14
A STUDY OF CEREBRAL PALSY IN THE CIHLDHOOD POPULATION OF EDINBURGH BY T. T. S. INGRAM From the Departmnt of Child Life and Health, University of Edinburgh (REasCEDv FOR PUBLICATnON AUGUST 30, 1954) There is increasing interest in the needs of children suffering from cerebral palsy for special education and medical treatment and for measures to render more of them employable. Many schemes to provide facilities for education and employment are being planned, but there are only a few scattered systematic surveys of the incidence of cerebral palsy in childhood, and so the size of the problem is largely unknown. This article presents the results of a recent investigation which was designed to assess the incidence of cerebral palsy in children resident in Edinburgh. The Plan of the Swvey The survey was confined to children born in the years 1938 to 1952 inclusive who lived within the boundaries of the City of Edinburgh. Since the population of this age group in the city was more than 100,000 it was clearly impossible to examine every child individually. On the other hand, the medical services of the city work in close liaison and the school medical and public health authorities are highly developed. Due largely to the propaganda of the Scottish Council for the Care of Spastics there is a great awareness of, and interest in, the problems of the child with cerebral palsy among doctors, teachers and medical auxiliaries. Therefore children in whom cerebral palsy was suspected tended to be over-examined rather than neglcted. Thus in many ways Edinburgh was an ideal city in which to obtain information about children with cerebral palsy and make a survey of its incidence. The Metds of the Swvey Details of the children who might be suffering from cerebral palsy were obtained in three ways in the first part of the survey. First, large numbers of case notes and reports from clinicians were examin- ed, and all cases in which cerebral palsy was sus- pected were noted. Secondly, all patients whose records showed that they suffered from conditions known to be associated commonly with cerebral palsy were listed. These included children with mental defect with clumsiness, epilepsy, hydro- cephalus, talipes equinovarus and arthrogryposis. At the same time all patients who gave histories of having suffered from conditions likely to lead to cerebral palsy were noted. These included patients with apparent birth injury, rhesus incompatibility, meningtis, encephalitis, cerebral thrombophlebitis and head injury. Thirdly, inspections and brief examinations were made of groups of children in whom a high incidence of cerebral palsy might be expected. Thus large numbers were examined in schools for the physically and mentally handicapped, in clinics for the premature infant and in institutions for the mentally defective. Sores of Asetimet The sources of information about possible and probable cases of cerebral palsy were very numerous and overlapped considerably. The more important sources of information will be mentioned. Access was given to the case notes of the officers of the School Medical Service, who were uniformly most helpful in providing information and facilities for the examination of their patients. In all, over 6,000 case notes were examined in various schools and over 400 patients seen in schools by arrangement with school nmdical officers. Information about educa- tional difficulties and progress was obtained in as many cases as possible from teachers. Fifty-five private schools were contacted and information sought about any children with dis- abilities suggestive of cerebal palsy. Replies were received from 53 of the private schools. The officers of the Maternity and Child Welfare Service were most helpful in giving information about children they felt might be suffering from cerebral palsy, and access was given to case notes and patients in their clinics. The results of the routine health visiting of the department were also made available to the investigation. The PubLic Health 85 copyright. on June 27, 2021 by guest. Protected by http://adc.bmj.com/ Arch Dis Child: first published as 10.1136/adc.30.150.85 on 1 April 1955. Downloaded from

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  • A STUDY OF CEREBRAL PALSY IN THE CIHLDHOODPOPULATION OF EDINBURGH

    BY

    T. T. S. INGRAMFrom the Departmnt of Child Life and Health, University of Edinburgh

    (REasCEDv FOR PUBLICATnON AUGUST 30, 1954)

    There is increasing interest in the needs of childrensuffering from cerebral palsy for special educationand medical treatment and for measures to rendermore of them employable. Many schemes toprovide facilities for education and employment arebeing planned, but there are only a few scatteredsystematic surveys of the incidence of cerebral palsyin childhood, and so the size of the problem islargely unknown. This article presents the resultsof a recent investigation which was designed toassess the incidence of cerebral palsy in childrenresident in Edinburgh.

    The Plan of the SwveyThe survey was confined to children born in the

    years 1938 to 1952 inclusive who lived within theboundaries of the City of Edinburgh. Since thepopulation of this age group in the city was morethan 100,000 it was clearly impossible to examineevery child individually. On the other hand, themedical services of the city work in close liaison andthe school medical and public health authorities arehighly developed. Due largely to the propagandaof the Scottish Council for the Care of Spastics thereis a great awareness of, and interest in, the problemsof the child with cerebral palsy among doctors,teachers and medical auxiliaries. Therefore childrenin whom cerebral palsy was suspected tended to beover-examined rather than neglcted. Thus inmany ways Edinburgh was an ideal city in which toobtain information about children with cerebralpalsy and make a survey of its incidence.

    The Metds of the SwveyDetails of the children who might be suffering

    from cerebral palsy were obtained in three ways inthe first part of the survey. First, large numbers ofcase notes and reports from clinicians were examin-ed, and all cases in which cerebral palsy was sus-pected were noted. Secondly, all patients whoserecords showed that they suffered from conditions

    known to be associated commonly with cerebralpalsy were listed. These included children withmental defect with clumsiness, epilepsy, hydro-cephalus, talipes equinovarus and arthrogryposis.At the same time all patients who gave histories ofhaving suffered from conditions likely to lead tocerebral palsy were noted. These included patientswith apparent birth injury, rhesus incompatibility,meningtis, encephalitis, cerebral thrombophlebitisand head injury. Thirdly, inspections and briefexaminations were made of groups of children inwhom a high incidence of cerebral palsy might beexpected. Thus large numbers were examined inschools for the physically and mentally handicapped,in clinics for the premature infant and in institutionsfor the mentally defective.

    Sores of AsetimetThe sources of information about possible and

    probable cases of cerebral palsy were very numerousand overlapped considerably. The more importantsources of information will be mentioned. Accesswas given to the case notes of the officers of theSchool Medical Service, who were uniformly mosthelpful in providing information and facilities forthe examination of their patients. In all, over 6,000case notes were examined in various schools and over400 patients seen in schools by arrangement withschool nmdical officers. Information about educa-tional difficulties and progress was obtained in asmany cases as possible from teachers.

    Fifty-five private schools were contacted andinformation sought about any children with dis-abilities suggestive of cerebal palsy. Replies werereceived from 53 of the private schools.The officers of the Maternity and Child Welfare

    Service were most helpful in giving informationabout children they felt might be suffering fromcerebral palsy, and access was given to case notes andpatients in their clinics. The results of the routinehealth visiting of the department were also madeavailable to the investigation. The PubLic Health

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  • ARCHIVES OF DISEASE IN CHILDHOOD

    Department physicians and surgeons providedinformation about patients with cerebral palsyvisiting their clinics.

