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Hemispherectomy in children
Medical Progress H E M I S P H E R E C T O M Y IN C H I L D R E N ARTHUR !1r I~APLAN, PH.D. ST. Lores, MO. of hemispherectomy in children is yet to be done. A start has been made in that a number of studies have begun to utilize objective and standardized measures wherever possible. I t is felt that while additional data need to be amassed and suff• experimental controls utilized, there is sufficient data available at this time to enable the pediatrician to answer some of the questions posed by parents.
INTRODUCTION
OTH the pediatric practitioner and the neurosurgeon are frequently confronted by parents who raise very practical, important, and difficult questions. To the parent who must make a decision regarding such drastic surgery as a hemispherectomy, consequences are of p r i m a r y importance. As u n h a p p y as parents are with a child's hemiplcgia, endless convulsions, and incorrigible behavior, the thought of surgery evokes certain anxieties and fears which must be dealt with by the pediatrician a n d / o r neurosurgeon. The probability of surgical survival is always uppermost in the minds of parents whose children undergo such major surgery. The next question is usually, " W h a t effect will such surgery have on my child's behavior ? " The question of surgical survival can be answered objectively in terms of probability based upon statistical data. The question regarding consequences of surgery has until recently been answered within the subjective, personal, and anecdotal realm. A definitive study dealing with the consequences
B
THE APPLICATION OF HEMISPHERECTOMY TO INFANTILE HEMIPLEGIA IIemispherectomy is a relatively new procedure. The first cerebral hemispherectomy was done by Dandy, in 1923, at the Johns Hopkins Hospital, and this case first appeared in the literature in 1928~--just 30 years ago. Since that time, numerous case reports have appeared. Mensh and his associates ~ listed 32 publications between 1928 and 1951. These represented 41 hemispherectomies. While a preponderance of this surgery was applied to the adult suffering from infiltrating tumors of the subdominant hemisphere, 1-12 hemispherectomy has of late been utilized and applied to a specific g r o u p - - t h e infantile hemiplegic. 13-26
F r o m t h e D i v i s i o n of M e d i c a l P s y c h o l o g y , Department of P s y c h i a t r y and Neurology, W a s h i n g t o n U n i v e r s i t y S c h o o l of :Medicine. 476
MEDICAL PROGRESS
F o r d 27 classifies this condition as one of either acute infantile hemiplegia of obscure etiology or congenital hemiplegia. Regarding the acute type, F o r d states : I n the e a r l y s t a g e s of t h e illness t h e affected h e m i s p h e r e m a y be so swollen t h a t ventrieulographie study may suggest brain tumor. L a t e r , of course, a t r o p h y becomes evident. Later the hemiplegia extremities become s p a s t i c a n d t h e u s u a l d e f o r m i t i e s develop. T h e a f f e c t e d l i m b s do n o t develop as well as t h e n o r m a l l i m b s a n d y e a r s l a t e r m a y be very s m a l l a n d distorted. T r e m o r or athetosis may appear. I n other cases, t h e f o c a l s i g n s m a y l a r g e l y or c o m p l e t e l y disappear. U n f o r t u n a t e l y , in a l m o s t h a l f of all cases convulsive seizures r e c u r f r o m t i m e to time.
The prognosis as presented by F o r d is one of low m o r t a l i t y and is unfavorable in outlook for recovery. E p i l e p s y is said to follow in about half of all cases, and mental retardation is to be expected. This type of hemiplegia is said to result f r o m a single gross vascular lesion of the brain. The congenital f o r m of hemiplegia is said to result from a birth injury. I n t r a u t e r i n e i n j u r y and disease are other explanations offered. F o r d describes the clinical features of the congenital t y p e as follows: F l a c c i d i t y of the p a r a l y z e d l i m b s is relatively more f r e q u e n t in c o n g e n i t a l hemiplegias t h a n a m o n g those o c c u r r i n g l a t e r in life. I n such cases, we find s t r i k i n g u n d e r development of t h e h a n d a n d less evident u n d e r d e v e l o p m e n t of the a r m a n d leg. T h e h a n d is of childish a p p e a r a n c e , b u t it is n o t in a n y w a y d e f o r m e d . D u e to t h e shorteni n g of t h e leg t h e pelvis m a y be tilted a n d t h e spine m a y show a p o s t u r a l curve. T h e t e n d o n reflexes a r e u s u a l l y s o m e w h a t increased on t h e affected side, b u t t h i s is never so s t r i k i n g as in s p a s t i c p a r a l y s i s .
