If one were to ask me to remember the most serious neurological case I can recall after over 50 years of practice I would have no difficulty answering.
She was a girl of 9 or 10 years of age brought to me with a history of weakness and numbness of both feet occurring within a few days. I made the diagnosis of Guillain-Barré Syndrome as the weakness started creeping insidiously up her body.
After reassuring the parents that almost all children and adults with this condition recover completely, I hospitalized the child. Perhaps I should not have been so optimistic, I thought, as the paralysis started creeping upward and finally involved her costal muscles.
There was respiratory difficulty so we placed the child in a respirator and soon were forced to do a tracheostomy with frequent suctioning to remove the mucus which collected in the bronchus. She was receiving moist oxygen and seemed in fairly good condition when I left the floor at 8 PM.
I always had difficulty sleeping when any of my patients were seriously ill, so I called die pavilion at 2 AM to inquire how Janet was doing. "She's doing OK," the resident answered, "but there's one funny thing - she isn't breathing with the respirator."
Suddenly agitated and almost tremulous I realized that the child was anoxic and that her body was struggling severely to obtain oxygen, so much so that the child was gasping violently against the slow mechanical breathing of the respirator.
I threw my clothes over my pajamas and drove quickly to the hospital, disregarding one red light after another. When 1 arrived on the floor Janet had just undergone a series of convulsive seizures. She was unconscious and was still fighting the respirations of the machine.
The moist oxygen was flowing but it was obvious that too little was reaching her lungs. I suctioned through the tracheostomy tube and quickly realized that there was a thick mucus plug below, too thick to relieve with simple suction. I was fearful that I would lose the child as she went through another severe convulsion.
I had to loosen the mucus so I sent a flow of warm nebulized water into her trachea, suctioning constantly. In a few minutes the plug loosened and I was able to suction the mucus out. Janet then resumed breathing with the respirator and slowly recovered.
As far as I could ascertain she suffered no brain damage. She later graduated from high school and college.
Like all of us I have had my share of children with traumatic neuropathies, even one girl with paraplegia. I have had a few cases of cerebral palsy, and several with muscular dystrophy. But most of the neurological complaints brought by parents relate-to habit spasms, overactivity, headaches and learning difficulties.
For this issue of Pediatric Annals devoted to neurology the Guest Editor is Dr. Lawrence T. Taft, Professor and Chairman of the Department of Pediatrics of Rutgers Medical School, University of Medicine and Dentistry of New Jersey. Dr. Taft is one of the leading pediatric neurologists in the US.
He has chosen for this symposium five areas of interest to all pediatricians - neonatal morbidity focusing on abnormal movements, intracranial hemorrhage, and asphyxia; headaches in childhood; peripheral neuropathy; tics; and cerebellar disorders in childhood.
The first article concentrates on abnormal neonatal movements, intracranial hemorrhage, and asphyxia. It is an excellent review of the most recent knowledge on these subjects and has been written by Dr. M. Richard Koenigsberger, Associate Professor of Neurosciences and Pediatrics at the New Jersey Medical School, University of Medicine and Dentistry of New Jersey.
The author opens this discussion by focusing on the motor and behavioral patterns of the newborn that signify seizures. He specifies in detail tonic postures, abnormal eye movements, abnormal respiratory patterns and repetitive stereotype movements. He notes that the gestational age of the infant as well as the type of seizure activity will often localize the cause of convulsive seizures.
Dr. Koenigsberger, in discussing intracranial hemorrhages emphasizes especially the intraventricular-periventricular hemorrhages (IVH-PVH) that account for 40% to 50% of those occurring in newborns weighing less than 1500 g.
He concludes by noting our present knowledge of prenatal and postnatal asphyxia.
The second article deals with the management of headaches in childhood and has been contributed by Dr. Gabor Barabas, Chief of the Division of Pediatric Neurology at Rutgers Medical School in New Jersey.
This is a presentation that should be of great interest and value to all practicing pediatricians for the author offers the reader an organized approach to the problem of headaches in children. Dr. Barabas carefully differentiates the pain from intracranial causes with those of extracranial etiology. Most of us are fairly capable of diagnosing the source of headaches if the cause is external (eg, eye strain, sinusitis, infected teeth, otitis media and bacterial or viral infections. We are less adept at pinpointing the pain of emotional etiology, and usually need extra procedures to diagnose intracranial lesions. Dr. Barabas warns against attempting to localize intracranial lesions from the localization of referred pain.