    All hospitals in Edinburgh concered with thediagnosis and treatment of children's diseases, ofwhatever type, and maternity hospitals in the citywere contacted and in each instance ready access tocase notes and patients was granted. Medical,nursing and auxiliary staff were questioned aboutpossible cases and approximately 2,500 case notesdating back to 1940 were examined. Out-patientstaffs and case notes were consulted as well as in-patient notes and staffs. Many consultants, phy-sicians and surgeons gave access to their privatepatients and went to considerable trouble to arrangefor the investigator to examine these children.The Scottish Council for the Care of Spastics gave

    free acces to their patients and records and muchvaluable information was obtained from the highlytrained members of their staff.The response of general practitioners to the

    request for information about their patients wasdisappointing. It was intended to contact allgeneral practitioners in Edinburgh by letter request-ing details of children who, they thought, mightbe suffering from cerebral palsy. As a first step, 25practitioners were circularized and asked for infor-mation. They were known at that stage of theinvestigation to have at least 28 patients with cere-bral palsy in their practices, but only two nameswere obtained and only one of these was unknownto the survey. General practitioners were not here-after contacted specifically to obtain informationabout the numbers of patients with cerebral palsy intheir practices, but whenever permission was soughtto examine known cases doctors were always askedfor details of any other cases known to them.

    Other research projects amongst children inEdinburgh provided a number of cases of cerebralpalsy. These included investigations of childrenwith mental defect, of children who had sufferedfrom meningitis, of infants with retrolental fibro-plaia, the prematurely born and children who hadsuffered cerebral birth injury.A few additional cases were reported by district

    nurses, infant clinics, speech therapists, childguidance clinics and teachers.

    DIrct Ascertaimnt of Cases ofCereb{ Palsy

    Direct ascertainment of children with cerebralpalsy was carried out in groups of children in whoma high incidence of cerebral palsy was to be expected.The usual procedure was to watch the children atplay for as long as possible and then when they

    attempted to sit still. Those in whom cerebralpalsy was suspected on the basis of these inspectionswere examined. After a few months, during whichexperience was gained, it was found that quite asmany cases of cerebral palsy were noted using thismethod as were discovered by detaied examinationof all the children in a group.Approximately 4,000 children were inspected in

    ordinary schools, special schools, nurseries, insti-tutions for the mentally defective and infant clinics.

    Resul of Plfnwry AswdmnAs a result of the first part of the investigation

    484 children were classified as possible cases ofcerebral palsy and 165 as probable cases. Probablecases were those children who had been diagnosedas suffering from cerebral palsy by other observersor were felt to suffer from the condition as a resultof the inspections of the investigator. Possible caseswere those children in whom a diagnosis of cerebralpalsy could not be excluded on the basis of informa-tion provided in case notes which had been inspected,or who had suffered from some condition known tolead to, or be associated with, cerebral palsy in aproportion of patients. It was felt to be desirable toexamine and obtain some details of the history of thedevelopment and course of the condition in all thechildren in both the possible and probable categories.

    Probable and Possie Cases. All the 165 childrenwho were considered to be probable cases of cerebralpalsy were examined with the exception of two forwhom permission for exammiation was refused. Infour cases a detailed history could not be obtained.Of the 484 possible cases of cerebral palsy all but

    85 were examined. Of the latter it was possible toobtain reliable information of recent medicalexaminations in 52 so that the presence of cerebralpalsy could be excluded. In the remaining 33patients it was impossible to obtain such informationand it was not found possible to examine them.The 399 patients who were examined were usuallyseen briefly at the time of the routine school medicalexamination for the first time and then examined inmore detail later if the presence of cerebral palsywas confirmed.The history was obtained from the mother when-

    ever possible and was suplemented by details ofhospital admissions and clinic visits, maternityhospital and infant clinic notes and the recollectionsof other relatives.

    In practically all the patients in the sers physicaexaminations were performed on more than oneoccasion, preferably in different surroundings andpreferably at intervals of a few months. Full

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  • CEREBRAL PALSY IN EDINBURGHclinical examinations were supplemented by specialexaminations, blood investigations, radiographs,intelligence tests and electroencephalograms in rela-tively few cases, but frequently these were availableas a result of previous hospital admissions or schooltesting.

    palsy are found together in categories of 'rigidity'and 'spasticity'. To eliminate such confusions it isdesirable to resort to the earlier, well tried types ofclassification by neurological syndromes.The present classification is based on that sug-

    gested by Freud (1893, 1897). shown in Table 2, with

    Classfition of Types of Cerebrl PalsyThe classification by neurological syndromes

    which was used during the survey is shown inTable 1. It will be observed that it is not based on

    TABLE ICLASSIFICATION OF CEREBRAL PALSY IN CHILDHOOD

    NeurologicalDiagnosis

    Hetmiplegia

    Extent

    I RightILeft

    Double hemiplegia

    DiplegiaHypotonicDystonicRigid or spastic J

    Ataxic diplegiaHypotonicSpastic

    AtaxiaCerebellarVestibular

    DyskinesiaDystonicChoreoidAthetoidTensionTremor

    I ParaplegicTriplegic

    l Tetraplegic

    ( ParaplegicTriplegicTetraplegic

    Severity

    MildModerately severeSevere

    ( idModerately severel Severe

    MildModerately severe

    l Severe

    I MildModerately severeSevere

    IPredominantly (MildUnilateral Moderately severe(Bilateral Severe

    MonoplegicI HemiplegicITripkegicI Tetraplegic

    MildModerately severe

    l Severe

    single clinical features, such as alteration of muscletone, as have many recent classifications (Phelps,1940; Hellebrandt, 1950-51). By laying stress onsingle clinical features of cerebral palsy greatconfusion has been caused. Too close adherence torigid classification on this basis has led to verbalabsurdities like categories of 'aspastic spastics' andnosological confusions like 'cerebellar releaseathetosis'. Cases of hemiplegia may be found underheadings of 'rigidity' and 'spasticity', though theonly difference in clinical features is that contractureis more marked in the former group. It is note-worthy that, when authors using some of thesecomplex modern classifications wish to compareseries of patients suffering from different forms ofpalsy, they immediately resort to grouping para-plegic, triplegic and tetraplegic cases together anddistinguish them from the hemiplegic forms. Yetin their classifications all these types of cerebral

    TABLE 2CEREBRAL PALSY IN CHILDHOOD AS

    CLASSIFIED BY FREUD (1897)

    Unilateral disorders Hemiplegiarightleft

    Bilateral disorders DiplegiaParaplegic ngidityGeneralized rigidityBilateral hemiplegiaChoreoathetosis

    Possibly other groups

    some modifications rendered necessary by thewidened scope of the term 'cerebral palsy' since histime, and by advances made in the knowledge ofthe nature of some forms of the condition. Freudincluded cases showing involuntary movements asthe predominant clinical findings in the categoryof diplegia, though with some misgivings. In thepresent classification cases showing involuntarymovements as the presenting symptom have beengrouped under the heading of dyskinesia, and classi-fied as suggested by Perlstein (1952). Largely toachieve some uniformity with contemporary Ameri-can classification Freud included cases of bilateralhemiplegia under the heading of 'diplegia' (Lovett,1888; Osler, 1889). In the present classificationthese cases have been placed in a separate category.In accordance with modern practice the categoryof ataxia has been added.By 'diplegia' is understood a condition of more or

    less symmetrical paresis of cerebral origin moresevere in the lower limbs than the upper and datingfrom birth or shortly afterwards. It will be observedthat separate categories have been made for patientsshowing diplegia without ataxia and those showingdiplegia associated with cerebellar ataxia. Theaetiologies of these disorders differ, and the courseand findings on examination are dissimilar.A high proportion of patients suffering from

    diplegia without ataxia are born prematurely (400%in the present series). Many show associatedmental defect, epilepsy and strabismus. There is aconstant pattern of development of the paresis,inadequately described in modern textbooks, inwhich only the final stage of diplegia, characterizedby spasticity and contractures, is mentioned. In atleast half the patients suffering from diplegia without

