Annual Meeting of the American Academy for Cerebral Palsy and Developmental Medicine, 1976

Develop. Med. Child Neurol. 1977, 19, 105-122

Annual Meeting Cerebral Palsy

of the American Academy for and Developmental Medicine,

1976 Reported bu Kevin Connollu

An Englishman at a Yankee Coiirt THE 30th annual meeting of the Academy, in this the bicentennial year, was held at the Century Plaza Hotel in Los Angeles between 12th and 16th September 1976. A significant decision was to change the name of the Academy to The American Academy for Cerebral Palsy and Developmental Medicine.

Any summary of a conference inevitably reflects the idiosyncrasies of the writer, and I would make it clear from the outset that this report is based entirely upon my own personal impressions. A large and important meeting such as the Academy’s annual get-together is of course about much more than the scientific papers presented. The various committees of the Academy transact business, instructional courses are mounted, honours are bestowed, old friends meet to discuss old problems and new friendships are made.

The venue chosen for the meeting itself merits comment. After a very long journey from London to Los Angeles, the surprise of an Englishman when helped from his taxi at 5 a.m. by a hotel doorman dressed in the uniform of a Yeoman of the Guard (vulgarly known as Beefeaters) can hardly be imagined. This awe inspiring sight set the tone for the hotel, everything larger than life-including the prices. The arrangements for the meetings themselves were excellent and those responsible are to be congratulated. But I wonder if i t is not possible to select some less costly location, perhaps in an academic setting. Many members of the Academy who attend and derive great benefit from these meetings, find difficulty in meeting such costs. However, even being stuck on a concrete island because of a transport strike had its compensations: it probably led to rather more professional and scientific interaction amongst delegates than might have otherwise been the case but for the enforced ‘closeness’.

The programme for the meeting was made up of twenty-four instructional courses, four symposia, twenty-four scientific papers, the Richmond Award Paper, a special evening session on non-verbal communication techniques and the Presidential Address. Over-all the standard was high and some excellent material was presented.

I managed to sample only about a quarter of the instructional courses, since eight courses ran in parallel on each of three mornings. The practical arrangements were excellent. None of the courses which I attended was poor, one was excellent and the others varied from good to rather ordinary. Inevitably there will be variations in quality between courses, variations reflecting the skill, thoroughness and level chosen by those presenting the course. My own experience, coupled with comments picked up during coffee breaks led me to form the view

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that the instructional course programme should be maintained, though perhaps the time has come for some change and some experiment. Instructional courses last for two and a half hours, from 8 a.m. The time is good but the duration is shocking. Few people can focus their attention and process densely packed information for such long periods, even given a short coffee break. Of course it is quite possible that the North American constitution and neural processing machinery are honed to a much finer point than the European, but I do wonder if i t might be possible to have instructional courses run over two successive days, with something like a one-hour session on each. Although some of the courses led to quite a lot of questions from the ‘students’, some way should be found to develop more of a ‘seminar’ atmosphere to encourage greater and deeper discussion.

The four symposia included in the programme dealt with widely varied issues; communication, perinatal monitoring and intensive care techniques for the newborn, cerebellar stimulation for the treatment of movement disorders and bioengineering in rehabilitation. This last symposium was in two parts, one dealing with gait analysis in cerebral palsy, the other with rehabilitation devices in cerebral palsy. The four short papers and associated discussions on gait analysis were most instructive. In the symposium on cerebellar stimulation, which was perhaps the most research orientated of the four, Drs. Penn and Gilman ably supported by Ms. Etzel presented some preliminary but sophisticated work which raised important theoretical, clinical and methodological problems. I am sure we shall hear more of this work in the years to come. The symposium on perinatal monitoring and intensive care of the newborn was directed at important and immediate practical questions and the views reported deserve to be widely known and discussed. The symposium on communication ranged over the widest area and included, amongst other things, a preliminary report on a twin study, spectrographic analysis of dysarthrias, anatomical abnormalities relevant to expressive speech, Blissymbolics and the Si/Comm communication system. Like the curate’s egg, this symposium was better in some parts than others, but it should provide rich vein to mine for future instructional courses on non-verbal communication. I very much hope this will be the case, with fundamental theoretical questions treated alongside and in conjunction with practical methods of training.

Limitations on time and space prevent anything other than a brief comment on the contributed papers, many of which were excellent. Hoffer and colleagues reported on a series of elegant and exciting experiments in which questions concerning the functional plasticity of central neurons were explored by investigations involving the transplantation of brain tissue into the eyes of rats. Most of the Academy’s members are concerned with the clinical care and educational management of children, but it is important that they also be kept aware of the purpose of basic research in the neurosciences and there can be fewer more successful ways of ensuring that this is done than by inviting excellent lecturers to describe their work. I am sure that the Programme Committee will continue to strive for a balanced programme, covering not only clinical research and new techniques but also keeping members abreast of developments in basic science. Improvements in practice have after all some relationship to developments in fundamental scientific knowledge. Drs. Segall and Gates gave a very clear presentation on new means of examining blood flow through the brain using a technique of computer tomography. Many other good papers were presented, but the area which seemed to this reporter most lacking was developmental psychology, though here I must admit of a special interest and perhaps a sharper level of criticism.

The Richmond Award paper Cerebral Palsy-Evidence of Failure .? was written by Dr. McManus and was presented most skilfully and eloquently by Dr. Mercer Rang. Special

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praise is merited for the superb visual aids which were used: it was quite a tour de force. The theme of the paper was that a substantial proportion of cases of cerebral palsy could be prevented by the application of our existing knowledge. The argument was compelling, and a clear duty falls upon the medical profession, amongst others, to implement their existing knowledge and so reduce the incidence of this tragic condition.

The Presidential Award for 1976 was made to Mr. Edward V. Roberts, a severely handicapped person recently appointed as Director of Services for the Handicapped in California and creator of the Center for Independent Living. In his short address in accepting the award, Mr. Roberts spoke of the emergence of the disabled as full members of the com- munity, but commented on the social and economic disincentives to independence. He said that not only does biomedical research on handicap need to be done but also social, economic and political changes are needed.