The article concludes with a discussion of migraine in children with diagnoses, treatment, and, when necessary, prophylaxis.
The third contribution discusses "Peripheral Neuropathy in Children" and has been written by Dr. Andrew K. Hodson, Assistant Professor of Pediatrics and Medicine (Neurology), Division of Child Neurology at Duke University Medical Center.
I would imagine that most practicing pediatricians when asked to name conditions of peripheral neuropathy would probably mention the Guillain-Barré Syndrome, muscular dystrophy, poliomyelitis and possibly the neuropathy that occasionally occurs in diabetes.
But along with these, as Dr. Hodson points out, there are numerous conditions in the peripheral neuropathy and these he carefully considers. The article is divided into two segments - the first covering myelinopathic disorders, and the second, neuropathic disorders such as those due to a dying back of nerves.
Fortunately the peripheral neuropathies during childhood are rare, which make the article all the more important by instilling an awareness in the reader. As I have often remarked, it is the ability to diagnose the unusual cases that differentiates the capabilities between pediatricians.
The next article is a discussion of "Tics in Childhood" and is authored by Dr. Gerald S. Golden of the Department of Pediatrics and Neurology of the University of Texas Medical Branch at Galveston.
At the outset Dr. Golden notes that the traditional viewpoint is that tics are primarily psychological in origin but then goes on to state that neurochemical studies " . . .are now focusing on specific brain abnormalities in the disorders associated with multiple tics." He even suggests that all tic disorders may have a primary neurochemical basis.
Dr. Golden classifies tics in two categories - simple tics and completed or multiple tics - the latter including Tourette Syndrome.
On the basis of my own considerable experience, I would definitely differentiate the simple from the complex type. There is no reason to doubt that most simple tics are essentially emotional in etiology. I have seen a number of instances where if one child in a class has a facial tic, others in the class soon develop similar tics. I have seen children under stress develop tics which subsided when they are relaxed. At times I have given low doses of tranquilizers to children with tics and in every instance have had the tics disappear within two weeks. Occasionally, however, the treatment had to be repeated but all tics cleared within a few months.
I have had very little experience with Tourette Syndrome with which Dr. Golden has had extensive experience. But even though this phenomenon is a complex of tics, I cannot help but feel that it is a different neurological condition from that of simple tics.
Dr. Golden gives an excellent description of the Tourette Syndrome with its genetic evidence, which even suggests in his words an autosomal dominant gene. The CT scans are normal and the EEGs, while often abnormal, are non-specific and of no diagnostic value. Medical treatment of the syndrome is specified. The condition is chronic and once it subsides may return under periods of stress. The evidence for possible abnormalities of the central nervous system dopamine metabolism is presented.
The final paper in this symposium concerns "Cerebellar Disorders in Childhood" and has been contributed by Dr. Joseph H. French, Deputy Director of the Clinical New York State Institute for Basic Research in Developmental Disabilities, Staten Island, New York, and Professor of Neurology and Pediatrics at Downstate Medical Center in Brooklyn, New York, and his co-author, Julius B. Familusi, M. B., F.R.C. P. (Glasgow), who is a visiting Fulbright scholar at the Clinical New York State Institute for Developmental Disabilities, Staten Island, and visiting Professor of Neurology at Downstate Medical Center, Brooklyn.
This is an excellent and well-organized review of the many entities that may cause cerebellar malfunction and the signs and symptoms accompanying these disorders.
After a scientific introduction describing the anatomy and histology of the cerebellum, Dr. French proceeds to discuss clearly the various clinical manifestations associated with cerebellar problems.
Many of us, as practicing pediatricians, have from time to time, seen certain of our patients with such signs as ataxia, tremors, hypotonia, and diminished reflexes, dysarthria, and disturbances of ocular motion. Each of these cerebellarrelated findings are carefully discussed as are the secondary effects of cerebellar disease such as headache, vomiting, papilledema, cranial nerve palsies, altered mentation, and certain vital sign changes.
But these are just the clinical manifestations of an underlying disease process. The problem is to define the etiology. This Dr. French covers step by step - the infections, whether viral, bacterial, fungal, or parasitic - and the tumors, including those most frequently encountered such as meduloblastomas, gliomas, and the astrocytomas. The accepted modern treatment, as well as the prognosis for each of these entities, is given. This is a most interesting and profitable review of an important subject.