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  • ARCHIVES OF DISEASE IN CHILDHOOD

    ataxia three stages may be distinguished in thedevelopment of the final clinical picture.For a period after birth the child may be thought

    to be normal, but in a proportion of patients theparents note that the baby is floppy and flaccid onhandling and shows a relative poverty of movementwhen compared with others of the same age. Onexamination at this stage these patients show general-ized hypotonia without any other apparent neuro-logical abnormality in most cases. The stage ofhypotonia persists for between six weeks and fivemonths in the majority of those showing it and issucceeded by the stage of dystonia.The stage of dystonia is first manifested by sudden

    generalized dystonic movements involving the trunkand limbs and precipitated by changes in the positionof the child, especially extension of the head relativeto the trunk. The involuntary movement consistsof a sudden extension of the head and trunk,extension of the hips and knees, and plantar flexionof the feet. The upper limbs are adducted andsomewhat internally rotated at the shoulders andextended at the elbows. The wrists and fingers aresemiflexed and the forearms are pronated. Onexamination when in this position marked rigidityis found in all the muscles of the trunk and limbs.The child maintains the position of extension of thetrunk and limbs for a few seconds during which timeconsciousness may seem slightly impaired, thoughit is never lost, and then the muscles again relax.Initially the sudden dystonic attacks occur relativelyinfrequently and only when the child's position issuddenly or grossly altered, but over a period of twoor three weeks the attacks become more frequent andmore readily precipitated. The generalized attacksseldom persist for less than three weeks or for longerthan six months. Towards the end of the dystonicstage they become less frequent and more difficultto produce. At about the time the child is able tohold the head up steadily the generalized dystonicattacks cease in most patients and simultaneouslyrigidity of the limbs first becomes apparent, markingthe end of the dystonic and the beginning of the rigidor spastic stage of diplegia. With the cessation ofthe generalized attacks the child tends to show anincrease in the range and control of voluntarymovements and begins to reach for objects. It isobserved at this stage that though voluntary move-ments or changes in position no longer result ininvoluntary extension of the trunk, the limbs con-tinue to assume positions similar to those notedduring the earlier dystonic attacks. Rigidity isusually first evident in the thighs. It increases ina matter of a few weeks and at the same timespreads to involve the distal parts of the lower limbs

    and, in all but paraplegic patients, the upper limbs.The rigidity tends to result in the limbs being heldin positions of predominant extension as in theearlier dystonic attacks, and these positions areaccentuated by voluntary movements or passivechanges in position. The phase of rigidity withpredominantly extensor associated movementsusually persists until the patient is able to sit withoutsupport with the knees flexed and handle objects.Commonly this is shortly before the age of I year inpatients who are not mentally defective. The phaseof rigidity merges imperceptibly into the phase ofspasticity at about this stage of the child's develop-ment. The tendon jerks become increased insteadof being difficult to elicit and stretch responsesappear in the muscles. The Babinski responsesbecome positive. Instead of extensor associatedmovements predominantly flexor associated move-ments accompany voluntary movements of thelimbs. When spasticity is present, contracture ofthe limbs in flexion almost invariably follows unlessthe child is treated, and the final stage of diplegia isreached.The stages which are apparent in the development

    of diplegic paresis appear to be of more significancethan mere indications of the diagnosis. In severecases the stage of rigidity or spasticity may never bereached and dystonic attacks may persist throughoutlife whenever the child attempts voluntary movementinvolving changes in posture. The child's neuro-muscular development may be regarded as havingbeen arrested at the relatively primitive stage ofdystonia. On the other hand, the infant in whomspasticity with flexor associated movements isevident, shows neuromuscular development whichmay be regarded as being more mature. The childwill be able to achieve more adequate motor controlthan will the child with persistent dystonia providedcontracture is prevented. One remnant of thegeneralized dystonic attack persists quite commonlyin patients showing predominant rigidity in themuscles and they may be regarded as being in anearly phase of the rigid or spastic stage of diplegia.It is found that when these children attempt to walka sudden extension of the neck, trunk and lowerlimbs ensues, a phenomenon known to physio-therapists as extensor thrust. By classifying thepatient with diplegia according to the stage of thedevelopment of his condition, a clear indicationis given of the maturity of his neuromuscular control.

    Patients with diplegia associated with cerebellarataxia show only two of the stages found in casesof diplegia without ataxia. Initially most of thechildren are hypotonic, but the majority showincreasing spasticity of the limbs, more marked in

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  • CEREBRAL PALSY IN EDINBURGH

    the legs than the arms from the age of 9 or 10 months.Attacks of dystonia are never found and the pre-dominant associated movements are flexor notextensor. The increase of tone is never rigid, butis spastic and the tendon jerks are invariably muchincreased. The Babinski responses are alwaysbilaterally positive. A high proportion of thesepatients and their relatives are mentally defective,and in three females of the 13 patients with diplegiaand cerebellar ataxia in the present series, there wasa similar condition in the mother or in femalesiblings. On examination, in addition to the find-ings of paresis of the limbs, with a spastic increase oftone, there is cerebellar ataxia, which may be pre-dominantly unilateral or bilateral.

    Cases which could not be conveniently classifiedin any of the foregoing categories were placed in thecategory of 'other'. They included only two patientsin the present series, one with arthrogryposis andcerebral palsy confined to the cranial nerves and onewith post-encephalitic Parkinsonism.

    Tbe Extent and Severity of the PalsyThe number of limbs so severely involved neuro-

    logically that their full normal functions were im-paired is stated for each category of cerebral palsy.Limbs involved neurologically but not affectedseverely enough to limit their functions were classi-fied as nc-mal limbs. Thus the extent of the palsywhich is stated is the extent of functional impair-ment and not of neurological involvement. It wasrare, for example, to find paraplegic patients whodid not show some evidence of slight affection of theupper limbs. The side affected by hemiplegia wasstated in every case.

    The severity of the palsy was somewhat arbi-trarily decided on the basis of different criteria foreach form of cerebral palsy. Thus mild cases ofhemiplegia were those in which the affected arm wascapable of being used, if somewhat clumsily.Moderately severe cases were those in which thearm was useful only as an assistant to the unaffectedarm, and severe cases were those in which there wasvery little if any functional use in the affected upperlimb. Mild cases of diplegia and diplegia withataxia were those in which the gait was clumsy butnot grossly impaired. Moderately severe cases werethose in which walking without support was possiblebut the gait was abnormal and activities were limitedbecause of this. Severe cases were those in whichwalking without support was impossible or possiblefor only very limited distances. Mild cases ofataxia and dyskinesia were those in which thechild's activities were not limited, moderately severecases were those in which some ordinary activities,such as dressing and feeding, were possible onlywith difficulty. Severe cases were those in which allnormal activities were severely handicapped. Bi-lateral hemiplegia was always a relatively severecondition. Moderately severe cases were thosewhich could stand or walk without support. Severecases were those in which walking was impossible,and, since the upper limbs were more handicappedthan the lower limbs, the former were generallyalmost functionless.