The Academy’s bicentennial President was introduced very cleverly by the Past President, Dr. Hans Zellweger, whose model of the Academy was taken from microbial genetics. He distinguished for the audience between structural genes, operator genes and regulator genes on his way to presenting Gene Bleck. In his urbane manner the President gave a very witty lecture in which he spoke of the importance of orthopaedics in child health and outlined the specialism of developmental orthopaedics with its own peculiar and important practical and theoretical problems. Dr. Bleck himself has also made a distinguished contribution to this important speciality.

1 was interested to witness again what 1 believe a distinguished member once called the tribal rites of the Academy. 1 thought that it was only in Britain that important personages were conducted to installation ceremonies as if in the charge of two jailers. I refer of course to new peers being introduced to the House of Lords flanked on either side by their spon- sors. I note, however, something of the pre War-of-Independence spirit still lingers in the United States as the President of the Academy is escorted to the podium flanked by a pair of past presidents.

Finally a cri de roeur. I would not be human if I let this opportunity pass without getting in a plea about something close to my heart-the English language. There is abroad these days what I have come to believe is a conspiracy against the English language (and also against the American language which is rich and vigorous). Not content with speaking and writing in such ugly codes as MBD or DPCS, which incidentally I shall do my utmost to suppress, there is an insidious tendency to invent unnecessary and often ugly words with which to confuse others. For example, while listening to one interesting paper 1 was com- pletely at sea when the author spoke of ‘desynthesising’ his data. (My copy of the Shorter Oxford English dictionary does not include this word). Eventually I realised that the author was speaking of the opposite of synthesis, which is not desynthesis but analysis-a per- fectly good word. Please may we stick to a clear and unambiguous use of our very satisfac- tory common language.

I enjoyed this meeting: it was stimulating, interesting and sometimes exciting. Clearly the Academy has enormous potential strength from the rich blend of professional skills and expertise which i t brings together.

Following are condensed summaries of many of the papers, in the order in which they were presented.

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A Study of Two Sets of Identical Twins, Male and Female, in Infancy: A Compari- son of Developmental Growth in All Areas, with Special Emphasis on Language. RUTH M . LENCIONE, JUDY HOWARD, KATHRYN TROWBRIDGE.

This study was undertaken to document longitudinally the similarities and differences in the total development of two sets of twins aged I1 to 21 months. The twins were monozygotic, one male and one female, and in each pair one was normal and one had mild to moderate spastic cerebral palsy. A complete pediatric history, physical and neurological examination has been recorded from birth, a Gesell Developmental evaluation has been done each six-month period, monthly audiotapes have been recorded of each infant’s vocalizations, and three videotapes were made at four-month intervals. The results were presented in each area of development, and an analysis of phonetic transcriptions of language and speech acquisition were demonstrated with videotapes.

Anatomical and Performance Abnormal- ities Pertinent to Expressive Speech. JAMES F. BOSMA.

Speech is accomplished by current anatomical structure of the larynx, phar- ynx and mouth. Early speech efforts of the infant employ motor implements which are anatomically much different from those of the child or adult.

Speech accomplished by malformed structures may require remarkable motor adaptations, as is seen in patients with hypoplasia of the tongue, of the velum, or of motor unit (Moebius syndrome).

Competence of current feeding, airway maintenance and various upper respiratory adaptations depends upon adequacy of the local sensory cues. In developmental perspective, the central representations of these performances are generated upon

a matrix of local sensory experience, later supplemented by sensory inputs from other sources, such as hearing. But this is not simply a matter of the quantity of local sensory input. It is noteworthy that adequate speech is achieved in most forms of primary genetic variation of mucosal sensibility.

Disability of speech is associated with impairments of perception from the speech apparatus. As a generality, central representation of speech is linked with central representation of more complex percep- tions in the oral region.

Clinical Implications from Spectrographic Studies of Dysarthrias in Cerebral-palsied Speakers. ALVIRDA FARMER.

This paper described individual and group habituatory speaking patterns found in dysarthrias. Data from a variety of spectrographic studies of the speech of 32 cerebral-palsied speakers ranging in age from four to 44 years were used to illustrate clear examples of specific behaviors. Segmental duration analyses of specific individual speaking strategies suggest that traditional articulation techniques are contra-indicated for this population. Gen- eral and specific electromyographic and acoustic feedback techniques appear to be more effective for modifying habituated speaking patterns.

Longitudinal Study of 20 Infants and Children with Cerebral Palsy using Sim- ultaneous Auditory Feedback Procedure for Language and Speech Management. RUTH M. LENCIONE.

A nine-month study of 20 cerebral- palsied children aged between three and eight years was undertaken to examine the effectiveness of an alternative system of language and speech management, using an adaptation of Berko’s (1965) Auditory Feedback Mechanism.

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Ten children (the experimental group) received 30 minutes per week of simultaneous auditory feedback therapy, and 10 children (the control group) received the same amount of traditional speech therapy. Monthly phonetic transcriptions were made of stop plosive words for each group. The results indicate that eight of the children receiving simultaneous auditory feedback therapy developed moderately intelligible competence with stop plosive sounds, and two children in the control group achieved essentially the same level of performance.

The Use of Blissymbolics as a Communi- cation Medium for the Non-speaking Person. SHIRLEY MCNAUGHTON.

Blissymbolics is a non-verbal and visual- graphic communication system which is based on simple pictographic and ideo- graphic components which can be combined and recombined to form new concepts. I t provides a total and compre- hensive communication system. The first application of Blissymbolics as a communication medium for non-speaking physically-handicapped children was at the Ontario Crippled Children’s Centre in 197 1. Its extended use is being standardized by the Blissymbolics Communication Foundation through dissemination of instruction materials, consultant services, instructor training workshops and establishment or resource centres. Symbol usage is being documented in an Ontario Ministry of Education evaluation study which provides detailed information per- taining to high and low symbol achieve- ment and offers criteria for initial selection, programming and continuing assessment. Results of the two-year study will be available in 1977 in the form of a handbook for parents, instructors and administrators. The population studied encompasses a wide range of intellectual capabilities,

degrees of disability and age-levels in both home and institutional settings.

Si/Comm: a System of Communication for Non-verbal Children-an Experimental Model. CARMELITA D. HEINER.

