hospitalfor childreni, - bmj

SOME DIFFERENTIAL CHARACTERISTICS OF CEREBRALMOTOR DEFECTS IN INFANCY

BY

EIRENE COLLISFrom the Cerebral Palsy Research Clinic and Parents' Advice Clinic, Queen Mary's Hospitalfor Childreni, Carshalton

(RECEIVED FOR PUBLICATION DECEMBER 7, 1953)

During the 13 years in which I have been studyingthe characteristics of physical activity of babies andyoung children with motor defects of cerebral originI have again and again been confronted withdiagnoses which describe presenting signs simply as'pyramidal' or 'extrapyramidal'. With the greatestdeference I would urge the importance of clinicalanalysis of available function in each affected child.Physical activity-the expression of interrelatedactivity of neural structures-appears as a singlething, a living mosaic showing cerebral functionboth mental and motor. Physical signs are signs offunction; where the brain is incomplete they showfunction which by its abnormality reveals theabsence of neural activity indispensable to normalfunction, that is, they show the presence of a lesionor lesions. A diagnosis of, for instance, 'pyramidaldefect' is thus based on the location, by deduction,of inactive cells. Yet the defect is manifest throughthe behaviour of active cells of infinitely complexfunction-motor and mental. Hence, the termspyramidal or extrapyramidal signs are not by them-selves adequate to describe the nature of thatremaining function upon which the child's poten-tialities and the planning of treatment depend.The presentation of such elementary data is

excusable on the grounds that little is known of thegrowth of function which lacks normal components,and it is only now becoming recognized that signsin the physical activity of a child with an incompletebrain show change as that brain matures. By com-parison with the physical activity seen in normalchildren of the same age the clinician may assessthe function, both mental and motor, remaining toa child with a non-progressive lesion of the brainand plan for its fullest exploitation. In this paper Iam confining myself to a description of some of theless striking characteristics of physical activity ingrowing children with abnormalities of motorfunction from which, I believe, the nature of theirresidual motor function may be deduced. (I have

described elsewhere (Collis, 1953) some character-istics of mental activity to be seen in the physicalbehaviour of infants.) The achievements of motor-deficient children rest not only on their mentalcapacity but also on the kind of physical handlingthey receive during the stages of 'plasticity' of thenervous system. For this reason it is important thattheir remaining equipment-mental, motor andspecial sense-should be assessed as early aspossible in life and help provided for those ofsufficient mental capacity.

Diagnosis of Motor Abnormality in InfancyIt is not yet universally accepted that diagnosis

of motor defect in infancy is important. It is im-portant because inappropriate handling of affectedbabies augments their motor abnormality, andhandling which is based on the assumption that anaffected baby is normal inevitably is inappropriate.It is not always accepted that differential diagnosisis important, yet it is important because treatmentmust be appropriate to the type of motor abnor-mality as well as to the child's mental status. Noris it generally recognized that diagnosis of brainabnormality in infancy is possible where there areno gross signs. We find, however, that a history ofdefective breathing and sucking throughout infancyis commonly given in cases of motor abnormalityand that most of these children are brought to thedoctor in babyhood because the parents suspect thatall is not well. This means that signs of neuralabnormality are indeed present in babyhood thoughthey are not the signs in the adult with neurologicalmotor defect. Neurological signs must be speciallylooked for in infancy to prevent delay in instructingparents in the techniques of handling their motor-deficient babies.For practical purposes, cerebral function in the

infant may be deduced from observation of theinfantile reflexes; these are fragments of the primaryactivity of the newborn baby and may be seen as he

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ARCHIVES OF DISEASE IN CHILDHOOD

is handled. The human brain is concerned, however,with thought as well as with movement, and inso-called voluntary movement, which includesspeech, thought finds expression through the reflexmotor apparatus. The change from infantile tomature activity happens in a gradual way and ismost easily seen by observing modifications of theinfantile reflexes which can be used as guides forassessing the normality or abnormality of developingmental activity.

