Pediatric Annals

EDITORIAL 

Cerebral Palsy-A Permanent Disability

Milton I Levine, MD

Abstract

There have been times in my life when I acted in a way that I have regretted ever since. One of these occurred during my high school years.

The boy had cerebral palsy of the athetoid type. He was a most unattractive teenager with twitching and writhing movements of his face, a ptosis of one eyelid, and a mouth that was constantly drooling. In addition, his clothes were often unkempt, and the fly of his trousers was always open. He was as bright as any of us in his schoolwork but he was rejected and friendless.

I felt sorry for this boy, realizing that he was not responsible for his tragedy, so I started speaking with him and once even walked him to his home.

But then I found him clinging to me every time he saw me in school, or waiting outside the entrance for me to come out. Soon I found that I was losing friends for they could not stand close association with my grotesque schoolmate.

So I started rejecting this boy. When I saw him coming I would turn in another direction, and when I saw him waiting in the doorway of the school I would turn and leave by another exit.

A few months later I graduated from high school and went on to college. I never saw this boy again. I do not even remember his name. But I have thought of him often and deplored my rejection of this lonely teenager who craved my friendship.

I went through medical school and learned about this strange and permanent abnormality. We knew that the athetoid type often followed kernicterus and we felt that much of it was due to intracranial hemorrhage which was thought to be the result of poor or difficult obstetrical procedures. These were the opinions of pediatricians in the mid-1920s and 1930s. I was on the staff of the Pediatric Department at Cornell Medical College at that time. In the late 1930s one of the pediatricians in our Department, Dr. Margaret Dann, surveyed premature infants and found that a high percentage of them had defects of various types. So we included prematurity among the possible causes of cerebral palsy.

Now, we thought, if we could only find a means of preventing kernicterus, improve obstetrical methods, and prevent prematurity we might have most of the problem solved.

Then in 1941 Dr. Philip Levine collected convincing evidence that erythroblastosis fetalis which led to kernicterus resulted from the isoimmunization of an Rh-negative mother by the Rh-positive red blood cells of the fetus. And, as we all know, this knowledge was followed by the use of exchange transfusions, and later by administering RhoGAM to negative, unsensitized mothers. So one of our great objectives had been accomplished - the practical disappearance of kernicterus.

This approach has without doubt been responsible for the decrease in the incidence of athetosis and ataxia. Dr. Neils L. Low noted in 1977 that in the 20 previous years this decline had been noted in the Cerebral Palsy Clinic of the Columbia Presbyterian Medical Center in New York City.

Other probable causes of cerebral palsy have been more difficult to prevent. Prematurity is one such factor for it has been found that 20% to 25% of all children with cerebral palsy have birthweights less than 2,500 grams. But what effective means have been found in the prevention of prematurity? The use of certain drugs such as Provera and diethylstilbesterol have been used, the latter drug being associated with an increased risk of clear cell adenocarcinoma of the vagina and cervix in the ,adolescent…

There have been times in my life when I acted in a way that I have regretted ever since. One of these occurred during my high school years.

The boy had cerebral palsy of the athetoid type. He was a most unattractive teenager with twitching and writhing movements of his face, a ptosis of one eyelid, and a mouth that was constantly drooling. In addition, his clothes were often unkempt, and the fly of his trousers was always open. He was as bright as any of us in his schoolwork but he was rejected and friendless.

I felt sorry for this boy, realizing that he was not responsible for his tragedy, so I started speaking with him and once even walked him to his home.

But then I found him clinging to me every time he saw me in school, or waiting outside the entrance for me to come out. Soon I found that I was losing friends for they could not stand close association with my grotesque schoolmate.

So I started rejecting this boy. When I saw him coming I would turn in another direction, and when I saw him waiting in the doorway of the school I would turn and leave by another exit.

A few months later I graduated from high school and went on to college. I never saw this boy again. I do not even remember his name. But I have thought of him often and deplored my rejection of this lonely teenager who craved my friendship.

