Develop. Med. Child Neurol. 1975, 17, 18-25
Locomotor Prognosis in Cerebral Palsy E. E. BZeck
when the child was suspended erect. These signs proved to be inaccurate when children who had them failed to walk independently after several years of observation. Reciprocal motion of the lower limbs probably represented the persistence of automatic marching commonly observed in infancy (AndrC-Thomas et al. 1960), and the ability to stand during infancy was probably the presence of an abnormally strong positive supporting reaction, or ‘extensor thrust’-always an abnormal response (Fiorentino 1963).
Introduction The questions most frequently asked by parents of children who have slow development and cerebral palsy are: “Will my child walk?” and “When will he walk?” This study was conducted to answer these questions. As the study evolved, other questions bearing on the prognosis for walking emerged: ( I ) how does the prognosis affect planning the management of the cerebral palsied child and parental understanding; (2) how does the prognosis affect decisions for orthopaedic surgical procedures; and (3) how does the prognosis affect judgments on the efficacy of various proposed ‘treatments’ for cerebral palsy if walking is the criterion for success? A few studies on prognosis in cerebral palsy have been published. Denhoff et a/. (1956), Denhoff and Robinault (1960), Jones (1962), Paine (1962), and Cohen and Kohn (1963) reported various aspects of the problem. The most detailed long-term study was that of Crothers and Paine ( 1 959), who pointed out the good prognosis for ambulation in hemiplegics and the poor prognosis in patients who had an obligate asymmetrical tonic neck reflex. My interest in locomotor prognosis based upon the presence or absence of infantile reflexes was prompted by the failure of traditional ‘good’ signs: reciprocal motion of the lower limbs and spontaneous standing
Materials Seventy-three children with the diagnoses of cerebral palsy, suspected cerebral palsy, or delayed motor development, were examined and followed from 1958 to 1973. Children who were not walking at the time of the first examination and who could be followed until at least the age of five years were included in the study (only four children were less than six years old when the study was completed for this report). The ages of the 73 children at the first examination ranged between 10 months and 54 months: 10 to 17 months (9 children); I8 to 26 months (25); 27 t o 36 months (19); 37 to 48 months (15); and 49 to 54 months (5). The diagnoses of these 73 children are delineated in Table I. Four children who
Chief, Orthopaedic and Rehabilitation Services, Children’s Hospital at Stanford, 520 Willow Road, Palo Alto, California 94304, U S A .
18
E. E. BLECK
had delayed motor development and suspected cerebral palsy were classified as mentally retarded when, at the time of follow-up, no movement disorder could be identified. Two children initially referred as cerebral palsy cases were subsequently classified as having had delayed motor development when no movement disorder was found at the time of walking and follow-up. One of these children had cerebral atrophy defined by pneumoencephalogram.
Methods E,varninaiion of Postural and Tonic Rej¶e.v Act i v i t j All children were initially examined for the following seven signs of postural and tonic reflex activity (infantile automatisms) (Bobath 1954, Fiorentino 1963, Paine t'r a/. 1964. Paine and OppC 1966). (1) Asymmetrical tonic neck reflex. ( 2 ) Symmetrical tonic neck reflex. (3) Moro reflex. (4) Neck-righting reflex. ( 5 ) Foot placement reaction. (6) Parachute reaction. ( 7 ) Extensor thrust. These reflexes were ascertained in the following manner ( 1 ) As~~nimetrical tonic neck rej7e.v. The child was tested supine. A positive response was extension of the upper and lower limbs on the face side and flexion of the opposite TABLE I Diagnoses of 73 children studied Diagnoses
N o . of' cli ildren
Spastic diplegia or paraplegia Spastic quadriplegia Spastic triplegia Spastic hemiplegia Tension athetosis (quadriplegia) Ataxia Athetosis Hemiathetosis Mental retardation Delayed motor development (one with cerebral atrophy)
23 12 3 8 13 4
2 2 4 2
limbs on the occiput side when the head was rotated. I t is never normal if it is obligate and imposable. It is a normal reflex in its partly developed form and is transient up to the age of seven months (Paine et al. 1964). ( 2 ) Symmetrical tonic neck reflex. The child was placed in the quadruped position, or, if this position could not be assumed, prone over the examiner's thighs. A positive response was elicited if ventral flexion of the head caused flexion of the fore-limbs and extension of the hind-limbs, and if extension of the head caused extension of the fore-limbs and flexion of the hindlimbs. A positive reaction is normal up to the age of six months. ( 3 ) Moro r e j e s . The classic response of sudden extension of the upper limbs followed by drawing them together in the em brace position was considered positive. The Moro reflex was sought for by suddenly jarring the examination table, dropping the child's head into 20- to 30" extension, or by pinching the epigastric skin. The reflex disappears by the age of six months. (4) Neck-righting refles. The child was tested supine. A positive response was recorded when the head was turned and the shoulders, trunk and pelvis rotated consistently in the same direction. An invariable response to head-turning so that the child can be rolled over and over like a log is never normal (Paine ~t al. 1964). ( 5 ) Foot placenient reaction. The child was suspended by the axilla and lifted upward so that the dorsum of his feet touched the underside of the table. Normally, the child will place one foot and then the other upon the table top--occasionally, both feet may be placed at the same time. The placing response can be found i n newborn infants and persists for an indefinite period. Probably it becomes a voluntary activity between the ages of three and five years. A positive score was given if this response was absent.
