INFANTILE HEMIPLEGIA TREATED BY REMOVING ONE

J. Neuirol. Neurosurg. Psychiat., 1950, 13, 243.

INFANTILE HEMIPLEGIA TREATED BY REMOVING ONECEREBRAL HEMISPHERE

BY

ROWLAND A. KRYNAUWFrom the Department of Neurosurgery, Johannesburg Hospital, South Africa

This preliminary communication is concernedwith what I believe to be a new approach to theproblem of the infantile hemiplegias. It is as wellhowever to point out at the outset that the terminfantile hemiplegia is employed here in a widesense, and that some of the cases would, in a strictattempt at classification, fall clearly into the Little'sgroup.The work on which the present report is based

has been carried out over a period of five years,during which time 12 hemispherectomies wereperformed on patients suffering from infantilehemiplegia. In no case was the hemiplegic mani-festation considered as a prime indication forsurgery. Convulsive and mental changes wereregarded as the indications for operation, and, aswill be seen from a study of the case records, I havecome to consider mental deviations in the absenceof epileptic phenomena, and also epileptic mani-festations in the absence of definite mental orbehaviour disturbances, as adequate indications forsurgery. In this series there was one death,Case 6 (J.S.). The age at the time of operation inthe remaining 11 cases ranged from 8 months inCase 12 (M.B.) to 21 years in Case 5 (Miss A.).

This choice of widely separated age groups hasbeen deliberate, as we wished to gain as muchknowledge as possible of the physiological, neuro-logical, and psychological adjustments at variousages after removal of so large a part of the brain.

Clinical ConsiderationsInfantile hemiplegia is a common condition, and

although the clinical features are well known, itmight be as well to present a resume of the symptomcomplex.

Hemiplegia.-The hemiplegic element usuallyaffects the arm, leg, and face, with the arm alwaysaffected to a greater degree than the leg and face.The weakness is usually of a spastic type, and inmost cases the arm assumes a typical posture ofadduction at the shoulder and flexion at the elbowand wrist. In some instances extensor movements

are almost impossible. In the lower limb thegreatest weakness is seen in the dorsiflexors andevertors of the foot. Contractures tend to fix thelimbs in paralytic attitudes. However incapablethe upper limb may be, most of these patientseventually learn to walk, with a greater or lesserdegree of weakness, spasticity, and deformity.Many cases have little contracture, but exhibit anataxic or athetoid awkwardness of the affectedlimbs, resulting in what Wilson described as a" gaucherie of volitional act ". Underdevelopmentof the affected limbs, both in length and bulk, is a

constant finding. Trophic and vasomotor changeshave been described, but these have been minimalin our experience. The face is little, if at all,affected, but a radiograph of the skull invariablydemonstrates a considerably smaller compartmenton the side of the affected hemisphere, which is theside opposite to that of the general growth im-pairment. Choreo-athetoid movements, sometimeswith the limbs at rest, but usually the accompani-ment of volitional endeavour, were a prominentfeature in all our cases, but here again the upper

limb seems most severely affected.

Epilepsy.-Epileptic convulsions occur in themajority of cases; these are often focal and of theJacksonian type, but generalized seizures, psycho-motor equivalents, and petit mal, have all beenobserved. Osler stressed that " one of the mostcommon and distressing symptoms is the occurrenceof convulsive seizures ", but he also said that," more distressing still to the relatives is the en-

feebled mental state which so often follows infantilehemiplegia ".

Mental Changes.-In the cases which have comeunder my observation it has been the mental statewhich has impressed me most strongly, and inmany, it has been the mental state, rather than thehemiplegia or epilepsy, which has led the parents toseek advice. The mental changes cover a widerange from virtual imbecility at the one extreme, tothe mildest retardation at the other, but this is

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ROWLAND A. KRYNAUW

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FIG. 1.-Basal stumpafter hemispherec-tomy.

often associated with an asocial outlook, whichmay well be related to the convulsive episodes. Inmost of these patients an outstanding feature hasbeen episodic outbursts of violent " temper tan-trums" which usually have no adequate cause, areshort lived, and suggest the possibility of their beingepileptic equivalents. Some patients attribute theiroutbursts to a compulsive drive which they areunable to resist. With regard to the languagefunction, young children who have learned to speakand then develop infantile hemiplegia may losethis function. In other cases, speech may belittle disturbed.

Pathological ConsiderationsNo neurologist would expect to find any single

pathological event subserving so large and variablea grouping of symptoms and concomitant signs.The gross pathological state in our series hasincluded porencephalic cysts, intracerebral para-ventricular cysts, arachnitic watery cysts, microgyralformations, and a small sclerosed hemisphere,associated with vascular alterations in theterritories of one or more of the main arteries.Each or any of these changes may be foundeither singly or in combination with the others.The list is not complete, but serves to indicate thewide range of pathological changes encountered.I would, however, like to stress that the findingsrepresent pathological " end states ", and are notto be regarded as progressive lesions. The patho-genesis of these established end states is not clearin all cases, but the protocols lead us to deduce thatbirth trauma, or later events during infancy, suchas trauma, infective, embolic, or thromboticepisodes, are the chief aetiological factors.

Air StudiesThe air studies play an important part in deter-

mining suitability for operation, but a less important

part in determining the scope and extent of theprocedure. Air pictures are presented with theindividual case records, and it will be seen thatapart from tne focal ventricular deformities encoun-tered in the porencephalic group, a considerabledilatation of the ventricle on the side of the affectedhemisphere has been encountered in all cases vithone exception, Case 10 (P.T.). We have come toexpect gross distortions in the air pictures, andprovided these remain unilateral the case is regardedas suitable for hemispherectomy.

OperationIn the past the vast majority of these patients

have had to rely on anticonvulsants for the manage-ment of their epilepsy. Orthopaedic surgeons havedirected their attention to the correction of defor-mities, and re-education by physical methods.Neurosurgical endeavour has been largely con-cerned with local and limited cerebral operationsfor epilepsy, where the episodes have been focal.Local excisions and incisions may in some casesstop fits, but in my experience they have no bene-ficial effect on the mental state, and I have notedlittle change in respect of physical disability.Because of this, and because of certain features ofthe electrocorticograms in these cases, I was led onto a more radical procedure embodying removal ofthe affected hemisphere, with the exception of thethalamus, the caudate nucleus and its tail. Withregard to the operation, a generous lateral osteo-plastic flap affords all the room that is required.The hemisphere is divided into four segments by avertical and transverse incision of the superolateralsurface extending into the ventricle. Each quadrantis dissected out, working from within the ventricle.The middle cerebral artery is taken just lateral toits antero-lateral ganglionic branches, that is, justlateral to the anterior perforated substance. Thechoroid plexus is carefully coagulated and removed

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SURGICAL TREATMENT OF INFANTILE HEMIPLEGIA

from the lateral ventricle. The dura is closedcarefully, and a burrhole made in the centre of thebone flap for post-operative aspirations. It will beseen that the whole of the cerebral cortex with theexception of that portion medial to the tail of thecaudate nucleus is removed, together with theputamen and globus pallidus. Fig. 1 is a photo-graph taken at operation to show the basal stumpafter the hemisphere has been removed.