Brain abnormality may affect mental processesand leave reflex motor activity intact. Modificationof the infantile reflexes shows abnormality in suchcases; in fact many workers have noted imperfectmodification of infantile characteristics in mentallydefective subjects; the grasping reflex, for instance,may persist in a relatively unmodified form up to alate age. At all ages mentally defective children showabnormality in their use of movement. On the otherhand, brain abnormality may directly disorder reflexmotor activity itself so that the infantile reflexes,the sucking reflex, for instance, are impaired andthe abnormality is in movement itself and hencedeveloping voluntary activity is distorted. Wide-spread abnormality of brain function affecting boththe mental and motor aspects of physical activityis shown by a deficient use of movement which itselfis abnormal. Though in the individual case move-ment itself, or its expressive use, or both parts ofphysical activity at once may be impaired by lossof brain cells, at examination the clinician is con-fronted with the two aspects simultaneously-physical activity. In order to diagnose abnormalityand recognize normality in either aspect he mustanalyse this physical activity and distinguishbetween the two parts.

Alterations of Muscle ToneThat the normal young infant shows hypotonicity

is well known, yet babies' limbs resist reflexly directattempts to alter their flexed attitudes; both thehypotonicity and the resistance diminish as develop-ment proceeds. These and other changes expressthe evolution of intact brain activity; similarly, withthe deficient brain, evolution of residual brainactivity is expressed by changes in physical activity.Reflex motor activity is gradually modified duringthe stages of infant development and throughoutlife it serves to express thought, so even where thisreflex function is defective motor signs change withmental development and movement is modified bythought. In mentally defective babies physicalexpression continues to be characteristically infan-tile; on the other hand, where developing mentalatfivity can find only distorted expression because

of motor insufficiency, there are characteristic signsof weakness and inadequate progress. Thus in casesof motor abnormality it is essential to distinguishmodifications of movement which are related tomental activity from morbid signs. Though thisdistinction may not be easy, when signs are viewedas a whole and due weight is given to effects of anycoexistent defect such as deafness, and if the malle-ability of nervous function in the young child is dulyremembered, it is usually possible to estimatecorrectly available function, both motor and mental,in any given case.While in children with the type of motor in-

sufficiency characterized by ataxia there is nostiffness, in other types, though hypotonus remainsas a sign, stiffening takes place as the child getsolder. (Stiffness or opisthotonus in the very youngbaby occur among other signs of gross cerebraldefect and present little diagnostic difficulty.) Wherestiffening gradually develops as the infant gets older,this may be neuro-pathological in origin or mayoccur as force is exerted by or emotion aroused inthe affected child. There are thus two possiblesources of stiffening-the motor apparatus and themental apparatus. In the child with an intact centralnervous system stiffening of effort or emotion, evenwhen widespread and associated with grimacing, isreadily correctly interpreted by the observer, but inthe motor-deficient child such stiffening may easilybe mistaken for added dysfunction and, as such,attributed to the presence of a further lesion of themotor apparatus. Signs of mental activity, includingthis normal characteristic of stiffening, are notdiscernible in the very young baby and are absentor greatly diminished at physiological rest, forinstance in sleep, in the older subject. When effectsof mental activity-normal and abnormal-aresubtracted from deficient physical activity, remainingcharacteristics indicate the nature of the motorfunction available to the child, and it is on thesecharacteristics, which may or may not includestiffness, that differential diagnosis of the type ofpalsy should be based.We have been able to distinguish only two types

of stiffening of neuropathological origin in cases ofinfantile cerebral palsy; these we call spasticity andrigidity. Stiffness of either type varies in degree fromcase to case, but the reactions characterizing eachtype remain constant. While the type of stiffness wedescribe as rigidity may be present in the very youngbaby, the type we describe as spasticity developsrelatively slowly. A difference in the early historiesof children showing these abnormalities is usual.Rigidity is often a sequel of prolonged jaundice orof white asphyxia, while spasticity may develop in

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a child who suffered birth trauma or in one who wasnormally born. Mental insufficiency (unless associ-ated with specific physical abnormality such asmicrocephaly) cannot be diagnosed in the newbornchild, but when gross signs of cerebral abnormalityare present in early infancy the prognosis is poor;if a child has fits a guarded prognosis should alwaysby given. In cases of rigidity a history of early signsis commonly given, and mental defect is, in ourexperience, invariable. In cases of spasticity and incases where stiffness of non-pathological origindevelops, a history of early signs may not be given;we think that in such cases less prominent signshave been missed. Usually, affected children showabnormal hypotonus and lack of normal advancethroughout infancy, but in the absence of grosssigns they are regarded as normal until they developdeformities.