477
I n other cases, the p a r a l y t i c l i m b s are very s p a s t i c since t h e muscle s p a s m develops w h e n the bones a r e plastic, e x t r e m e d e f o r m ity of t h e h a n d is produced. I n a l m o s t all cases, t h e r e is m e n t a l def i c i e n c y - c o n v u l s i v e seizures occur in Mmost 40 or 50 per cent of all cases. E v e n severe h e m i p l e g i a s of c o n g e n i t a l origin m a y not be a p p a r e n t u n t i l t h e child is several m o n t h s of age.
The prognosis is said to depend upon the amount of i n j u r y to the nervous system. " L i t t l e i m p r o v e m e n t is to be expected. The presence of convulsions makes the outlook unfavorable, for deterioration often occurs in such eases." I t would certainly seem f r o m the above that the prognosis of infantile hemiplegia accompanied by convulsive seizures is indeed gloomy. I t is with this in mind t h a t surgical intervention occurred. K r y n a u w is worked primarily with children and carried out twelve hemisphereetomies over a fiveyear period. I n all cases, hemiplegia per se was not a sufficient condition for surgery. Convulsive seizures and behavioral changes (uncontrolled emotional outbursts) were also evident. Thus, a triad of symptoms were considered before hemisphereetomy was undertaken--hemiplegia, convulsive seizures, and the presence of lowered intellectual functioning a n d / o r uncontrolled emotional outbursts as behavioral manifestations. Occasionally, surgery was u n d e r t a k e n in the absence of seizures or in the absence of emotional outbursts while the other symptoms were present. Thus, it was only a little over seven years ago that a n y specific formulation developed in the application of henfispherectomy to children.
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T H E J O U R N A L OF P E D I A T R I C S
FINDINGS
A survey of the American and British literature pertaining to hemispherectomies in children (ages from birth to 16 years) yielded 29 cases. Table I summarizes the data reported by each investigator. I t is interesting to note that investigators differ in the amount and kind of information reported. F o r example, one investL gator ~9 relied on parental report and subjective evaluation of change in intellectual functioning. Another investigator 25 focused on the social adjustment of the child and reported no TABLE I .
EFFECTS OF CEREBRAL HEMISPHERECTOMIES
HEIViI ~ S AGE P H E R EACT-
AUTHOR
Krynauw
(1950)
IIENIISPHERE
TOMY
SEX
9 7 3 ]0 2 9
F F I~ M M N[
L L L L R L
2 15
F F
L L
14 I3
M
R
F
L L
7 too.
F F
4 II 4 4
M l~ M M
E L R L
13
M
R
12 4 8 9
3/[ F M F
Fleischacker (1954)
12 ].3 12
Ueeker (1954)
McK:issoek (1953)
Munz & Tolor (1955)
IN
CIrIILDREN
II~TELLECCONVULSIVE
REMOVED
7
Cairns (1951)
data regarding the particular hemisphere removed, the effect on the child's seizures, and hemiplegia. Still another investigator 2~ concluded that intellectual functioning increases following hemispherectomy, yet gave no specific data to support his conclusions. While systematic and quantitative measures arc at times lacking and like the blind men, each investigator touches a different part of the huge elephant, the impressions and findings that are reported do seem to indicate specific trends. Tables I I - V I I I present these trends.