DEVELOPMENTAL MEDICINE A N D CHILD
All children were in a cerebral palsy preschool nursery program up to the age of three years, then in a cerebral palsy nursery school program until the age of five, and afterwards in a school program for cerebral palsied children, in schools for the mentally retarded, day care centres, or in normal schools. All programs were on an out-patient basis. Even though the prognostic examination was done on children older than 24 months, all the patients in this study had been in treatment programs by the age of 24 months. Treatment was given for one hour three times per week in the pre-school program, and three to five times per week in the special school. No formal treatment was given during the Christmas, Easter and summer holidays. Parents were involved in the management of their child, and parent counseling was an important aspect of the management. No braces were used prior to ambulation. Only 12 children had orthopaedic surgery done prior to walking. Treatment designed to achieve walking was abandoned in children over the age of seven years and management was then redirected towards helping them with activities of daily living, wheelchair use, and getting in and out of a chair on their own or with assistance, depending upon the motor capabilities of each child. The criterion for ambulation was the ability to walk at least 15 meters independently on a level surface (i.e. a carpeted or uncarpeted floor, a clipped lawn, or an outdoor smooth surface) without falling. The only external supports permitted were crutches; usually these were of the forearm type. The ability to walk between parallel bars, with saw horses or walkers, or with braces and supported by a physical therapist, did not qualify as ambulation in this study. If a child was not ambulatory at the time of follow-up his final score was nought. If he was ambulatory (as defined above) he was
(6) Parachute reaction. The child was held prone and then was suddenly lowered to the table top. A normal response is extension of the upper limbs with the fingers spread as if to break the fall. This protective reaction appears at around the age of nine months and no later than 1 1 months. It persists throughout life. In this study, an absent parachute reaction was given a positive score. (7) Extensor thrust. The child was picked up by the axillae and then was lowered so that his feet made contact with the tabletop or the floor. A positive response was a marked and persistent extensor tonus of the lower limbs (often accompanied by hip adduction) progressing upward to the trunk. This response is never normal (Gesell and Amatruda 1958). Method of Scoring,for Prognosis Each abnormal reflex found (or, in the case of the parachute reaction and foot placement reaction, when not found) was scored as one point. The point scores for each child were totalled. The total score determined the prognosis for walking : ( a ) a zero score was a good prognosis; ( 6 ) a one point score was a guarded prognosis; and (c) a two point or greater score was a poor prognosis (Table 11). Folloiv-up and Treatment
The children were re-examined at two to six monthly intervals. The treatment given might more appropriately be called management (BIeck and Headley 1961). TABLE 11 Accuracy of prediction in 73 children studied
No. of
Prognosis
childreii
.score
I
Results Ambulatory Noniunhulatory
49 7 17
0 1
2+
'
46 7 1
NEUROLOGY. 1975, 17
16
~~
Prognosis score: 0 good prognosis; I guarded prognosis; 2 + poor prognosis.
20
graded A. We were able to follow all children after they had left the cerebral palsy nursery-school programs for other schools mentioned above.
was accurate in 94.5 per cent of 73 children studied (Table 11). Prognostic signs. The five most frequently appearing prognostic signs in non-ambulatory children were: extensor thrust (12 patients); absent parachute reaction ( I I ) ; asymmetrical tonic neck reflex (10); Moro reflex (nine); and neck-righting reflex (four). Age ut anihulution. Of the 54 children who walked, 36 did so by the age of four years. Five children walked between the ages of six and seven years. Because eight of the 14 children who did not walk until after the age of five years had had orthopaedic surgery prior to walking, we thought that surgery might account for the delay. However, four children who had had orthopaedic surgery prior to walking did walk before the age of five (46 to 50 months). The age at which walking is achieved can be correlated with the diagnosis, providing that the diagnosis is accurate. In this series, spastic hemiplegic children with a mean age of 27 months walked earlier than the other children. The earliest age at which a child with hemiplegia walked was 18 months. Spastic diplegic and paraplegic children had a mean walking age of 47 months. Triplegic, quadriplegic, athetoid and ataxic children walked later (mean age 54 months). How tlie children Mulkrtl. The 39 children who walked without crutches did so with a gait pattern typical of spasticity, athetosis or ataxia, or a combination of such movement disorders. All children walked with a forward progression. None of these children regressed to a non-walking state. Those children who walked independently without crutches were able t o run. albeit abnormally. All children who walked without crutches were able to climb stairs. either in the normal way or by taking one step at a time (f.e. one foot was placed on the step and the other foot brought up
Results Ambulatory cliildren. Of the 73 children studied, 54 became ambulatory. Of the 49 children who had prognosis scores of zero (good prognosis), 46 walked and three cases did not. Of these three, one child was first seen at 10 months, when he had a score of zero and was given a good prognosis. At 16 months, however, his prognosis score was 3. The reliability of our prognostic testing before the age of 12 months is therefore questionable. The second child was still not walking at the age of seven years despite a good prognosis score initially. He had been put on large doses of multiple anticonvulsants. and the sedative effects of these medications may have precluded walking. The third child had a good prognosis score initially but did not walk until seven years of age and then only five meters, with her mother or the examiner touching her little finger. Non-ambulatory children. Of the I 7 children who had scores of 2 points or more (poor prognosis), 16 are not walking to date. One child had a score of 2 but did manage to walk 15 metres with crutches at the age of seven years. At the time of follow-up ages ranged from 12 to 14 years (eight children), 10 to 1 I years (four), eight years (three) and one child was five and a half years old. All 16 children had quadriplegia: five were spastic and I1 had tension at hetosis. Guarded-prognosis cliildren. Of the seven children who had scores of one point (guarded prognosis). all are now walking. Five of this group use crutches. (Only 15 children in the entire study of 73 children require crutches to walk.) Accwrnrj. qf prediction. The prognosis
21
DEVELOPMENTAL MEDICINEA N D C H I L D NEUROLOGY.
