March, 1961 P H Y S I O T H E R A P Y Page 3 Recent Advances in Cerebral Palsy with Special Reference to South Africa* BEN E P S T E IN , M.B., B .C h.(R and), M .R .C .P.(L ondon), D .C .H .(L ondon). Paediatrician, Pretoria. Reprint from S .A . M edical Journal, Vol. 34, 13th February, 1960. NOT long ago those in South A frica who interested themselves in cerebral palsy were considered cranks and impractical visionaries. I t was thought th a t to spend nnev on cerebral-palsied children was sheer waste, because othine could be done for them. B ut times have changed, d in the last 10 years an entirely new concept has been a" ted N o t only has the public attitude altered, thus pivins the parents new hope, but the C entral G overnm ent has recognized the justice o f the claim fo r support and the medical profession is devoting m ore time to the study and treatm ent o f this condition. In this country, centres for the treatm ent o f cerebral palsy have been established in Pretoria, Johannesburg, Cape Town and P ort Elizabeth. These centres (schools), subsidized by the D epartm ent o f E ducation, A rts and Science, provide treatm ent and schooling. They are all organizing residential facilities for platteland children. In addition, facilities are provided fo r som e types o f cerebral-palsied children in institutions such as M eerhof H ospital (Pretoria), the H ope Home (Johannesburg), U plands O rthopaedic C entre (Pieter­ maritzburg), the Elizabeth C onradie School (Kimberley), the School for V ocational Training (Kimberley), and the Open Air School (D urban). There is a clinic and hostel fo r in­ educable cerebral palsied children at Townsview, Johannes­ burg and a similar centre is being established in Pretoria in the near future. T o date, the to tal num ber o f children attending these institutions in South A frica is ab o u t 450. Developments are taking place very quickly in this field and it is im portant th a t the medical profession should keep abreast o f events. W H A T IS CEREBRAL PALSY ? Cerebral palsy is an unscientific term , and therefore difficult to define precisely. A year ago the International Study G roup on Cerebral Palsy, which m et in O xford, E ngland, discussed the definition for m any hours (4 different definitions were put forward) and did n o t reach unanim inity. There has also been difficulty in translating the term into A frikaans. It is only recently th a t serebraal verlamming was accepted as the m ost suitable term. In South A frica the following definitions and criteria has been adopted as standards for admission to a cerebral palsy school: Cerebral palsy is a term used to designate any abnorm al alteration o f movem ent o r m otor function arising from a defect, injury o r disease o f the nervous tissue contained within the cranial cavity. T he lesion m ay be localized or o r diffuse and it m ay be caused by factors arising before, during, o r after birth. Among the signs and symptoms which m ay be present are the following: M otor dysfunction, convulsions, speech defects, m ental retardation, behaviour disturbances o f organic origin, and sensory losses, particularly in hearing and vision. It is recommended th a t the non-m otor-handicapped brain-injured child, defined as follows by Strauss and Lehtinen,1 be considered under certain circumstances for admission to a cerebral palsy school: ‘A brain-injured child is one who before, during or after birth has received an injury to, or suffered an infection o f the brain. As a result o f such organic im pairm ent, defects o f the neurom otor system may be present o r absent. However, such a child m ay show disturbances in perception, thinking, and em o­ tional behaviour, either separately o r in com bination.’ T he criteria fo r admission are as follows: 1. All children suffering from cerebral palsy in term s o f the above definition. 2. These children m ust be able to benefit by the corrective and educational treatm ent provided at the school. 3. T he brain-injured child (Strauss and Lehtinen) may be adm itted provided he can fit into and benefit by the school program m e and is not aggressive. 4. Initial admission should be on a trial basis. In E ngland the ‘Little C lub’ has recently suggested the following definition:2 Cerebral palsy is a persistent b u t no t unchanging disorder o f m ovem ent and posture, appearing in the early years o f life, and due to a non-progressive disorder o f the brain, the result o f interference during its development. Persistence o f the infantile type o f m otor control such as m ay be seen in intellectually handicapped children, is not considered to be ‘cerebral palsy’. A t the Oxford conference considerable differences o f opinion on term inology and classification were voiced. Terminology and classification are im portant because scientific papers should be universally understood. D uring the dem onstration o f cases at the Radcliffe Infirm ary there