has very restricted mobility, When admitted to a residential unit for cerebral palsied children at the age of six years she could support herself with the aid of two sticks and could take four steps per minute. Her intelligence quotient was 65 and she showed some impairment of tactile and spatial sense. Her progress was slow but steady so that after three years she could walk well still taking support from two aids but could not balance with one or kick a ball. She was working in school up to her ability level and was socially advanced. Compared with her school fellows, physically and socially, she was less handicapped than any and at this stage her physical progress ceased and her educational attainments began to lag. It was felt that progress should have continued and that with adequate stimulation greater advances could be made. The decision was made to transfer her to a residential school for educationally subnormal children. Here continuous demands were made upon her to conform to a pattern of activity slightly ahead of her current ability. She rapidly progressed until after one month she walked at the speed of normal children, after two months could lay down the one stick she now used and mount a swing and get off again without losing balance and after three months was sufficiently sure of her balance to kick a ball. Educationally attainments are up to her ability level and her intelligence quotient remains 65. At a residential school the classroom work, therapy and play of the child can be integrated with his social life in a manner not possible elsewhere. Waking or sleeping he is in a controlled environment which can be adapted to his needs in a way that his home usually cannot. In his own home he is one member of a family all of whom have rights to parental attention and domestic order. Any one child can only receive an excess if some other member of the family is deprived. This deprivation, whether of another child or of a parent, characterises the home life of a majority of cerebral palsied children of the more severe grades. The weight of the physical and emotional burden of caring for the child is often realised and acknowledged by the parents only after they have agreed
.$70
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to his entering a residential unit or school. This acknowledgment is an important step towards realistic acceptance. The family must ultimately adjust to the child, to his disability and accept him as an individual in his own r i ~ t . There is much to be said for the social work so necessary in the home being a logical extension of the work of the school staff with the child and being carried out by them. The case for a residential school should therefore rest primarily on assessment of social and emotional grounds and have regard to the more complete treatment it provides. CONCLUSIONS
Cerebral palsy in other than minimal cases is an enduring disability. The afflicted can never be completely normal. It is important to appraise the prognosis and the ultimate limitations on the individual's life without sentimentality or prejudice. The extent to which the individual fails to defeat or circumvent his disability is the final measure of his personal handicap whilst the extent to which he fails to attain his potential is a measure of society's failure in its obligation to help. ACKNOWLEDGMENTS
I am indebted to Dr. G. H. Gibson, County Medical Officer, Leicestershire, for permission to quete current statistics and to Dr. W. J. Matheson, Paediatrician, and Dr. N. R. W. Simpson, Consultant in Physical Medicine for permission to refer to cases seen at their Cerebral Palsy Clinic at Leicester Royal Infirmary.
Male Fema:e Total
Category Quadriplegia ... Hemiplegia--right --left.. 91 Paraplegia ... T o t a l spastic ... Athetoid ... Ataxic ...... T o t a l cases
...
APPENDIX C e r e b r a l p a l s y in L e i c e s t e r s h i r e at 30th June, 1961. C a s e s a g e d 0 t o 16 years. ... 81 Aged 0 to 4 years ... ... 63 ,, 5 ,, 16 , , ...
33 I11
...
144
144
Total
.........
Classification a n d a s s o c i a t e d disabilities. A s s o c i a t e d disabilities N u m b e r o f cases D e f e c t i v e vision* Epilepsy Defective hearing 26 2 6 2 28 3 7 1 40 10 12 1 19 2 1 Nil 113 10 21
17 3 6
26 3 5
144 26 34 * D e f e c t s o f vision n o t including refractive errors.
4 l Nil 5
BOOK
REVIEWS
Incidence : 1.7 per 1,000 children aged 5 to 16 years. Educability : Pre-school age : Educable ...... 10 1neducable ...... 12 l'qot yet assessed ... 11 Total
......
371
School age : Educable Ineducable
33
w •
75 36
~
111
Total
Disposal o f School Age Educable : To a local residential unit for C.P . To residential schools for E.S.N . . To other residential schools . . . Receiving home tuition . . . . . . To normal day school . . . . . .
. . . . .
. . . . .
. . . . .
. . . . .
. . . . .
. . . . .
. . . . .
. . . . . . . . . . . . . . . . . . . . . . . . . . . . .
. . . . . . . . . .
. . . . .