SEIZURES
HEMIPLEGIA T U A L F U N G TIONING
Gone Gone Gone Gone
Improved No change Improved Improved Died None before No change surgery Not reported Improved Gone No change
Improved Improved Improved Improved
EMOTIONAL
OUTBURSTS
Goue Improved Improved Improved
Not reported Gone Improved Improved
Improved None before surgery
Not reported Not reported Not reported Improved Not reported Not reported Improved
Improved
Improved
Improved
L L L R
Gone Improved Gone Gone Gone Gone Improved Gone Gone Gone
No change Worse Improved No change No change No change Worse No change No change No change
Not Not Not Not Not Not Not Not Not Not
Gone Gone Gone Gone Gone Gone No change Gone Gone Gone
M M 3/[
L R L
Improved Gone Improved
Worse Improved No change
Not reported Improved Not reported Improved Not reported No change
13 13 15
F M M
? ?
Not reported Not reported No change Improved Not reported No change Gone Not reported No change
Improved Improved Not reported
14
F
L
Gone
Gone
No change
reported reported reported reported reported reported reported reported reported reported
No change
479
MEDICAL PROGRESS
Of the 29 hemispherectomized children, one or 3 per cent died. This occurred to a 2-year-old male and is reported b y K r y n a u w . 19 His condition is said to have given no cause for alarm d u r i n g surgery. He became v e r y restless while r e t u r n i n g to the w a r d and suddenly collapsed and died. Thus, the m o r t a l i t y rate as a function of surgery is seemingly low. TABLE I I . AGE ])ISTRIBUTION OF ALL CASES REPORTED IN THE LITEBATURE
AdE DISTRIBUTION < 1 2 3 4 5 6 7 8 9 10 11 ]2 ]3 14 15 16 No
year years years years years years years years years years years years years years years years age reported
I
N 1 2 1 4 0 0 1 1 3 1 1 3 5 2 2 0 1
I
% 4 7 4 14 0 0 4 4 10 4 4 10 17 7 7 0 4
Table I I indicates t h a t the eases reported in the literature are d r a w n f r o m all age groups except years 5 and 6. W h e n the sample is categorized into preschool (ages f r o m birth to 6 years), middle childhood (ages 7 to 12 years) and adolescence (ages 13 to 16 years) we find little difference between the n u m b e r included in each developmental category. F o r 27 reported ages, 30 per cent come f r o m the preschool group, 37 per cent f r o m the middle childhood group, and 33 per cent f r o m the early to middle adolescent period. According to Tables I I I and IV, boys underwent hemispherectomy more often t h a n girls, and the left hemi-
sphere was r e p o r t e d l y removed more frequently than the right one. The sex differences m a y not necessarily be significant since these differences are relatively small. There does, however, a p p e a r to be a greater proclivity for the left hemisphere to be removed. More t h a n half of the cases consisted of the removal of the ]eft hemisphere. Of the 26 cases in which the p a r t i c u l a r hemisphere removed is indicated, 19 or 65 per cent r e f e r r e d to the removal of the left hemisphere. Ten per cent failed to indicate which hemisphere was removed. TABLE I I I . SEX DISTRIBUTION OF ALL CASES REPORTED IN THE LITERATURE
1
N I
Boys Girls TABLE :IV.
59 41
~IEMISPHERE RE1V[OVED
I
(% oF REPORTED
I-IE]~KISPttERE REIV[0VED
N
Left Right N o t reported
17 9 3
TABLE
V.
