For many pediatricians, the medical examination of an infant who might have cerebral palsy is a diagnostic exercise in which the presence of cerebral palsy is confirmed or denied. They do not realize that a wealth of additional information can be obtained from the medical examination that can provide therapeutic and prognostic guidance. That this situation exists is not surprising when it is recalled that all the conditions grouped together under the general term of cerebral palsy occur rarely, and pediatricians who are not specializing in this work will encounter only a few cases. Nevertheless, cerebral palsy does occur from time to time in every pediatrician's practice, and as each case provides work over many years it will probably be useful to alert pediatricians to the kind of information that can come from their clinical examinations. Three aspects will be considered: early diagnosis, the follow-up examination, and prognosis.
It is to be hoped that the diagnosis of cerebral palsy is made in infancy. Early diagnosis has been described on many occasions, and Taft's description in 1973' is especially valuable as a guide. Early diagnosis depends upon the following elements:
1. Suspicion, from (a) the history of risk factors and (b) the symptoms.
2. Developmental delay, especially delay in motor development.
3. Persistent neonatal reflexes.
4. Specific neurologic features.
History. Most parents and physicians are well aware that cerebral palsy is more likely if the infant is at risk from a variety of conditions, such as premature birth, asphyxia, and neonatal jaundice. Consequently, both groups usually are alert to the possibility of cerebral palsy when these factors are present. In the majority of these babies, fortunately, everything is all right. But it is important to parents that they be fully assured that this is so.
The physician, then, must perform the examination meticulously and repeatedly. It is very tempting in the case of an asphyxiated premature baby who has come through a stormy neonatal period to say to the anxious parents, when examination at three months shows no abnormality, that everything is all right and their baby does not have cerebral palsy.
But a single examination is not enough in these cases. Reported examination for at least a year and preferably for two years is necessary before one can be quite confident that the baby is all right. This situation arises because there is often a latent period of several months before all the signs of cerebral palsy appear. The physician should take care, therefore, that his natural desire to please and reassure anxious parents does not lead him into making a hasty and possibly false prognosis.
Symptoms. A variety of nonspecific symptoms occur in infants with cerebral palsy, including irritability, sleep disturbances, poor feeding, and vomiting. Such symptoms may be due to many different causes, of course, but when all other causes have been excluded cerebral palsy should be considered.
When a child has these symptoms and all other causes have been ruled out, it is easy to assume that they are due to cerebral palsy - and are, in fact, an inevitable part of the clinical picture. An attempt may be made to eradicate the symptoms by treating the cerebral palsy. Such interpretations may be erroneous and can lead to an unsatisfactory management of the case. In the first instance, accepting the symptoms as an inevitable accompaniment of cerebral palsy does not help the parents, who are left struggling to deal with them as best they can. And attempting to remove the symptoms by treating the cerebral palsy assumes that they are causally related, which is not always so.
My experience leads me to conclude that some of the general symptoms arise as a result of a tense mother-child interaction. A vicious spiral occurs. The mother is anxious because she senses that her baby is not quite right. She becomes tense. The baby reacts by becoming irritable and more difficult to feed. The mother becomes more tense, and so it continues.
The extent to which symptoms are due to this cause of course varies from case to case, but in some cases it is the major factor. Consider the mother and baby shown in Figure 1. The photograph is one of a 15-week-old baby with cerebral palsy. Note the tense posture, anxious face, retracted head, persistent tight fisting of the hands, and extension, adduction, and crossing of the legs. Now note the strained mother holding her infant. Note her anxious expression, the awkward manner in which she holds her baby, and the tenseness of her hold, which causes blanching of her fingers.
In situations like this it is our practice to begin by working with the mother. In this case, we helped her to understand her baby's problems. We helped to show her how well she herself could manage her boy. As her confidence increased, she became more relaxed and her baby lost his tenseness and irritability and was soon free of these distressing symptoms. When this stage was reached it was possible to introduce measures to treat the basic cerebral palsy.