Post-Operative ResiduaThe vast implications arising from such a

procedure are a source of considerable speculation,and will be dealt with more fully in a later com-

munication. It is perhaps worth noting, however,that even when an apparently functioning motorcortex, area 4, is present, as determined bythyratron stimulation, its removal together with thelentiform complex does not result in more than a

transient increase of weakness of voluntary motorpower. Some movement has been present in allcases the day after operation, and in some cases a

few hours afterwards. The return to pre-operativepower is rapid, and is associated later with a

considerable lessening of the spastic athetosis whichis such a significant feature in these cases. It isfurther to be noted that this improvement is notjust transient, and back to the pre-operative level,but is a definite progressive improvement, exten-ding over a period of many months or years

Case 1 (A.H.).In the sensory sphere there is at first a profound

loss of all cortical sensory modalities. Compen-sation seems to occur soon, and in all cases has beenso complete within a few months that the changesno longer constitute a subjective disability. In no

case has the so-called thalamic syndrome developed.Quantitative and qualitative appreciation of painand tactile stimulation as tested by pin prick andcotton wool have returned to normal by comparisonwith the opposite side, and within the limits ofroutine tests. A contralateral homonymous hemia-nopia with sparing of the macula has been inducedin all the cases in which this was not already presentbefore operation, and up to the present this hasremained constant. The disability is, however, one

of which the patients are quite unaware, exceptwhile being specifically tested for it, a finding inaccordance with other experience. In Cases 3, 8,and 12, the remarks concerning residual phenomenaonly apply within the limits of our ability to examinesuch young patients. Other factors relating tobehaviour, mentality, the intellectual sphere,voluntary and involuntary movements, spatialorientation, tactile discrimination, are all consideredin individual case records.

Electro-Encephalographic ChangesEEG studies in all our cases have shown very

considerable deviations from normal. In mostcases it has been a non-specific dysrhythmia notconfined to the affected hemisphere. In someinstances a quiet area has been disclosed, whichat operation later has been found to be the regionoverlying the porencephalic cyst. EEG recordstaken after removal of the affected hemispherehave shown the electrical picture rapidly settlingdown to within normal limits. It would appearreasonable, therefore, to suppose that the dysrhyth-mia encountered in the remaining hemisphere wasdue, in the first instance, to an overflow from thegrossly abnormal high amplitude components fromthe opposite and pathological hemisphere via theinterhemisphere communicating pathways. Herein,to my mind, is objective support for my contentionthat the entire hemisphere on the side of abnormalpathology should be removed. I feel that if anypart of the hemisphere is retained, it will continueto inflict its unruly patterns on the opposite,anatomically normal side, with consequent disrup-tion of its natural physiological activities, particularlythose more especially concerned with the highestintellectual integration.

I think that it is even possible that the continuedbombardment of the unaffected hemisphere by suchunruly elements is capable of inducing secondaryepileptogenic foci. Support is given to thiscontention by a study of case records, particularlythose of Case 3 and Case 9, in which there werefrequent petit mal episodes before removal of thehemisphere. In the past many of our clinicaldeductions regarding localization and function inthe central nervous system have been based on theconcept of non-function and negative activity ofareas of pathology, and due regard has not alwaysbeen given to the fact that these areas may often bethe site of increased, albeit distorted activity ; inother words, zones of dysfunction rather than of non-function. At operation electrocorticograms havebeen recorded, and are included later with theindividual case reports, together with photographsof the brain with markers in position indicating theregions from which bipolar recordings were made.It will be seen that zones far removed from the siteof macroscopic pathology are often those showingthe greatest dysrhythmia, and it was this fact whichfirst led me to remove the entire hemisphere in thesecases.

Case ReportsCase 1 (Serial No. 1624, A. H., Female).-This child

was brought to me on January 16, 1945, at the age of9 years. There was a story of prolonged, difficult

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ROWLAND A. KRYNAUW

labour. The following is a letter from the child'smother when she made the appointment two monthsearlier. It is such a good presentation of the case, thatI take the liberty of setting it down in place of my ownroutine history.

"Dear Doctor,My little girl aged eight years was injured at birth by

forceps. The injury was over the left eye, and, I wastold, would cause no trouble. However, three daysafter she was born, she developed a jaundiced con-dition, had a collapse, and became slightly paralysedon the right side. She was given calcium andrecovered, but has grown up with her right side affected.At first I considered it only a physical injury. Shedrags the right foot slightly and leaves her right armhanging unnaturally, and her right eye droops. Now,however, I realise that her brain too is affected. Sheis liable to go into unreasonable and unmanageabletempers, and lacks the power to concentrate, andshe has had mental lapses and rigors which I am toldare epileptic. I regret I was not told the birth injurywas the root ofthe trouble. I was given the impressionthat it was a physical fault on my part and have hadno other children."

The child had been placed in an institution becauseof the temper tantrums and irritability which madediscipline impossible.In the past 18 months .she had developedmental lapsesin whichthere occurred grind-ing of the teeth, rtalkingcompletenon-sense, uncontrolledshouting, and theuttering of queernoises lasting fromone to two minutes.Mentally she was slowand backward, andhad not learned towrite or read.On examination,

the cranial nerves didnot show any devia- -tions worth recording,the optic discs wereof normal appear- FIG. 2 (Case 1. A.H.).-Veniance, and the visualfields were full.The right arm was shorter and less well developed thanthe left: posture was typically hemiplegic, tone was

increased, and purposive movements exhibiled a grossathetoid component. The right lower limb was shorterthan the left, with slight equinus deformity and toneabout natural. There was moderate weakness of distalmuscle groups, both dorsi and plantar flexors. Gaitwas clumsy. The tendon reflexes were brisker in theright upper limb than on the left, but about equal on

both sides in the lower limbs. Abdominal reflexeswere all present. No significant loss was encounteredfor any of the sensory modalities. Ventriculographywas carried out and showed a large porencephalic defect

situated in the posterior half of the left hemisphere(Fig. 2).

Operation (February 9, 1945, Hemispherectomy).-Oncoming round from the anaesthetic the child was able tomove the right arm and leg as well as before operation,but she now exhibited a right facial weakness. She wasallowed up on February 21, and was discharged fromhospital on March 10. It is noteworthy that duringthe post-operative period she was sweet tempered andcooperative: "altogether a different child ". Hermother had taught her the alphabet, and within threeweeks of operation she was reading and understandingsimple sentences such as " The cat sits on the mat ".She was also making progress with her first steps inwriting, e.g. making pot hooks, and printing, with theleft hand. The most important feature was that sheexhibited a desire to apply herself to these matters.I last saw the child on January 13, 1949, and the followingis an extract from my follow-up note:Follow-up Note.-This child is now 13 years of age.

She is rather stout. Her mother tells me that herperiods started about one year ago and these are quiteregular. With regard to the mental state, she has beenat school for about two years, and is in Standard 3,

triculogram. Antero-posterior and right(Negative touched up.)

lateral.

that is, she is in a class with children about 10 yearsof age. She is good at some subjects, particularlyhistory; arithmetic is weak, but reading and writing sheseems to have mastered all right, although she is notreading for her own pleasure.With regard to her conduct, her mother tells me that

her association with other children is entirely normal;she is at a boarding school for normal children andlikes it. Owing to some clumsiness of the right armshe is not partaking in general sport, but she is good atswimming. With regard to memory, she has a goodretention of poetry. There has been no evidence offurther attacks, or any of the emotional outbursts

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SURGICAL TR-EATMENT OF INFANTILE HEMIPLEGIA

which were the main presenting features when I firstsaw her. Her span of attention is good; she is anexcellent witness, and cooperates well throughout theexamination. In attitude she is a little shy and reservedduring the first few minutes of the consultation, butsoon settles down to a normal approach. Generalhealth has been good. The mother has become awareof the right homonymous field defect, and tells methere is sometimes a drooping of the right eyelid.Extreme ocular movements are not quite perfect. Herwalking is now nearly normal, and any disability thatthere is is not due to spasticity, or an athetoid element,but due to a relative slight shortening (about c in.) ofthe right leg, with a slight shortening of the tendoachillis and some degree of pes equinus.