1975, 17
1961). Only one family sought a different method of treatment. (This was the child who eventually walked at seven years of age but only by touching the little finger of her mother or the examiner. This child is mentally retarded and her need for the barest touch of another person seems to be an emotional one.) The parents of those children who had a poor prognosis for ambulation were advised of the prognosis so that they might not have unreasonable expectations. Treatment goals based upon the child's disability could be set early; major efforts could then be expended on other needs-e.g. feeding, and wheelchair manipulation. Because treatment by physiotherapists is, by necessity, not continuous (none during weekends or holidays), its r6le became more one of advice and assistance to parents, thus becoming more rational both to parents and therapists. The effect was a change to a more relaxed and patient attitude to a child's progress if he had a good prognosis for walking-even though walking was delayed. Orthopaedic surgery. Although the number of surgical cases in this study is insufficient to reach a firm conclusion, there is a hint that orthopaedic surgery designed to improve function delayed the onset of walking. I would therefore suggest prudence and defer surgery on pre-school, non-ambulatory children with a good prognosis until a walking pattern has been established. The exception to this advice would be to do surgery in the pre-school years if structural changes are manifest, particularly in the hip joint (subluxation or dislocation is more frequent in the child with a poor prognosis), or if contractures of the lower limb joints are present. Better results from surgery for functional improvement by restoring muscle balance have been obtained once the child had an established walking pattern. Improved over-all analysis of abnormal muscle
after it to the same step before progressing to the next step). While a study of the equilibrium reactions of these children is beyond the scope of the present study, it was observed that all the hemiplegic children had normal standing equilibrium reactions, whereas those children with spastic diplegia or paraplegia were usually deficient in the posterior equilibrium reactions and would fall backwards with the slightest push (Hagberg et a/. 1972). Children who walked with crutches had deficient anterior and posterior standing equilibrium reactions and fell easily without support. (Full details of all children studied are available from the author.)
Comment Feasibi1it.y and securitj) in loconiotor prognosis. The ability to predict ambulation in a child with delayed motor development and definite cerebral palsy (by definition due to a non-progressive lesion), and excluding children who have an obvious flaccid paralysis (e.g. spinal muscular atrophy, myelomeningocele), appears feasible and reasonably accurate when based upon the examination for postural and tonic reflex activity. Because of the late appearance of the parachute reaction (up to 1 1 months), it seems prudent not to make a prognosis on any child until the age of 12 months. The results obtained in this study parallel those of Crothers and Paine (1959). In their study of the age of walking of 289 patients with cerebral palsy, 100 per cent of the children with spastic hemiplegia were able to walk by the age of seven years, and 70 per cent of the children with spastic quadriplegia did so. In other words, the probability of a child walking after the age of seven is remote. Parental counseling. In the group of children studied here, a high degree of parental understanding and trust of medical personnel was achieved (Bleck and Headley 77 --
t.. E. BLECK
by conscious efforts or, more usually, by geographic happenstance, will always yield a certain percentage of good results if walking is the criterion applied for the success rate. In this study, 22 per cent of the children failed to walk. Thus it can be said that we had a 78 per cent success rate. One can easily see how, in treating a nonselected sample of children with any method-be it ice to the feet, brushing the skin, long leg braces and parallel bar gait training, orthopaedic surgery, or singing Bulgarian, Japanese or English nursery rhymes-good results will always be obtained in the majority of children, depending upon the epidemiology of cerebral palsy in the region or country. Cerebral palsy due to perinatal anoxia, or to erythroblastosis fetalis is decreasing. Spastic diplegia associated with pre-term birth is now the preponderant cause of cerebral palsy, at least in the region of Northern California. In the present study, children with spastic diplegia generally had a better prognosis for walking than those who had spastic quadriplegia or quadriplegia with tension athetosis; these conditions are usually associated with perinatal anoxia or erythroblastosis fetalis (kernicterus). Because of the changing etiologies of cerebral palsy and what appears to be a less severe type (spastic diplegia), physicians and therapists might conceivably be misled as to the efficacy of their particular current treatment programs, if walking is the criterion for success. The study reported here suggests that other criteria might be used to judge the efficacy of treatment programs, and that a perspective o n the intensity and duration of treatment might be an important consideration.
function can be obtained by observation of the gait and by electromyography. Analwing the results oj’ various physical therapy treatment systems. Gesell has stated that walking is not a learned activity: “The normal child does not need to be taught to walk. The urge to assume upright posture and then to step forth is part of development” (Gesell and Amatruda 1958). However, one should question whether walking is an inherent trait set in the central nervous system and whether it is present not only in normal children but also in those who have a degree of brain damage. Walking may well be a biological rhythm, as suggested by Becker (1974). The beat of the heart, the movement of the intestines, and the respiratory cycle are all examples of biological rhythms (Bath 1940). Indeed, the results of tracking and recording the trajectories of gait in both normal and cerebral palsied ambulatory children strongly suggest that the normal relaxation oscillations of gait are present in children with cerebral palsy, but in exaggerated and altered forms. From this present study it appears that the brain damage may be such as to preclude the ability to walk. Most of these postural reflexes are in the form of infantile automatisms elicited through proprioceptive sensory endings in the muscles and joints of the cervical spine and possibly represent subcortical functions (Paine et a/. 1964). A persistence of such reflexes after the first year of life possibly indicates that only subcortical functioning for motor control is present. When analyzing the results of numerous physical therapeutic systems in the treatment of cerebral palsy, it seems prudent to ask what kind of child we started treatment with. Pre-selection of case material either
23
DEVELOPMENTAL MEDICINE A N D CHILD NEUROLOGY.