The Si/Comm Board is a picture- language system which was designed to meet some of the communication and personal needs that occur in many activities of daily living for the non- verbal severely cerebral-palsied child.

The plan of the board provides left to right progression of word order (syntax) which is color-coded to designate personal pronouns, proper nouns, nouns, verbs and modifiers.

At present the Si/Comm model is in use in more than 100 clinics and schools in the United States and in England. This report summarized the progress made with three non-verbal children with whom the model was used.

Implications and Considerations for the Application of Non-vocal Communication Techniques and Aids. GREGG C. VANDERHEIDEN.

Probably the most crippling of all handicaps is the inability to communicate, to interact with and relate to others in a meaningful way. Many children and adults are unable to- communicate because of severe physical impairment. Techniques and aids have been developed which can provide these people with the effective communication needed for cognitive development and personal growth. Tech- niques have been developed which can be used by the most severely physically handicapped and by severely mentally retarded children and adults. The major reason there is not widespread application of these techniques is the lack of information on them and the fear that their use may deter speech. With the results of

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research and clinical programs now showing that these techniques do not deter speech and, in many cases, facilitate its development, and with the recent increase in publications in this area, these obstacles are beginning to diminish. Awareness of and familiarity with these techniques is an important factor for those physicians and therapists working with the severely physically handicapped child or adult.

Prognosis of Children Surviving after Ventilatory Assistance in the Newborn Period. JOHN D. JOHNSON, NATALIE C. MALA- CHOWSKI, PHILIP SUNSHINE.

One of the major concerns about the use of neonatal intensive care techniques has been that an increased number of infants will survive with severe handicaps. Evalua- tion was made of the long-term prognosis of infants surviving respiratory insufficiency with the aid of ventilatory assistance as a means of assessing the success of neonatal intensive care. Follow-up data were obtained on 54 survivors of ventilator treatment born between 1962 and July 1969. Mean age at follow-up was 5 -7 years. Only 20 per cent of the survivors had significant neurological handicaps (cerebral palsy, deafness, blindness) and/or IQ

scores less than 85. Mean IQ for the total group was 110. Significant handicaps were seen more frequently in children who had had birthweights below 1500g.

Follow-up of infants born between August 1969 and December 1972 gave almost identical results. However, preliminary data on the follow-up of infants born in 1973 and 1974 indicate an improved prognosis, especially for infants with birthweights below 1500g (neurological handicaps in five out of 31 (16 per cent) of the survivors weighing less than 1500g at birth, vs 14 out of 27 (52 per cent) in previous years). Factors which might have contributed to improved outcome

in the group of infants born in 1973 and 1974 include earlier institution of ventilatory assistance and improved methods of ventilation, with the use of continuous gas flow, positive end-expiratory pressure and inspiratory pressure plateau. The incidence of chronic lung disease (bron- chopulmonary dysplasia) in infants receiving ventilatory assistance has also decreased in the more recent years.

Continued refinements in neonatal intensive care are necessary in order to further reduce the incidence of neurological, intellectual and pulmonary complications in survivors.

Cinematographic Analysis of Neonatal Automatic Walking-a Pilot Study. VENITA LOVELACE-CHANDLER, BEN LOVE- LACE-CHANDLER.

The purpose of this study was to determine the physical characteristics of the walking reflex in the normal neonate. One of the characteristics was measured: the knee-joint angle during the step cycle. 18 neonates were selected for study. The examiner elicited a placing reaction, followed by a slight tilting forward to obtain automatic walking. The procedure was filmed with a 16mm movie-camera and analyzed frame-by-frame with a ‘Vanguard’ motion analyzer. It was ascer- tained that it is possible to use cinemato- graphy to analyze movement patterns in the neonate. The gait and the range of motion at the knee were variable, but the gait was consistent in those infants who demonstrated more than one step cycle.

The Behavior of the Full-term but Under- weight Newborn Infant. H. ALS, E. TRONICK, LAUREN ADAMSON, T. BERRY BRAZELTON.

A newborn infant greatly influences the care he receives from those around him by the way he behaves. 10 thin but other-

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wise healthy full-term newborns (Ponderal Index <2*3) were compared with 10 healthy full-weight newborns (Ponderal Index >2.3) on the Brazelton Neonatal Behavioral Assessment Scale, administered on days I , 3, 5 and 10 after birth. This behavioral examination differentiated the two groups clearly on the reflexes of walking, crawling, passive movements of arms and legs, rooting and sucking, and, more importantly, on those behatiors which are of key value for the caretaker of the baby-attractiveness, need for stimulation, interactive processes and motor processes. The thin newborns did substantially more poorly than their full-weight peers. At a later point during the first year, the underweight infants showed temperamental organizational difficulties and some indication of psycho- somatic reaction to stress. It is speculated that the underweight newborn’s fragile organization elicits anxiety in the caretaker which makes a smooth interaction and stabilization of organization difficult. The possibility of subtle cerebral dysfunction resulting from poor intra-uterine nutrition cannot be ruled out. (This paper has been published in full in Develop- mental Medicine and Child Neurology, 1916, 18, 590-602.)

A Comparison of the Psychosocial Prob- lems of Deaf, of Visually-impaired, and of Non-handicapped Children. ROGER D. FREEMAN, SUSAN F. MALKIN.

In two different studies in the Greater Vancouver area, deaf children (N = 120; ages five to 15 years) and legally blind children (N=92 ; ages infancy to 20 years) were compared by means of question- naires and home visits with non-handicapped ‘neighborhood’ controls. Multi- handicapped children comprised 25 per cent of the deaf and 54 per cent of the blind groups. Contrary to clinical lore, family breakdown was no more frequent

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than for the control children. Delay in diagnosis and parental confusion were much more severe with the deaf group. Both groups were admitted to hospital more frequently in early life than the controls.

Management by physicians of the original parental suspicion and the con- firmation of the diagnosis was a much more serious problem for the deaf: parents were often reassured that with good education and effort the child would become like a normal child (untrue), and most parents never learned to communicate effectively with their child. On the other hand, parents of the blind often had to learn that their child would not be as helpless as they feared.