I went through medical school and learned about this strange and permanent abnormality. We knew that the athetoid type often followed kernicterus and we felt that much of it was due to intracranial hemorrhage which was thought to be the result of poor or difficult obstetrical procedures. These were the opinions of pediatricians in the mid-1920s and 1930s. I was on the staff of the Pediatric Department at Cornell Medical College at that time. In the late 1930s one of the pediatricians in our Department, Dr. Margaret Dann, surveyed premature infants and found that a high percentage of them had defects of various types. So we included prematurity among the possible causes of cerebral palsy.

Now, we thought, if we could only find a means of preventing kernicterus, improve obstetrical methods, and prevent prematurity we might have most of the problem solved.

Then in 1941 Dr. Philip Levine collected convincing evidence that erythroblastosis fetalis which led to kernicterus resulted from the isoimmunization of an Rh-negative mother by the Rh-positive red blood cells of the fetus. And, as we all know, this knowledge was followed by the use of exchange transfusions, and later by administering RhoGAM to negative, unsensitized mothers. So one of our great objectives had been accomplished - the practical disappearance of kernicterus.

This approach has without doubt been responsible for the decrease in the incidence of athetosis and ataxia. Dr. Neils L. Low noted in 1977 that in the 20 previous years this decline had been noted in the Cerebral Palsy Clinic of the Columbia Presbyterian Medical Center in New York City.

Other probable causes of cerebral palsy have been more difficult to prevent. Prematurity is one such factor for it has been found that 20% to 25% of all children with cerebral palsy have birthweights less than 2,500 grams. But what effective means have been found in the prevention of prematurity? The use of certain drugs such as Provera and diethylstilbesterol have been used, the latter drug being associated with an increased risk of clear cell adenocarcinoma of the vagina and cervix in the ,adolescent female children of such treated mothers.

So far the prevention of prematurity has been generally elusive, although care of the premature infant has greatly advanced in recent years.

Another important etiological factor in the development of cerebral palsy is hypoxia. Some advance has been made in this area during the perinatal period by the modern approach of monitoring infants during labor. But there are undoubtedly many cases of hypoxia in the prenatal period that cannot be determined at the present time.

There are certain cases of cerebral palsy due to neurological damage during the early gestational period such as by drugs taken by the mother, radiation or through infections. In addition, there are probably a number of cases of genetic origin.

So the problem of prevention is not only difficult but multifaceted. The diagnosis of cerebral palsy is most difficult during the first 12 months of life. Furthermore, basic neurological damage is permanent and the condition will exist for the life span of the child.

The pediatrician must be the mainstay of this child's life during infancy, childhood and adolescence - and he or she must strongly support the parents as well.

This has become of increasing importance in recent years when so much has been accomplished in aiding the affected child to reach the highest potential both physically and emotionally. As an example, Dr. Martin Diamond in his excellent article in this present symposium, writes of the values of orthopedists, psychiatrists, neurologists, physiotherapists, urologists, occupational therapists, speech therapists, recreational therapists, orthotists, biomedical engineers, specialized teachers, nurses and social workers. This same article mentions computer-driven devices responding to the voice.

The pediatrician who would provide the best care for a patient with cerebral palsy will see to it that the child receives the full advantage of all the optimal services.

As Guest Editor for this important subject we have once again called on Dr. Lawrence T Taft, Professor and Chairman of the Department of Pediatrics of the Rutgers Medical School in New Brunswick, New Jersey. Dr. Taft, an eminent pediatric neurologist, has for many years taken special interest in the child with cerebral palsy.

This symposium covers a broad review of the subject including newer knowledge on the etiology, diagnostic measures, and the latest methods of treatment.

The first article deals with "Etiologic Factors in Cerebral Palsy" and has been written by Dr. Nigel Paneth of the Sergievsky Center, the Division of Epidemiology, and the Department of Pediatrics College of Physicians and Surgeons, Columbia University.

This most interesting review covers our recent knowledge of the various causes of cerebral palsy. A great deal of knowledge was gained from the Collaborative Perinatal Project, but the author also has a large reference list through 1984 from which he draws his conclusions.

As I mentioned at the outset of this introduction, many of us thought that morbidity due to cerebral palsy would be greatly reduced if we could conquer kernicterus and provide better obstetrical care. That was more than 40 years ago. Dr. Paneth now reports that in spite of medical advances the number of CP cases has not declined - possibly because at the present time we are keeping more premature infants alive.