1975, 17
SUMMARY
A study of the prognosis for walking of 73 pre-school children who had cerebral palsy
or delayed motor development is reported. The importance of prognostic signs, based upon the presence or absence of postural reflex activity, was confirmed. The prognosis was accurate in 94.5 per cent of the 73 children studied. The method of scoring a child’s reflex responses and relating this to the prognosis seems to be a simple procedure, easy to understand and to apply. It is suggested that the assessment of treatment methods should include an analysis of the child’s walking potential, based upon the methods delineated in this study, before treatment is started.
RCSUM~ Pronostic de la locomotion dans I’injirmite‘ motrice cPrPbrale L’auteur rapporte une Ctude pronostique sur le deplacement de 73 enfants d’bge prescolaire, presentant une infirmite motrice cerkbrale ou un retard de developpement moteur. La valeur pronostique de symptomes, basis sur la presence ou I’absence d’activitks reflexes posturales a ete confirmee. Le pronostic a ete correct chez 94.5 pour cent des 73 enfants Ctudits. La mtthode d’appreciation de I’activitC reflexe d’un enfant et la facon de relier les rksultats aux pronostics apparaissent un procede simple, facile a comprendre et a appliquer. L’auteur suggtre que les mCthodes d’appreciation des therapeutiques incluent une analyse de potentiel de marche de I’enfant, b a d e sur les methodes precisees dans cette Ctude avant qu’aucun traitement ne soit entrepris.
ZUSAMMENFASSUNG
Prognose der motorischen Entwicklung bei Cerebralparese Der Autor berichtet uber eine prognostische Studie an 73 Vorschulkindern mit Cerebralparese oder verzogerter motorischer Entwicklung. Die wichtigen prognostischen Zeichen basierend auf dem Vorhandensein oder nicht Vorhandensein von Lagereflexen wurden gepruft. Die Prognose stimmte in 94.5 Prozent der 73 untersuchten Kinder. Die Methode, die Reflexaktivitat eines Kindes zu analysieren und zur Prognose in Beziehung zu setzen, scheint ein einfaches Vorgehen zu sein, das leicht zu verstehen und anzuwenden ist. Der Autor schlagt vor, darj die Beurteilung der Behandlungsmethoden eine Analyse des Gehpotentials des Kindes einschlierjen sollte und zwar unter Berucksichtigung der in dieser Studie ausgefuhrten Methoden, bevor eine Behandlung begonnen wird.
RESU MEN
Prondstico locomotor en la yardisis infantil El autor aporta un estudio pronostic0 de la deambulacion en 73 niiios preescolares con paralisis cerebral o retraso en el desarrollo motor. Fueron confirmados 10s signos pronosticos importantes, basados en la presencia o ausencia de actividad postural refleja. El pronostico fue acertado en 94,5 par ciento de 10s 73 niiios estudiados. Parece que el mktodo de puntuar la actividad refleja del niiio y relacionarla con el pronostico, es un procedimiento simple y fhcil de aprender y aplicar. El autor sugiere que la valoracion de 10s metodos de tratamiento deberia incluir un andisis del potencial ambulatorio del niiio, basado en 10s metodos perfilados en este estudio, antes de iniciar el tratamiento.
24
E. E. BLECK
REFERENCES Andr&Thomas, Chesni, Y ., Saint-Anne Dargassies, S. (1960) The Neiirologicd €.tmninar,on of the Infirnt. Little Club Clinics No. I . London: National Spastics Society with Heinemann Medical. Bath, van der P. (1940) ‘Biological rhythms considered as relaxation oscillations.’ A d a Medica Scanclinavica. 104, 76. Becker, J . C. (1974) Personal communication. Bleck, E. E., Headley, L. (1961) ‘Treatment and parent counseling for the pre-school child with cerebral palsy.’ Pediatrics, 27, 1026. Bobath, B. (1954) ‘A study of abnormal postural reflex activity in patients with lesions of the central nervous system.’ Physiofheraphy, 40,259,295,326, 368. Cohen, P., Kohn, J. (1963) ‘Fifteen year follow-up of cerebral palsy patients.’ Paper read at the Annual Meeting of the American Academy for Cerebral Palsy, November 1963. Crothers, B., Paine, R. S. (1959) The Nafural History of Cerebral Pal.sj~.Cambridge, Mass.: Harvard University Press. Denhoff, E., Holden, R. H., Silver, M . C. (1956) ‘Prognostic studies in children with cerebral palsy.’Joirrnnl of the Atnerican Medical Association, 161, 781. - Robinault, 1. (1960) Cerebral P a l . ~ jutid , Related Di.sorders. New York : McGraw-Hill. Fiorentino, M. R. (1963) Reflex Tesfing McthoilvJor Evaluating CNS Developnient. Springfield, Ill. : C-harles C Thomas. Gesell, A,, Amatruda, C. S. (1958) Dewlopmental Diagnosis. New York: Paul B. Hocker. Hagberg, B., Sanner, G., Steen, M. (1972) ‘The dysequilibrium syndrome in cerebral palsy.‘ ,4cta Poediatrica Sranifinuvica. Suppl. 226. Jones, M. H. (1962) ‘Preschool program for children with cerebral palsy.’ Jorrrnd o f f h e American Meilicirl Wom~n’sAssociafion, 17,713. Paine, R. S. (1962) ‘On the treatment of cerebral palsy.’ Pediatrics, 29, 605. - Brazelton, T. B., Donovan, D. E., Drorbaugh, J. E., Hubbell, J. P., Sears, E. M. (1964) ‘Evolution of postural reflexes in normal infants and in the presence of chronic brain syndromes.’ Neurology, 14, 1036. - (1964) ‘Evolution of postural reflexes in normal infants and in the presence of chronic brain syndromes., Neiirology, 14, 1036. - Oppe, T. E. (1966) Neurological E.rirtnination ofChili/ren. Clinics in Developmental Medicine Nos. 20/2 I . London: Spastics Society with Heinemann Medical.