    Inidene of Cerebal Palsy by Year of BirthIn Table 3 is shown the incidence of cerebral palsy

    in Edinburgh children born between 1938 and 1952as ascertaied during the survey. It will be seen

    TABLE 3INCIDENCE OF CEREBRAL PALSY BY YEAR OF BIRTH IN EDINBURGH*

    Number of Patients Incidence per 1,000 in 3-year PeriodsYear ofBirth Population Hesniplegia Diplegia All Types Hemiplegia Diplegia All Types

    1938 6,7491939 6,5741940 6,209

    194119421943

    6,0996,3886,538

    1944 6,5331945 5,9931946 7,902

    194719481949

    8,9007,7007,500

    1950 7,2001951 7,1001952 6,900

    1938-52 104,285 75 79 208 0-719

    Based on figures obtained from the Education Authority and estimates of the Registrar-General.

    89

    1033

    444

    748

    867

    5

    380

    24S

    1137

    7413

    363

    171382

    10I13124

    249

    19)i211424)1185 i

    0-819

    0-631

    0-930

    0-871

    0-330

    0-563

    0-578

    1-028

    0-996

    0- 66

    1-945

    1 -839

    2-546

    2-448

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  • ARCHIVES OF DISEASE IN CHILDHOOD

    that for the two three-year periods from 1944 to1949 the incidence of cerebral palsy is approximately2-5 cases per 1,000 of the population. For theperiods before 1944 and after 1949 the figures forincidence are lower.The apparently lower incidence of cerebral palsy

    in children bom between 1950 and 1952 is partlydue to there being a smaller proportion of acquiredcases of cerebral palsy than in those born between1943 and 1949. Though the majority of cases ofacquired cerebral palsy occur before the age of3, a few occur after this age, and clearly a numberof children in the group born between 1950 and 1952have yet to develop the condition. The importantcause, however, of the smaller number of patientsfound among children born in this period comparedto those born in earlier years is defective ascertain-ment. This is true for all the categories of thecondition and for congenital as well as for acquiredcases.The reasons for defective ascertainment of patients

    in the younger age group are of some importance.In a large proportion of patients the early mani-festations of the disorder had been misinterpretedor ignored by the doctor to whom the mother tookthe child. Thus in the 79 cases of diplegia in theseries, 64 had symptoms before the age of 18 months.In 58 cases the symptoms resulted in the child beingtaken to the doctor before this age. Yet the diag-nosis of some form of cerebral palsy was made inonly 18 cases and the majority of patients had towait for between 18 months and two years beforediagnosis and treatment were undertaken. A delayof this duration resulting in children appearing fortreatment only at the age of 3 years or more meansthat many have reached the stage of contracturebefore anything is done to limit the effects of thecondition. Because the diagnosis of many patientswith cerebral palsy born between 1950 and 1952 wasunknown to doctors and the patients had notreached clinics or treatment centres, they were notascerained during the survey.The apparently lower incidence of cerebral palsy

    in children born between 1938 and 1943 than inthose born between 1944 and 1949 appears to be dueto a number of factors. It was difficult to discoverwhy ascertainment should have been more defectivefor this age period than for those born in the laterperiod. The same sources of information wereavailable and the numbers of routine medical exami-nations in schools and clinics was greater in theolder groups than in the younger. It might havebeen expected that ascertainment would be more,not less, effective in the older children. It seemedlikely that the apparently lower incidence of cerebral

    palsy in those born between 1938 and 1943 com-pared with that of those born between 1944 and 1949was either a true reflexion of a difference in thenumbers of patients developing the condition or ofthe proportion of patients surviving in spite of it.The death rate in children suffering from cerebral

    palsy is unknown, though various estimates based onunpublished or inadequate evidence have been made.It seems probable, however, that the commonestcause of death among children suffering from cere-bral palsy is infection, especially of the reiratorytract in those with bulbar involvement complicatingsevere bilateral affection of the limbs. Since theadvent of antibiotics these infections can morefrequently be controlled and a higher proportion ofseverely affected children with cerebral palsy may beexpected to survive. In the present series there is,in fact, a higher proportion of severe forms of thecondition, especially diplegia of tetraplegic dis-tribution and bilateral hemiplegia, among childrenborn between 1944 and 1949 than in those bornbetween 1938 and 1943.

    In addition to the increase in the number ofpatients with cerebral palsy born in 1944-49 com-pared with those born in 1938-43 due to the lowerdeath rate in the later period, there is an increase inthe numbers suffering from the condition as a resultof certain aetiological factors. The increase ismost marked in cases of acquired ataxia and hemi-plegia. The numbers suffering from the latter whowere born in the periods 1938-43 and 1944.52 areshown in Table 4. It will be observed that there is

    TABLE 4RELATIVE NUMBERS OF CASES OF ACQUIREDHEMIPLEGIA IN 1938-43 AND 1944-52 COMPARED

    No. of Cases ofNo. of Cases of No. of Cases of Acquired

    Period Hemiplegia Acquired HemiplegiaHeniplegia Treated with

    1938-43 28 8 2*1944-52 47 25 171938-52 75 33 19

    * Both developed infections which were treated with penicilli after1943.

    a significantly higher proportion of patients in thesecond group who suffered from acquired hemi-plegia, the result of the cerebral complications ofacute infections. It seems probable that the effect ofantibiotic therapy has been to lower the mortalityfrom these acute infections and that a higher propor-tion of patients suffering from them are surviving toshow the effects of cerebral damage. Similarly,there appears to be an increase in the numbers of

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  • CEREBRAL PALSY IN EDINBURGH

    patients born in the period 1944-49 compared withthose born in 1938-43 who show ataxia as a resultof tuberculous and septic meningitis.

    It is more difficult to assess the effects on theincidence of cerebral palsy of the improvements inobstetric and infant care which have taken placesince 1938. Between 1938 and 1952 the infantmortality in Edinburgh was halved. Much of thisimprovement was due to a reduction in the numberof infants dying from infections, but the mortalityfrom other conditions was also reduced. Unfor-tunately the extent of the reductions in the deathrate during the past 15 years is unknown for patientswith cerebral birth injury, rhesus incompatibility,and other disorders likely to result in cerebral palsy.Nor can the relative importance of these disordersin the aetiology of various forms of cerebral palsybe established in many instances. There does seemto have been an increase in the proportion of pre-maturely born infants surviving in the past 15 years,indicated in one Edinburgh hospital by a 39%reduction in the neonatal mortality in infants withbirth weights between 3 and 51 lb. It has beenestimated that the incidence of diplegia in pre-maturely born children who survive is approxi-mately 1 %, and a large proportion of these patientsgive a history of neonatal asphyxia, pulmonaryatelectasis and respiratory infections (Ingram andKerr, 1954). Clearly, if an increasing number ofpremature infants survive, especially of those suffer-ing from these complications, an increase in theincidence of diplegia must be expected. When thenumbers of patients with diplegia born in the periods1938-43 and 1944-49 are compared, it is found thatthe incidence of diplegia is increased in those deliver-ed in the later period and that a higher proportionwere premature than in the earlier (Table 5).

    TA.BIE 5NOS. OF DIPLEGIC PATIENTS AND OF PREMATURELY

    BORN DIPLEGIC PATIENTS ASCERTAINED INEDINBURGH BORN IN THE YEARS

    1938-43 AND 1944-49

    No. of DiplegicPeriod of Birth No. of Diplegic Patients Born

    Patients Prematurely

    1938-43 22 51944-49 45 19

    It seems probable that there has been someincrease in the numbers of infants surviving theeffects of rhesus incompatibility and cerebral birthinjury in the past 15 years, but the extent of thedecrease in mortality and its effects on the incidence

    of cerebral palsy, and particularly of congenitalhemiplegia and dyskinesia, cannot be estimated.