SpasticityThe neuromotor abnormality we describe as

infantile generalized spasticity can usually bedifferentiated from other types of motor defectbefore it attains its recognized clinical form andbefore the usual clinical tests for pyramidal defectare of value. Though spastic stiffness developsrelatively slowly, two early signs indicate thepresence of the neurological defect underlying itsdevelopment. Where both these signs are presentin a child up to 2 or 3 years of age the developmentof spasticity should be expected. First, with the childsupine, the ankles are firmly grasped, the thighsrotated inward and sharply abducted (Fig. 1)resistance is abruptly encountered. Second, with thechild prone, with his pelvis held in contact with thesurface on which he is lying, one of his legs ispassively flexed at the knee and the sole of thatfoot stroked firmly with the examiner's thumb nail(Figs. 4 and 5); the hip on the same side flexes.(Each reaction may be obtained repeatedly, and itshould be noted that the second reaction differsfrom the well-known infantile 'withdrawal' whichmay persist in mentally deficient children whetheror not motor dysfunction is also present.)

In infantile generalized spasticity postures of thelimbs and body of the older infant show little changefrom those seen in theyoung baby; in most cases signsof mental defect are found. Though theyoung affectedchild is noticeably inactive and hypotonic, the Mororeflex is readily evoked; the sharp contrast betweenhis slow and laboured voluntary activity and thishyperreflexia is often mistakenly regarded asevidence of abnormal 'nervousness' and fear. It isnot fear; even the normal baby shows this sign atthe time of birth befQre the emotion of fear can

safely be attributed to him. Later on fear and 'thebody jerk' may become related, but we cannotassume the presence of fear in the child with motordysfunction who exhibits this hyperreflexia as afeature of his condition. Such a child cannot developnormal security of posture and is easily thrown offphysical balance; it seems to me that physicalinsecurity, spastic stiffness, and inadequate modifica-tion of infantile reflexia sufficiently account for thesupposed 'highly nervous' state of many affectedchildren.With the development of voluntary activity the

deficient reflex motor apparatus produces thatstiffening which indicates the nature of the abnor-mality; this spasticity is subject to modification anddecreases as the child becomes more adept andspeedy in the purposeful use of his limbs. In olderchildren the gross clinical picture of Little's diseaseis seen only where there is severe mental defect, butin many children there is marked spasticity only inthe legs; this develops during the period of evolutionof use of the upper limbs when movements of thelegs are still largely reflex. Voluntary activity of thebaby's arms sets in train reflex leg movements; thebaby drags his lower limbs when trying to crawlforward by using his forearms for support and soactivates his abnormal reflex leg patterns; wherethese are subsequently reinforced by prematureattempts to assist the development of walking, theimpaired reflex apparatus has full play to establishgrossly distorted lower limb activity. Such childrenrequire skilled assistance in all early achievements ifthe development of an abnormal gait is to be as faras possible averted.As children with early signs develop, further signs

characteristic of spasticity appear. The child's limbsresist stretching only when force is sharply appliedto counter the postural tendency, and this resistanceyields to sustained traction. Force meets no resist-ance where it assists the return of the limb to itshabitual posture. Then, the two signs already notedin the infant become more marked, and with theolder child lying in the supine position, when theankles are firmly grasped, the thighs rotated inwardand sharply abducted, the hips and knees flexabruptly and the lumbar curve increases as resistanceoccurs (Fig. 2). With the child in the prone positionwith the legs extended, the pelvis held firmly downand the hip adducted, when one leg is passivelyflexed at the knee, the hip on the same sideflexes; at this stage no plantar stimulus is requiredto evoke the reaction. With the child placed andsupported on his side with his legs extended in linewith the trunk, when the uppermost leg is passivelydrawn backward and abducted, the under leg rises