Both groups of handicapped children played less with children of their own age, and were permitted to do less by their parents. Behavioural difficulties were most common in the blind, but there was also an increased incidence among the deaf. Most children did not present serious psychiatric problems, nor were there large adverse effects upon the families, in spite of many stresses. Generally parents can function well as an integral part of the ‘management team’, since often they become experts in their child’s type of disability.

An Evaluation of Two Educational Treat- ment Programs for Hyperactive Children. MARGARET G. PEREBOOM, SALLY CREAG-

To evaluate the possibility of interven- tion with ‘hyperactive’ children within a public school system, 10 children fiom each of three different schools (in Grades three to six) were given a 10-week pilot treatment program. These 30 children were assigned randomly to either relaxation therapy or specific academic tutoring groups.

The relaxation therapy groups were

HAN, ANNE-MARIE MEEHAN.

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taught specific relaxation exercises. The academic tutoring groups were given subject matter remediation in the child’s area(s) of specific academic weakness.

Although behavior rating forms indicated general improvement, pre- and post-testing with objective measures indicated that no significant gains were made by either group.

Physical Therapy as a Determinant of Change in the Cerebral-palsied Infant. ALFRED L. SCHERZER, VALERIE MIKE, JEROME ILSON.

Twenty-four children under I8 months of age with an initial diagnosis of cerebral palsy were studied for a minimum of six months on either experimental or control physical therapy programs, using a double- blind study design. Medical and therapy evaluations indicated definite changes in motor, social and management areas for those subjected to the experimental procedure, and particularly among children expected to show higher intelligence. Change was found to be somewhat correlated with age at entry to the study and was more often associated with less actual treatment time. The findings suggest the need for additional treatment for the youngest children, who are most likely to have the greatest global involvement.

Evaluation of Training Voluntary Inhibition in Patients with Dystonic Cerebral Palsy. DANIEL HALPERN, LINDA EILERS, BARBARA MEYERS, KRIS BIRKMEIER, ROBERT BECK, RICHARD MCHUGH.

Nine adults with severe spastic, athetoid or mixed cerebral palsy were treated with two different training methods: (a) conscious volitional inhibition of dystonia through verbal, tactile and proprioceptive feedback; and (6) positional and direc- tional control through conscious attention and similar feedback. A cross-over design consisting of three eight-week treatment

periods was used, enabling each patient to act as his own control. Standard motor tasks were recorded photographically and five aspects of motion were scored objectively: range, direction, speed, regularity of rate and path tortuosity. Both training methods showed clear evidence of improved performance, but relative effectiveness varied in the five aspects. The patients’ learning ability in each aspect was a function of the training procedure, the amount of training and the previous training experience.

Brain Tissue Transplanted to the Anterior Chamber of the Eye: Implications for the Study of Functional Plasticity of Central Neurons. B. HOFFER, R. FREEDMAN, D. TAYLOR, L. OLSON, A. SEIGER.

One of the fundamental problems in the pathophysiology of cerebral palsy is the extent to which functional plasticity can compensate for neonatal brain injury. This question was studied by examining the growth, cytochemical organization and electrophysiological properties of rat fetal and neonatal brain-tissue, homologously transplanted to the anterior eye chamber of adult female rats. The transplants provoked a rapid stromal ingrowth from the host iris, and the establishment of an extensive vascular plexus was seen within a few days. Cerebellum, hippocampus and cerebral-cortex transplants proliferated 10- to 20-fold in oculo and developed a normal laminar architecture histologically. Golgi stains revealed that the hippocampal pyramidal neurons elaborated an extensive apical and basilar dentritic tree. Electro- physiological studies showed intrinsic ex- citatory and inhibitory synaptic circuits in all three types of transplants. Electro- encephalographic evidence of hippocam pal theta rhythm and penicillin-induced in- terictal spikes was also readily seen. Adrenergic and cholinergic fibers from

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the iris autonomic ground plexus invaded the transplants. Stimulation of these inputs evoked specific electrophysiological changes indicative of the formation of functional adrenergic and cholinergic syn- apses on the transplanted central neurons. Taken together, these data suggest that, if placed in the proper milieu, neonatal brain tissues possess a marked capacity for physiological reorganization after extensive lesions. The identification of those factors that underlie this plasticity might provide new therapeutic approaches to reversing the functional deficits in cerebral palsy.

Transtympanic Neurectomy : A Solution to the Drooling Problem. GRADY ARNOLD, CHARLES W. GROSS.

Drooling is a troublesome problem in the care and management of brain-dama- ged patients. An effective, safe procedure for long-term control was investigated. 30 patients were selected and bilateral sectioning of the chorda tympani nerve and the tympanic neural plexus was performed through a transcanal approach. An 87 per cent improvement rate was noted between six and 20 months later. Two patients developed post-operative otitis media with subsequent perforations, but no serious complications occurred. Of the several methods used in controlling drooling, trans-tympanic neurectomy has proven to be the safest and most effective.

Therapeutic Handling Techniques for the Athetoid or Spastic Cerebral Palsy Patient in the Dental Setting. GEORGE BEEDLE, NANCY TUCKER.

Therapeutic handling and positioning techniques for relaxation of hypertonicity were developed to facilitate delivery of service to the severely involved cerebral- palsied child undergoing dental treatment. A sensorimotor approach, including techniques to reduce general tone followed by

therapeutic positioning to maintain that reduction, and specific oral facilitation techniques to inhibit primitive oral reflexes were used.

The success of such an approach requires re-evaluation of original professional rales and boundaries, so that the patient is handled as a total being rather than as a collection of fragmented body parts. 60 athetoid and spastic patients have been treated using this approach. Dental res- torations and tooth extractions have been completed without the use of restraint, pre-medication or general anesthesia.

Audio-visual material was presented to demonstrate specific techniques.

Cerebrovascular Disease in Infants and Children: A Study of Incidence, Clinical Features and Survival. BRUCE S. SCHOENBERG, JAMES F. MELL- INGER, DEVERA G . SCHOENBERG.