Neurological examination reveals a right homonymoushemianopia with macula sparing, and the fundi aresomewhat pale. The corneal reflex on the right is notso brisk as the left, but pin prick and cotton wool arenormally appreciated in the trigeminal field on bothsides. There is no facial weakness. The tongue protrudesnormally. Her speech is a little slurred; this has alwaysbeen so, and her mother tells me that in the home circlethis entirely disappears.

There is a slight relative shortening of the right upperlimb compared with the left, but this is less noticeablethan it was originally, and the muscle bulk is good.There is still weakness of extension of the wrist ; flexionis good and equal on both sides. Tone is perhaps slightlyincreased in the flexor muscles of the wrist. Reflexesof the right arm are only slightly increased as comparedwith the left. She is not using the right arm too well,as, for instance, when she is dressing herself, tying hershoelaces, fastening a buckle. Posture is equallymaintained on both sides. In the finger-nose-fingertest there is now only a small residue of the grossspastic athetoid element which was there before opera-tion.

In the lower limb power is good and equal in allmuscle groups, except for some resistance of dorsi-flexion of the right foot as a result of a slight contractureof the achilles tendon as noted above. Purposivemovements in the lower limbs are very accurately carriedout, and the " heel-knee-shin " test was faultless onboth sides. The muscle bulk is good and equal, andalthough there is shortening, this is now relatively lessthan it was four years ago. In the lower limbs thereflexes are about equal, and somewhat brisk on bothsides. The right plantar is still extensor, and on theright, the abdominal reflexes, although present, are slightlyless brisk than on the left.

Pin prick and cotton wool are equally well appreciatedover all areas of the body, although on the first testingshe thought it was perhaps a little sharper on the leftthan on the right, but later was quite certain that thiswas not so. Joint sense was carefully tested in thefingers of both hands, and at the wrists. She madeseveral mistakes, about three out of ten in the rightindex finger and more mistakes for the little finger, onthe right side, whereas the left was absolutely accurate.Wrist movements through a small range were equallyappreciated on both sides; two point discrimination,

thenar eminence, 1-2 cm., occasional mistake right side,accurate on the left. With eyes closed she had difficultyin finding the right forefinger with her left hand, but wasvery accurate in finding the left forefinger with the righthand. Localization of pain and touch was accurate onboth sides.

Case Comment.-A year has passed since thisnote was made, and I have not had theopportunity of seeing the child again. Her mothertells me that she continues to make satisfactoryprogress in all spheres, and that she still remainsfree from any fits, five years after operation. It isto be noted that she has not received anticonvulsantsedation since operation.

Case 2 (Serial No. 2832. C.M., Female).-The patientwas born at full term and instruments were used.According to the father the child was perfectly normaland healthy until the age of 2 years, when she suddenlyhad a convulsive episode which resulted in paralysisof the right arm and leg. After six weeks power startedto improve, but she was left with a clumsy weaknessof the right side. Three years later, at the age of 5,she started having fits. The father said that the fitscame on without apparent warning, and that con-sciousness was lost, the eyes turned up, and the legswere twitching. Since the age of 5 years she has beenhaving fits almost nightly, and as many as 12 during a24-hour period. Uncontrollable emotional outburstsand destructive tantrums were frequent events.On questioning the child said that she usually knew

when an attack was coming on. She would have afeeling of fear and depression, and she would thenfeel her right arm beginning to pull upwards.

*'.'.

FIG. 5 (Case 2. C.M.).-EEG tracings at operat ion.

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ROWLAND A. KRYNAUW

bFIG. 3

FIG. 3 (Case 2. C.M.).-Encephalo-gram: antero-posterior and rightlateral.

FIG. 4 (Case 2. C.M.).-Cortex ex-posed at operation. EEG markersin position.

FIG. 4

FiG. 6 (Case 2. C.M.).-Motor cortexas determined by stimulation.

FIG. 6

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The father said that she never used the right arm orhand, and that she dragged the right foot and walked-with a limp.

The child exhibited a marked and irritating restlessness,and it was impossible to hold her attention for morethan a few seconds.There was no disturbance of the language function

but her vocabulary was very limited. Schooling hadbeen attempted with little result.The visual fields were full to confrontation tests, and

the fundi were of normal appearance. There was nofacial weakness, and no abnormality was detected inexamination of the other cranial nerves. The rightarm and hand were found to be much smaller andshorter than the left, with the limb adducted, the elbowflexed, the wrist dropped, and the fingers extended andflaccid, whereas the rest of the limb was rather spastic.Purposive movements showed a marked athetoid clumsi-ness. Tendon reflexes were all brisker on the right,the abdominals were present, and the plantar responsewas of the extensor type on the right side. Pin prickand cotton wool were normally appreciated throughout.Considerable loss of joint sense in both the hand andfoot on the right side was present. Air encephalographywas carried out, and showed a gross dilatation of thelateral ventricle on the left side (Fig. 3).

Operation (June 20, 1947, Hemispherectomy).-Theconvolutions of the promotor and motor areas wererather narrow and felt leathery to the advancing brainneedle. The frontal pole was also of a rather toughconsistency, but posteriorly in the parietal and occipitalzones the brain had a more normal feeling, but theconvolutions were rather wide and flattened, and perhapssomewhat pale, quite unlike those anterior to Rolando.Bipolar electroencephalographic recordings were takenfrom the positions indicated in the operation photograph(Fig. 4) which shows the markers in position. Fig. 5shows the EEG recordings taken from these positions.

Thyratron stimulation was also carried out and themotor cortex mapped out as shown in Fig. 6.The day after operation it was noted:" She is drinking and swallowing well and passing

urine normally. There is some movement of the rightarm, which is spastic. The right leg is atonic, but themovement is good."On July 10, 1947, there is the following note in the

records:" She is doing occupational therapy in order to

train her right hand to greater advantage." By August 8her walking was " quite good ", and she could extend herwrist slightly. A letter from C. M.'s father, datedFebruary 14, 1949, states.

.I am pleased to tell you that she is in perfecthealth. Mentally there is a big improvement. Thereare no signs or symptoms of the fits returning andshe seems to be more balanced and contented in hermind. C is still, however, more childish in heroutlook than her age would indicate, probably on apar with a child of ten, which does not mean to saythat she is stupid. Her appearance is quite normaland bright. Unfortunately her hand and leg are stillshowing no signs of improvement; they are as b2fore,

but she is very active and tries to use her handsometimes."Case Comment.--I last heard of this child in

January 1950, two and a half years after operation.Mentally she continues to make marked andgratifying improvement. She mixes well with otherchildren; she is tractable and well balanced, andexhibits none of her former emotional outbursts.There have been no further fits since operation.No sedative medication has been given her.