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In Fig. 1, with the child (aged 3, spasticity not fully developed) supine, the ankles aregrasped, the thighs rotated inward and sharply abducted, the hips and knees flex abruptly.Fig. 2 shows an older child (aged 5) in whom the sign is more marked. Fig. 3 shows thereaction of a normal child for comparison.

In Fig. 4, when with the child prone and the pelvis held in contact with the table, oneof his knees is passively bent and the sole of that foot stroked firmly, the hip on the sameside flexes. Fig. 5 shows a similar reaction of the other hip (same child as Figs. 1 and 6).

Fig. 6 shows same child as in Figs. 1, 4 and 5 pushed into a sitting position and Fig. 6aa normal child for comparison.

Fig. 7 shows same child as in Fig. 2 supported on his side with the legs extended in aline with the trunk; when the uppermost leg is passively drawn backward and abducted theunder leg rises off the table.

Fig. 8 shows a normal child suspended by one ankle. This child is the twin sister of thechild with spasticity shown in Figs. 8a-8d. Figs. 8a and 8b show retained extension of thefreed leg on release of either ankle.

Fig. 8c shows the freed leg pushed into flexion. Fig. 8d shows the return of the freedleg to extension when pressure is released. Compare the postures of the head and armswith those of the normal twin child.

FIG. 8a. FIG. 8b.

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off the surface on which it was placed (Fig. 7). Withthe child suspended by the ankles, the feet showinversion and plantar flexion (in contrast to thenormal straight, strongly dorsiflexed feet), and onrelease of one ankle the freed leg may remainextended (Figs. 8a and 8b) or if the leg flexes thethigh rotates in; if the leg remains extended, whenthe examiner pushes it into flexion and then releasespressure from the sole of the foot, the leg spon-taneously returns with characteristic precision to itsformer extended position (Figs. 8c and 8d). Whenthe child is pushed from a supine into a sittingposition with the examiner's hand behind his neck,his head may fall forward or 'poke', the back isrounded as in a young infant or is noticeably convexbackward in the lumbar region; the legs showinternal rotation at the thigh, flexion at the knee,plantar flexion at the ankle; the child's weight restson the sacrum instead of on the tuber ischii (Fig. 6).By the time these signs are present, whatever other

abnormalities are found, a diagnosis of generalizedspasticity could generally be made on better knownpyramidal signs. The tendon jerks frequently showbriskness. 'Spastic' briskness, moreover, is character-istic, the response being identical on repetitivestimulation, each response being of sharply limitedrange. This is usually particularly well seen at theknee joints. Clonus which is abnormally sustainedmay frequently be easily elicited at the ankle joints,especially with the child in the prone position withthe knee kept passively flexed during application ofthe stimulus. The great toe may extend in a deliberatemanner when the sole of the foot is firmly stroked.In addition to these well-known signs in these cases,with the child lying supported on his side with thelegs extended, when the uppermost leg is passivelydrawn backward, abducted, and then released, itfalls and remains with the hip flexed on the surfaceon which the child is lying. This sign is invariablypresent in hypotonic infants who later developspasticity. In children with gross spasticity in whomthis sign persists dislocation of the hips may bepresent or may supervene. Attitudes of affectedchildren are determined by the character of theirphysical activity which includes the voluntary aswell as the reflex aspect of movement. Abnormallysustained attitudes constitute deformity and thisdevelops in most cases because voluntary movementis slowed and limited by the motor defect.