    If the incidence of prematurely born diplegicpatients, the incidence of acquired hemiplegia andthe incidence of severe bilateral forms of cerebralpalsy had been as high in children born between 1938and 1943 as it was between 1944 and 1949 the figuresfor the total incidence of cerebral palsy in theseperiods would have been very similar. It seemsprobable, therefore, that the apparent increase in theincidence of cerebral palsy since the period 1938 to1943 is due largely to the widespread use of anti-biotics and to improved obstetric and infant care.

    Comparison of Incidence of Cerebral Palsyin Present Svvey and with Other Surveys

    An attempt has been made to compare the inci-dence of cerebral palsy found during the presentsurvey with the figures obtained during other specialinvestigations. Some comparable figures are shownin Table 6. It will be seen that estimates of the

    TABLE 6INCIDENCE OF CEREBRAL PALSY IN CHILDREN OF

    SCHOOL AGE IN VARIOUS URBAN AREAS OFGREAT BRITAIN

    Incidence ofAuthority Town 'Cerebral Palsy

    per 1,000

    Selected rban areasBritish Council for the Welfare (Wallasey 2-4

    of Spastics (1948) . orwich 2-0Salford 2-0(oldam 1-9

    Cohen (1954) .. Sheffield 1-9Smallwood (1953) .. .. .. Bristol 2-0This survey (1954) . . Edinburgh 2-3

    incidence of the condition in children of school agevary between I 9 and 2 4 cases per 1,000 of thepopulation. A figure of between 2 and 2 5 casesper 1,000 of the school population is probably a fairestimate of the incidence of significant handicaps asa result of cerebral palsy in urban areas.

    During the survey records were kept in every caseof the authorities who were aware of the existence ofeach patient with cerebral palsy (Table 7). It washoped to compare roughly the incidence figures ofother authors whose sources of ascertainment weremore limited than in the present survey. It will beobserved that if only the school medical service, thepublic health authorities and general practitionershad been contacted, the incidence of cerebral palsywould have been approximately 1 per 1,000 of thepopulation under the age of 15 years. With casesknown to hospitals added the incidence becomes

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  • ARCHIVES OF DISEASE IN CHILDHOODTABLE 7

    SOURCES OF ASCERTAINMENT IN 208 PATIENTS WITHCEREBRAL PALSY IN EDINBURGH

    Nos. of Cumulative IncidenceAuthority Patients Totals per 1,000

    School medical service andschool for spastics -. 97

    Public Health Department.. 16General practitioners .. 1

    Total known to authonitesbefore present survey . . 114 1-094

    Hospitals .. .. .. 52 156 159Institutions for the mentally

    defective .. .. .. 8Personal cases 34

    Grand total .. .. .. .. .. 208 1991

    1 - 59. These figures may be compared with thefigures of a number of recent surveys shown inTable 8. On the basis of these figures it seemsunlikely that the incidence of cerebral palsy variesgreatly in different large towns though, necessarily,this conclusion must be regarded as somewhattentative.

    Comparison of Prootonal Distrion ofForms of Cerebal Palsy in Different SwveysIt is rather difficult to compare the incidence of

    the different forms of cerebral palsy in differentsurveys owing to the wide variations in terminologyused and to the different types of classificationswhich are found. In Table 9 an attempt has beenmade to compare some of the larger recent series ofcases with those found during the present survey.Owing to the inadequate data available the reclassi-fication of cases in other surveys is somewhatspeculative.

    TABLE 8INCIDENCE OF CEREBRAL PALSY IN SCHOOL CHILDRENASCERTAINED AS A RESULT OF LOCAL AUTHORITY

    RETURNS AND RECENT SURVEYS

    IncidenceAuthor Source of Data Plac per 1,000

    British Council forthe Welare ofSpastics (1948) .. Local authorities England 1-0

    Asher and Schonell School Medical(1950) .. .. and Public

    Health Author-itiesandgeneralprattioners Birmingham I-O

    Dunsdon (1952) .. Local authorities Various 1-3

    Holoran (1952) .. School Medicaland PublicHealth Author-ities, somehospitals Leeds 1-6

    There is a considerable difference in the propor-tion of patients suffering from the various forms ofcerebral palsy in the surveys. In general, the seriesbased on the examination of candidates for specialschools for children with a physical handicap orcerebral palsy show a higher proportion of patientswith dyskinesia and a lower proportion of those withhemiplegia. The latter can more frequently managein normal schools, whereas the former, though themajority of them are relatively intelligent, requirespecial educational provision. Thus Evans's (1948)and Dunsdon's special school candidates show asmaller number with hemiplegia than are found insuch surveys as Dunsdon's regional survey, thesurvey of Asher and Schonell (1950), or the presentseries. The lower figure obtained for the incidenceof dyskinesia in the present series is also partly dueto the fact that patients showing the dystonic stage

    BIF 9

    PROPORTIONAL DISTRIBUTION OF VARIOUS TYPES OF CEREBRAL PALSY IN RECENT SURVEYS

    This Asher andSurvey Schonell Evans Hellebrandt Dunsdon

    Year .1953 1950 1948 1950 1952

    No. of patients .208 349 112 531 780

    Special SelectedSchools Areas

    Hemiplegia .. 36-05% 29' . 9// 24'. 13% 35-3%Diplegia .( ..-..). (37 95%)Typenotstated - 0- 6. 2'. 7. - -Paraplegia .13-93% 26% 10/. 26% 25-5%Triplegia .11-06% 5'% 4'%Tetraplegia .. 12 96, J 42Bilateral hemipgia .3-86, 222'. ,19% 137% 19-6%Diplegia and ataxia .5-78. . . - - -Ataxia .7-23-i 1 ' 3'. 8'. - -Dyskinesia .8-18x. 10', 40' 27'. 13% 7-8%Other. 0-95% 0-3% - %Mixedforms .- 6-0', 4-0% - 11% 11-8%

    100% 99-9% 100% 100% 100% 100%

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  • CEREBRAL PALSY IN EDINBURGH

    of diplegia or rigidity with some dystonic mani-festations have been classified as diplegia and not ascases of dyskinesia.

    It will be noted that the figures quoted for theincidence of ataxia in the present series and in thatof Hellebrandt (1950) derived from a specializedcerebral palsy clinic are higher than those of theother series. This is probably due to the fact thatchildren with quite severe cerebellar or vestibulardeficit may compensate to a remarkable extent byusing the eyes. In many of these patients theRomberg test is negative and very careful examina-tion is necessary to demonstrate even moderatelysevere ataxia.

    It is interesting that the proportional distributionof patients in the other categories of cerebral palsyin this series and in the regional surveys of Asherand Schonell and Dunsdon are broadly comparable.

    Sex Distnbuto of Cases of Cerebrl PalsyIn this series, 60 5% of the patients were males

    and 39 5% females. This distribution is similar tothat reported in a number of other recent regionalsurveys (Asher and Schonell, 1950; British Councilfor the Welfare of Spastics, 1948). In other surveysa more equal distribution is reported (Cohen, 1954).

    In the present series females outnumber males intwo forms of cerebral palsy only, bilateral hemi-plegia and dyskinesia. The higher proportion ofmales in the groups of hemiplegic patients wasespecially marked.

    The Severity of the PalsyIn Tables 10 and 11 is shown the distribution of

    cases by their severity and the numbers of limbsinvolved. By 'normally useful upper limb' isimplied a limb which can be used for normalactivities, such as dressing and feeding withoutgross clumsiness being evident, whether neuro-logical involvement is present or not.