. . . . .
10 11 4
. . . . .
1
49* 75
Total * Six o f these already due for transfer to residential school for E,S.N. Distribution of intelligence quotients of the school age educable. Range o f I.Q. Number of
Category
Quadriplegia ... Hemiplegiawright wleft.. Paraplegia ... Athetoid ... Ataxic . . . . . . Total
...
120 to 130
110 to 120
100 to 110
90 to 100
80 to 90
70 to 80
60 to 70
50 to U n d e r 60 50
1
1 1
1 -
2 . -
4 -
6 9 2 1
3 4 5 2
2 4 1 .
2 1 5 1
1 2 .
-
-
1
-
1
2
-
-
6
2
I
I
3
2
7
20
14
7
15
5
1
cases
Over 130
7 17 25 9 4
. 1
13
75
.
.
.
.
Book Renews FAM ILY WELFARE CENTRE. B y N A N C Y E. L I N G A R D , psychiatric social w o r k e r ; M A R G A R E T PLATT, M.B., ¢H.B., O.P.M., a n d B A R B A R A O L D rt A M, M.B., CH.B. P u b l i s h e d f o r p r i v a t e c i r c u l a t i o n by the F a m i l y W e l f a r e C e n t r e , M a n c h e s t e r . (Pp. 44. Price 5s. post free to m e d i c a l w o r k e r s . ) F r o m e a n d L o n d o n : Butler & T a n n e r L t d . , 1961. This i n t e r e s t i n g b o o k l e t describes the b e g i n n i n g s in 1948 a n d the activities o f t h e M a n c h e s t e r F a m i l y W e l f a r e Service. T h e service was established b y t h e late L a d y Jefferson with the valued support o f Dr. (now L a d y ) Platt and a psychiatric social w o r k e r a n d w i t h the active e n c o u r a g e m e n t o f D r . C. M e t c a l f e B r o w n , m e d i c a l officer o f h e a l t h o f M a n c h e s t e r , a n d with t h e s o m e w h a t belated s a n c t i o n o f the Ministry of Health.
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well to become more knowledgeable in at least one of the handicaps which children are likely to develop, and none is more interesting than the opthalmological handicaps.
4.
The Need for Attractive Frames Finally, in any ideal ophthalmic service for children, consideration must be given to the provision of attractive frames for glasses where necessary. Up to the age of 10 the present free range of glasses is adequate if not attractive. The National Health Service Act, 1961, which came into force on 16th May, 1961, makes it possible to supply free lenses in certain frames bought by parents to children aged I0 or over, and either under 16 years or aged 16 and over, who are still receiving full-time school education. This regulation will do little to encourage adolescent children to wear their glasses if parents are unable or refuse to buy special frames for them. Furthermore, these new regulations only apply to older children receiving full-time school education at an establishment which is a school within the meaning of the Education Act, 1944. This interpretation excludes children over 16 years who are in fulltime attendance at a Technical College. Every effort should be made to provide fashionable frames, so that our young people will wear the spectacles prescribed. (1 wish to thank Dr. C. B..Crane, Medical Officer of Health and Principal School Medical Officer of York, for her help and guidance in writing this article and for permission to publish it.)
CEREBRAL PALSY--THE CURRENT OUTLOOK By
R. W. K I N D ,
M.R.C.S., L.R.C.P., D.P.H.