In the absence of hearing deficiency clear speechdevelops in intelligent children with spasticity; onthe other hand, where there is mental defect,development of speech may be retarded and thisabnormality is often mistaken for dysarthria,especially where other signs of retardation include

imperfect mastication and dribbling; in such casesthe palatal reflex is unimpaired, as it is in mentallyabnormal children with spasticity whose speech isclear but 'chattering'. In contrast, in other types ofmotor disorder the palatal reflex is usually defectiveand there is dysarthria in addition to dribbling andimperfect mastication. Among signs of mentalabnormality, squinting is common in children withspasticity, but this defect seldom persists throughoutchildhood and should be distinguished from per-sistent strabismus of other origins.

RigidityThe other type of neuropathological stiffening we

find where signs of spasticity are absent, and this wecall rigidity. We have never found this condition apartfrom signs of mental defect which include incoher-ence of movement often so gross as to be termed bymany 'athetotic movements'. A history of rhesusincompatibility or of asphyxia pallida is obtainedin these cases; in some there is a history of pro-longed jaundice in infancy. If a young baby is verystiff and shows opisthotonus and other signs ofwidespread cerebral defect, a diagnosis of motorabnormality is easily made in addition to that ofmental defect. If, however, stiffening is slight andnot constant and infantile hypotonus is prominent adiagnosis of motor deficiency may be more difficultin the early stages. Yet, in all cases of rigidity signsof both motor and mental abnormality develop asage advances, and when signs of mental activity areseparated out from the physical activity of thesechildren, neuropathological stiffness remains whichdiffers from that of spasticity. In most affectedinfants the eyes tend to roll upward and the babiesoften appear to be blind because they do not focus;blindness is an uncommon complication, however.When these babies are brought into a sitting positionthe eyes no longer roll upward. To induce a sittingposition in an infant who shows marked stiffnessit is necessary to flex his head sharply, when thechild's body flexes as it does in the Landau reflex.Many infants with pathological rigidity are found tobe partly deaf, but deafness is often overlookedwhere the mental defect is gross. Abnormal responsesto skin stimulation are often found.

In children with rigidity even where there is severemotor and mental defect, motor characteristicschange somewhat with increasing age. Where stiff-ness was constant it becomes intermittent, whilethose babies who were only slightly stiff developgreater rigidity which also is intermittent. Thus. theclinical features which develop are identical in caseswhich show rigidity, though the degree of abnor-mality varies from child to child. The combination

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of stiffness and gross incoherence of movementwhich develops in most affected children is theproduct of both motor and mental processes whichare impaired in varying degree. When signs ofmental activity are subtracted from physical activitythe rigidity which remains is the diagnostic featureas far as motor abnormality is concerned. Childrenwith rigidity of neuropathological origin showingintermittent stiffness are those whom we describe ashaving variable rigidity, and in these resistance topassive movement of a limb may abruptly cease andthe limb become limp. In contrast to the resistanceof spasticity that of rigidity is not altered by alteringthe speed of the passive movement. Stiffness andlimpness of the child's limbs and body alternateabruptly. The alterations are not like those of'cog-wheel' rigidity but are complete changes in thepliability of the limb and are usually associated withalterations of attitude of other parts of the body.Simultaneous changes of pliability are found indifferent limbs. This type of neuropathologicallydetermined behaviour of the parts of the body seemsto be unresponsive to the child's will; movementsare usually stiffly performed with the limbs and bodycontorted, and incoherence of these movements maybe very marked. Attitudes of head, limbs, and body,that is, postures characteristic of the mental andmotor activity remaining to affected children,usually become abnormally sustained. For example,the arms may be rigidly extended with internalrotation at the shoulder, sometimes so extreme thatthe back of the elbow joint is turned toward the frontof the body; the legs may similarly be rigidlyextended and internally rotated at the hip joint.Even where these attitudes are so persistent as toconstitute deformity and are even maintained duringsleep, they may yield suddenly under the examiner'shands. In addition to these signs, the palatal reflexis abnormal. Rarely this is found to be hyperactive,in most cases it is almost inactive, dribbling ofsaliva is usually present, and swallowing, chewingand speech are deficient. Abnormality of respiratorymovements is frequently marked throughout earlychildhood, and digestive troubles, including flatu-lence and vomiting, are common. Affected childrenare often ill nourished, and, as they grow older,deformity of the chest becomes marked in manycases. Sustained clonus, especially at the anklejoints, is often present, though the tendon jerks,when obtained, are normal, and, after infancy, theplantar response is equivocal or flexor. Tendon jerksand a clear plantar response are difficult to obtainin the presence of sustained rigidity.