%
17 12
THE
CASES) N ~ 26
%. 58 32 10
65 35
STATUS OF
CONVULSIVE
SEIZURES FOLL0WIN~ SURgEry (% OE STATUS OF CONVULSIVE SEIZURES Completely gone Improved l~lo change Absent before surgery No d a t a reported Expired
N
%
15 5 0
52.0 17.0 0.0
1 7 1
3.5 24.0 3.5
I~EPORTED CASES) N ~ 20 75 25
Table V indicates that a m a j o r consequence of hemispherectomy is the absence or reduction of convulsions. While close to one fourth of the reported cases did not deal with the preand poststatus of convulsions, of the
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THE JOURNAL OF PEDIATRICS
20 eases which did deal with this variable, 75 per cent no longer experience convulsions, and 25 per cent are improved in t h a t the frequency a n d / o r intensity of the convulsions have diminished. This is one v e r y positive finding. Of the three presenting complaints usually considered as the criteria for hemispherectonly, one m a y v e r y well be able to predict t h a t convulsions will become extinct or at least become g r e a t l y reduced in severity in all or at least in the m a j o r i t y of eases. A second criteria for hemispherectomy, hemiplegia, is more difficult to assess. Investigators focus o n different aspects and with more or less specificity. W h e n the findings are categorized in a general w a y according to improved, no change in status, or condition worse folIowing hemisphereetomy, the results are less f o r t u n a t e t h a n is the ease with convulsions. Table V I indicates that of the 23 TABLE u
THE STATUS OF THE I-IE~IPLEGIA FOLLOWING SURGERY
STATUS OF HE~IPLEGIA Improved No change Condition worse No data reported Expired
% 8 12 3 5 1 29
28 41 10 17 4
( % OF REPORTED CASES) N ~ 23 35 52 13
cases in which this variable is considered, 35 per cent showed some form of improvement. This varied behaviora]ly f r o m improved balance to decreased spasticity of the affected limbs. F i f t y - t w o per cent did not show a n y change, and 13 per cent becanle worse. The hemiplegia became worse in two cases reported by Me-
Kissock. 2a He states, " [Cases Nos, 2 and 7] suffered as a result of operation: both of these were errors of j u d g m e n t on m y part. One developed an increase in hemiplegia which has f o r t u n a t e l y been offset by disappearance of the severe behavior disorder, whilst the other has acquired an increased speech defect. This latter ease should have been subjected only to a local excision of abnormal parietal cortex r a t h e r than to ablation of the h e m i s p h e r e . " Thus, while occasionally one can expect worsening of the hemiplegia, in 87 per cent of the cases the patients were no worse off and some (35 per cent) improved. The t h i r d criteria, emotional outbursts, also diminish to a great extent. Table V I I indicates that of 24 cases in TABLE VIIi.
CONTIgOLOF EI~IOTIONAL OUTBURSTS
STATUS
N
%
Improved No change Worse None present p r i o r to surgery No d a t a reported Expired
21 3 0
72 10 0
1 3 1
4 10 4
( % OF REPORTED CASES) N--__24 87 13
which severe emotional outbursts are reported prior to surgery, 21 or 87 per cent show i m p r o v e m e n t in this area. Only 13 per cent of the reported eases indicate no change in behavior. I n no ease did this behavior become worse. I t would seem then that along with control of convulsions, one can anticipate effective control of emotionnl outbursts. Little systematic attention has been given to the actual measurement of
48l
MEDICAL PROGRESS
intellectual functions. While the consensus of opinion seems to be that intellectual functioning improves as a result of hemisphereetomy, few studies ~3, ==, ~ have actually reported objective measurements. Table V I I I indiTABLE
VIII. THE FUNCTIONING
STATUS OF INTELLECTUAL FOLLOWING SURGERY
t STATUS OF INTELLECTUAL
EUNCTIONING Improved No change Worse No data reported Expired
N 7 4 0
% 24 14 0
17 1
58 4
NUMBER BASED NUMBER UPON O~BASED ,JECTIVE UPON IM- 3gEASUaF,PaXSSlON MENTS 6 1 2 2 0 0
cates t h a t no data were reported with respect to intellectual functioning for 58 p e r cent of the cases. While close to one fourth report improvement, 86 p e r cent of these are based upon subjective evaluations. Of the 14 per cent who report no change in intellectual functioning, only 50 per cent are based upon subjective evaluat i o n s - t h e other 50 per cent being based upon objective measurements. Assessing this variable is extremely complex. No one can measure intelligence per se but only the functions of intel/igenee as manifested in behavior. W h a t is being measured is intellectual functioning and not native intelligence. The latter is i n f e r r e d ; the former is observed and ascertained through standardized and objective measures. The appraisal of intellectual functioning is f u r t h e r complicated b y the numerous factors which alter one's level of functioning'. Some o f the more i m p o r t a n t factors are: the ability to see and h e a r ; level of motivation including' the desire to co-
operate and do as well as one is able; and adequate control of impulses. Howells and K a y 2s state: I n our experience in testing eases of infantile hemiplegia before operation we have become aware of the difficulties involved in ascertaining reliable and stable indices of tile intellectual ability. In particular, we have noted that as well as the child's physical handicap in completing performance tests, his frequent distractibility, memory defects, short lapses of attentim b lack of confldence~ and perhaps Mso his often eonsideralfle gap in formal education, have lowered his test results to an undetermined extent.