Case 3 (Serial No. 3331, W. O., Female).-Thischild was first seen on February 10, 1948, aged 3 years.She was a premature infant. The mother's pregnancyand confinement were normal. Development appearedto be normal up to the age of 10 months, by whichtime the child had started to crawl and to use a fewwords. At this stage she developed a febrile illness inwhich a squint of the right eye and paralysis of theright arm and leg were noted. Some power returnedto the limbs and the child was able to walk in duecourse, but with difficulty, and she did not regain thevoluntary use of the right upper limb. In February,1947, one year before examination, right-sided epilepti-form seizures developed. These occurred daily withoutloss of consciousness. The child's speech did notdevelop normally; and at examination her vocabularywas limited to three words, " Mama ", " Baba "," Ta-ta ". The child was obviously grossly mentallyretarded. She cried a great deal and became very noisyfor no apparent reason. The fontanelles were closed andthe head of average size. There was a suggestion of avisual field defect but this could not be checked. Therewas a mild bilateral ptosis. Otherwise, as far asexamination was possible, no anomaly could be detectedin the cranial nerves. There was a spastic paralysis ofthe right upper limb, the growth of which was markedlyretarded. The growth of the right lower limb wasretarded and there was less spontaneous movementthan in its fellow. There was a right pes equinusdeformity. The tendon jerks were increased on the rightside, the right abdominal reflexes were absent, and theplantar response was extensor. The gait was grosslyunsteady and reeling. Air studies were carried out andshowed a considerable dilatation of the left lateralventricle (Fig. 7).

Operation (April 19, 1948, Hemispherectomy).Fig. 8 shows the exposed cortex with markers indicatingpoints from which bipolar EEG recordings were taken.The EEG tracings are reproduced in Fig. 9.The entire left hemisphere, with the exception of the

thalamus, caudate nucleus and its tail was removed.It was interesting to note that thyratron stimulationproduced no movement in this case, so that there wasno direct evidence of functioning motor cortex. Imme-diately on regaining consciousness it was evident thatthe movements in the right leg were not impaired. Thepost-operative course gave no reason for undue anxiety.Movements in the right upper and lower limbs dis-appeared on the first post-operative day, the limbs onthe right side becoming spastic. The child was parti-

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cularly apathetic for the first 11 post-operative days, andit may well be that this was the cause of the apparentinability to move the right-sided limbs.On May 5 movement of the right lower limb returned

and the child took more interest in her surroundingsand responded to attention. By May 12 she was playingwith her toys and was greeting the attendants with acheerful smile. By May 17 the head wound was wellhealed. The child was attempting to express herselfby making peculiar little noises which were, however,quite unintelligible. She was particularly bright andhappy and taking a lively interest in her surroundings.There was some movement of the right upper limb andthe movements of the right lower limb were maintained.Movements were mainly in the proximal parts of thelimbs, but there were no voluntary movements of thehand or foot.On May 24 physiotherapy was begun. The child

was discharged from hospital on June 10. It was thoughtat the time that there had been a considerable improve-ment in the mental sphere since operation. She wasmuch more responsive, was paying more attention toher toys than before operation, seemed to be attemptingto form words, and the bouts of noisiness and cryingwere no longer present. When she left hospital onJune 10 there were still only slight shoulder and thighmovements on the right side. The upper limb was stillmore spastic than the lower; the reflexes remained asbefore operation. There had been no epileptiformseizures since operation.The child returned on August 14, 1948. We were all

delighted at the progress shown. She was walking andrunning, although the right lower limb was still spasticand showed a fixed equino-cavus deformity. Balanceappeared to be perfect; movements of the right upperlimb were still minimal. The father expressed thedeepest pleasure at the child's progress. There hadbeen no seizures since operation and the child hadremained consistently sweet tempered. The vocabularyhad extended considerably and the child appeared to beprogressing very rapidly in the mental sphere ; shecould understand commands and carried them outproperly.

Case Comment.-The child was seen again inNovember, 1949, and one was struck by her remark-able growth and general physical development.The spastic disability of the right arm and leg wasconsiderable, although she was walking well andattempting to use the right hand. Her understandingof the spoken word was excellent, and speech wassurprisingly good; she prattled away in themanner one would expect of any child between theage of three and four. There have been no furtherfits or suggestive episodes.

Case 4 (Serial No. 2582, R. v.d. V., Male).-The boy,aged 10, was admitted on January 27, 1948.A few days after a normal birth, the infant became very

ill and weakness of the limbs on the right side wasnoticed. This was accompanied by convulsions. Thechild was speaking at one year, and walking at 17

months. His mentality subsequently appeared to bebelow average. Petit mal episodes began two,years before examination, and were increasing infrequency. He had also developed episodes, precededby complaints ofepigastric pain, in which the eyes becameset and the right hand was drawn up and remained fixedfor a second or two. The hemiparesis of the right side7persisted.On examination, the positive findings included a

right homonymous hemianopia, external divergentstrabismus, a right-sided facial weakness of theupper motor neurone type, right spastic weakness ofthe arm associated with athetoid movements, andspastic weakness of the right leg. The limbs on theright side had lagged behind in growth. There wasgross impairment of three-dimensional sensation onthe right side. The child was left handed and showeda distinct tendency to " mirror writing"; for this.reason he had great difficulty in school. He displayedan endless drive to activity which manifested itself inthe performances of numerous drawings and paintings,which showed a good colour sense, a general apprecia-tion of form, but a lack of appreciation of the relativeposition of the main objects represented. EEG showedgeneralized non-specific dysrhythmia, and a large areaoverlying the left occipital and parietal regions fromwhich there was little or no activity (Fig. 10).

Air studies revealed a large, left-sided porencephalicdefect, occupying the posterior half of the left hemisphere-(Fig. 1 1).

Operation (March 22, 1948, Hemispherectomy).-EEG records were taken from the exposed cortex(Figs. 12 and 13). Stimulation and mapping out of themotor cortex was followed by removal of the entirehemisphere, with sparing of the caudate nucleus andthalamus (Fig. 14).The day after operation no movement was detected

in the right arm, which was spastic. There was some-slight movement of the right leg. Speaking and swallow-ing were not impaired. On March 24 movement hadreturned to the right upper limb. On Marclh 25 move-ments in the right upper limb were carried out quitewell at the pre-operative level, when first asked to doso, but the child found it extremely difficult to repeatthe movement more than once in a short space of time.On April 7 the child was carrying on normal conversa-tion. The spasticity of the limbs on this date was notedas being less than in the immediate post-operative phase.On April 14 the child was walking very satisfactorily-at a level corresponding with the pre-operative state.On May 10 he was discharged from hospital. At this.time he was up and about and played happily with theother children in the ward. Attention was now easilyobtained and well held. Comprehension and orienta-tion were unimpaired. Memory appeared to be goodfor both recent and remote events. He appeared tohave lost his spatial disorientation. He felt that henow appreciated the relative position of objects, and,when in the subsequent months he was kept underobservation, it was noted from his drawings that thisappeared to be so. The tendency to " mirror writing'was no longer present. He was already aware of a

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FIG. 10

FIG. 10 (Case 4. R.v.d V.).-Pre-operative EEG tracings.

FIG. 11 (Case 4. R.v.d.V.).-Ven-triculogram: A.P. and rightlateral.

FIG. 1Z (Case 4. R.v.d.V.).-Exposed cortex at operation.

I.

FIG. 12

252





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FIG. 13 FIG. 13 (Case 4. R.v.d.V.).-'FIG. 14 (Case 4. R.v.d.V.).-

stimulation.

FIG. 15 (Case 4. R.v.d.V.).-I

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EEG tracings at operation.-Motor cortex as determined by

Post-operative EEG tracings.

FiG. 15

253




ROWLAND A. KRYNAUW

great change in his ownmental state and discussedhis mental outlook as com-pared with the pre-opera-tive state. He said that hisright arm and leg were lessstiff and more easily con-trolled than previously. Heno longer felt restless, but /would concentrate for any 1ilength of time on matters ,\which interested him, such -Ias drawing and painting.He admitted that before*the operation he wouldhave uncontrollable urgesto do naughty things, thathe could not and did notwant to learn, and that hismind never seemed tosettle. Now, after theoperation he no longer feltrestless, was interested inhis painting, and wantedto improve his art and waskeen to proceed with his schooling. His Iported all this and said he was no longer juchild, but an interested and interesting mehousehold.