M.O.H. Wigston, Oadby and Market Harborough ; Assistant Medical Officer, Leicestershire C.C. A QUARTER Of a century ago the demonstration of a case of cerebral palsy was an interesting and unusual event which evoked no more emotional response than any other case of gross paediatric pathology. To-day this situation has altered. Cerebral palsy is the subject of its own pressure groups. The emotions of those personally involved with the problem are no longer concealed, are the subject of publicity and even exploitation. Two national bodies have come into being to concern themselves with the problem and the balance sheet of one of them testifies to the energies of its organisers on the one hand and the response of the public on the other. We work therefore in a climate of opinion in which it is increasingly
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necessary and increasingly difficult to maintain a global perspective of the problem. CLASSIFICATION Cases of cerebral palsy are classified on a basis of clinical manifestations° The following categories are widely accepted : - 1. Spasticity, characterised by an exaggerated stretch reflex, increased tendon jerks and an extensor plantar response, and further differentiated according to the three common forms of presentation into:-(i) Quadriplegia in which all four limbs are involved. (ii) Paraplegia in which the lower limbs only are affected. (iii) Hemiplegia where the involvement is of the arm and leg on one side. 2. Athetosis. 3. Ataxia. 4. Tremor, characterised by involvement of the whole body in a rapidly repeated and rhythmic tremor. 5. Rigidity, characterised by resistance of muscles to movement over their full range but associated with no exaggerated stretch reflex. INCIDENCE
Published figures suggest that the incidence of cerebral palsy varies between one and seven per 1,000 according to the country studied. The overall incidence is reported between one and two per 1,000 in Great Britain. The established incidence in Leicestershire where some attempt is being made to include all minimal cases lies between one and two per 1,000 and details are shown in the appendix. The reported incidence of the different categories shows a similar variation over a wide range. It is generally agreed that tremor and rigidity are the least common variants at under 1 ~ . Spasticity predominates, accounting for some 70 to 80~o of cases. Published analyses are commonly weighted by the inclusion of an excess of cases presenting features to which the investigator was directing particular attention. The appendix is no exception. For reasons which are not apparent cerebral palsy manifests itself in males more frequently than in females. DIAGNOSIS
Early recognition of cerebral palsy depends upon the early detection of departures from the normal sequence of developmental milestones and the acceptable age limits within which they occur. The problem bristles with diagnostic pitfalls, not merely because cerebral palsy can range from a minimal handicap which passes undetected until school age to a disability so gross as to be immediately apparent, but because disturbed maturation can be a manifestation of other conditions.
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Frequent reappraisal of cases is essential. Frequent minimal cases can be found where the perinatal history is suggestive, where a transient spasticity is manifest in the early stages, where milestones are delayed but where by the age of two or three years no apparent disability remains and the child's development and intelligence are within normal limits for his family. On the other hand in some minimal cases the full appearance of athetosis is delayed. The following case histories illustrate the need for continued observation. Case o n e was first seen at the age of one month because her mother thought that 'she was stiffer than her other children'. The perinatal history was highly suggestive and it was evident that she had a minimal spasticity of the right arm and leg. She was kept under observation. Her milestones were delayed but she appeared to improve. At four years of age function was unimpaired, no spasticity was evident and her intelligence quotient (Terman-Merrill Scale) was 110. She was subsequently admitted t6 school where her progress has been normal. Case t w o was first seen at the age of two months at his general practitioner's request. The perinatal history contained nothing suggestive. Examination showed a well developed child with some adductor spasticity and generalised hypolonia of the trunk muscles. He was kept under observation. His milestones were delayed. He sat up at 10 months, walked with support at two years and walked alone at two years and six months. No further spasticity had been detected. At three years athetoid movements supervened to immobilise him. Case t h r e e was admitted to hospital at the age of nine days with an intra-cranial haemorrhage following a difficult instrumental delivery. No active treatment was called for and at one month he was discharged fit and well. He was observed at very frequent intervals up to two years and six months when all milestones had been passed early and he was alert and active. He was next seen at four years when he developed major epilepsy and had an obvious, if slight, right hemiplegia. Vision and hearing were unaffected, his spatial sense was disordered, his intelligence quotient was 100. It is hard to resist the observation that closer scrutiny should have brought the condition to light earlier. Case o n e is not exceptional. Among 1,200 consecutive babies presen~d for examination at infant welfare centres over a period of four years five similar cases came to light as did three other cases with a grosser disability. ASSOCIATED DISABILITIES Cerebral palsied children commonly exhibit additional disabilities arising out of their brain damage. These can be conveniently grouped under the following headings of which a numerical illustration appears in the appendix. I. Epilepsy. Convulsive episodes are a relatively common feature associ-