Had the mother and baby been referred for "treatment" when cerebral palsy was first diagnosed, the symptoms would have become worse. The mother would have seen her fears that her baby had a serious problem confirmed by the fact that he was being referred for special treatment, and she would have felt herself even more of a failure because a therapist had to take over. By taking time at first to deal with the mother-child interaction, by helping the mother to understand the problems, and by restoring her confidence in her own abilities as a mother, the physician can create an atmosphere in which both mother and baby can relax. Therapy can then be introduced more effectively. In this case, the mother continued to play the major part in her child's care and the therapist advised about but did not take over the handling of the baby. The outcome was most satisfactory - the boy walked normally just before his second birthday.
In summary, then, in cases of cerebral palsy, interpret the symptoms carefully.
Developmental delay. The possibility of cerebral palsy must be considered in any child showing developmental delay, and especially if the delay is either solely or principally in the areas of motor development. Recognition of developmental delay should not stop at the diagnostic level. The implications should be considered.
Figure 1. A 15-week-old baby with cerebral palsy
Developmental delay in cerebral palsy is seldom due only to the cerebral pathology. Other factors play a part, and these should be investigated because something can be done about them. For example, parents frightened by their child's condition often adopt a subdued and apprehensive approach to his care - hardly the most favorable basis for early child development. They may be uncertain about what is the best thing to do - rearing a normal, healthy child has enough problems and difficulties, but parents faced with rearing a child with cerebral palsy may be completely overwhelmed. If the child is going to make the kind of developmental progress that is possible, his parents need support and developmental guidance."
The specific delay in motor development that is so characteristic of cerebral palsy means that mobility is seriously impeded. Movement is essential for the development of physique, exploration of and manipulation of the environment, and even emotional development.2 Consider two contrasting examples. A normal two- or three-year-old child is very active. He is on the go all the time, running, jumping, and climbing. As he moves about, he explores different parts of his surroundings. All this activity stimulates his muscular development and provides innumerable opportunities for learning.
Now, contrast this situation with that of a cerebral-palsied child who cannot move about. How does he develop his muscles and learn about his surroundings? Do we do enough to provide such a child with all those learning opportunities he is being denied as a result of his immobility? One can see the difference in the two cases in comparing what happens in each household when the father arrives home from work. A normal child can run to greet him, jump up to him, and fling his arms around his neck. What an enriching experience for the child, and what a contribution to his emotional development! The cerebral-palsied child is denied this; he is immobile. So the pediatrician should help the parents find other ways to provide their child with suitably enriching emotional experiences.
The delay in motor development interferes with other aspects of development and so produces a distorted pattern. Consider the following examples. A normal baby can hold his head quite steady by four months of age, and from that steady base can use his eyes and ears to explore his surroundings with great accuracy. The ability to localize the source of a sound develops rapidly between four and 10 months of age, and at the end of this period an infant can directly locate a sound source with an accuracy of a few degrees. A child with cerebral palsy may not be able to hold his head steady even when four years old, so there is little wonder that his sensory-function development is impaired. Interpreting the significance of delay in development in this way leads to therapeutic recommendations - namely, that a child with cerebral palsy with poor head control should have periods of head stabilization and reinforcement of auditory and visual stimuli so as to provide him with the developmental experiences he needs. Developmental guidance is an essential part of pediatric management.3
Persistent neonatal reflexes. A characteristic feature of many children with cerebral palsy is the persistence of neonatal reflexes long after they should have subsided, reflexes that often become excessively strong and obligatory. As long as these reflexes persist, they dominate a child's posture and movements. The following are common examples:
Moro reflex. A strong and persistent Moro reflex will be triggered every time the child is picked up or moved about. It will interfere with anything the child may be doing at the time and is a very disturbing experience for both the child and his mother.