Case Comment.-I have seen this boyintervals; he often comes up to thetalk to us, and to show us his latestHis improvement has been progressive;at school and doing well, and as anshowing great promise. There have beeifits or petit mal episodes, spasticity in tlessening, although the shortening ardeformity of the right leg and foot cconsiderable disability. The athetoid cohis purposive movements is now veryPin prick and touch are normally appr

FiG. 17 (Case 5. Miss. A.).-Ventriculof

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---.

FIG. 16 (Case 5. Miss A.).-Pre-operative EEG tracings.

parents sup- localization of these modalities is accurate. Jointist a difficult sense is considerably impaired in the fingers of thember of the right hand, but is accurate at the wrist. He has a

right homonymous hemianopia with macularat regular sparing. The right lower facial weakness is still

hospital to more pronounced than it was before the operation.t drawings. An electroencephalographic check-up 12 months afterhe is back operation showed the responses to be within normal

artist he is limits (Fig. 15), with no activity on the right siden no further from which the hemisphere had been removed.

hd luins s Case 5 (Serial No. 3082, Miss A., Female).-Thisnstitute a young woman was first brought to me on January 16,1948, at the age of 21 years. There was a story oftmponent in delivery by Caesarian section, and the infant was bornmuch less. with weakness of the right arm and the right leg whicheciated and has persisted. Fits started at the age of 9 years. These

were of the tonic clonictype, starting in the rightarm with spread to the rightleg and right side of theface, and occurring at in-tervals of five to seven days.More recently, in additionEd ? ::'shehad developed minor

- _2 w fepisodes during which shefelt she was " wearing anuncontrollable grin ", andduring which there seemedto be a momentary suspen-sion ofconsciousness. Theseepisodes were occurringseveral times each week.;On examination, the

mental age was assessed atgram: postero-anterior and right lateral. 10 years 4 months, and the

254




SURGICAL TREATMENT OF INFANTILE HEMIPLEGIA 255

_3 i,/\ r.FUjrv pMa9>t.JN> -- 3

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FIG. 18

FIG. 19

FIG. 18 (Case 5. Miss A.).-Lefthemisphere exposed at operation.

FIG. 19 (Case 5. Miss A.).-EEGtracings at operation.

FIG. 20 (Case 5. Miss A.).-Motorcortex as determined by stimulation.

FIG. 20




ROWLAND A. KRYNAUW

l.Q. at 70. There was no dis-turbance oft[e language function.Visual fields were full. The limbson the right side were shorterand less bulky than on theleft, and displayed a spasticathetotic state with increasedjerks and extensor plantar res-ponse. Joint sense was poorin the fingers of the righthand. There was also in-accuracy of two point dis-crimination and impairment ofvibration on the right side. Thisgirl was unhappy, depressed, andacutely aware of her disabilities.She had no amusements, didnot read, and was afraid to mixwith other people. She would notgo to places of entertainment"for fear of an attack ". Herpersonal appearance meantnothing to her and she statesthat she had " nothing to live for ". The rEEG and air studies are shown inand 17.

FIG. 22 (Case 5. Miss A.).-Post-operatiLeft.-Five w;eks after operationRight.-Six months after operatio

FIG. 21 (Case 5. Miss. A.).-Post-operative EEG recordings.pre-operative Operation (August 25, 1948. Hemispherectcmy).-

Figs. 16 After the cortex had been exposed by an appropriateleft lateral flap EEG recordings were taken (Figs. 18and 19).The motor cortex was mapped out by thyratron

stimulation (Fig. 20). This picture also shows thethinned out gliosed cortex in the lower two-thirds ofthe line of Rolando. The arrangement of the bloodvessels is noteworthy, as is the peculiar distribution ofstimulable motor area. Complete hemispherectomy, asdefined earlier in the report, was then carried out.By the next day Miss A. was speaking normally, and

r movement of the affected limbs had returned to thepre-operative level.

Progress in this case ha, been extremely gratifying,and re-examination on February 1, 1949, revealed thefacts and impressions which were recorded as follows:

This girl's general appearance presented a verystriking improvement on the pre-operative state.Previously, she had looked years older than her age,and was particularly dowdy and careless about her dress.This morning she was smartly dressed and made-upand appeared to have developed an interest in herappearance. One also gained the impression that shehad a certain poise which previously had been totallylacking. In conversation she stated that she had muchmore confidence in herself than before the operation.Whereas formerly she had tended to avoid company,she now sought out her fellow beings, whose companyshe enjoyed and with whom she felt more comfortable

^- than previously. She also states that she has developeda lively interest in entertainment, such as the cinema,and feels that her memory and appreciation of currentevents has much improved. She has taken to readingthe newspaper and to general reading for the pleasureof the occupation alone, and is certain she would now

ive pictures. be able to learn more easily than formerly. She statesI. that in carrying out occupational therapy she is usingbn. the right hand and arm more freely than before, and is

256




SURGICAL TREA4TMENT OF INFANTILE HEMIPLEGIA

FIG. 23

FIG. 24

FIG. 23 (Case 6. Baby S.).-Air studies antero-posteriorand right lateral.

FIG. 24 (Case 6. Baby S.).-Exposed cortex at operation.

FIG. 25 (Case 6. Baby S.).-EEG tracings at operation.

FIG. 25

257




ROWLAND A. KRYNAUW

-enjoying the work given to her. There have been no-seizures of any kind since the operation.

" Neurologically, she had anosmia on the left side,*with a right-sided homonymous hemianopia with-sparing of the macula. Ocular movements were fulland without defect, and there was no facial weaknessto be noted. On the motor side there has been a very-dramatic alteration in the tone of the right upperlimb. In fact, once her confidence is gained, there isno spasticity to be noted at all, although she still tendsto carry the limb flexed at the elbow and adducted atthe shoulder. One gains the impression that tone is*entirely normal compared with the contralaterallimb. The fixed deformity at the wrist, resulting mainly-from former surgical intervention, complicates the-examination, but there is no evidence of spasticity inthe muscles acting on this joint. Strangely enough,she does not exhibit a spontaneity of volitional niove-ment one might expect with the general improvementin tone. Movements at the wrist, hand, and fingers,have shown least return to a normal state. There isstill accession of flexion at the interphalangeal joints.

FIG. 26 (Case 7. H.B.).-Photograph 3 months afteroperation.

There is no forced grasping. Testing of purposivemovements in the upper limb shows useful control overthe muscles acting on the proximal large joints. Thereis no gross dysmetria, and no tremor. There is nochange in the relative size of the limbs on the two sides.Gait is no longer awkward. Miss A. volunteers - theinformation that she has much greater power in thelower limb, and does not experience her earlier iendencyto fall. Tone is still somewhat increased in the rightlower limb which still exhibits slight clonus. Objectivelytested, power in the lower limbs is good; dorsi-iexionat the ankle approximates nearly to that on the normalside."In the sensory sphere, there is a lack of ability to

localize pin prick in both the affected limbs. It isinteresting to note, that whereas she tends to localizepin prick more distally than its actual position, shetends to localize touch with cotton wool more proximally.Vibration sense is still grossly impaired in both affectedlimbs. Spatial orientation of the affected limb is stillpoor. She has great difficulty in placing the normallimb in a position comparable with that of the affectedlimb. The tendon jerks are present and equal in theupper limbs, but in the lower limbs they are briskeron the right side than on the left. It was impossible tostudy the plantar reflex in this case by the usual methodbecause of the extreme ticklishness of the patient. Itwas interesting to note that the Rossolimo, Mendel-Bechterew, and Hoffmann signs appeared to be negative,but the Oppenheim gave an upgoing toe on the rightside."EEG studies six months after operation showed

responses to be within normal limits in the remaininghemisphere, with no activity on the hemispherectomizedside (Fig. 21).