appeared to be disagreement even on clinical findings am ong the em inent experts present. T he classification m ost com m only used is th a t o f the A m erican A cadem y o f C erebral Palsy, which is as follows: (1) Spasticity (quadriplegia, paraplegia and hemiplegia), (2) athetosis, (3) ataxia, (4) trem or, (5) rigidity, (6) atonic form , (7) mixed form. D IAGNOSIS E arly diagnosis is obviously im portant. There is, however, frequently an applicable and em barrassing passage o f time between the voicing o f the m other’s fears ab o u t the norm ality o f her child, and the specific diagnosis made by the doctor. Increasing knowledge is lessening this gap. I t is, I think, essential th a t a m edical practitioner who cannot answer a m other’s questions should no t hesitate to say, ‘I do no t know .’ T he comforting answer frequently given, ‘There is nothing to w orry a b o u t; your child will be quite well in two or three years’ tim e,’ is worse than useless. H onesty can only enhance the d octor’s prestige; the parents will appreciate the referral o f the patient to a doctor who has m ade a study o f child development. In practice it inevitably happens th at parents will, on their own initiative, seek another opinion if they get no helpful lead from their family doctor. W hat are the criteria for the determ ination o f early cerebral palsy? These are based on the studies o f child developm ent a t various schools. A rnold Gesell and M yrtle M cG raw , of the U .S.A ., and A ndre T hom as o f France, have contributed greatly to the understanding o f the abnorm al, through their study o f the norm al developm ent o f the infant. T he signs to look for are the ocular signs, the M oro response, the grasp reflex, the cardinal-points sign, the crossed-extension response, the righting reaction, the trunk-flexion reflex, and autom atic stepping. O ne m ust be careful not to draw definite conclusions on the results o f one exam ination, but * P a p e r p re s e n te d a t th e 4 2 n d S o u th .A frican M e d ic a l C o n g re s s (M .A .S .A .), E a s t L o n d o n , C .P ., S e p te m b e r-O c to b e r, 1959. R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) these results m ay serve to w arn one to keep a careful check on a suspected case; for example, a case b o m with a history o f an abnorm al pregnancy o r labour o r a case th a t has convulsions early in life. I t should be possible to diagnose most cases o f spasticity by the age o f 6 m onths, and o f athetosis by the age o f 1 year. Early diagnosis m ay be the greatest factor in the prevention o f deformities and o f the development o f bad posture and gait. A great deal o f attention is now being given to this problem. We can expect additions to o u r knowledge in the near future from the A ndre T hom as School in Paris, where Minkowski and Mme. Saint-Anne Dorgassies and others are studying the early life o f prem atures and full-time neonates along clinical, histological and electro-encephalo- graphic lines. T he nutritionists, the biochemists and the geneticists are adding their quota to o u r inform ation. A knowledge o f the disorders o f lipid metabolism , carbohydrate m etabolism (as in galactosaemia) o r amino-acid metabolism (as in phenylketonuria) m ay help to solve some o f the problems associated with abnorm alities o f the cerebral functions. T he R ussians have draw n attention to the im ­ portance o f enzymatic systems, such as cytochrom e oxidase and succinic-acid dehydrogenase, in m aintaining the n o r­ mality o f the brain. A great deal is being done, but much still eludes us. TREATMENT The treatm ent o f cerebral palsy is still in a state o f confusion. Perhaps the multiple problem s presented, and the great variability o f the physical and m ental disabilities o f the children, will always result in lack o f unanim inity. A num ber o f capable and intelligent individuals have w orked out different methods o f treatm ent, and have achieved some success. D ifferent schools present completely divergent views, and sometimes success claimed by som e cannot be repeated by others. Some use bracing, which others consider harm ful. Some use corrective surgery, to which others are opposed. Some use techniques for breaking down various types o f reflexes. M ost schools use a com bination o f different methods, depending o n the child’s condition. Phelps,3 one o f the w orld’s leading experts in cerebral palsy, enum erates the basic principles o f the various m eth o d s: 1. C onditioning to establish reciprocal m otion (Phelps). 2. R elaxation and m otion from the relaxed position (Phelps). 3. Increasing awareness o f contraction by resistive therapy (K abat). 4. Utilization o f residual patterns and o f pathological reflexes (Temple Fay). 5. Inhibition o f abnorm al reflex patterns (Bobath). 6. Stim ulation o f contraction and consequent relaxation o f antagonists (R ood). 