    It will be seen that 52 of the 208 patients (25 %)

    TABLE 10SEVERITY OF CEREBRAL PALSY IN 208 PATIENTS

    No. of Moder-Type of Palsy Mild ately Severe Totals

    Cases Severe

    Hemiplegia 27 26 22 75Diplegia 13 35 31 79Diplegia and ataxia 4 4 4 12Ata .. 4 8 3 15Dyskinesia.. .. 4 6 7 17Bilateral hemiplegia 0 0 8 8Other .. .. 0 0 1 2

    Totals.. .. 52 80 76 208(25'.) (38%) (37%) (100,)

    were classified as slightly handicapped by cerebralpalsy, 80 children (approximately 38 ) as moder-ately severely handicapped and 37% as severelyhandicapped. Apart from those suffering fromhemiplegia severely handicapped patients usuallyneeded assistance in looking after themselves andwere unsuitable for employment in present con-ditions. In patients classified as being moderatelyseverely affected, the possession of one functionallynormal upper limb was of crucial importance indeciding if a child required special help in lookingafter himself or not.

    TABLE 11EXTENT OF CEREBRAL PALSY IN 208 PATIENTS

    Two One NoUseful Useful Useful

    Type of Palsy Upper Upper Upper TotalsLimbs Limb Limb

    Hemiplegia 27 48 0 75Diplegia 33 19 27 79Diplegia with ataxia 4 6 2 12Ataxia 15 0 0 15Dyskinesia 3 3 11 17Bilateral hemiplegia 0 0 8 8Other I.. .. 1 1 2

    Totals 83 76 49 208(40%) (37%) (23 (100.)

    Associated Manifesations of Cerebal PalsyIf paresis of the limbs was the only maRifestation

    of cerebral palsy the vast majority of patients wouldbe educable. It is because of the associated effectsof the condition, in particular, intellectual impair-ment, epilepsy, speech defects, visual abnormalityand specific learning difficulties, that the majority ofpatients need special educational help. In Table 14some of the more important handicaps found in theseries of208 patients with cerebral palsy are recorded.

    Inteectual Impaiment in Patientswith Cerebral Palsy

    Routine intelligence tests were not performed onall the patients examined during the survey, butintelligence quotients were obtained for most ofthose of school age from schools, hospitals and child-guidance departments (Table 12). Since the esti-mates were obtained as a result of different tests,in the hands of different testers in differing circum-stances, too much reliance cannot be placed on theresults quoted. However, they are comparable tothe more accurate and better controlled resultsobtained by other surveys (Table 13).The majority of the children with cerebral palsy

    in the present series were of subnormal intelligence.Approximately 44% of the tested patients had

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  • ARCHIVES OF DISEASE IN CHILDHOODTABLE 12

    INTELLIGENCE QUOTIENTS OF 208 PATIENTS WITH CEREBRAL PALSY

    I g Quoient Over 115 100-114 85-99 70-4 55-69 Under 55 Untested Totals

    HemiplegiaMild. 1 2 4 4 8 5 3Moderately severe .2 4 2 4 7 4 3Severe 0 4 4 9 2 2 1

    Total hemipegia .3 10 10 17 17 11 7 75

    DilegiaParaplegic. 1 5 4 6 7 1 5Triplegic .2 2 0 5 3 3 8Tetrapegi.c 0 0 2 4 3 11 7

    Total diplegia .3 7 6 15 13 15 20 79

    Diplegiawih ataxia .0 2 3 1 1 2 3 12Ataxia .0 . 1 3 6 3 0 2 15Dyskinesia .1 2 2 4 2 1 5 17Bilateral p0 0 0 0 0 8 0 8Oter .1 0 0 0 1 0 0 2

    Total. . . 8 22 24 43 37 37 37 | 208

    Percentagedistnrbutn 3 -8 10-6 11-5 20-7 17-8 17-8 17-8 100

    Peracagedistnbution .. 49 12-8 14-3 24-2 21 9 21-9 - 100

    TABLE 13PERCENTAGE DISTRIBUTION OF INTELLIGENCE QUOTIENTS IN THIS SURVEY COMPARED WITH

    OTHER RECENT INVESTIGATIONS AND A GROUP OF UNAFFECTED SCOTrISH ClJILDREN

    IntelligenceQuotients 130orMore 115 to 129 100 to 114 85 to 99 70 to 84 55 to 69 Under55

    Dunsdon (1952) 0-5 2 6-2 155 17 2 23-6 35

    This survey .. 4-9 12-8 14-3 24-2 21 9 21-9

    130orMore 110 to 129 90 to 109 70 to 89 50 to 69 25 to 49 Under25 Untestable

    AsherandSchonell(1950) 0-6 3 -4 20-1 26-8 22-9 10-7 11-6 3-9Holoran(1952) .. - 2-8 22-0 36-2 21-3 8-5 3-5 5-7

    Unaffected children(Macmeeken, 1939) 4-7 21-2 48-6 24-3 1-3

    intelligence quotients of 69 or less. They were,therefore, in need of special educational provisionwhatever the severity of their palsy. Fifty-four ofthe patients, or 32% of those tested, had intelligencequotients of 85 or more and may, charitably, in somecases, be regarded as of normal intelligence.

    I 1denc of EpilepsyIn Table 15 are shown the numbers of patients

    who suffered from epileptic attacks of various types.Sixteen patients suffered from petit mal or similarattacks involving brief impairment of consciousness.Some of these patients also suffered from grand mal.Petit mal occurred more frequently in those withsevere or extensive cerebral palsy than in those witha mild disability. It was, therefore, a nuisance tothe already severely handicapped children ratherthan a cause, in itself, of serious limitation ofactivities. Grand mal and attacks of Jacksonian

    type with more prolonged severe impairment or lossof consciousness were present in 53 patients orapproximately 25% of those suffering from cerebralpalsy. In the majority attacks occurred betweenonce a month and once a year, in spite of anti-epileptic drugs. The proportion of children suffer-ing from severe cerebral palsy who showed epilepsywas not much higher than the proportion of mildlyaffected patients. The attacks were, therefore, animportant cause of limitation of activities in somepatients.

    Inience of Speech DefectsThe crude criterion used during the present survey

    to determine if a child suffered from significantspeech defect or not was whether comprehensionof what he said was rendered difficult. Minorspeech defects which were not sufficiently severe toimpair comprehension of what was said were

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  • CEREBRAL PALSY IN EDINBURGHTABLE 14

    NO. OF PATIENTS WITH CEREBRAL PALSY BETWEENAGES OF 6 AND 11 FOUND REVERSING LETTERS WHENREADING OR WRITING IN A SMALL EDINBURGH SURVEY

    No. of, BothType of Patients Reading Writing! ReadingPalsy Ques- Only Only and Totals

    tioed Writing

    Right hmipea.. 12 1 4 3 8Lefthem ia .. 13 0 1 1 2Diplegia .. 24 0 3 2 5Ataxia and diplegia

    with ataxia .. 5 0 2 0 2Dyskinesia.. 3 0 0 0 0

    Totals 57 I 10 6 17

    ignored. Generous allowance was made for retard-ation of speech in children with mental defect.One hundred and two of the patients (approxi-

    mately 49%) were considered to suffer from signi-ficant speech defects. This may be compared to thefigure obtained in one recent series where, in all,79% of the palsied children of school age were foundto suffer from speech defects, including minor defectsand probably some patients with aphasia (Dunsdon,1952). Of the patients in the present series, 10 5%had no more than one or two words of speech andare included in a total of 24-5% who had speech

    defects of such severity that they could not expresstheir ideas adequately in speech.