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ated with cerebral palsy. The proportion of cases reported to be so affected ranges from 14 ~o 40 %. 2. Sensory disabilities. (i) Defective vision. Serious defects of vision other than blindness include squint and abnormalities of muscle balance, nystagmus and herrdanopia. Hemianopia is seen more often in hemiplegics. The oiher ocular defects do not appear to correlate closely with the type of cerebral palsy. Refractive errors appear some two to five times commo~er than among normal children. (ii) Defective hearing. Defects of hearing are variously reported as affecting between 3 and 20 % of cases of cerebral palsy. Defective hearing for high frequencies is relatively commoner among athetoids particularly where a history of kernicterus or severe anoxia is established. A unilateral hearing loss is not uncommon and presents a greater handicap to the cerebral palsied than the normal child. (iii) Defective touch and position sense. Defective discrimination of touch, position sense and stereognosis are among the additional burdens borne by the hemiplegic. Athetosis and ataxia are not usually associated with discriminative failure of this kind. (iv) Laterality. In a world peopled with and planned by a preponderance of the dominant right hand the left-handed individual is at a slight disadvantage. To the cerebral palsied child this is disproportionately so. Crossed laterality is twice as common among hemiplegics as normal children and whilst it may present little ultimate problem to the normal child it is one more burden to those already handicapped. 3. Intelligence. The intelligence of cerebral palsied children varies over the range encountered in physically normal children. Only the mean is different and this deviated in a downward direction. Psychological handicaps. The cerebral palsied child responds to the climate of emotional feeling surrounding him as does any normal child. The parent of the afflicted child however cannot be regarded as an average parent. Parental attitude is characteristically anxious, apprehensive, shocked, demanding reassurance, failing to secure it, looking elsewhere for it, feeling guilt and ultimately reaching acceptance. In all ef this there may be increasing demands on the child of false expectations for his future. Expectations may be frustrated often and rejection of the child by the parent may be a sequel, commonly masked by overprotection. .
ASSESSMENT
By assessment is implied a procedure which views the child from all aspects
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PALSY--THE
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and attempts some definition of prognosis in order to fit the available facilities for treatment and education to him in the best possible way. Assessment is continuous over a long period of time and is an exercise in co-operation. Paediatrician and physical medicine specialist may combine to establish the diagnosis and initiate treatment, defective hearing or vision may involve the audio!ogist or ophthalmologist. Establishing the extent of the intellectual handicap and emotional imbalance of the child calls for a psychologist more clinical than educational, a field in which an experienced school medical c,fficer might work to good effect. The occasional child presents features calling for consultation with the neurologist whilst the orthopaedic surgeon fulfils an important role in treatm,mt. Behind all of these stands the family's general practitioner who must be kept informed of every stage of progress. His vital task must be supportive to the parents for it is he who will be first involved when the parents become frustrated and dissatisfied with progress and wish for 'another opinion'. One individual should co-ordinate the work of all of these agents. It matters little who the co-ordinator is so long as he has an appreciation of the work of all specialities involved in the case and can ensure that they are subordinated to the needs of the child as a person and do not become ends in themselves. TREATMENT
It is not possible to consider the physical, educational, social and emotional aspects of treatment separately without obscuring the situation by over simplification. All of the~;e aspects are necessarily coincident in time and overlap in practice. Any failure to integrate them will result in a less satisfactory end result for the child. The physical disability may vary from the imperceptible to complete immobility. The associated disabilities may be negligible or maximal with defects of sight and hearing and epilepsy coexisting. The intellect may be relatively unimpaired or grossly defective. The home may be emotionally warm and the parents co-ope~ative and understanding or the child may be rejected. The personality of the child may vary as does that of normal children, drive and motivation will show their usual variety. The possible combinations ensure that no two cerebral palsied children are the same and that no one pattern of treatment will serve more than a few children. Physical treatment, including speech therapy, must recognise that nothing can be done to replace nerve ceils that have been destroyed and that no therapy can fill the functional gap which remains. Physical treatment therefore aims at developing the residual potentiality and concentrates on the achievement of as near normal function as is possible. The procedures which different schools adopt to further these common aims show a wide variation of apparent technique and principle. That all progressive authorities achieve much the same levels of progress reflects on the