Asymmetric tonic neck reflex. When this persists in a strong form it will prevent a child from rolling over because, as he turns his head in the direction he wishes to roll, the arm on that side will extend stiffly and block his movement. An abnormal reflex also will interfere with self-feeding. Suppose the child sees a desirable ice cream. He looks towards it, and this makes his arm extend towards it automatically. This is useful because it gets his arm in the right direction. However, after he has taken hold of the ice cream he cannot flex his arm to take it to his mouth. If he turns his head away to allow his arm to flex, the ice cream reaches the back of his head and not his mouth. A thoroughly frustrating experience!
Oral reflexes. These are useful at the right time - the neonatal period - when they facilitate breast feeding. When the cheek and corner of the mouth touch the breast the infant's mouth opens and the head turns to the stimulated side, and as a result the nipple is well received into the baby's mouth. But in cases of cerebral palsy the reflexes may persist after infancy and the excessive oral sensitivity makes feeding difficult. Whenever the lips are touched, the mouth opens just at the time when the lips should be closing on the spoon in order to take off the food. Feeding is very troublesome in these circumstances.
Figure 2. This boy's strong asymmetric tonic neck reflex has been influential in causing dislocation of his hip joint.
Interpreting the effects and significance of persistent reflex patterns provides the pediatrician with guidance about treatment. For many pediatricians the treatment of cerebral palsy is bedeviled by a series of systems that seem to conflict and sometimes even seem to be incomprehensible. It is some help to approach them from the point of view of the reflex patterns and to find out whether they aim to inhibit and reduce the reflexes or to make use of them to control posture and to develop movement.
In general, the physician will want to avoid the frequent stimulation of the reflexes because to do so perpetuates and enhances stereotyped movements and delays the acquisition of motor skills. He will probably also learn to curb the enthusiastic therapist who wants to develop motor activities in the face of the persistent reflexes.
Some forms of therapy make use of the reflexes to produce a movement or to control an abnormal movement. The pediatrician may wish to make use of these methods from time to time but should do so only with a full knowledge of the advantages and disadvantages of the various techniques. The interested reader is recommended to consult some of the texts on the subject, such as Gillette4 and Levitt.5
The observation of persistent reflexes during the examination is also a guide to future problems and deformities. Two examples are the following:
A strongly persistent withdrawal reflex of the legs produces flexion of the hips and knees and dorsiflexion of the feet. In these cases, one watches for and seeks to prevent flexion deformity of the hips and knees. Also, as long as the withdrawal reflex is the dominating influence, the feet will dorsiflex in response to an appropriate stimulus so equinus of the feet is unlikely to become a problem. Sometimes, however, the situation is not so straightforward and other reflexes will be present that promote the development of equinus.
When the asymmetric tonic neck reflex is very strong, the legs will be affected as well as the arms. When the head is turned to one side, the arm on that side extends stiffly, and the leg on that side flexes and adducts at the hip. This action jeopardizes the hip joint. Figure 2 is the photograph of a boy with a strong asymmetric tonic neck reflex that has been influential in causing dislocation of the right hip joint.
The reflex patterns in every child with cerebral palsy should be studied carefully, and all possible information collected from them will assist therapeutically and prognostically.
Other specific neurologic features. There are a number of other neurologic features associated with cerebral palsy, and three will be discussed here: exaggeration of the stretch reflexes, cocontraction, and dystonia.
Exaggeration of the stretch reflexes, as shown by abnormally brisk knee jerks, is often thought to be an important sign of cerebral palsy, but this is a carryover from adult neurology, and they are not as important as some of the other features previously mentioned. When it is present, the practical effect is that any quick movement can excite them and result in sudden intense counteraction.
Cocontraction - i.e., the simultaneous contraction of agonist and antagonist muscles - dampens down movement and makes any movement slow, stiff, and laborious. Cocontraction occurs quite frequently in cerebral palsy but is not always easy to recognize. I have seen therapists urging children to do more in the belief that they are not putting any effort into moving their leg or arm or whatever the task may be. In fact, the children are striving hard but cannot produce any movement as evidence of their efforts because the movement is blocked by cocontraction. The presence of cocontraction may be recognized clinically by the alert therapist and pediatrician, and it can be demonstrated quite easily by surface electromyography.6 Indeed, simple surface electromyography is a useful adjunct to therapy in cerebral palsy.