25
Locomotor Prognosis in Cerebral Palsy E. E. BZeck
when the child was suspended erect. These signs proved to be inaccurate when children who had them failed to walk independently after several years of observation. Reciprocal motion of the lower limbs probably represented the persistence of automatic marching commonly observed in infancy (AndrC-Thomas et al. 1960), and the ability to stand during infancy was probably the presence of an abnormally strong positive supporting reaction, or ‘extensor thrust’-always an abnormal response (Fiorentino 1963).
Introduction The questions most frequently asked by parents of children who have slow development and cerebral palsy are: “Will my child walk?” and “When will he walk?” This study was conducted to answer these questions. As the study evolved, other questions bearing on the prognosis for walking emerged: ( I ) how does the prognosis affect planning the management of the cerebral palsied child and parental understanding; (2) how does the prognosis affect decisions for orthopaedic surgical procedures; and (3) how does the prognosis affect judgments on the efficacy of various proposed ‘treatments’ for cerebral palsy if walking is the criterion for success? A few studies on prognosis in cerebral palsy have been published. Denhoff et a/. (1956), Denhoff and Robinault (1960), Jones (1962), Paine (1962), and Cohen and Kohn (1963) reported various aspects of the problem. The most detailed long-term study was that of Crothers and Paine ( 1 959), who pointed out the good prognosis for ambulation in hemiplegics and the poor prognosis in patients who had an obligate asymmetrical tonic neck reflex. My interest in locomotor prognosis based upon the presence or absence of infantile reflexes was prompted by the failure of traditional ‘good’ signs: reciprocal motion of the lower limbs and spontaneous standing
Materials Seventy-three children with the diagnoses of cerebral palsy, suspected cerebral palsy, or delayed motor development, were examined and followed from 1958 to 1973. Children who were not walking at the time of the first examination and who could be followed until at least the age of five years were included in the study (only four children were less than six years old when the study was completed for this report). The ages of the 73 children at the first examination ranged between 10 months and 54 months: 10 to 17 months (9 children); I8 to 26 months (25); 27 t o 36 months (19); 37 to 48 months (15); and 49 to 54 months (5). The diagnoses of these 73 children are delineated in Table I. Four children who
Chief, Orthopaedic and Rehabilitation Services, Children’s Hospital at Stanford, 520 Willow Road, Palo Alto, California 94304, U S A .
18
E. E. BLECK
had delayed motor development and suspected cerebral palsy were classified as mentally retarded when, at the time of follow-up, no movement disorder could be identified. Two children initially referred as cerebral palsy cases were subsequently classified as having had delayed motor development when no movement disorder was found at the time of walking and follow-up. One of these children had cerebral atrophy defined by pneumoencephalogram.
Methods E,varninaiion of Postural and Tonic Rej¶e.v Act i v i t j All children were initially examined for the following seven signs of postural and tonic reflex activity (infantile automatisms) (Bobath 1954, Fiorentino 1963, Paine t'r a/. 1964. Paine and OppC 1966). (1) Asymmetrical tonic neck reflex. ( 2 ) Symmetrical tonic neck reflex. (3) Moro reflex. (4) Neck-righting reflex. ( 5 ) Foot placement reaction. (6) Parachute reaction. ( 7 ) Extensor thrust. These reflexes were ascertained in the following manner ( 1 ) As~~nimetrical tonic neck rej7e.v. The child was tested supine. A positive response was extension of the upper and lower limbs on the face side and flexion of the opposite TABLE I Diagnoses of 73 children studied Diagnoses
N o . of' cli ildren
Spastic diplegia or paraplegia Spastic quadriplegia Spastic triplegia Spastic hemiplegia Tension athetosis (quadriplegia) Ataxia Athetosis Hemiathetosis Mental retardation Delayed motor development (one with cerebral atrophy)
23 12 3 8 13 4
2 2 4 2
limbs on the occiput side when the head was rotated. I t is never normal if it is obligate and imposable. It is a normal reflex in its partly developed form and is transient up to the age of seven months (Paine et al. 1964). ( 2 ) Symmetrical tonic neck reflex. The child was placed in the quadruped position, or, if this position could not be assumed, prone over the examiner's thighs. A positive response was elicited if ventral flexion of the head caused flexion of the fore-limbs and extension of the hind-limbs, and if extension of the head caused extension of the fore-limbs and flexion of the hindlimbs. A positive reaction is normal up to the age of six months. ( 3 ) Moro r e j e s . The classic response of sudden extension of the upper limbs followed by drawing them together in the em brace position was considered positive. The Moro reflex was sought for by suddenly jarring the examination table, dropping the child's head into 20- to 30" extension, or by pinching the epigastric skin. The reflex disappears by the age of six months. (4) Neck-righting refles. The child was tested supine. A positive response was recorded when the head was turned and the shoulders, trunk and pelvis rotated consistently in the same direction. An invariable response to head-turning so that the child can be rolled over and over like a log is never normal (Paine ~t al. 1964). ( 5 ) Foot placenient reaction. The child was suspended by the axilla and lifted upward so that the dorsum of his feet touched the underside of the table. Normally, the child will place one foot and then the other upon the table top--occasionally, both feet may be placed at the same time. The placing response can be found i n newborn infants and persists for an indefinite period. Probably it becomes a voluntary activity between the ages of three and five years. A positive score was given if this response was absent.