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multi-factorial nature of cerebral palsy and suggests that the enthusiasm and personality of the therapist is not the least important of many variables. Defects of hearing and vision are so common among the cerebral palsied as to warrant routine consultation with the audiologist and ophthalmologist. The importance of even small departures from normality is such as to introduce another handicap. The following case illustrates this. Case four, an athetoid boy aged nine years with good mobility, moderately dysarthric speech, intelligence quotient 75, had been screened by pure tone audiometry on several occasions. He was variously reported as having no hearing loss or having no loss greater than 10 db. His teacher regarded him as behaving in school as a partially deaf child and reported that his attainments lagged behind his ability level by one year. He was provided with a hearing aid. His behaviour improved rapidly and in the following six months his progress in reading and number was equivalent to two years. On retesting his intelligence quotient had appreciated to 95. Squint is common among hemiplegics and quadriplegics. There is commonly an associated cerebral defect and stereoscopic vision fails to develop. Nystagmus may restrict visual efficiency whilst athetoids frequently fail in their ability to move the line of sight progressively in a controlled direction. Refractive errors occur more frequently than among normal children and correction is relatively more important. Particularly is this so in ataxic children where normal vision may be the principal pathway open to allow balance and muscular co-ordination to be improved. Defects of peripheral vision occasionally have the bizarre effect of making the score on a performance test critically dependent upon the relative positions of the child and the test material. Ascertainment and frequent reappraisal of the child's intelligence is very important. Firstly, because low intelligence levels exert a profound effect on physical development and can constitute a serious diagnostic dit~culty. Secondly, because the benefits of physical treatment are substantially related to the child's ability to co-operate. Low intelligence is a limiting factor. Thirdly, because the appropriate educational treatment depends upon it. The Terman-Merrill Scale is still the most reliable and most widely applicable. A proper understanding of the aims and principles of each test item allows for slight detail modifications of administration. Since no deviation of the required standard of response is involved the validity of the resultant score is not significantly reduced. It is of the greatest importance that the administrator of the test should be familiar with the responses of normal children to the many situations which can arise. Only in this way can the handicapped child's response be kept in perspective and discriminated. The mutual adjustment of the child and his parents has to be kept in mind at every stage of treatment. The intelligence of the parents may place a limit on the extent to which they can co-operate in therapy whilst their emotional
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involvement with the child may restrict his progress. These factors can be decisive when the question of day or residential school arises. Case five was a very attractive girl aged four years. She had a limited quadriplegia. Intellectually handicapped (I.Q. 60) she is covertly rejected by her parents in favour of her normal twin. At home there is little co-operation either in therapy or in the provision of social stimulation. Admitted for intensive therapy she progresses well during the week only to show regression on Monday mornings following a week-end at home. A fully residential unit suited her needs much better. Case six is an athetoid boy aged nine years. He is over-protected by his mother whose husband has recently deserted her. She confides all her troubles to him. When he was first admitted his maladjustment was as great a handicap as his athetosis. His intelligence quotient when first seen was 45. As he has become better adjusted this has appreciated to 90. He shows a slight setback after each week-end at home and a pronounced regression after each holiday. Case seven is a paraplegic girl aged nine years. She has an intelligence quotient of 120, no additional handicaps and is socially and emotionally well adjusted. Her parents are highly co-operative and accept her disability. She has been admitted to a local junior school where her self-propelled chair allows her full mobility in single-story buildings. Educationally and administratively she presents little difficulty. Case eight is a mobile hemiplegic boy aged 10 years having an intelligence quotient of 65. He has very understanding parents who accept his limitations. His admission to school was delayed until the age of six years when he joined the five-year-old stream at his local infant school. At the age of eight .years he transferred to the junior school where, because classes are not strictly streamed by ability, he was able to fit into a suitable group. Socially and emotionally his development is commensurate with his normal coevals. From an educational point of view the disposal of the cerebral palsied follows initially much the same path as that of normal children. The first question to be decided is that of educability. The grossly defective leave no doubts. Only with regard to the doubtful and the definitely educable do arguments arise. Should they be admitted to day or residential schools? To schools exclusively for cerebral palsied or with normal children ? Or given home tuition ? Underlying all these questions is the more im~portant one . . . educated to what purpose ? The ideal purpose must be to become self-supporting, non-dependent, members of society. However, a majority of the cerebral palsied will never become self-sufficient. This majority will include many who are technically educable. Consequently a more limited objective must be aimed at, that of becoming as nearly normal socially, emotionally and culturally as limited iablities will permit.