Miss A. recently sent me a snap of herself, takensix months after the operation, and this is reproducedin Fig. 22 with a picture taken at the time of theoperation.

Case Comment.-The last news I had concerningthis patient was in September, 1949, when heruncle, an eminent physician of Capetown, told usat a meeting of the Medical Congress that theresults in this case were " nothing short of miracu-lous ". There had been no further fits or minorepisodes. Her disposition had become happy andcongenial, and above all she was able to hold downa job in an office.

Case 6 (Serial No. 3565, J. S., Male).-This child,aged 2 years 3 months, was first seen by me on May 15,1948. The confinement had been a short one, andthere was no evidence of birth injury. On the thirteenthday after birth the parents noticed that there was some-thing wrong with the left hand, and at one monththey noticed that the left leg was stiff, held straight,and that the child would not kick with it. Fits startedabout the sixth month and have persisted, occurringsome six to eight times a day. The parents were notable to give us any information of localizing value

I

258





FIG. 27 (Case 7. H.B.).-Ventriculogram.

concerning these convulsions. The child had notattempted to sit up, crawl, or talk.On examination, the fortanelles were found to be

closed; there was a suggestion of a left visual fielddefect to menace. The left limbs were shorter than theright and the left armtended to be spastic,the limb held in flexionwith the fingers closed.Power and movementin the left leg was notedto be deficient, butrelatively better thanin the left arm. Theleft plantar responsewas strongly extensor,but on the right sidethe response was doubt-ful. The child madeno attempt to sit upor crawl.

In September, 1948,the child was admitted,and during the pre-operative period inhospital, which lastedsome six weeks, he lay inbed screaming most of

c

the day, and throwing the right arm and leg around in arepetitive circular type of movement, which would go onfor hours on end. There were feeble attempts at movingthe left arm. Ventriculography was carried out andrevealed a considerable dilatation of the right ventricle(Fig. 23).

Operation (October 22, 1948. Hemispherectomy).-Considerable malformation, in the nature of a poren-cephaly with an adjacent paraventricular cyst, wasfound in the right hemisphere. The thinned-out brainsubstance will be seen underlying the EEG markersNos. 7, 8, and 9 in the operation photograph (Fig. 24).Fig. 25 shows the EEG tracings recorded from themarker positions indicated in the previous figure. Theflat records from the positions 7, 8, and 9 overlying thecystic malformations are interesting, but do not needexplanation.

Routine hemispherectomy was carried out. Thechild's condition gave no cause for alarm during oper-ation. On returning to the ward he became veryrestless, and then suddenly collapsed and died.

Case 7 (Serial No. 3615. H.B., Male).-This child,aged 9 years 5 months, was admitted to the neuro-surgical department of the Johannesburg Hospital onFebruary 7, 1949. His mother was distresseJ becausethe right arm and leg were weak and spastic, and becausehe was "very backward mentally ".' He was thesurvivor of twins delivered by Caesarian section. Hehad never used the right limbs as well as the left, and ashe grew older the shortening and clumsiness of the rightarm and leg became more obvious. No fits or minorepisodes occurred at any time, but during the past threeto four years he had been having increasingly frequentbouts of violent tempers. These would last from a fewminutes up to half an hour, and during these bouts hewould salivate profusely, scream, kick, and attackany one near him. Mentally he was very retarded, andin this respect he was becoming progressively worse.

f-.-',---'- ----, . - - -

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FIG. 28 (Case 7. H.B.).-Pre-operative EEG tracings.

259




ROWLAND A. KRYNAUW

FIG. 29 (Case 7. H.B.).-Cortex ex-posed at operation.

FIG. 29

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FIG. 30 (Case 7. H. B.).-Electrocorticograms.

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Two years previously he could do simple additions, buthad lost this facility, and his ability to read had alsodeteriorated. He had become restless, and his span ofattention was poor. Of particular interest here was thefact that two and a half years before admission tohospital he was considered a fairly bright child, but nowhe appeared to have forgotten all that he had previouslylearnt. Apart from a limited vocabulary there was nodisturbance of language function, and he was left-handed.On examination, the cranial nerves showed no abnor-

malities worth noting, and the visual fields were full toconfrontation tests. He had a right spastic hemiplegiawith contracture deformities, and shortening of theaffected limb. These points are well brought out inthe accompanying photographs (Fig. 26).On the sensory side pin prick, cotton wool, and

vibration were normally appreciated throughout. Hispostural sensibility was somewhat diminished in thefingers of the right hand. Tendon jerks were all briskeron the right, the right abdominal reflexes were absent,and the right plantar was of the extensor type.

Ventriculography showed considerable dilatation ofthe body of the left ventricle, but the left temporal horndid not fill. Some dilatation of the right ventricle wasalso noted (Fig. 27).EEG records were taken and are shown in Fig. 28.Operation (March 14, 1949. Hemispherectomy).

At operation the left hemisphere was exposed, and alarge circular surfacing " watery cyst ", 4 cm. in dia-meter, was encountered in the lower Rolandic line, andextending into the temporal lobe and down to the wallof the ventricle. The cyst can be clearly seen in thephotographl (Fig. 29) underlying the marker No. 2.

Electrocorticograms were then recorded and arereproduced in Fig. 30.

Thyratron stimulation of the cortex was then carriedout, but no responses were obtained. The reason forthis became clear later in the operation when the regionof the internal capsule was found to be replaced by amultilocular cystic formation, surrounded by leatherygliosis. I would like to draw attention to the fact thatthis patient had never had convulsive episodes, manifestin the motor somatic sphere, but that he had hadnumerous emotional outbursts which might be regardedas epileptic equivalents.

Case Comment.-During the past nine monthsthis boy has made satisfactory progress, apartfrom the fact that the function of the right armand leg is of the same order as before the operation.There have been no further emotional outbursts.He has become a quiet and thoughtful boy andapplies himself diligently to tasks and amusements.

Case 8 (Serial No. 3859. R.P., Female).-Thischild, aged 2 years 9 months, was admitted to theJohannesburg Hospital in February, 1949. She hadbeen perfectly healthy until the age of 10 months, wascrawling well, and could say " Mama ", " Dada ". Atthis time she was suddenly taken ill with convulsionsand acute otitis media. Penicillin was given and aparacentesis performed. About 36 hours after this

the right arm and leg were found to be paralysed. Aftersome months power on the right side gradually returned,but the limbs remained very weak and spastic. Somemonths after the illness she started having convulsiveepisodes, starting on the right side, and these increased infrequency, and, at the time of admission, she was havingseveral episodes each day despite sedation. She hadbeen seen by the late Dr. George Riddoch, who diagnoseda venous thrombosis and offered a very poor prognosis.On examination, she was found to be extremely

restless, and responded to attempts at examination byviolent outbursts of screaming, kicking, and fighting.Her speech had shown but little development duringthe two years since her illness, and although the parentshad worked very hard at this aspect of her educationshe could only use some five or six simple isolated words.She was starting to walk, but clumsily, and with assistance.Detailed examination of the cranial nerves was impos-sible, but she did appear to have a field defect on theright side, and there was some right lower facial weak-ness. The right arm and leg were less well developedthan on the left; they were spastic and the movements ofthe right side were extremely clumsy. Ventriculographyrevealed a gross generalized dilatation of the left lateralventricle (Fig. 31).