DEVELOPMENTAL MEDICINE A N D CHILD
All children were in a cerebral palsy preschool nursery program up to the age of three years, then in a cerebral palsy nursery school program until the age of five, and afterwards in a school program for cerebral palsied children, in schools for the mentally retarded, day care centres, or in normal schools. All programs were on an out-patient basis. Even though the prognostic examination was done on children older than 24 months, all the patients in this study had been in treatment programs by the age of 24 months. Treatment was given for one hour three times per week in the pre-school program, and three to five times per week in the special school. No formal treatment was given during the Christmas, Easter and summer holidays. Parents were involved in the management of their child, and parent counseling was an important aspect of the management. No braces were used prior to ambulation. Only 12 children had orthopaedic surgery done prior to walking. Treatment designed to achieve walking was abandoned in children over the age of seven years and management was then redirected towards helping them with activities of daily living, wheelchair use, and getting in and out of a chair on their own or with assistance, depending upon the motor capabilities of each child. The criterion for ambulation was the ability to walk at least 15 meters independently on a level surface (i.e. a carpeted or uncarpeted floor, a clipped lawn, or an outdoor smooth surface) without falling. The only external supports permitted were crutches; usually these were of the forearm type. The ability to walk between parallel bars, with saw horses or walkers, or with braces and supported by a physical therapist, did not qualify as ambulation in this study. If a child was not ambulatory at the time of follow-up his final score was nought. If he was ambulatory (as defined above) he was
(6) Parachute reaction. The child was held prone and then was suddenly lowered to the table top. A normal response is extension of the upper limbs with the fingers spread as if to break the fall. This protective reaction appears at around the age of nine months and no later than 1 1 months. It persists throughout life. In this study, an absent parachute reaction was given a positive score. (7) Extensor thrust. The child was picked up by the axillae and then was lowered so that his feet made contact with the tabletop or the floor. A positive response was a marked and persistent extensor tonus of the lower limbs (often accompanied by hip adduction) progressing upward to the trunk. This response is never normal (Gesell and Amatruda 1958). Method of Scoring,for Prognosis Each abnormal reflex found (or, in the case of the parachute reaction and foot placement reaction, when not found) was scored as one point. The point scores for each child were totalled. The total score determined the prognosis for walking : ( a ) a zero score was a good prognosis; ( 6 ) a one point score was a guarded prognosis; and (c) a two point or greater score was a poor prognosis (Table 11). Folloiv-up and Treatment
The children were re-examined at two to six monthly intervals. The treatment given might more appropriately be called management (BIeck and Headley 1961). TABLE 11 Accuracy of prediction in 73 children studied
No. of
Prognosis
childreii
.score
I
Results Ambulatory Noniunhulatory
49 7 17
0 1
2+
'
46 7 1
NEUROLOGY. 1975, 17
16
~~
Prognosis score: 0 good prognosis; I guarded prognosis; 2 + poor prognosis.
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graded A. We were able to follow all children after they had left the cerebral palsy nursery-school programs for other schools mentioned above.
was accurate in 94.5 per cent of 73 children studied (Table 11). Prognostic signs. The five most frequently appearing prognostic signs in non-ambulatory children were: extensor thrust (12 patients); absent parachute reaction ( I I ) ; asymmetrical tonic neck reflex (10); Moro reflex (nine); and neck-righting reflex (four). Age ut anihulution. Of the 54 children who walked, 36 did so by the age of four years. Five children walked between the ages of six and seven years. Because eight of the 14 children who did not walk until after the age of five years had had orthopaedic surgery prior to walking, we thought that surgery might account for the delay. However, four children who had had orthopaedic surgery prior to walking did walk before the age of five (46 to 50 months). The age at which walking is achieved can be correlated with the diagnosis, providing that the diagnosis is accurate. In this series, spastic hemiplegic children with a mean age of 27 months walked earlier than the other children. The earliest age at which a child with hemiplegia walked was 18 months. Spastic diplegic and paraplegic children had a mean walking age of 47 months. Triplegic, quadriplegic, athetoid and ataxic children walked later (mean age 54 months). How tlie children Mulkrtl. The 39 children who walked without crutches did so with a gait pattern typical of spasticity, athetosis or ataxia, or a combination of such movement disorders. All children walked with a forward progression. None of these children regressed to a non-walking state. Those children who walked independently without crutches were able t o run. albeit abnormally. All children who walked without crutches were able to climb stairs. either in the normal way or by taking one step at a time (f.e. one foot was placed on the step and the other foot brought up
Results Ambulatory cliildren. Of the 73 children studied, 54 became ambulatory. Of the 49 children who had prognosis scores of zero (good prognosis), 46 walked and three cases did not. Of these three, one child was first seen at 10 months, when he had a score of zero and was given a good prognosis. At 16 months, however, his prognosis score was 3. The reliability of our prognostic testing before the age of 12 months is therefore questionable. The second child was still not walking at the age of seven years despite a good prognosis score initially. He had been put on large doses of multiple anticonvulsants. and the sedative effects of these medications may have precluded walking. The third child had a good prognosis score initially but did not walk until seven years of age and then only five meters, with her mother or the examiner touching her little finger. Non-ambulatory children. Of the I 7 children who had scores of 2 points or more (poor prognosis), 16 are not walking to date. One child had a score of 2 but did manage to walk 15 metres with crutches at the age of seven years. At the time of follow-up ages ranged from 12 to 14 years (eight children), 10 to 1 I years (four), eight years (three) and one child was five and a half years old. All 16 children had quadriplegia: five were spastic and I1 had tension at hetosis. Guarded-prognosis cliildren. Of the seven children who had scores of one point (guarded prognosis). all are now walking. Five of this group use crutches. (Only 15 children in the entire study of 73 children require crutches to walk.) Accwrnrj. qf prediction. The prognosis
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DEVELOPMENTAL MEDICINEA N D C H I L D NEUROLOGY.