The Mayo Clinic experience with cerebrovascular disease in children under the age of 15 years was analyzed for the period 1965 to 1974. Stroke associated with birth or trauma was excluded. Two neurologists independently reviewed all potential cases, yielding 69 stroke patients. 100 per cent follow-up through 1974 was achieved. There were 38 cases of ischemic stroke (18 with pre-existing heart disease; three with basal arterial stenosis with telangiec- tasia (Moyamoya disease); four with other associated predisposing conditions; and 13 without known predisposing conditions) and 31 cases with hemorrhagic stroke (13 with arteriovenous malformation ; six with aneurysm; five with other associated predisposing conditions, and seven without known predisposing conditions). Chil- dren with cerebral infarcts, without known predisposing conditions, had the best survival. Patients with hemorrhagic stroke secondary to' predisposing conditions other than aneurysm or arteriovenous malformation, had the poorest survival rate.

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Residual disability resulted most frequently from cerebral infarcts.

The medical record-linkage system for residents of Rochester, Minnesota, makes it possible for the first time to study cerebrovascular disease in a well-defined childhood population. Records from all medical facilities serving Rochester revealed four strokes over a 10-year period, occurring among a mean population of 15,834 resident children (average annual incidence rate 2.52 cases/100,000/year).

Hereditary Spastic Paraplegia. W. KRAMER.

In three generations of one family, spasticity of the legs progressively developed from the age of 15 years. More- over, EEG abnormalities were registered in all six children examined, four being of a classical spike and wave pattern. Only two of the patients had clinically manifest epilepsy. A man from the first generation died at the age of 74, and post-mortem examination was done. Spasticity had started at the age of 12 years and had led to complete invalidity at the age of 32. In the cord, degeneration of the corticospinal tracts and of the posterior columns was found. Loss of motoneurons was conspicuous. The clinical and post-mortem findings were discussed in relation to cases described since 1880.

Computerized Tomography of the Brain in Children. HERVEY D. SEGALL.

Computerized tomography is making a remarkable impact in the field of neuro- radiology. This is because these examina- tions may be performed without hazard or even discomfort to the patient and permit diagnoses which sometimes elude cerebral angiography and pneumography. Intra- venous contrast materials may be injected to provide further information, with the same risk-rate as for an IVP. A complete

examination gives an x-ray dose roughly equivalent to a conventional skull series.

The various cerebral tissues and CSF differ in their x-ray absorption coefficient. These small differences in x-ray absorption are not appreciated with conventional x-ray techniques but can be exploited with computerized tomography.

Thousands of x-ray readings are recorded by extremely sensitive detectors which move about the patient’s head in parallel with the x-ray tube. The ultimate picture recorded on Polaroid or x-ray film represents a ‘mosaic’ of relative absorption values within tiny individual zones of tissue. Usually eight or more different ‘slices’ (at 8 or 13mm thick) are obtained during the course of the examination, ranging from the skull-base upward to the vertex. The usual radiological convention is preserved so that the most dense structures appear in extreme white while those structures absorbing the least amount of x-ray photons are in peak black. Intermittent gray scale tones are linearly related to x-ray absorption coefficients.

In the course of this presentation, examples of various forms of cerebral abnormality in children were demonstrated. Cerebral lesions which can be demonstrated by computerized tomography include neoplasms, hematomas, cysts, hygromas, infarcts, infections, mal- formations, cerebral atrophy and hydrocephalus.

Radionuclide Detection of Strokes in Childhood. GARY F. GATES.

Cerebral perfusion abnormalities in children are easily detected by a scintilla- tion camera and digital computer, a combination used in analyzing cerebral passage of 99mTc-DTPA in children with ‘strokes’. The method produces images of cerebral perfusion plus time-activity histo- gram curves of isotope passage through

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the hemispheres. Characteristic images and curves allow separation of stroke patients from normal children. Differing patterns of collateral flow into an ischemic hemisphere are apparent and are prog- nostically significant. Correlation with static imaging (nuclide or computerised tomography, both of which may be normal with ischemic, non-infarcted brains) results in a useful combination of comple- mentary diagnostic studies.

Cerebellar Stimulation for the Treatment of Movement Disorders. RICHARD D. PENN, SIDNEY GILMAN, MARY LIZ ETEL.

Chronic cerebellar stimulation recently has been introduced as a treatment for children and adults with cerebral palsy. The anatomical and physiological basis of such an approach, the physical therapy required, and quantitative evaluation of implanted patients, their degree of functional improvement and the possible long-term effects of stimulation were discussed. The results at present suggest that limited clinical trials should be continued, but objective measurements and long-term follow-up will be necessary before the procedure can be adequately evaluated.

Computerized Axial Tomography of Long- standing Major Brain Injury in Children. JOYCE D. BRINK, JOHN S. MARSH, ROBERT E. FLORIN.

In 12 severely brain-injured children, computerized axial tomography has permitted a graphic demonstration of the physical status of the brain. Sample cases were presented in summary form and a representative sample of the scan was projected. Ventricular enlargement and distortion were readily seen and hydrocephalus could be diagnosed without the danger and morbidity of intracranial air

injection. Subdural fluid collections can be localized without the risk and technical problems associated with angiography in the young. Porencephalic and other atro- phic cavities consequent to post-traumatic cerebral-tissue loss can also be demonstrated. Computerized axial tomography has proven to be a major development in the evaluation of chronic brain-injured patients.

Brachial-plexus Injuries in Children. ROGER W. WICKENDEN.

Forty-five children with 47 brachial- plexus injuries have been evaluated in the Brachial Plexus Clinic from 1968 to 1976. These include 11 traumatic and 36 birth- related injuries. The birth-related injuries were subdivided into 25 with typical Erb's palsy, seven with mixed Erb-Duchenne- Klumpke, and four with posterior-cord injuries.

A new operative procedure was described for the treatment of the child with Erb's-type paralysis. These children have glenohumeral tightness, with limited abduction and external rotation of the shoulder. The literature contains little information or new innovations in the surgical care of these children.

Nine children have had the latissimus dorsi and teres major transferred from their insertion to the rotator cuff as an active external rotator. The pectoralis major is also released and myodesed to the anterior-inferior deltoid to allow improved external rotation.

Post-operative follow-up ranges from 15 to 62 months (average 34 months). Functional ranges of motion are markedly improved, with an average increased abduction of 65" and external rotation of 4 1 '. Average age at the time of surgery was five years four months. There were no complications among these nine patients.