Operation (March 18, 1949. Hemisphrectomy).-The left hemisphere was exposed. No gross focalpathology was seen, but the whole hemisphere appearedsomewhat shrunken and the gyri small. Needling of thehemisphere revealed a general toughness of the wholestructures (Fig. 32).

Electrocorticograms were recorded and the tracing isreproduced in Fig. 33.The cortex was stimulated with a view to mapping

out the motor cortex, but no responses were obtained.The post-operative course was uneventful and within

a few days the movements of the right arm and leg hadreturned to the pre-operative level. Of particularinterest was the fact that when I visited her the dayafter operation and asked her where her mummy was,she responded by saying, " Mummy outside". Fromthat time on she started learning new words rapidly,and also started putting them together to constructsentences.

Case Comnient.-The child wa, discharged fromhospital a month after operation, and I have notseen her since, but a letter fron her mother sevenmonths after operation gives the following informa-tion:

"First let me say that her mental improvement isremarkable. She has a normal vocabulary for a childof her age, if indeed it does not exceed normal, andher pronunciation is perfect. She has an extremelysharp perception of all that goes on around her.She runs about a lot, the movement of her leg havinggreatly improved. She takes much more notice ofher right hand, and it is gaining strength and usefulnessfrom the many movements she attempts throughoutthe day."

Case 9 (Serial No. 4158, D.S., Female).-This girl,aged 15 years, was admitted to hospital on May 22,

261




ROWLAND A. KRYNAUW

,FF 2 (

FIG. 32 (Case 8. R.P.).-Cortex exposed at operation.

FIG. 32

FIG 31

FIG. 31 (Case 8. R.P.).-Ventriculogram.

FIG. 33 (Case 8. R.P.).-Electrocorticograms.

FIG. 33

I'

w4,

262





1949. There was a story of difficult birth, but mentaland physical development were regarded as beingentirely normal up to the age of 4 - years. At 4, yearsof age she became very ill, and was admitted to theChildrens' Hospital with a diagnosis of encephalitis.She remained in hospital for six months, and duringthis time weakness and clumsiness of the right handwere first noticed. Soon after leaving hospital shestarted having " blank spells" which were diagnosed as

petit mal by a senior piediatric consultant. The natureof the episodes changed during the next year or two.They were still of a relatively minor character, but thehead and eyes would turn to the left and both arms

would be drawn up and out to the right. During the 18months before she was admitted under my care shedeveloped in addition to these attacks, generalizedconvulsive episodes. In all she was having three toseven major and minor episodes each day, despitedetermined sedative measures. This child h2d a quiet,placid temperament, and was not prone to emotionalupsets. Her school record had been quite good andabove average for her age. Of late she had become lessinterested in things and people, but not more so thanone would expect in a child who was having numerousfits, combined with a generous presentation of sedativedrugs.On examination she was seen to be a well-built girl

tending to adiposity. The menarche was at 13 years,and her periods were regular. She was a good witnessand showed good insight. With regard to the cranialnerves she was found to have generalized constrictionfor all isoptres in the right homonomous field, and avery slight right lower facial weakness for emotionalmovements.The right arm was less well developed than the left

and movements, especially distally, were weak andspastic, while purposive movements showed an athetoidclumsiness. There was little fault to be found with theright lower limb except that the tendon jerks were

comparatively slightly increased and the right plantarresponse was regarded as equivocal.The examination of sensory functions revealed a

normal appreciation and localization of pin prick andlight touch, but slight loss of all the cortical sensorymodalities. Electroencephalography revealed a per-sistent slow wave focus from the left frontal region withsuggestion of wave and spike patterns (Fig. 34).

Air encephalography showed the left ventricle to bedilated, but mainly in its posterior half and temporalhorn.

Operation (June 15, 1949. Hemispherectomy).-Thecortex was exposed in the usual manner, and in generalappearance seemed quite normal (Fig. 35).

Electrocorticograms were recorded and are shownin Fig. 36.

In view of these, and because of the ventriculardistortion, hemispherectomy was carried out. Threedays later, because of haemorrhage into the cavity, theflap had to be re-elevated and the clot dealt with.Thereafter her post-operative course was uneventful,except that movements of the right arm and leg wereslow in returning. This raises what I consider to be an

important point regarding the function of the caudatenucleus, to which reference will be made later.

Case Comment.-It is now seven months sincethis patient was operated on. She is bright andcheerful and mentally alert, and looking forwardto returning to school in a few weeks' time. Move-ments of the right side are improving satisfactorilybut are not yet up to the pre-operation level. Thisapplies particularly to the right lower limb. Therehave not been any fits or suggestive episodes sinceoperation.

Case 10 (Serial No. 2453, P.T., Male).-This boy,aged 14 years, was referred to the Neurosurgical Depart-ment on April 11, 1948. He is an only child. Birthwas difficult and instruments were used. When he was6 months of age the parents first noticed that the leftarm and leg were weak. Mentally his development wassatisfactory, but walking was delayed until he was over2 years of age. The left arm seemed to become pro-gressively more clumsy, and lagged behind in generaldevelopment and growth. At about 21s years of age hestarted having " funny turns ", in which he went verypale and giddy. At 8 years of age he was operated onby an orthopsedic surgeon for the contracture deformityof the left foot. By this time he was having frequentepisodes in which he complained of" funny little shozks "in the left hand which were associated with a drawing upof the left hand and arm, but without I )ss of conscious-ness. The left arm became gradually more spastic,deformed and useless. More recently he had becomeaggressive and bad tempered, and terrified of impendingattacks. Sedative medication failed to control theepisodes.On examination the cranial nerves showed no devia-

tions worth tecording. The left arm and leg were not sowell developed as the right, being both shorter andthinner. The left arm was flexed and adducted, thegrip on the left was poor, and he was unable to extendthe wrist or fingers. The tendon jerks on the left sidewere all increased by comparison with the right, and theplantar response was of the extensor type. In thesensory sphere he showed defective postural sensibility,and impaired two point discrimination. Pin prick andcotton wool were normally appreciated, but localizationof these modalities was not accurate in the left upperlimb. Ventriculography was carried out. The brainfelt leathery to the advancing brain needle, and t'ie airpictures showed slight dilatation in the region of thevestibule of the right ventricle. Electroencephalographyrevealed a generalized dysrhythmia with a focus of highamplitude three to four per second discharge from theright temporo-parietal zone.

First Operation (May 14, 1948).-A right lateralosteoplastic flap was turned and revealed a zone ofmicrogyral formation occu,ying a considerable part ofthe right parietal lobe and extending forwards to theRolandic fissure. The motor cortex was mapped out bystimulation. Block excision to a depth of 3 cm. of theparietal lobe, to include the microgyral formations andextending forward to incluJe the motor arm area, was

263




ROWLAND A. KRYNAUW

,k " ,

FIG. 34

FIG. 35

FIG. 34 (Case 9. D.S.).-Pre-operative EEG tracings.FIG. 35 (Case 9. D.S.).-Cortex exposed at operation.

FIG. 36 (Case 9. D.S.).-Electrocorticograms.

FIG. 36

264

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carried out. He was discharged from hospital threeweeks later. Movement on the left side showed someimprovement on the pre-operative level.