Children with variable rigidity and incoherentmovement must be distinguished from those in

whom incoordination is of motor origin only andthe stiffness which develops with age is the result ofunimpaired mental processes activating impairedmotor processes, and they must also be distinguishedfrom children with spasticity. In children withvariable rigidity the following abnormal reactionsare found. When, with the child supine, the anklesare firmly grasped, the thighs rotated in andabducted, resistance may be encountered; in con-trast to that of spasticity, such resistance is notaugmented by increased speed of passive abduction,and instead of 'melting' under sustained tractionit may abruptly yield at any phase of the passivemovement or be sustained throughout. Attempts toflex the extended arm or leg may meet with similarresistance. Often there is no resistance to anattempt to extend the same limb immediately after-wards. When, with the child placed prone, with hispelvis held in contact with the surface on which heis lying, one of his legs is passively flexed at the kneeand the sole of that foot firmly stroked, a with-drawal reaction takes place. This is characteristic of'retardation' and occurs in a cumulative series ofjerks as the stimulus is repeated; it thus differs fromthe reaction found in the presence of spasticity.With the child placed and supported on his sidewith his legs extended, the uppermost leg momen-tarily remains in abduction when lifted and releasedunless this manoeuvre is performed during a phasewhen resistance abruptly wanes. With the childsuspended by the ankles, the feet show plantarflexion, and on release of one ankle the leg mayremain stiffly extended, though the knee is notusually turned in as it is in the child with spasticity;when pushed into flexion the leg usually remains inthe flexed position. When the child is pushed froma supine into a sitting position with the examiner'shand behind his neck, the trunk may fall forwardwith the head flexed and the back rounded.If this reaction is not immediate, passive flexion atthe neck will usually produce it; the legs may bewidely abducted and extended or may show flexionwith the feet plantar flexed. In affected childrenmental development is slow and limited and sym-metrical attitudes of the limbs only gradually succeedthe tonic neck reflex attitude described by manyauthors for early infancy and for decerebraterigidity. The physical characteristics of the olderchild, apart from the intractable fluctuating rigidityfrom which we have named this condition, dependlargely on his mental capacity and hence are subjectto modification by mental and emotional develop-ment.

IncoordinationIncoherence of mental origin in physical activity

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differs from incoordination of motor origin. We havebeen able to distinguish only two types of motorincoordination in children with infantile cerebralpalsy. These we call respectively ataxia and athe-tosis. Incoordination of either type varies in degreefrom case to case, but the reactions characterizingeach type remain constant. While ataxia may bepresent in children with a history of gross signs ofcerebral abnormality in early infancy, athetosis isfound only in children with a history of asphyxialivida neonatorum who showed no gross early signs,or, rarely, in those with a history of early normalityand subsequent asphyxia during a paroxysm ofwhooping-cough.

Infantile ataxia is, in our experience, a relativelyrare type of palsy and the clinical picture whichdevelops conforms rather closely with that seen inadult ataxia. When signs of mental activity aresubtracted from physical activity the motor defectis that there is too little stiffening. The child withataxia thus finds himself unable to move effectivelyagainst even the ordinary opposition of gravity. Inthe supine and prone positions he lies with widelyabducted, externally rotated and extended limbs.with his feet in eversion and plantar flexion, andshows no resistance to passive displacement. Withthe child placed and supported on his side, theuppermost limbs fall when lifted and released. Theleg falls into flexion if the hip is flexed before thelimb is released and extension if it is extended. Withthe child held suspended by the ankles, the headfails to extend; the feet show eversion, and onrelease of one ankle the free leg falls into abnormallywide abduction. Well recognized signs, suchas nystagmus or other eye defects, slurring speech,absent knee jerks, 'pendulum' reactions of the limbs,may also be found. In some cases hearing is defec-tive. Where there were gross early signs mentaldefect is shown as development proceeds.The unsteadiness of movement which underlies