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Cerebral palsied children irrespective of intellectual ability seem to resemble educationally subnormal children in displaying relative social and emotional backwardness. Many of them, for example, have no idea of how to play. The skills and abilities acquired by the normal child in playing with his fellows are commonly denied the handicapped child by lack of opportunity, lack of ability to participate and lack of ability to profit. It is necessary in the education of the cerebral palsied to recognise this and to allow time for much social education before learning readiness for more formal subjects is reached. The educational treatment received should be appropriate to the child's abilities, intellectual, sensory and motor. Ideally no cerebral palsied child should be educated alongside normal children unless it can be shown that no serious sensory loss is present, that motor function is adequate for the performance of work and that no significant maladjustment exists. All children require some individual attention, require to be recognised and accepted as individuals. Handicapped children require even more. This is plainly impossible in classes of 40. Hence the ordinary day school is useless for more than a very small percentage of cerebral palsied childrer~. The child of average or below average intelligence associated with sensory disabilities but reasonable motor function is better off in a school for educationally subnormal children where teaching techniques specifically applicable to such disabilities are more likely to be found and classes are smaller. A difficult disposal problem is posed by the child with a gross sensory disability and superior intelligence. He needs highly individual attention involving specialised techniques but carried out in a framework which allows him social contact with normal children. Under present arrangements he is likely to be found in an ordinary day school where remedial teaching is not available. Case nine was referred to a school medical officer at the age of 12 years. He had been admitted to his secondary modern school one term previously and on a basis of his junior school record had been placed in the D stream. His work was such that he remained at the bottom of this class. His form master and headmaster however expressed the view that in practical situations he showed superior intelligence but that his inability to write and his difficulty with reading were keeping him back. Inquiry revealed a suggestive perinatal history and examination showed an ataxic cerebral palsy with severe disturbance of spatial sense. His intelligence quotient was 125. Further inquiry showed that he had been absent from school on the dates of routine medical examinations and that neither his parents nor previous infant or junior school teachers had noticed anything wrong. The child with motor handicaps which render him substantially immobile will require continuous physical treatment. His progress will be determined to a large extent by his will to co-operate. Motivation can best be secured in a
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social or work setting. Therapy detached from real life situations is apt to be unproductive. Treatment should therefore be fully integrated with school work and social life. This presupposes a specialist establishment with provision for therapy within it. The suggestion that the cerebral palsied child can well share the provisions for physiotb.erapy in schools for the physically handicapped is less realistic than at first appears. Few of the more severely affected cases of cerebral palsy escape sensory losses. It is well recognised that such sensory losses impose limitations on learning and demand teaching techniques which are not necessarily to be found in schools catering for acquired physical defects. There is also the case of the child being cared for in an environment including many more severely disabled. The situation may then develop in a dull child where lack of motivation brings progress to a standstill. Case ten is an ataxic girl aged 10 years. She has very restricted mobility, When admitted to a residential unit for cerebral palsied children at the age of six years she could support herself with the aid of two sticks and could take four steps per minute. Her intelligence quotient was 65 and she showed some impairment of tactile and spatial sense. Her progress was slow but steady so that after three years she could walk well still taking support from two aids but could not balance with one or kick a ball. She was working in school up to her ability level and was socially advanced. Compared with her school fellows, physically and socially, she was less handicapped than any and at this stage her physical progress ceased and her educational attainments began to lag. It was felt that progress should have continued and that with adequate stimulation greater advances could be made. The decision was made to transfer her to a residential school for educationally subnormal children. Here continuous demands were made upon her to conform to a pattern of activity slightly ahead of her current ability. She rapidly progressed until after one month she walked at the speed of normal children, after two months could lay down the one stick she now used and mount a swing and get off again without losing balance and after three months was sufficiently sure of her balance to kick a ball. Educationally attainments are up to her ability level and her intelligence quotient remains 65. At a residential school the classroom work, therapy and play of the child can be integrated with his social life in a manner not possible elsewhere. Waking or sleeping he is in a controlled environment which can be adapted to his needs in a way that his home usually cannot. In his own home he is one member of a family all of whom have rights to parental attention and domestic order. Any one child can only receive an excess if some other member of the family is deprived. This deprivation, whether of another child or of a parent, characterises the home life of a majority of cerebral palsied children of the more severe grades. The weight of the physical and emotional burden of caring for the child is often realised and acknowledged by the parents only after they have agreed
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to his entering a residential unit or school. This acknowledgment is an important step towards realistic acceptance. The family must ultimately adjust to the child, to his disability and accept him as an individual in his own r i ~ t . There is much to be said for the social work so necessary in the home being a logical extension of the work of the school staff with the child and being carried out by them. The case for a residential school should therefore rest primarily on assessment of social and emotional grounds and have regard to the more complete treatment it provides. CONCLUSIONS
Cerebral palsy in other than minimal cases is an enduring disability. The afflicted can never be completely normal. It is important to appraise the prognosis and the ultimate limitations on the individual's life without sentimentality or prejudice. The extent to which the individual fails to defeat or circumvent his disability is the final measure of his personal handicap whilst the extent to which he fails to attain his potential is a measure of society's failure in its obligation to help. ACKNOWLEDGMENTS
I am indebted to Dr. G. H. Gibson, County Medical Officer, Leicestershire, for permission to quete current statistics and to Dr. W. J. Matheson, Paediatrician, and Dr. N. R. W. Simpson, Consultant in Physical Medicine for permission to refer to cases seen at their Cerebral Palsy Clinic at Leicester Royal Infirmary.