C(ase Comnent.-For three months the boy wasperfectly well and then the attacks started again,but were somewhat modified. There was nosensory component. but the head and eyes wouldbe pulled over to the left and he would then haveclonic twitching in the left arm. The left leg wasnot involved. These attacks were of particularinterest to us because the motor arm area had beenremoved at operation. He was re-admitted inOctober, 1949, because of the attacks being asfrequent as before operation, and because theydid not respond to aggressive sedative measures.In view of my previous experience with limitedcortical excisions I decided to remove the entirehemisphere.Second Operationi (November 1, 1949: Hemispherec-

*omy).-His post-operative course was uneventful andhe was up and about three weeks after operation, withmovements quite up to the pre-operative level. After afurther week he was allowed home. His progress willbe carefully followed.

Case 11 (Serial No. 120, E.G., Female).-This child,aged 13, was first seen by me in June, 1943, when shewas 7 years of age. She exhibited a right hemi-plegia of the infantile type, was having fits starting inthe right arm, and also numerous petit mal episodes.Mentally she was very retarded and was difficult tohandle. She was put in an institution where she remainedfor the next six years, until she was admitted to theNeurosurgical Department. Her mental age wasassessed at 5 years. Air encephalography revealedsome dilatation of the left ventricle (Fig. 37).EEG studies were carried out and are shown in Fig. 38.Operationt (December 14, 1949: Hemispherectomy).-

The cortex was exposed in the usual manner. Therewas no evidence of microgyral formation or grosspathology, apart from the fact that the sub-arachnoidspace was very wide, and contained large, loopingarteries, which looked like small pulsating worms, overthe entire extent of the cortex. No motor responseswere obtained when the cortex was stimulated. Laterduring the operation the region of the internal capsulewas found to be the site of a firm leathery gliosis. Itis now only a few weeks since operation and too early tomake any comment regarding her progress.

Case 12 (Serial No. 4557, M.B., Female).-The child,aged 7 months, was admitted on December 22, 1949.On November 21, 1949, she had fallen from a bed ontothe floor. One hour later she became unconscious andremained so for two days. It was then noticed that theright arm and leg were paralysed. On November 29the surgeon, to whom the case had been referred,trephined the left side of the skull and evacuated a largeextradural clot through an opening 1 in. in diameter.The paralysis improved remarkably for a couple of

weeks. The right arm and leg then became weak again,and the region of the trephine opening bulged, andfinally broke down discharging a serosanguinous fluid ina pulsatile manner. I decided to turn a flap and dealwith any remaining extradural collection, and at thesame time expose and inspect the cortex.

Operation (December 28, 1949: Hemispherectomy).-The anaesthetic used was 7 c. cm. syrup of chloral, andlocal 1% novocaine.A left lateral flap was turned and a large organizing

tarry extradural blood clot was found and dealt with.This clot was found to be overlying the entire hemi-sphere posterior to the Rolandic line. On opening thedura the posterior half of the hemisphere behind Rolandowas found to be pale, oedematous, and with convolu-tional widening and a generally necrotic appearance.Needling revealed a multilocular cystic formation, thecystic fluid being of a milky appearance. The cystswere not communicating with the ventricle. I startedby amputating the posterior half of the hemisphere, buton encountering a gross generalized dilatation of theentire left ventricle, I proceeded to carry out a completehemispherectomy as defined in this report.

Case Comment.-This baby is making excellentpost-operative progress. Movements on the rightside were present immediately after the hemispherewas removed, but by the next morning there wasvery little movement and the right arm and legwere rather spastic. On the fifth post-operativeday power in the arm and leg started to return,and now two weeks later are regarded as good ason the opposite side, although the patient definitelyfavours the left side. She is an extremely bright.observant, and happy child. Her subsequentprogress will be followed with the greatest interest.

DiscussionIn the foregoing protocols I have tried to confine

myself to the more positive aspects of each case.No mention is made of C.S.F. pressures andanalysis, Wassermann reactions, or blood counts,because no significant changes in these were noted.The number of cases reported here is too few, andin most cases the interval since operation too short,to be dogmatic about results, but one can at leastsay that these are promising.

Epilepsy, either focal or generalized, was presentin 10 out of 12 cases, and in all these the epilepticmanifestations have ceased after operation withoutsedative medication. Disorders of behaviour andpersonality are a marked feature of this group ofcases, and the profound betterment in respect ofmentality in all cases exceeds our best expectationis.It should be pointed out that this improvement inthe mental sphere, presupposes, and is dependentupon, the integrity of the remaining hemisphereand its ability to function normally once it has been

265




ROWLAND A. KRYNAUW

Fio. 37 (Case I1. E.G.).Ventriculograms.

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FIG. 38 (Case 11. E.G.).-Pre-operativeEEG.

FIG. 38

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266





released from abnormal influences from the patho-logical side.Although return of function and power to the

pre-operative level was predicted and expected inthese cases, improved tone and function beyondthis level has been a happy by-product of theoperation, and is perhaps a Jittle difficult to explain.If the caudate nucleus is removed with the hemi-sphere there is little or no return of voluntaryand useful motor activity. This point is well broughtout by a study of the earlier cases of hemispherec-tomy as carried out by Dandy, Gardner, and othersin cases of brain tumour. What of the lentiformcomplex, putamen, and globus pallidus? Theresults indicate that this should be included in theremoval, a hypothesis supported by a carefulconsideration of anatomical pathways, and thefrequency with which we find associated pathologyin the region of the lentiform complex in these casesof infantile hemiplegia. It would appear reasonableto assume that the ipsilateral hemisphere has somecontrol over the volitional act, but that this controlis via the caudate nucleus of the contralateral side.On the sensory side we have found that localiza-

tion of pin prick returns after a few months withonly a minimal defect of accuracy. Joint sensehas also returned with the lapse of time in at leasttwo cases. It is accurate at the larger joints, shoulder,elbow, hip and knee, and in one case accurate atthe wrist, and again in one case accurate for grossmovements of the fingers. Two point discriminationwith an increased threshold-about three times thenormal-has been present in two of the casesexamined.With regard to the language function, I suppose

one might argue that hemisphere dominance hadadjusted itself before removal of the affected hemi-

sphere, and that it was the minor hemispherewhich was removed in all cases. Such an argumentwould be comfortable, and in conformity withaccepted principles. Nevertheless, careful study ofsome of the cases reported above, particularlyCase No. 8, makes one wonder whether such aneasy acceptance of the position is not causing u;to overlook some of the most interesting andimportant facts which may emerge from this work.

It is, I think, worth noting that apart from theloss of cortical sensory modalities referred to above,there has been an entire absence of those changes,such as spatial disorientation and disturbance ofbody image, which we have come to associatewith disturbances of the parietal cortex.

SummaryHemispherectomy has been carried out in 12*

cases of infantile hemiplegia. There has been onedeath in the series.

Epilepsy and mental changes, either singly or incombination, are regarded as indications for theoperation.

Post-operative return of motor power withlessening of the spasticity and clumsiness has beena feature in these cases.Marked improvement in personality, behaviour,

and mentality has been noted in all cases.

I should like to take this opportunity of thankingMr. K. Lewer Allen, my first assistant, Dr. I. Siff, myanaesthetist, and all the other members of the medicaland nursing staff for their unfailing enthusiasm andtireless attention to the patients whose welfare formsthe theme of this communication.

*Since going to press eight more cases have been operated uponand are making satisfactory progress.

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CEREBRAL HEMISPHERETREATED BY REMOVING ONE INFANTILE HEMIPLEGIA

Rowland A. Krynauw

doi: 10.1136/jnnp.13.4.2431950 13: 243-267 J Neurol Neurosurg Psychiatry

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INFANTILE HEMIPLEGIA TREATED BY REMOVING ONE