Dystonia. Muscle tone in some children with cerebral palsy is distributed unequally and fluctuates with position. The term dystonia is applied to this phenomenon. Dystonia produces problems for parents. There is little more distressing than to see a small child sitting on his mother's lap suddenly shoot backwards in extensor spasm. Dystonia is a valuable diagnostic sign; its significance for the handling of the child should be analyzed and this information used in determining management.
THE FOLLOW-UP EXAMINATION
Once the diagnosis is made and treatment started, many pediatricians feel lost. One told me once that every time he saw a child in a follow-up examination he would ask how he was, tap his knees, and then was at a loss as to what to do next. The follow-up examination is important. It should monitor the child's progress, reveal whether earlier aims have been achieved, and help physician and parents anticipate future problems. The examination consists of inquiry, observation and examination.
Inquiry. Obviously, it is necessary to know about the child's current problems, so my first inquiries are in this direction. Since it is so easy to overlook a problem in an area other than that of immediate concern (i.e., the motor problems), it is advisable to make a systematic inquiry. In order to do so I have devised a system of classification of disabled children7 that I have found to be of great value. It is named PULHESTIB, after the first letter of each of the nine points to be covered. (See box).
Much of the inquiry must relate to what the child does. With respect to abilities that were present at an earlier examination, I want to know if these are now being performed as well, less well, or better than previously. I also want to know about abilities that have disappeared and ones that have developed.
The other important inquiry attempts to decide what the child can do and what he actually does do. There is often a considerable difference between these two items. When a child fails to do all that he can, a serious investigation into his management is called for. This valuable "can do- does do" distinction was, I believe, first brought to notice by Chess, Korn, and Fernandez8 in 1971.
Such a rhorough inquiry as part of a follow-up examination may seem excessive, but it does pay handsome dividends in terms of ensuring completeness of cover and accuracy in planning treatment and management.
Observation. Throughout the inquiry and examination it is useful to observe the child and his parents, since their composure and actions and reactions are worth noting. It is through observation that one obtains many clues to the parents' attitude to their child and, from this, their acceptance of the physician's therapy recommendations. Sometimes I will excuse myself so as to be able to watch child and parents from a distance. For example, observing a remoteness in the parents' attitude to their child at this stage prepares me for their later questions about prognosis and increases the accuracy of my speculations about what they are hoping for and what they will accept prognostically.
Examination. Isn't this the same child with cerebral palsy that I saw some months ago? Why repeat the examination, since the signs of cerebral palsy that I saw then are likely to be the same now? A pediatrician may well ask such questions, but the point of subsequent examinations is that you are not just concerned with abnormal clinical signs. The physician must learn to break away from concentration on clinical signs and look more at the child, his development, and his abilities. I like to see whar the child can do and note the quality of his performance.
For example, I might see two children who can stand alone for at least two minutes, but although their abilities with respect to standing might be similar they could present different problems. The first might show a good attempt to pull to a standing position and do so with a normal sequence of movements. In this case I would encourage frequent standing and pulling to standing, and I would anticipate fairly rapid progress towards independent walking. The second child might stand for a short period when placed in that position but have no idea at all of the sequence of movements for pulling to standing, and any attempts he might make in that direction would be very abnormal and distorted. If such were the case I would be much more pessimistic about his ability to walk alone in future, and during physical therapy I would want the therapists to concentrate on the earlier stages, such as knee standing and sideways cruising.
Most deformities in cerebral palsy are not part of the basic disorder but are secondary complications that could and should be prevented. To achieve success it is necessary to check for deformity-producing situations at each follow-up examination.
The items to which I give especial attention include the following:
* Marked discrepancy in strength between opposing muscles.
* Persistent adoption of particular postures.
* The range of abduction of the hips.
* The development of any tendency towards asymmetry either in posture or in movement.
* Observation of the back for any sign of developing scoliosis or kyphosis.