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Flexion Contractures and Involuntary Flex- or Bias in the Upper Extremities at El- bows: Its Surgical Management. MOHINDER A. MlTAL

This was an updated report on the surgical procedure of elbow flexor lengthening which was originally presented to the American Academy of Orthopedic Surgeons in 1975 (abstract Journal of Bone and Joint Surgery, 57A, 1031). Its goal is cosmetic and functional improvement in the limb, and the procedure has now been performed on 32 elbows of children with spasticity and/or flexion contractures at the elbows. After a mean follow-up of 28 months, the reach continues to remain permanently increased and the spasticity decreased, not only at the elbows but in the whole limb. The procedure remains unmodified from that reported earlier and consists of excision of the laceratus fibrosis, Z lengthening of the biceps tendon and aponeurotomy of the brachialis muscle.

Familial and Social Acceptance of Myel- odysplastic-hydrocephalic Children. E. J . TREMBATH, CAROL ANGLE, JACK A. STARK, WILLIAM STROND.

The purpose of this study was to analyze the effects of a handicapped child on family stability, in order to ascertain the importance of this variable in medical treatment.

A longitudinal study between 1962 and 1975 was made of 222 children, 144 with myelodysplasia and 78 with hydrocephalus. The factors investigated were mortality, ambulation, medical problems, socio- economic status, socio-educational programs, and family stability. There was a mortality rate of 24 per cent for the group with myelodysplasia and 14 per cent for the hydrocephalic group, resulting in 177 survivors.

Current ambulatory status showed 47 per cent to be without crutches, 23 per cent

to be with crutches, 21 per cent who used a chair and 9 per cent with no independent movement. The major medical problems were incontinence (70 per cent), recurrent seizures (22 per cent), uncorrected squint (35 per cent), abnormal head size (30 per cent), abnormal facies (10 per cent), hyperactivity (10 per cent) and behavior disorders (9 per cent). The socio-economic status of these families was representative of the general population. Some 63 per cent of the 136 children who had reached school age were in regular schools at a Grade level appropriate to age 1 2 years, 19 per cent were in special education classes, 3 per cent were in educable programs, 7 per cent were in programs for the trainable and 8 per cent were in institu- tions.

A remarkable ability of families to cope is reflected in an over-all divorce rate of only 10 per cent, as opposed to 31 per cent for families with non-handicapped children. Only 7 per cent of the children were in foster homes. These findings are contrary to previously reported studies, and are attributed to extensive medical, economic, social and educational supFort; to cultural ethics; to rural location and to large and extended families.

The Results of Hip Surgery for Myelody- splasia. E. N. FEIWELL, T. BLATT, D. N. SAKAI.

The results of 116 operative hip procedures in 67 myelomeningocele patients followed at Rancho Los Amigos Hospital were compiled. Surgery benefited hip reduction, but did not improve ambulation. Fixed pelvic obliquity was the most significant factor in limiting both hip reduction and independent ambulation- Complications of surgery caused de. creased ambulation and limitation of hip motion affecting sitting. The importance of a level pelvis and a good range of hip motion is paramount. Multiple procedures

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and difficult reductions are to be avoided. The functional need of the patient is the surgical guide.

Bus Safety for the Handicapped Child. VIRGINIA BADGER, 0. CHASE, L. VAUGHAN.

The safety of handicapped children during transport to and from school is of prime importance to those entrusted with their care, medically, educationally and legally. Considering the force generated by a limp child being thrown through the air at the time of a bus accident, and what happens to the child at the instant of deceleration, illustrates the need for adequate restraint systems for head, shoulders, full body and sometimes wheelchairs. In counselling parents about the safe trans- portation of their children, it has been found that parents are poorly informed of the reasons for the use of simple, adequate protective devices.

It is reported that 34,000 of the nation’s 250,000 school buses are involved in accidents each year, and that 80 per cent of the injuries are suffered by pupils thrown against unyielding metal and plastic seats and poles inside the bus. For the wheelchair-bound child with a severely disabling disease such as cerebral palsy, paraplegia or muscular dystrophy, specially designed restraint devices are essential.

In the Special School District of St. Louis, orthopedically handicapped, multi- ply and profoundly handicapped, deaf and blind, and mentally retarded pupils are transported daily in large yellow buses and vans. 232 buses travel over 3,060,000 miles via some 300 bus routes. Over a three-year-period, 958 accidents to students were reported by the bus drivers. 292 of these incidents were related to the bus or its equipment. Injuries were most frequent to the head or face (32 per cent) and to the extremities (21 per cent). 47 per cent of injuries occurred from a fall off the bus seat or out of the wheelchair, and

30 per cent from a fall forward while seated. To prevent injuries to head, face and extremities in these handicapped children, restraint systems are mandatory. Individual disabilities must be considered in their design.

A System of Seating for the Physically Handicapped Child. ELAINE TREFLER, RUTH DUNKEL.

Commercially available seats, e.g. wheelchairs or Hogg chairs, often do not adequately fulfill the needs of children who are physically handicapped. Because of many problems-such as spasticity, athe- tosis, floppy postures or severe fixed deformities of extremities or spines-these children require an individual approach to their seating problems.

At the Rehabilitation Engineering Pro- gram in Memphis, at the University of Tennessee Center for the Health Sciences, a series of seating systems for physically handicapped children are being developed. In our experience the severity of physical or mental involvement of the child are more reliable indicators of appropriate seating choice than are the clinical mani- festations of the disorder. Although each child and family is individually assessed, a modular system of seating with inter- changeable components (neck supports, roll seats, pommels, chest straps or harnesses) can be incorporated into a seating system to meet the individual’s needs.

The R81e of the Thoracic Suspension Orthosis in Management of Neuromuscular Spinal Deformities. JAMES C. DRENNAN, PATRICK J. FAZZARI, BURR H. CURTIS, SIEGRIED W. PAUL.

As an adjunct to the care of the paralytic patient, a thoracic suspension orthosis has been used with 36 patients at Newington Children’s Hospital. The ages ranged from three to 19 years and diagnoses included

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myelomeningocele, muscular dystrophy, cerebral palsy, Werdnig-Hoffman disease and traumatic spinal-cord injury.