1975, 17
1961). Only one family sought a different method of treatment. (This was the child who eventually walked at seven years of age but only by touching the little finger of her mother or the examiner. This child is mentally retarded and her need for the barest touch of another person seems to be an emotional one.) The parents of those children who had a poor prognosis for ambulation were advised of the prognosis so that they might not have unreasonable expectations. Treatment goals based upon the child's disability could be set early; major efforts could then be expended on other needs-e.g. feeding, and wheelchair manipulation. Because treatment by physiotherapists is, by necessity, not continuous (none during weekends or holidays), its r6le became more one of advice and assistance to parents, thus becoming more rational both to parents and therapists. The effect was a change to a more relaxed and patient attitude to a child's progress if he had a good prognosis for walking-even though walking was delayed. Orthopaedic surgery. Although the number of surgical cases in this study is insufficient to reach a firm conclusion, there is a hint that orthopaedic surgery designed to improve function delayed the onset of walking. I would therefore suggest prudence and defer surgery on pre-school, non-ambulatory children with a good prognosis until a walking pattern has been established. The exception to this advice would be to do surgery in the pre-school years if structural changes are manifest, particularly in the hip joint (subluxation or dislocation is more frequent in the child with a poor prognosis), or if contractures of the lower limb joints are present. Better results from surgery for functional improvement by restoring muscle balance have been obtained once the child had an established walking pattern. Improved over-all analysis of abnormal muscle
after it to the same step before progressing to the next step). While a study of the equilibrium reactions of these children is beyond the scope of the present study, it was observed that all the hemiplegic children had normal standing equilibrium reactions, whereas those children with spastic diplegia or paraplegia were usually deficient in the posterior equilibrium reactions and would fall backwards with the slightest push (Hagberg et a/. 1972). Children who walked with crutches had deficient anterior and posterior standing equilibrium reactions and fell easily without support. (Full details of all children studied are available from the author.)
Comment Feasibi1it.y and securitj) in loconiotor prognosis. The ability to predict ambulation in a child with delayed motor development and definite cerebral palsy (by definition due to a non-progressive lesion), and excluding children who have an obvious flaccid paralysis (e.g. spinal muscular atrophy, myelomeningocele), appears feasible and reasonably accurate when based upon the examination for postural and tonic reflex activity. Because of the late appearance of the parachute reaction (up to 1 1 months), it seems prudent not to make a prognosis on any child until the age of 12 months. The results obtained in this study parallel those of Crothers and Paine (1959). In their study of the age of walking of 289 patients with cerebral palsy, 100 per cent of the children with spastic hemiplegia were able to walk by the age of seven years, and 70 per cent of the children with spastic quadriplegia did so. In other words, the probability of a child walking after the age of seven is remote. Parental counseling. In the group of children studied here, a high degree of parental understanding and trust of medical personnel was achieved (Bleck and Headley 77 --
t.. E. BLECK
by conscious efforts or, more usually, by geographic happenstance, will always yield a certain percentage of good results if walking is the criterion applied for the success rate. In this study, 22 per cent of the children failed to walk. Thus it can be said that we had a 78 per cent success rate. One can easily see how, in treating a nonselected sample of children with any method-be it ice to the feet, brushing the skin, long leg braces and parallel bar gait training, orthopaedic surgery, or singing Bulgarian, Japanese or English nursery rhymes-good results will always be obtained in the majority of children, depending upon the epidemiology of cerebral palsy in the region or country. Cerebral palsy due to perinatal anoxia, or to erythroblastosis fetalis is decreasing. Spastic diplegia associated with pre-term birth is now the preponderant cause of cerebral palsy, at least in the region of Northern California. In the present study, children with spastic diplegia generally had a better prognosis for walking than those who had spastic quadriplegia or quadriplegia with tension athetosis; these conditions are usually associated with perinatal anoxia or erythroblastosis fetalis (kernicterus). Because of the changing etiologies of cerebral palsy and what appears to be a less severe type (spastic diplegia), physicians and therapists might conceivably be misled as to the efficacy of their particular current treatment programs, if walking is the criterion for success. The study reported here suggests that other criteria might be used to judge the efficacy of treatment programs, and that a perspective o n the intensity and duration of treatment might be an important consideration.
function can be obtained by observation of the gait and by electromyography. Analwing the results oj’ various physical therapy treatment systems. Gesell has stated that walking is not a learned activity: “The normal child does not need to be taught to walk. The urge to assume upright posture and then to step forth is part of development” (Gesell and Amatruda 1958). However, one should question whether walking is an inherent trait set in the central nervous system and whether it is present not only in normal children but also in those who have a degree of brain damage. Walking may well be a biological rhythm, as suggested by Becker (1974). The beat of the heart, the movement of the intestines, and the respiratory cycle are all examples of biological rhythms (Bath 1940). Indeed, the results of tracking and recording the trajectories of gait in both normal and cerebral palsied ambulatory children strongly suggest that the normal relaxation oscillations of gait are present in children with cerebral palsy, but in exaggerated and altered forms. From this present study it appears that the brain damage may be such as to preclude the ability to walk. Most of these postural reflexes are in the form of infantile automatisms elicited through proprioceptive sensory endings in the muscles and joints of the cervical spine and possibly represent subcortical functions (Paine et a/. 1964). A persistence of such reflexes after the first year of life possibly indicates that only subcortical functioning for motor control is present. When analyzing the results of numerous physical therapeutic systems in the treatment of cerebral palsy, it seems prudent to ask what kind of child we started treatment with. Pre-selection of case material either
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DEVELOPMENTAL MEDICINE A N D CHILD NEUROLOGY.