Thus, a careful examination of the type outlined above will go a long way toward anticipating and preventing deformities in the child with cerebral palsy. Every pediatrician, of course, will add other items to the list shown here that he has found through his own experience to be especially useful.
Sometimes events in the child's life can increase the risk of development of a deformity, and so at these times the physical examination should be performed with special care. An illness, especially one requiring a period in bed, can produce quite rapid deterioration. A sudden increase in the child's physical abilities is usually hailed with delight, but it may result in an increase of deformities and then the pediatrician is in a therapeutic dilemma. Should he reduce the risk of deformities by curbing the child's activities, or should he accept the increase in abilities with pleasure and regard the deformities as the price to pay for progress?9 Adolescence is a time for careful follow-up. The growth spurt accentuates the risk of deformities, and the emotional adjustments require careful, sensitive management.
Often this subsequent examination will show results identical to those found when the child was examined six months earlier. There has been no "progress." When the situation is viewed in this way, there is little cause for satisfaction. But the fact of the matter is that in cerebral palsy, maintenance of the situation may indeed be progress; if there had been no treatment, the child's condition might well have deteriorated.
So the correct interpretation of the child's condition and change of condition from one follow-up examination to the next is an important aspect of the management of the case. Such an interpretation can lead to realistic guidance of the parents and to supportive advice to the therapists who are engaged in the continuing program.
Prognosis. In current medical practice and training, the skill of diagnosis receives a great deal of attention and the art of prognosis very little. Prognostication, however, is quite important in cerebral palsy. Parents want to know what the future holds. What is the worst that they may have to face? What is the best they can expect? The mother and father usually will want to know about the type of cerebral palsy, and its severity, and about the time such essential skills as walking will be acquired.
Severity. It is difficult to judge the severity of cerebral palsy in its early stages. A mistake made frequently is to assume that mildness of clinical signs is synonymous with mildness of condition.
In attempting to make a diagnosis as early as possible, one has to seek out clinical signs when they are just appearing and before they have reached their fully developed state. It is difficult for parents to realize that their baby has cerebral palsy when all there is to show for it is slight motor delay and tenseness of the muscles. In these circumstances the pediatrician is tempted to be kind and say, "it is only a mild case." Resist this temptation. The pediatrician who has the task of counseling even a single set of parents who have been given this advice during their child's infancy and now are bringing their child to him, six years later, asking why the child is still unable to sit or stand, will realize full well the dangers in such premature advice. So do not rush into making a prognosis in early cases.
Parents will be concerned about the prognosis for their child's intellectual development even more than they are about his motor disability. "How severe is he?" usually means "How mentally retarded is he?" It often will be difficult to be certain. Avoid subjective judgments. You may not be able to believe that the lovely round-faced, wavy-haired girl could be very retarded, and you are tempted to be overoptimistic. The unlovely child who does nothing may mislead you in the other direction. Have we not all seen a child with quite reasonable abilities whose parents were told when he was a year or two old that he would never be able to do anything?
Clues can be obtained from such signs as the head circumference, degree of apathy, and ability in eye pointing, which give some guidance to the probable mental ability of the child. Even so, I find prognostication in the early years so difficult that I usually say that I do not know but would wish to give as good a training program as possible for two or three years and then decide. Parents accept this approach, and if at the end of the training period they are given a gloomy outlook they accept it more readily because they know it is based on a period of lengthy observation.
Type. There exists a crude clinical classification of the different types of cerebral palsy. Being able to assign a particular child to one type helps prognostically because we know something of the expected progress of the different types. It also helps parents who feel they are dealing with a specific entity, and they may know other parents whose child is also, say, an "athetoid."
Nevertheless, the clinical classifications have grave limitations. They are very imprecise. What one pediatrician may label athetoid another may label dystonic. The classifications are approximations. For example, about 30 per cent of children labeled as having congenital spastic hemiplegia show abnormal signs on their good side when they are subjected to scrupulous examination. These children are not hemiplegic, but it is convenient to leave them so labeled because their involvement is so strongly asymmetric. The terminology is unsatisfactory. Thus, spasticity is a well-defined neurophysiologic entity. True spasticity is seldom encountered in a pure form in clinical practice, yet many children are labeled "spastic" because the term is taken to be synonymous with "cerebral palsy." If we are to be able to plan therapy more effectively and to provide more accurate prognostications, a more precise classification of cases of cerebral palsy must be developed. Our present nosology leaves much to be desired.