Male Fema:e Total
Category Quadriplegia ... Hemiplegia--right --left.. 91 Paraplegia ... T o t a l spastic ... Athetoid ... Ataxic ...... T o t a l cases
...
APPENDIX C e r e b r a l p a l s y in L e i c e s t e r s h i r e at 30th June, 1961. C a s e s a g e d 0 t o 16 years. ... 81 Aged 0 to 4 years ... ... 63 ,, 5 ,, 16 , , ...
33 I11
...
144
144
Total
.........
Classification a n d a s s o c i a t e d disabilities. A s s o c i a t e d disabilities N u m b e r o f cases D e f e c t i v e vision* Epilepsy Defective hearing 26 2 6 2 28 3 7 1 40 10 12 1 19 2 1 Nil 113 10 21
17 3 6
26 3 5
144 26 34 * D e f e c t s o f vision n o t including refractive errors.
4 l Nil 5
BOOK
REVIEWS
Incidence : 1.7 per 1,000 children aged 5 to 16 years. Educability : Pre-school age : Educable ...... 10 1neducable ...... 12 l'qot yet assessed ... 11 Total
......
371
School age : Educable Ineducable
33
w •
75 36
~
111
Total
Disposal o f School Age Educable : To a local residential unit for C.P . To residential schools for E.S.N . . To other residential schools . . . Receiving home tuition . . . . . . To normal day school . . . . . .
. . . . .
. . . . .
. . . . .
. . . . .
. . . . .
. . . . .
. . . . .
. . . . . . . . . . . . . . . . . . . . . . . . . . . . .
. . . . . . . . . .
. . . . .
. . . . .
10 11 4
. . . . .
1
49* 75
Total * Six o f these already due for transfer to residential school for E,S.N. Distribution of intelligence quotients of the school age educable. Range o f I.Q. Number of
Category
Quadriplegia ... Hemiplegiawright wleft.. Paraplegia ... Athetoid ... Ataxic . . . . . . Total
...
120 to 130
110 to 120
100 to 110
90 to 100
80 to 90
70 to 80
60 to 70
50 to U n d e r 60 50
1
1 1
1 -
2 . -
4 -
6 9 2 1
3 4 5 2
2 4 1 .
2 1 5 1
1 2 .
-
-
1
-
1
2
-
-
6
2
I
I
3
2
7
20
14
7
15
5
1
cases
Over 130
7 17 25 9 4
. 1
13
75
.
.
.
.
Book Renews FAM ILY WELFARE CENTRE. B y N A N C Y E. L I N G A R D , psychiatric social w o r k e r ; M A R G A R E T PLATT, M.B., ¢H.B., O.P.M., a n d B A R B A R A O L D rt A M, M.B., CH.B. P u b l i s h e d f o r p r i v a t e c i r c u l a t i o n by the F a m i l y W e l f a r e C e n t r e , M a n c h e s t e r . (Pp. 44. Price 5s. post free to m e d i c a l w o r k e r s . ) F r o m e a n d L o n d o n : Butler & T a n n e r L t d . , 1961. This i n t e r e s t i n g b o o k l e t describes the b e g i n n i n g s in 1948 a n d the activities o f t h e M a n c h e s t e r F a m i l y W e l f a r e Service. T h e service was established b y t h e late L a d y Jefferson with the valued support o f Dr. (now L a d y ) Platt and a psychiatric social w o r k e r a n d w i t h the active e n c o u r a g e m e n t o f D r . C. M e t c a l f e B r o w n , m e d i c a l officer o f h e a l t h o f M a n c h e s t e r , a n d with t h e s o m e w h a t belated s a n c t i o n o f the Ministry of Health.