    of Visuma Defects

    Visual defects due to other causes than refractiveerrors were found in 35 patients (Table 15). Theincidence of partial blindness was much higher inpatients with severe cerebral palsy and mentaldefect than in those who were mildly affected. Alleight of the patients with bilateral hemiplegia, forexample, had only partial vision. In seven patientswho were considered to be educable the visualdefects were thought to be sufliciently severe tonecessitate education by non-visual means, now orin the future. Four of these patients were sufferingfrom retrolental fibroplasia, which was found in sixpatients in the series in association with diplegia.It was interesting to note the very good compensa-tion achieved by some patients who suffered fromsevere field defects.

    of Behaviour Disorder

    The majority of the children with cerebral palsyin the present series, especially those with acquiredcerebral palsy, showed functional behaviour dis-

    TABIE 15ASSOCIATED DISABILITIES AND SCHOOLING IN 208 PATIENTS SUFFERING FROM CEREBRAL PALSY

    Epilepsy SchoolingVisual Speech Over-

    assifiation No. of Grand Petit Aphasia Defect Defect acivityof Palsy Cases Mal Maland Normal P.H. M.H. Inedu- Not yet

    Jack- Other School School School cable atsonian School

    HemiplegiaMild .... 27 6 0 2 1 11 3 9 2 8 4 4Moderately severe 26 10 4 6 4 8 4 6 4 5 1 10Severe .. 22 8 0 4 6 2 2 8 5 2 4 3

    Total .. .. 75 22 4 12 11 21 9 23 11 15 9 17

    DiplegiaParaplgia .. .. 29 6 1 0 2 7 0 7 9 5 0 8Triplegia .. .. 23 9 3 3 3 14 0 3 3 5 4 8Tetraplegia .. .. 27 7 5 1 8 14 0 1 0 6 9 11

    Total .. .. 79 22 9 4 13 35 0 11 12 16 13 27

    Dipkgia with ataxiaTriplegia .. .. 9 1 2 0 0 8 1 2 3 1 2 1Tetraplegia .. .. 3 0 2 0 1 2 0 0 1 1 0 1

    Total 12 1 4 0 1 10 1 2 4 2 2 2

    Ataxia .. 5 3 1 0 2 I 1 4 2 7 3 0 3

    Dyskinesia 17 0 4 0 0 15 1 2 7 2 1 5

    Bilateral bemiplegia 8 5 0 0 8 8 1 0 0 0 8 0

    Other forms of cerebralpalsy .2 0 0 0 0 2 0 0 2 0 0 0

    Grand total .. 208 53 22 16 35 102 16 40 43 38 33 54

    Pecentage .. 28-4 10-5 7-7 16-8 48-8 7-7 19-3 20 7 18-2 15-9 25-9

    Percentage ofschool age 26-0 27-9 24-7 21-4

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  • ARCHIVES OF DISEASE IN CHILDHOOD

    order at some time or another, the result of difficultyin adapting to their disability. In addition, anumber of children showed a rather typical form ofover-ctivity which was characterized by an intensepreoccupation with the immediate surroundings,gross impairment of concentration and attention-span, and complete lack of common sense or insight.These patients were never still. All objects in theroom, furniture and people attracted great superficialmomentary interest. Objects were handled andfrequently put to the mouth, chewed and then dis-carded. The over-activity appeared to be mostsevere up the age of 8 or 10 and then gradually todecrease. This form of behaviour disturbance wasfound in 16 patients and was most frequent in thosewith hemiplegia, especially when it affected the rightside, and was associated with epilepsy (Table 15).Until the over-activity lessened most children suffer-ing from it were ineducable.

    Incdec iof Aphasia and DyspasiaAphasia or dysphasia was evident in 16 patients

    (approximately 8%) with cerebral palsy. Twelveof the patients suffered from hemiplegia, only one ofthem on the left. All but two of the 16 aphasic ordysphasic patients suffered from moderately severeor severe cerebral palsy, and a mild or moderatedegree of mental defect was present in nine. In all,however, the degree of verbal difficulty was out ofall proportion to the severity of the mental impair-ment. Cases of severe mental defect in which thereseemed to be some specific difficulty in comprehen-sion were classified as mental defective but not alsoas aphasic.The most important effects of aphasic and dys-

    phasic disorders were seen in the first years at schoolwhere a number of the patients were considered to bedefective until careful intelligence testing wasperformed.

    Specific Lrning Difficulties in Childrenwih Cer Palsy

    In addition to the handicaps imposed on thechild's ability to learn by motor impairment, visualdefects, aphasia and speech defects a number ofpatients showed specific educational difficulties.Some appeared to have great difficulty in recognizingand forming letters, numbers and shapes of smallwords, and learning to read and write was thereforemuch retarded. Mild manifestations usually lastonly a few months and take the form of reversals ofletters and small words in writing, and, less com-monly, in reading. In its more severe forms, how-ever, the laning disability deserves the name of true

    word-blindness. Ataxic patients appeared to showparticular difficulty in writing letters, and letterreversals and word inversions were especially fre-quent and severe.

    It was impossible to obtain details of the earlyeducational progress of all the children in the survey,but in 57 patients, whose intelligence quotients variedfrom 60 to 125, details were obtained and the resultsare shown in Table 14. It will be seen that a rela-tively high proportion of patients, especialy thosewith right hemiplegia, showed directional con-fusions in writing.A high proportion of patients with cerebral palsy

    showed rather poor powers of concentration and avery limited attention span, even when intellectualimpairment was relatively slight. Very skilteaching is necessary.

    The EdMcin of Children wi Cerebral PasyIn Table 15 are shown the numbers of children of

    school age in the series who attended various typesof school or were classified as ineducable. Thefigures obtained during this survey were comparedwith those of other similar surveys. The proportionsof children in different schools vary in differentsurveys. This is probably a reflexion on theselective nature of some series and of the differencesin the types of schools available in different districtsrather than a measure of differences in the types ofhandicap suffered by children in different areas.Nevertheless, the surveys show that not more thanone quarter of the children who suffer from cerebralpalsy are capable of benefiting from normal educa-tional methods. Only three patients in the presentseries were thought to be capable of taking theirhigher leaving certificates. Over half the patientsin normal schools suffered from hemniplegia and,therefore, had at least one functionally normalupper limb. In contrast, only 11 patients withdiplegia were in normal schools and there wastetraplegic paresis in only one of these. In general,types of cerebral palsy which tended to involve alllimbs resulted in a smaller proportion of children innormal schools. Thus, in spite of the relatively lesssevere mental impairment than in other forms ofcerebral palsy, only two patients with dyskinesiawere in normal schools. Of the 12 patients ofschool age who showed ataxia, there were onlytwo in normal schools.