For total contact, a ‘Vitrathene’ spinal orthosis is carefully contoured beneath the rib cage in the form of an undercut, using the lower thoracic cage for partial weight- bearing. Further suspension can be achieved by attaching the orthosis to a wheelchair by means of a bilateral clamp and hook apparatus, so as to suspend the patient, using the weight of the pelvis and lower limbs as a corrective force.

Indications for use included postpone- ment of surgery, support for a patient who cannot tolerate surgery, and control of disease for which surgery is not indicated. Goals were to retard progressive deformity, to maintain sitting alignment and balance, to relieve pressure, to relieve pain, to preserve respiratory function and to enhance independence.

This orthosis is so designed that a patient with good upper-extremity strength can engage himself in the wheelchair. It also provides good lateral stability, no swaying when suspended, and possible adjustment of anteroposterior movement. Of the 36 patients, only one has rejected its use.

Thoracolumbar Scoliosis in Cerebral Palsy. Results of Surgical Treatment. CHARLES A. BONNETT, JOHN C. BROWN, DENNIS N. SAKAI.

Fourteen per cent of 294 patients with cerebral palsy were noted to have clinically significant scoliosis. 33 were treated sur- gically: 10 by posterior fusion with Harrington rod, 18 by anterior fusion with Dwyer instrumentation, and five by a combined anterior and posterior fusion. Post-operative results showed relief of pain in 17, improved sitting tolerance in 17, less use of equipment or improved functional use of equipment in nine, transfer to a facility requiring less care in three,

and improved eating patterns in two. The combined anterior and posterior procedure gave the best correction, with lowest incidence of pseudoarthrosis.

Non-invasive Measurement of Tension in the Dwyer Spinal Orthosis for Correction of Scoliosis. C. MCDONALD, J. HALL, D. ROWELL.

Many spinal deformities not amenable to bracing or posterior fusion are now treated using the anterior spinal orthosis developed by Allan Dwyer in the late sixties. No hard data exist on the forces to which this system is subjected during and after surgery. Information was sought regarding tensions on implanted cables affixed to the vertebral bodies for correction of scoliosis, which would be used to help guide surgical and post-operative management .

An inductively-powered biotelemetry unit has been developed which is contained within a load cell positioned on the cable so as not to alter the clinical orthosis. Six implants have been tested in virro and calibrated for compressive loading. Cali- bration data have fitted well with an algebraic relation derived from transducer characteristics-cable tension is a function of output frequency. This calculation is programmed to a portable microprocessor unit, providing analog and digital readout of tensions. In vivo experiments were designed to test long-term biocompatibility of the units and to evaluate an ability accurately to monitor intra-operative and post-operative forces. Dwyer cables were affixed to the anterior spines of six dogs, simulating full clinical procedures. Meas- urements provided useful information, corresponding to clinical practice. Chronic information has been obtained for up to 12 months in the animals. Post-operative evaluations have included wakeful and anesthetized measurements, enabling manual bending of the dogs, with exten-

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sive radiological assessments in conjunction. A seventh animal was implanted differently under fixed compression to evaluate the effects that maturing scar might exert. Final measurements, radiology, and sacrifice demonstrated an ability to monitor a temporal decrease in cable tensions measured with bending, as the loading is taken up by the fused vertebrae. This should prove predictive of interbody fusion and recovery for patients. Implant biocompatibility and reliability of the non-invasive technique have been demonstrated. Final observations will involve a select patient group.

Genu Recurvaturn in Spastic Cerebral Palsy : Preliminary Report. S. R. SIMON, S. D. DEUTSCH, R. K. ROSENTHAL.

The patho-mechanics of genu recurvatum and its correction with a below-knee orthosis were determined by gait analysis in 10 children with spastic cerebral palsy. Results were compared with clinical evaluations. Body motions, EMG activity in the involved limb, and foot-floor reaction forces were monitored. All children demonstrated poor ankle plantar flexion function. Additional pathological factors created recurvatum by altering the normal movements of the femur to the tibia over time. The below-knee orthosis prevented recurvatum by locking the ankle, but did not change walking ability as measured by velocity, cadence and stride length. Clinical evaluation recognized one abnormal factor, but not the multitude present and their complex interrelation- ship.

Adductor Transfer for Spastic Cerebral Palsy. WILLIAM H. COUCH, G. PAUL DEROSA, FRANK B. THROOP.

Thirty-five patients with spastic diplegia underwent bilateral adductor transfer to

decrease their crouched, adducted and scissoring gait. 32 were available for follow-up at an average of 30 months post-operative1 y.

Objectively, the measurement of the width of base of gait and abduction range improved in all patients. Rotational position of feet and knees while ambulating improved significantly in all but five patients. The need for ambulatory aids was decreased in over half the patients. Measurement of stance phase knee-flexion revealed a decrease in half the patients. Subjectively, the gait and appearance was improved in all but one patient.

Complications of the procedure were few, consisting only of two superficial wound infections.

The Adductor Transfer for Adductor Spas- ticity : A Clinical and Electromyographic Gait Analysis. P. P. GRIFFIN, W. W. WHEELHOUSE, R. CHIAVI.

Transfer of the adductor muscle origin to the ischial tuberosity has been performed in order to improve the functional ambulatory status of patients with adductor muscle spasticity. Physical examination, videotape recordings, and electromyographic gait analysis were performed pre-operatively and post-operatively to evaluate the adductor muscle function. Adductor muscle transfer was found to improve gait, increase hip abduction, and stabilize the hip in selected patients with adductor spasticity.

Crouch Gait in Spastic Diplegia. D. H. SUTHERLAND, LES COOPER.

Gait analysis techniques were used to investigate the abnormalities associated with exaggerated stance knee flexion.

Measurements of movement were made by a photographic technique of triangula- tion from movie film. Simultaneous force- plate recordings and electromyograms were obtained.

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The most frequent abnormalities observed were: (1) exaggerated knee flexion and ankle dorsiflexion in stance; (2) variation in the first peak and diminished second peak in vertical force; (3) markedly increased hip and knee torque; (4) pro- longed stance-phase activity of the quadriceps and gluteus maximus muscles. These deviations are the individual bits of an awkward and cumbersome gait pattern. The high-knee torque values correlate well with an instrumented cadaver experimental study which measured the quadriceps force required to stabilize the flexed knee.