7. M ethods o f Pohl, Swartz, D eavers, and Collis. Conditioning is ta u g h t fo r the purpose o f establishing fundam ental patterns o f motions autom atically acquired by the norm al baby and child. T he sequence is th at o f the norm al developm ent o f the infant. Relaxation and motion, depending on the teaching o f Jacobson. Progressive relaxation is ta u g h t by contraction and relaxation o f the extremities. N um erous techniques are used to achieve this purpose. Resistive therapy is utilized from the beginning o f treat­ ment. Instead o f therapy o f isolated muscles, patterns o f mass movement are utilized, achieving m axim um response in the muscles w ith each effort. Mechanical and m anual m ethods are used to produce resistance. Utilization o f residual patterns and pathological reflexes. Temple Fay has been using prim itive reflex patterns to enable movements to occur. H e has utilized the am phibian and reptillian type o f movem ent to achieve these results. Inhibition o f abnormal reflex patterns. D r. and Mrs. B obath have recently spent 6 weeks in Cape Town teaching therapists from all over South A frica their theories and techniques. A cerebral-palsied child develops m any varied Page 4 and abnorm al patterns which interfere with the co-ordinated use o f the trunk and extremities. Lesions at different levels of the central nervous system produce different abnorm al reflex reactions, and B obath techniques aim at inhibiting these reactions so th at the child can relax. D uring the period o f relaxation therapists, such as physio-, occupational or speech therapists, train the patient in their own sphere. Stimulation o f contraction and consequent relaxation o f antagonists. This m ethod was w orked o u t by. Miss M. S. R ood who operates on the two prim ary functions o f muscles, viz. (a) movem ent by contraction with reciprocal inhibition o f antagonists, and (b) holding sim ultaneous contraction of antagonists and agonists. While physiotherapy is the basic treatm ent, adjuncts to treatm ent are available, viz., (1) Bracing as practised by Phelps and D eavers in the U .S.A. and by C rosland in the U .K ., (2) orthopaedic surgery, (3) drugs, and (4) special adjuncts. Orthopaedic Surgery Surgery has a definite place in treatm ent. F o r some time heavy attacks have been made on various surgical techniques. Some o f them have been justifiably discarded, but others have \ been im proved on. O rthopaedic treatm ent m ay be operative or conservative. A great deal o f its activity should be devoted to the preven­ tion o f deformities. N o cerebral-palsy u n it can function adequately w ithout orthopaedic assistance, and w ithout the help o f specialists in physical medicine. T he day-to-day control o f treatm ent m ust be in their hands. T hose centres th at lack adequate help o f this type m ust inevitably suffer. G . A. Pollock, o f Edinburgh, an eminent orthopaedic surgeon in this field, visited South A frica in 1955, when he participated in a sym posium on cerebral palsy a t the Medical Congress in Pretoria. H e recom m ends4 that surgery should be used m ainly in spastics, but occasionally in athetoids. N eurectom ies such as Stoffel’s operation have fallen into dis­ repute and are rarely used nowadays. Pollock advises th at surgery should, in selected cases, be regarded as one o f the means o f facilitating and enhancing the effect o f conserva­ tive treatm ent, rather than as a m easure o f desperation to be used only when conservative treatm ent has failed. The com m on types o f operation practised a r e : (a) length­ ening o f the tendo Achilles, with capsulotom y if required, and (b) the Pollock slide operation on the gastrocnemius. Failure o f these operations have been due to (i) inadequate excision o r prem ature lengthening, (ii) lengthening o f the tendo Achillis when tightness is present in the gastrocnem ius alone, and (iii) the comm onest cause, failure to m aintain the correction obtained at operation by subsequent p ro ­ longed use o f braces o r splints and physiotherapeutic care. O perations have also been devised fo r talipes calcaneus, talipes valgus, talipes varus, pes cavus, hallux valgus, flexor deform ity o f the knees, and the num erous other deformities occurring in the upper and lower limbs. Neurosurgery has until recently had very little to offer. A t present hemispherectomy5 is occasionally done for hemi­ plegia if associated w ith fits. Irving C ooper’s w ork6 on chemo-pallidectomy for cases o f chorea-athetosis m ay open the way for m ore extensive attacks on the prim ary lesion in the brain. Perceptual Defects O ne o f the m ost im portant advances in the holistic con­ ception o f cerebral palsy is the increasing knowledge o f the perceptual difficulties o f these children. All cases o f cerebral palsy are brain-injured—hence the adm ission o f brain- injured children w ithout m o to r defect to o u r schools. The signs and sym ptom s depend on the