This paper discusses the major as well as the minor risks, such as environmental toxins and alcoholic mothers. This is an excellent and most informative discussion, but much more is still to be learned.

The second paper discusses "Early Signs and Differential Diagnosis of Cerebral Palsy." It is presented by Dr. Gabor Barabas, Associate Clinical Professor of Pediatrics and Neurology and Dr. Lawrence T Taft, Professor and Chairman of the Deparment of Pediatrics, both at the University of Medicine and Dentistry of New Jersey-Rutgers Medical School.

The authors note at the outset that cerebral palsy is a clinical syndrome due to many causes and is not a distinct disease. They follow with a classification of the neurological signs and describe the various types of the condition. An excellent discussion of the differential diagnosis and the prognosis follows.

The third contribution, entitled "Early Neuromotor Reflexes in Infancy" discusses the early diagnosis of cerebral palsy during the first 6 to 12 months of life. It is presented by Dr. Arnold J. Capute, Associate Professor of Pediatrics at the Johns Hopkins University School of Medicine, and VicePresident for Medical Affairs of the Kennedy Institute for Handicapped Children in Baltimore.

Dr. Capute starts by examining the primitive reflexes, including those of the intrauterine life as well as those that are postnatal. These early primitive reflexes gradually subside, but if any remain for an unusual period of time or are exaggerated they might indicate a cerebral impairment or injury.

The various early reflexes are carefully described and discussed. The author couples the neurological findings with the developmental. With this knowledge the pediatrician, watching for signs of primitive reflex appearance and suppression, would have the ability for early detection of marked motor handicaps. This is a valuable guide for those who would detect the early evidence of cerebral palsy.

The following paper is an excellent guide to the modern approach to the rehabilitation of children with cerebral palsy. It is written by Dr. Martin Diamond, Pediatric Psychiatrist at the Children's Specialized Hospital, Mountainside, New Jersey. Dr. Diamond is also Assistant Clinical Professor of the Department of Physical Medicine and Rehabilitation of the New Jersey School of Medicine, University of Medicine &. Dentistry of New Jersey.

I mentioned earlier in this introduction the numerous members of the rehabilitation team who work together to give the greatest opportunity to the child, both physically and emotionally. One by one Dr. Diamond describes the various methods of therapy and what they aim to accomplish. He notes that the most recent approach is by "neurodevelopment" therapy - a therapy aimed at inhibiting abnormal primitive reflex patterns, and emphasizing the development of trunk and extremity movements separately.

The infant stimulation programs are presented where, as soon as the diagnosis is made, infants are placed under the guidance of early intervention learning specialists, with the extra aid of physical, occupational and speech therapists. The use of drugs to relax muscle tension, controlled intensity electrical stimulation, peripheral nerve blocks, as well as bracing and adaptive equipment are all discussed.

Dr. Diamond concludes that the child's pediatrician ought to be aware of what should be done in a rehabilitation program so that the maximal functional independence is achieved.

The final contribution, "Counseling Families of Children with Cerebral Palsy" is authored by Dr. Mark L. Wolraich, Associate Professor of Pediatrics at the University of Iowa, and Medical Supervisor of their Division of Developmental Disabilities.

This covers an area which is among the most difficult for the pediatrician. What to tell the parents? When to tell the parents? How to tell the parents? What to tell the siblings? What about other relatives? This thoughtful article deals with all these problems and offers advice on how to communicate with parents and patients with humaneness.

Dr. Wolraich suggests participation in parent groups where experiences can be shared, but realizes that some parents may not be ready to talk to others immediately. He also discusses the importance of counseling the siblings who so often resent the amount of time being given to their handicapped brother or sister.

A further important directive in this article is for the pediatrician to correlate all the information obtained from the many disciplines involved in the development of the cerebral palsied child both physically, educationally and emotionally.

Dr. Wolraich mentions that children with cerebral palsy frequently have psychosocial needs, the fulfillment of which is important for all children but especially for adolescents with cerebral palsy. Once again I remember my "friend" of those long-ago high school days.

10.3928/0090-4481-19860301-04

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