developing voluntary activity of children falling intothe remaining group of infantile cerebral palsies isusually described as athetosis. However, the termathetosis (without fixed position) is often used todescribe any incoherence or incoordination of move-ment irrespective of its origin, and this is confusingbecause there is no other diagnostic term to dis-tinguish this last group from those in which signsalready described are found. For lack of a diagnosticterm for these cases, we have restricted to them ouruse of the term athetosis. In them, the sole abnormalcharacteristic of physical activity is generalized un-steadiness of movement including that of speech,and this abnormality must be distinguished fromthe incoherence of movement associated with mental

defect and from the hyperreflexia already described,as well as from the ataxic type of incoordination.Partial deafness is occasionally found in childrenwith athetosis. Though affected infants show abnor-mality of breathing, sucking and swallowing as asequel of blue asphyxia, and though hypotonus isa sequel of their motor insufficiency, since grosssigns are absent, early diagnosis is rare. Yet, in thesecases early diagnosis is particularly desirable as evenwhere the motor defect is of severe degree, normalityof mental processes is found and hence underappropriate management the prognosis is favour-able. With mental development, however, andespecially where there is also deafness, the bizarreexpression enforced upon mental activity by themotor disorder may mislead the observer. We areaccustomed ordinarily to deduce people's mentaland emotional processes from the expression ofthese in physical activity-in attitudes, in speech,in facial play, in gesture and in achievements. Weassess mental growth in children by alteration intheir physical expression and, normally, this includesnot only increase in physical attainments but alsodecrease in physical insecurity and incoordination.Where, however, the motor activity underlyingdeveloping expression lacks the normal attribute ofsteadiness, affected children inevitably shqw in-security and incoordination disproportionate totheir mental growth. Hence, their physical insta-bility may create a false impression of emotionalinstability or even of mental deficiency. Becauseordinary achievements are too difficult for themsuch children grimace and stiffen with effort andwhen emotionally disturbed. These are normalreactions and it is normal to throw out the armsand plantar flex the feet when physically insecure,but in these children even these reactions are subjectto distortion equally with other fragments of motoractivity. Hence, when compared with normal easymovement, difficult movement and the effects of thedifficulty may alike be taken for morbid signs andthe results attributed to two coexistent motorlesions: a diagnosis of combined extrapyramidal andpyramidal abnormality is then given.

Stiffening of effort tends to 'fix' attitudes ofinsecurity and of effort, and, in time, these sustainedattitudes constitute deformity. Further, as is wellknown, the tendon jerks may be affected by physio-logical stiffening, and incoordination may mask thenature of the plantar response; these irregularitiesmay be mistaken for pyramidal signs. Since, how-ever, the abnormal degree of stiffening exerted bythese children is associated with effort it diminishesas effort is relaxed and tendon jerks are then foundto be normal. Further, stiffening tends to mask

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motor incoordination associated with mentalactivity. The motor state may be difficult to assessunless the complex interaction of motor abnormalityand its results are separated. Reactions associatedwith mental processes are not found in earlyinfancy, during sleep or at other times when mentalactivity is quiescent, in these phases 'athetosis' isnot seen. When signs of mental activity are sub-tracted from the physical activity of affected childrenthe diagnostic characteristic of motor incoordina-tion remains. In contrast to the incoordination ofataxia where there is inadequate stiffening, theincoordination of athetosis results from inefficacioussteadying mechanisms.With the child in the supine and prone positions

no abnormal resistance or lack of resistance is foundwhen the legs are passively abducted, though thechild may stiffen with effort. With the child placedand supported on his side, when the uppermost legis lifted, the lowermost leg remains on the supportingsurface beneath it; when released the upper-most leg momentarily remains abducted andusually shows marked unsteadiness and some stiff-ness of effort may be present. With the childsuspended by the ankles, the feet show absence ofthe normal strong dorsiflexion and remain plantarflexed; on release of one ankle the leg may fall ormay remain extended. When it falls the fall iswavering; if the extended leg is pushed down itstays down. Other signs of motor insufficiency inthese cases include deficiency of the palatal reflex,inability to frown, raise the eyebrows, close the eyesnormally or smile normally. Squinting is a normalphenomenon of early infancy, but it does not persistin children with athetosis, though the fists may beheld closed, the infantile grasping reflex is notfound, nor are other signs of mental retardationpresent.