area and extent o f brain involvement. Large num bers o f children have difficulties in which, ap art from m otor involvement, the power o f percep­ tion is im paired. T his handicap m ay give a w rong impression o f a child’s potentialities and, unless tackled along the correct pedagogic lines, will give rise to all kinds o f difficulties for th e child and the teacher. Fortunately, in South Africa March, 1961P H Y S I O T H E R A P Y R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) March, l t d P H Y 5 1 0 Wedh? h e V n e estCudlieclt S s and tney are engaged in research into it. implicati j d;ffi | ties m anifest themselves in different Per ,hP, most obvious being in the hemiplegic child who has W? ^ n ^ i s and is unable to differentiate between various as ereognosi gd hand M ore subtle are t he intellectual difficulties_the aphasic child, and the one who is unable to ™ a picture in its proper perspective and thereby to S e d a t e the significance o f figures, letters, and words. These may appear in the form o f m irror image reversed, o r H id e -d o w n As an illustration there is the child who draws a fare with the eyes outside the head. a Mhe Oxford conference7 it was pointed o u t th at agnosias resulting from inadequate practice o r experience should be distinguished from those resulting from specific cortical S n f fchieflv parietal). The form er could be im proved by special educational procedures m ore readily th an the latter. The phrase ‘body im age’ is becoming more frequently used, though it is not an exact reflection o f w hat is meant By ‘hodv image’ we m ean a constant state o f awareness o f the nnsition o f the body; this is fundam ental to norm al develop­ ment and behaviour. In the cerebral-palsied child its ultim ate effect is largely determined by the age at which treatm ent is started because if it is tackled early on one m ay be able to overcome some o f the difficulties. These children require a great deal o f movem ent and the opportunity to crawl and roll and feel their own bodies. I t is im portant for them to dress and undress and to do for themselves whatever they can This type o f direction is being extensively practised in Bristol by D r. G race E. W oods.8 Mme. Stella J. A lbitreccia,8 of Paris, the fam ous speech therapist, has draw n up detailed tests for diagnosis and a plan for treatm ent o f this very serious handicap. SOUTH A FRICA N W O R K In South Africa a fair am ount o f experimental and research work is being carried out, but not all o f it has been published. Significant contributions to our knowledge have been made by two very successful courses organized by the Cerebral Palsy Division o f the N atio n al C ouncil for the C are of Cripples in South Africa. One was on the ‘E ducation of cerebral palsy children,’10 and the other was on the ‘T h era­ pies in cerebral palsy.’11 Prof. B. F. N el,12 D irector, Child Guidance Clinic, University o f Pretoria, has published a book on Die Serebraalgestremde Kind, which is a study of the physical and psychological handicaps o f the brain- injured child. Several logopaedic students at the University o f W itwatersrand have handed in M. A. theses on the speech problems o f the cerebral-palsied child. T he m ost im portant contribution has ju s t been published by the N ational Bureau o f Educational and Social Research o f the D epartm ent of Education, Arts, and Science. T his is A Survey o f the Physical and M ental Status o f Cerebral Palsied European Children at School in the Union o f South Africa,1'3 a book w ritten by D r. C. H. de C. M urray, Inspector o f Psychological Services in the D epartm ent o f Education, A rts, and Science, and formerly Principal o f the Pretoria School for C erebral Palsy. This survey, which covers also adults w ith cerebral palsy, and which was undertaken prim arily at the request o f the N ational Council for the C are o f Cripples in South Africa, obtained inform ation, by m eans o f detailed questionnaires, about numbers, age, sex, hom e language, diagnosis, limbs involved, degree o f disability, personal traits, intellectual ability, and scholastic status. A survey o f this kind is very complicated and difficult to carry o u t because o f the num ber o f individuals on w hom one has to draw fo r inform ation; terminology, classification, and outlook on cerebral palsy varies, and correlation is not easy. D r. M urray has per­ formed a gigantic task ; his study is a classic, and bears favourable comparison w ith similar studies m ade elsewhere, r o r those o f us who are w orking in this field in South Africa jt is a source o f invaluable inform ation th at will guide us ln determining a course o f action for the increasing numbers ot affected children who, in the near future, will be leaving our schools. T here is a great deal o f statistical inform ation T H E R A P Y Page 5 th at has already been presented. O u t o f 380 cases a t the