Clinical Use of a Gait Analysis Laboratory. JOHN L. HAGY.

For the past seven years, Shriners Hospital for Crippled Children in San Francisco has been investigating and implementing a system of gait analysis for the study of abnormal gait. It is important in studying such gait that a patient be left as free as possible from any apparatus so as to walk in his ‘normal’ way. By combining three separate systems, simultaneous data are gathered on forces, electromyography and motion analysis.

Explanations of the laboratory and the three basic systems that have been integra- ted were presented. There was also an explanation of how these systems are used to help the cerebral-palsied patient, and how engineering ties in with medicine to produce a pre- and post-operative evaluation of treatment.

Cerebral Palsy Gait Evaluation by EMG and Footswitch Data Analysis. GEORGE BEKEY, DANIEL ANTONELLI.

The goal of gait analysis at Rancho Los Amigos Hospital is to provide a quantitative basis for evaluation of muscle function and potential surgical procedures in the lower extremity.

Data are acquired using 50-micron intramuscular EMG wire-electrodes and

footswitches under the heels, 1st and 5th metatarsals and great toes. Electromyo- graphy and footswitch data are transmitted by FM-FM and digital telemetry, recorded on magnetic tape and reproduced on a light-sensitive strip chart.

Analysis of these data yields the temporal relationships between the active period of individual muscles and the gait cycle. Prediction by computer analysis of multiple EMG signals of potential gait changes resulting from surgery is at present under clinical evaluation.

Biofeedback Therapy in Cerebral Palsy. C. MCLAURIN.

This presentation was continuously illustrated with a colour film showing the need for special training with cerebral- palsied children and how this may be enhanced with biofeedback. Specific biofeedback systems included the head position trainer, joint positioner trainer used at the knee and hip, muscle training with myo-electrics, postural seating training and an attempt at control of drooling. Methods of evaluation and some results were shown.

Orthotic Devices for Assistance in Cerebral Palsy. WALLACE M. MOTLOCH.

At the Rehabilitation Engineering Center we have four new items. The first is the Stanford Children’s Weight-Relieving Am- bulator-a supportive device which at- taches to the patient’s trunk. It provides an increased sense of balance, partially relieves the weight, and enables patients to stand with both hands free. The second is the Stanford Children’s slotted control for electric wheelchairs or communicators. This control minimizes the requirements on patient’s voluntary control. All the patient needs to do is to select the direction of motion and the electronics take over and accelerate the chair slowly until the top speed is reached.

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The third items are policing devices, a number of aids designed to remind the patient of incorrect head, leg or trunk position. This same kind of electronic bracing can be used in the management of scoliosis and tissue trauma. In the fourth group are portable printers, displayers and speech generators. These communication devices for the non-speaking fill one of the greatest needs that cerebral- palsied children and adults have. 'Mini- Comm' is an example of a light visual- display unit with hard copy print-out capabilities and a weight of only 3.5 Ibs.

Electrical Stimulation of the Spinal Mus- cles to Correct Scoliosis. M. A. HERBERT, W. P. BOBECHKO, H. G. FRIEDMAN.

Scoliosis is a common orthopaedic deformity, affecting up to 14 per cent of the population. Its rapid progression during puberty requires positive action to prevent collapse of the curve, with the associated problems later in life. Serious curves must be arrested and partially corrected, using surgical fusion combined with metallic rods or cables. While this leaves part of the spine immobile, it prevents further collapse of the curve. Lesser curves are usually treated with a Milwaukee brace, which has a tight-fitting pelvic bucket with upright posts and pressure pads to apply force to the spine. In the majority of cases it is successful in stopping the progression of the curve, but its main drawbacks are the lack of correction and rejection by patients of the bulky device.

We have designed a system which uses electrical stimulation of the paraspinal muscles on the convex side of the curve to arrest and correct the progression of the curve. A small receiver is implanted under the skin and connected to three miniature platinum electrodes located in the paraspinal muscles about the apex of the curve,

ANNUAL MEETING

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on the convex side. A modulated radio frequency signal is sent transcutaneously from a small antenna worn on the skin over the receiver site. The antenna is connected to a transmitter placed at the bedside. The transmitter contains the battery, cycling and amplitude controls for the system. The stimulation program is carried out during the night only, using a one-second pulse train repeating every 10 seconds.

A total of 47 curves are presently being treated, with follow-up of up to 23 years. To date 45 per cent of these curves have been arrested, 38 per cent have shown an improvement of 5" or more on x-ray, while 17 per cent have increased by 5" or more. Patient acceptance of this treatment has been excellent.

Suppression of Intention Tremor by Appli- cation of Viscous Damping. M. J. ROSEN, J. W. GESINK, D. ROWELL.

Intention tremor is frequently among the symptoms of cerebellar damage, multiple sclerosis and Friedreich's ataxia, and may render a limb useless in activities of daily living despite the fact that usable levels of strength and voluntary control are present. While there remains no accepted successful treatment for this tremor, modification of normal and abnormal tremors by application of forces or mechanical elements to a limb has been observed in clinical and experimental settings.

The usefulness of viscous damping as a means of selectively suppressing intention tremor was tested. The initial study was performed on a 29-year-old male patient suffering from severe post-traumatic cerebellar intention tremor, intractible to L-Dopa, dantrolene and cryothala- mectomy. A thermoplastic hinged brace was prepared for his left wrist, incorporat- ing a rotational potentiometer as a wrist angle transducer and a linear pneumatic


dashpot. The patient was asked to maintain the co-linearity of two adjacent oscilloscope traces-one driven by a sinusoidal target signal and the other by his wrist extension-flexion angle.

The following results were obtained : this patient's abnormal wrist tremor occurred near 3Hz, with a random amplitude having peak values near 10". Attachment of the damper to the brace reduced the amplitude of the tremor in all trials with a better than 0.05 significance.

The amplitude of the average purposeful component of the patient's wrist motion showed either no significant change, or a substantial increase in the presence of damping, giving a more accurate tracking performance.

It is concluded that the application of mechanical loads for selective suppression of intention tremor is worthy of more elaborate general study and such a study is under way.

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Documents

Annual Meeting of the American Academy for Cerebral Palsy and Developmental Medicine, 1976