HemiplegiaEpilepsy, which is sometimes associated with

combined mental and generalized motor defects,may also occur in cases of infantile hemiplegia.This last condition is usually easily distinguishedfrom generalized motor defect; early flaccidity ofone side of the body is sometimes noticed by vigilantparents. The signs which develop on one side arenot dissimilar from those seen in generalizedinfantile spasticity; involuntary irregular movementsof the disordered limbs may be seen in epilepticchildren with hemiplegia. In some cases thewhole affected side is smaller than the otherfrom early infancy; in other cases a discrepancybetween the two sides of the body is not noticeduntil a later stage of development and seems

to be accounted for by the relative inactivity of thepalsied side in the absence of effective treatment.In the prone position with both knees flexed thechild's body curves to the affected side. In ourexperience signs of mental abnormality are usuallyfound in these children and their speech is usuallyretarded.

Conclusion

In conclusion, I have, perforce, recounted onlysome of the details of physical activity of motor-deficient children which, I think, help to differentiatetheir motor defects, and I have not described differ-ing types of mental defect. Nor have I referred tolikeness in details of physical activity. Yet, intactstructures in incomplete brains have commonfunction with similar structures in complete brains,and hence there are likenesses in physical activityto be described; these demand interpretation no lessthan do differences. I think that when both differ-ences and likenesses are considered together reason-able explanations occur for some of the features ofmotor abnormality which are often regarded aspuzzling. For instance, the apparent left-handednessof numbers of affected children, the relatively normalspeech of most children with spasticity, the mannerof development of characteristic deformity in eachtype of motor defect, the good use of the handsusually developed in children with spasticity and thepoor use of the hands so commonly found inchildren with athetosis.

Summary

From observation of developing physical activityin babies and young children the clinician deducesthe normality or abnormality of their maturingbrains.

In physical activity, however, related motor andmental functions of the brain are simultaneouslyrepresented and each is of considerable complexity.Hence, deficiency in either must be separatelyassessed and remaining function analysed, for onthe nature of this depend the planning and theoutcome of appropriate management.

Deficiencies of cerebral motor function are dis-cussed in this paper. An account of some of thesimpler details of physical activity from which thetype of defect may be deduced is given. Signs ofmental defect are not described here.

Differences distinguishing the four types ofabnormal motor activity covered by the generalterm the infantile cerebral palsies are described; twoof these four types are characterized by abnormalstiffening and two by incoordination. The two typesof stiffness and the two types of incoordination are

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122 ARCHIVES OF DISEASE IN CHILDHOODeach separately described and differentiated. Like-nesses in physical activity of affected children arenot described. Relationship has been found betweentypes and early histories.

Establishment of effective utilization of a deficientmotor apparatus depends not only on the affectedchild's own efforts but also on the kind of help hereceives in infancy. Thus rational physical handlingcan help all affected children apart from those inwhom there is gross mental deficit. In a smallproportion-perhaps 20% or even less-of allcerebral palsied children mental processes are

entirely normal even where the motor disorder isextreme, and in these the prognosis is excellent ifearly appropriate help is given. For these especiallyshould be made available the inestimable advantagesof differential diagnosis and appropriate care duringthe stages of maximal plasticity of central nervousfunction.

I would like to thank Dr. David Lawson, the PhysicianSuperintendent of Queen Mary's Hospital for Children,for permission to publish the photographs.

REFERENCECollis, E. (1953). Lancet, 1, 416.



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hospitalfor childreni, - bmj

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