schools, 222 were males, and 158 females. (O n this basis hostel accom odation is being arranged). D r. M urray p u t these figures to a statistical test an d found th a t this sex inequality was n o t significant and was due to chance. H e also found th at in the hands o f a com petent psychologist the IQ rating, in spite o f all the difficulties in testing, was reasonably accurate; he subsequently confirmed this by the critical test o f children’s perform ances at school. One serious gap in South Africa is the lack o f facilities for the treatm ent o f the A frican cerebral-palsied child. We do not know how m any children are involved; m y own attem pts in P retoria to m ake a sam ple survey o f an area like L ady Selbourne proved a failure, even w ith the help o f an A frican social w orker. There were very few cases o f cerebral palsy to be found, owing, I think, to tw o factors, viz. (1) early death due to difficulties in rearing a child u n d er primitive living conditions, and (2) the custom o f Africans in the towns o f sending such children away to the country. T his custom is now a diminishing factor, for millions o f Africans have become detribalized and established in the urban areas. F o r those A fricans who present themselves for treatm ent no special facilities are available in the large u rb a n areas. A t Edenvale H ospital, Johannesburg, cases o f cerebral palsy are adm ittted to the wards for diagnosis and investigation. Physiotherapy and occupational therapy are begun and the m others are shown how to carry on with such treatm ent at home, and asked to report back regularly as out-patients to the D epartm ent o f Physical Medicine, under D r. Cyril Adler. A proper school for A frican patients has been started in U m tata by the Glen A vent R om an Catholic Mission, and there are prospects o f this becoming a big school with financial support from overseas. C O NCLUSIO N In this very superficial survey o f recent advance in cerebral palsy I have inevitably had to limit myself to a few aspects o f the problem ; the field is far too wide to be covered in one paper. I should like to conclude w ith an appeal to the medical profession in South A frica to accept the fact th a t rehabilita­ tion (or, as some people call it, habilitation) has become an im portant facet o f cerebral palsy. T he profession m ust, by the nature o f the condition, co-operate closely w ith n o n ­ medical personnel such as teachers, social w orkers, and em ploym ent officers, and w ith ‘near-medical’ personnel such as psychologists and physio-, occupational and speech therapists. The cerebral-palsy patient needs the active assistance o f paediatricians, orthopaedic surgeons, specialists in physical medicine, psychiatrists, neurologists, neuro­ surgeons, ophthalm ologists, and audiologists. T he general practitioner plays an im portant p a rt in this set-up because it is to him th a t the first approach to diagnosis will be made. T here are at least a thousand cases o f cerebral palsy am ong the E uropean section o f o u r population, and there m ust be m any thousands am ong the non-E uropean section; it is therefore im portant to teach our medical students ab o u t this condition. R E F E R E N C E S 1. S tra u s s , A . A . a n d L e h tin e n , L . E . (1 947): P syc h o lo g y a n d E ducation o f the B rain-Injured C hild, v ol. 1, p. 4. N e w Y o r k : G r u n e S tr a t to n . 2. M a c K e ith , R . C ., M a c k e n z ie , I . C . K . a n d P o la n i, P . E . (1959): C e r. P a lsy B u ll., 5, 23. . , 3. P h e lp s , W . M . In Illin g s w o rth , R . S. e d . (1958): R e c e n t A d v a n ces m C erebral P alsy, p. 252. L o n d o n : C h u rc h ill. 4. P o llo c k , G . A . In Illin g w o rth , op. c it.,3 p . 287. t 5. K x y n au w , R . A . (1950): J. N e u ro l, N e u r o s u r g . P sy c h ia t., 13, 243. 6. C o o p e r, I. S. (1956): T he N eu ro lo g ica l A lleviation o f P a rkin so n ism . S p rin g fie ld , III.: C h a rle s C . T h o m a s . 7. R e p o r t o n O x fo rd C o n fe re n c e (1958): C e r. P alsy B u ll., 4, 4. 8. W o o d s , G . E . (1958): Ib id ., 4, 10. 9. A lb itre c c ia , S. I. (1958): Ib id ., 4, 12. 10. E d uc ation o f C erebral P a lsy Children (1956): J o h a n n e s b u r g : N a ti o n a l C o u n c il f o r th e C a r e o f C rip p le s in S. A . 11. T herapies in C erebral P a lsy (1957): J o h a n n e s b u r g : N a ti o n a l C o u n c il f o r th e C a re o f C rip p le s in S .A . _ T 12. N e l, B . F . (1955): D ie Sereb ra a lg e stre m d e K ind. P r e to r i a : J . L . v a n 13. M u r r a y , C . H . d e C . (1959): A S u r v e y o f the P h y sica l a n d M e n ta l S ta tu s o f C erebral P a lsied European Children at S ch o o l in the U nion oj S o u th A fric a . P r e to r ia : N a ti o n a l B u re a u o f E d u c a tio n a l a n d S o cia l R e s e a rc h . R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. )