1975, 17
SUMMARY
A study of the prognosis for walking of 73 pre-school children who had cerebral palsy
or delayed motor development is reported. The importance of prognostic signs, based upon the presence or absence of postural reflex activity, was confirmed. The prognosis was accurate in 94.5 per cent of the 73 children studied. The method of scoring a child’s reflex responses and relating this to the prognosis seems to be a simple procedure, easy to understand and to apply. It is suggested that the assessment of treatment methods should include an analysis of the child’s walking potential, based upon the methods delineated in this study, before treatment is started.
RCSUM~ Pronostic de la locomotion dans I’injirmite‘ motrice cPrPbrale L’auteur rapporte une Ctude pronostique sur le deplacement de 73 enfants d’bge prescolaire, presentant une infirmite motrice cerkbrale ou un retard de developpement moteur. La valeur pronostique de symptomes, basis sur la presence ou I’absence d’activitks reflexes posturales a ete confirmee. Le pronostic a ete correct chez 94.5 pour cent des 73 enfants Ctudits. La mtthode d’appreciation de I’activitC reflexe d’un enfant et la facon de relier les rksultats aux pronostics apparaissent un procede simple, facile a comprendre et a appliquer. L’auteur suggtre que les mCthodes d’appreciation des therapeutiques incluent une analyse de potentiel de marche de I’enfant, b a d e sur les methodes precisees dans cette Ctude avant qu’aucun traitement ne soit entrepris.
ZUSAMMENFASSUNG
Prognose der motorischen Entwicklung bei Cerebralparese Der Autor berichtet uber eine prognostische Studie an 73 Vorschulkindern mit Cerebralparese oder verzogerter motorischer Entwicklung. Die wichtigen prognostischen Zeichen basierend auf dem Vorhandensein oder nicht Vorhandensein von Lagereflexen wurden gepruft. Die Prognose stimmte in 94.5 Prozent der 73 untersuchten Kinder. Die Methode, die Reflexaktivitat eines Kindes zu analysieren und zur Prognose in Beziehung zu setzen, scheint ein einfaches Vorgehen zu sein, das leicht zu verstehen und anzuwenden ist. Der Autor schlagt vor, darj die Beurteilung der Behandlungsmethoden eine Analyse des Gehpotentials des Kindes einschlierjen sollte und zwar unter Berucksichtigung der in dieser Studie ausgefuhrten Methoden, bevor eine Behandlung begonnen wird.
RESU MEN
Prondstico locomotor en la yardisis infantil El autor aporta un estudio pronostic0 de la deambulacion en 73 niiios preescolares con paralisis cerebral o retraso en el desarrollo motor. Fueron confirmados 10s signos pronosticos importantes, basados en la presencia o ausencia de actividad postural refleja. El pronostico fue acertado en 94,5 par ciento de 10s 73 niiios estudiados. Parece que el mktodo de puntuar la actividad refleja del niiio y relacionarla con el pronostico, es un procedimiento simple y fhcil de aprender y aplicar. El autor sugiere que la valoracion de 10s metodos de tratamiento deberia incluir un andisis del potencial ambulatorio del niiio, basado en 10s metodos perfilados en este estudio, antes de iniciar el tratamiento.
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E. E. BLECK
REFERENCES Andr&Thomas, Chesni, Y ., Saint-Anne Dargassies, S. (1960) The Neiirologicd €.tmninar,on of the Infirnt. Little Club Clinics No. I . London: National Spastics Society with Heinemann Medical. Bath, van der P. (1940) ‘Biological rhythms considered as relaxation oscillations.’ A d a Medica Scanclinavica. 104, 76. Becker, J . C. (1974) Personal communication. Bleck, E. E., Headley, L. (1961) ‘Treatment and parent counseling for the pre-school child with cerebral palsy.’ Pediatrics, 27, 1026. Bobath, B. (1954) ‘A study of abnormal postural reflex activity in patients with lesions of the central nervous system.’ Physiofheraphy, 40,259,295,326, 368. Cohen, P., Kohn, J. (1963) ‘Fifteen year follow-up of cerebral palsy patients.’ Paper read at the Annual Meeting of the American Academy for Cerebral Palsy, November 1963. Crothers, B., Paine, R. S. (1959) The Nafural History of Cerebral Pal.sj~.Cambridge, Mass.: Harvard University Press. Denhoff, E., Holden, R. H., Silver, M . C. (1956) ‘Prognostic studies in children with cerebral palsy.’Joirrnnl of the Atnerican Medical Association, 161, 781. - Robinault, 1. (1960) Cerebral P a l . ~ jutid , Related Di.sorders. New York : McGraw-Hill. Fiorentino, M. R. (1963) Reflex Tesfing McthoilvJor Evaluating CNS Developnient. Springfield, Ill. : C-harles C Thomas. Gesell, A,, Amatruda, C. S. (1958) Dewlopmental Diagnosis. New York: Paul B. Hocker. Hagberg, B., Sanner, G., Steen, M. (1972) ‘The dysequilibrium syndrome in cerebral palsy.‘ ,4cta Poediatrica Sranifinuvica. Suppl. 226. Jones, M. H. (1962) ‘Preschool program for children with cerebral palsy.’ Jorrrnd o f f h e American Meilicirl Wom~n’sAssociafion, 17,713. Paine, R. S. (1962) ‘On the treatment of cerebral palsy.’ Pediatrics, 29, 605. - Brazelton, T. B., Donovan, D. E., Drorbaugh, J. E., Hubbell, J. P., Sears, E. M. (1964) ‘Evolution of postural reflexes in normal infants and in the presence of chronic brain syndromes.’ Neurology, 14, 1036. - (1964) ‘Evolution of postural reflexes in normal infants and in the presence of chronic brain syndromes., Neiirology, 14, 1036. - Oppe, T. E. (1966) Neurological E.rirtnination ofChili/ren. Clinics in Developmental Medicine Nos. 20/2 I . London: Spastics Society with Heinemann Medical.
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