    Thirty-three of the patients of school age (approxi-mately 21-5 %) were classified as ineducable. Inpractice this category was found to consist almostentirely of severely mentally defective children.Thirteen of the patients classified as ineducable werein institutions for the mentally defective. The

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  • CEREBRAL PALSY IN EDINBURGHremainder were in occupation centres or at home.Twenty-one were bedridden and in need of constantcare and attention. Eighty-one of the patients ofschool age (52 6 attended special schools for thementally or physically handicapped. The decisionas to which type of school was best for the childwas decided on the basis of the relative severity ofthe palsy and of the intellectual impairment. Thoughthere was some overlapping of the types of casefound in these schools; in general, those withintelligence quotients of under 70 were found inschools for the mentally handicapped and thosewith intelligence quotients of over 70 were in schoolsfor the physically handicapped. Forty-three chil-dren, approximately 28% of those of school age,were in schools for the physically handicapped, ofwhom six were in residential schools.

    Prospects of Regular Employment forChldre with Ceal Palsy

    During the survey some experience was gained ofthe employment difficulties encountered by a numberof patients between the ages of 16 and 18 whosuffered from cerebral palsy. On the basis of thisexperience an attempt was made to assess theprospects of regular employment and the need forcare of the cases of school age ascertained duringthe survey. Patients were placed in three categories,probably employable, possibly employable, orunemployable (Table 16).

    TIhe majority of patients who attended normalschools were considered to be probably employable

    with the exceptions of two relatively severely affectedpatients with diplegia, who were placed in the'possibly employable' group. Three patients withhemiplegia and two with diplegia in schools for thephysically handicapped were felt to be probablyemployable. In all 43 patients, or 28% of those ofschool age, were considered to be probably em-ployable.

    Patients with intelligence quotients of less than 70were automatically classified as unemployable.Because of intellectual impainment they were onlyfitted for unskilled manual work, which they wereunable to do on account of their physical handicaps.Patients with intelligence quotients of between 70and 85 might be expected to be suitable for unskilledwork, but the majority would only be capable ofthis if the palsy was of a relatively slight degree.Therefore those who were moderately severely orseverely affected were classified as unemployable,and patients with mild cerebral palsy were classified,somewhat optimistically, as being possibly employ-able. In all 86 patients, approximately 56% ofthose of school age, were classified as unemployable.

    Twenty-five patients were classified as possiblyemployable, 16% of those of school age. Two casesin this category came from normal schools, 20 fromschools for the physically handicapped, includingfive patients with ataxia and six with dyskinesia.Three patients came from schools for the mentallyhandicapped. They had intelligence quotients ofbetween 70 and 85 and relatively mild cerebralpalsy.

    TABLE 16ASSESSMENT OF PROSPECTS OF EMPLOYMENT IN PRESENT CIRCUMSTANCES OF

    154 CHILDREN OF SCHOOL AGE WITH CEREBRAL PALSY

    Unemployable Nos. of Possibly Employable Nos. of Probably Employable Nos. ofCriteria Patients Criteria Patients Critena Patients

    Inteligence quotient below 70Hemiplegia with epilepsy Hemiplegia

    Hemiplegia.. 22 P.H. schools .. . 5 Normal schools .. .. 23Mild hemiplegia Hemiplegia

    Diplegia .25 M.H. schools .. .. 2 P.H. schools. . . 3Paraplegia Paraplegia

    Other 15 P.H. schools .. . I Normal schools .. .. 7

    Total I.Q. under 70 . .. 62

    InteUigence quotient betwen 70 and85, but severely affected

    Hemiplegia..Triplegia

    3 Normal schoolsP.H.M.H. ,,

    P.H. schools ..

    2

    Tetraplegia TriplegiaDiplegia .10 Normal schools .. .. 1 Normal schools .. . 2

    P.H. ,, .IOthers Others

    r Other . 11 P.H. schools .12 Normal schools .. . 6

    Total I.Q. between 70 and 85 .. 24

    Total unemployable .. .. 86 Total possibly employable . 25 Total probably employable 43

    Percentage .56 Percentage possibly Percentage probablyemployable .. 16 employable .. 28

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  • 98 ARCHIVES OF DISEASE IN CHILDHOODIt seems clear from this somewhat speculative

    assessment of the chances of regular employmentfor children with cerebral palsy that not more thanone-third are likely to be self supporting in presentconditions. A number of the patients who doobtain regular work will obtain only jobs in whichprospects are poor. In present labour conditionsrelatively few of the 16% of children in the 'possiblyemployable' group are likely to obtain regularemployment, though in favourable conditions andunder some form of supervision many might makea contribution to their own maintenance.The 560% of patients in the unemployable group

    are unlikely to be able to make any contribution totheir own maintenance. The majority will be inneed of constant care for many years. Twenty-oneof the patients in this group are bedridden andprobably all will eventually be in need of constantnursing attention in institutions.

    Summary and ConcluiosA description has been given of a recent survey

    in Edinburgh designed to ascertain the incidence ofcerebral palsy in children born between 1938 and1952. Evidence is presented which suggests thatthe incidence is between 2 and 2 5 cases per 1,000children, and this estinate is compared with theincidence of the condition found in other surveys.The number of children suffering from cerebral

    palsy born between 1950 and 1952 who were ascer-tained was small. It is shown that this is largelydue to the present great delay in the diagnosis ofchildren with early symptoms of cerebral palsy.A smaller incidence of cerebral palsy was found

    in the six-year period 1938 to 1943 than in theperiod 1944 to 1949. It is suggested that thedifference in incidence is partly due to more patientswith cerebral palsy born in the later period survivingbecause complicating infections could be controlledby antibiotics. A more important cause of thegreater incidence amongst children born between1944 and 1949 is the fact that the survival rate forthose suffering from conditions likely to lead tocerebral palsy was increased in this period byimprovements in infant care and the widespread useof antibiotics.The severity of the handicaps imposed on the child

    because of cerebral palsy and associated physical andmental abnormalities are described. The relatively

    low proportion of patients capable of benefitingfrom normal educational methods, and the highproportion who are likely to be a burden to thecommunity throughout life is stressed. An assess-ment is presented of the numbers of patients likelyto obtain regular employment eventually and thenumbers who will require care and attendance.

    It is pointed out that earlier diagnosis and treat-ment of children with cerebral palsy might lead toa reduction of the physical handicap resulting fromcontractures of the limbs. Though not likely to befinancially rewarding, it is apparent as a result ofthe survey that much more attention must be paid toobtaining training and employment, if necessaryunder supervision, for patients with cerebral palsy.

    This survey could not have been made without thecooperation of so many physicians and surgeons inEdinburgh that it is impossible to mention them indivi-dually. I am especially grateful to Dr. W. N. BoogWatson, of the School Medical Service, and Dr. H. P.Tait, of the Maternity and Child Welfare Service, fortheir unfailing help, and to the paediatricians of theRoyal Hospital for Sick Children and Western GeneralHospital, Edinburgh. I owe thanks to the records officersof these and other hospitals and institutions, and to MissE. Cruickshank for much secretarial assistance.

    I am deeply indebted to Professor R. W. B. Ellis forhis guidance and encouragement during the survey andfor his help during the preparation of this paper.

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    School Children in Sheffield. Personal communication.Dunsdon, M. I. (1952). The Eduability of the Cerebral Palsied Child.

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    and Royal Burgh of Edinburgh.Evans, P. R. (1948). Archives of Disease in Chidfihood, 23, 213.Freud, S. (1893). Zur Kenntniss der Cerebralen Diplegien des Kinide-

    salters. Viennra(1897). Die Infantile Cerebrallahmurg. In Specielle Pathologieund Therapie, ed. Nothagel, H., Vol. 9, Pt. 2(2). Vienna

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