Specific prognosis: walking. "Doctor, will he walk?" "When will he walk?" "Why can't he walk better?" Questions such as these are pressed on pediatricians. Bleck10 found that evidence he had once relied on to make locomotor prognosis in cerebral palsy (such as reciprocal movements of the legs and intensive therapy) did not provide a useful guide when the same children were examined years later. And so he investigated several new criteria, especially the reflex patterns, and came to the conclusion that walking was unlikely to develop if the neonatal reflexes persisted and if the later postural reflexes, such as the parachute reflex, failed to appear. He comments on some of these results in his article elsewhere in this issue of PEDIATRIC ANNALS.
It is, in fact, possible to suggest a number of items that, if present, make it likely that the child will learn to walk, and, if absent, make it unlikely. Favorable signs for a good prognosis for walking include:
* Absence of neonatal reflexes
* Presence of supporting and parachute reflexes
* Ability to pull up to standing
* Ability to transfer weight from leg to leg, as in sideways cruising
* Ability to stand
Great ability in a motor skill not directly concerned with the development of vertical stability does not necessarily mean that walking will develop. For example, a two-year-old cerebral-palsy child could crawl rapidly and with a perfectly normal pattern yet could not walk then or even four years later.
Crudely, the better the intelligence the better the chances of walking. But there are reservations. Possession of an above-average intelligence cannot produce walking in the face of overwhelming motor impairment. This fact is shown by some athetoid children. Mental retardation may reduce the likelihood of walking and reduce the effectiveness of therapy but should never be used as an excuse for not trying to get the child walking.
Children with ataxic cerebral palsy seem to be little affected on examination, but when they attempt to walk they are seriously impaired. In these children the development of walking is often delayed much longer than the pediatrician expects.
The presence of dislocation of the hip and of deformities of the legs do not necessarily account for the failure to walk. Pediatricians should be wary of advising surgery in these cases in the expectation that it will assist the child to walk. Usually, when there exists the potential for walking the child will do so despite the dislocation or deformity.
With experience, prognostication of walking and other attainments will become easier, although both pleasant and disappointing surprises will dog the pediatrician's footsteps. This will continue to be so until we know more about the chronic neurologic and developmental disorders of childhood.
1. Taft, L. T. Early recognition of cerebral palsy. Pediatr. Ann. 2 (December, 1973), 30-46.
2. Holt, K. 5. How and why children move in movement and child development. In Holt, K. S. (ed.). Developmental Paediatrics. London: William Heinemann, Ltd., 1975.
3. Holt, K. S. Developmental guidance. Child Care Health Dev. (in press).
4. Gillette, H. E. Systems of Therapy in Cerebral Palsy. Springfield, Ill.: Charles C Thomas, Publisher, 1969.
5. Levitt, S. The Treatment of Cerebral Palsy and Motor Delay. Oxford: Blackwell Scientific Publications, Ltd., 1977.
6. Holt, K. S. Facts and fallacies about neuromuscular functions in cerebral palsy as revealed bv electromyography. Dev. Med. Child Neurol. 8 (1966), 337.
7. Holt, K. S. A suggested medical classification of handicapped children. Arch. Dis. Child. 32 (1957), 226.
8. Chess, S., Korn, S. J., and Fernandez, P. B. Psychiatric disorders of children with congenita) rubella. New York: Brunner/Mazel, 1971.
9. Holt, K. S. Deformity and disability in cerebral palsy. Der. Med. Child. Neurol. 5 (1963), 629.
10. Bleck, E. E. Locomotor prognosis in cerebral palsy. Der. Med. Child Neurol. 17 (1975), 18.