In view of these m a n y variables, one needs to be cautioned against conclusions which infer t h a t native intelligence r a t h e r than intellectual functioning becomes or does not become elevated. K r y n a u w a~ indicates : Improvement in the mental sphere presupposes and is dependent upon~ the integrity of the remaining hemisphere and its ability to function normally once it has been released front abnormal iitfluences from the pathological side.
I t would seem then that once the pathology is removed and the motor "drivencss" of the individual restored to a more stable level, intellectual functioning as measured by standardized tests, is able to become more adequately maximized. DISCUSSION
A review such as this brings to the fore m a n y questions and problems for the researcher in this area. While investigators are idiosyncratic in their interests and necessarily focus on different aspects of the problem, there is a need for greater systematization in reporting clinical case accounts if a complete understanding of the hemisphereetomized patient is to be
482
T H E J O U R N A L OF P E D I A T R I C S
achieved. This discussion will consider the ways in which investigators can communicate and make their findings more meaningful to others as well as present suggestions for f u t u r e research. It would seem that all investigators should report their face sheet data, i.e., a patient's age, color, religion, socioeconomic level, etc. Furthermore, one would need to consider dominance, presenting symptoms, the specific time in the total course of study when certain observations are made, and the particular hemisphere removed. These data are available to all and should be reported if systematic comparisons are to be made between investigators. I f a follow-up study is done one would also need to indicate the sources from which particular data stem and the time factor in relation to the date of hemispherectomy. Thus, it is important to indicate whether the investigator is reporting his own observations based on specific tests or is reporting anecdotal material related by the patient's family: One would also need to indicate whether the observations are made while the patient still resides in the hospital or as an outpatient. There is also a tendency to speak collectively of drugs r a t h e r than to specify particu]ar drugs and dosage. I t is insufficient to indicate that a patient no longer has seizures following hemispherectomy. One also needs to spell out to what extent this is evident when the patient is maintained on anticonvulsive drugs and to what extent this is so when the patient is free of drugs. There are also investigators who describe ~in a general manner that a child has emotional outbursts, but there are no indications as to what
situations instigate the outbursts and just what the outbursts entail. Also, quantitative measurements should be utilized whenever possible. It was pointed out earlier that subjective evaluations arc made regarding intellectual functioning and also regarding the appraisal of social and emotional development and adjustment. This is u n f o r t u n a t e since there are tools available29, 3o which can quantitatively measure changes in specific intellectual functions such as general f u n d of information, comprehension, arithmetical reasoning, immediate recall, vocabulary, planning ability, perceptual skills, visual-motor coordination and integration, and ability to handle abstract ideas. I n like manner, there are scales which can measure social development, sl and projective techniques32, 3a, 34 which can measure specific personality variables such as hostility and aggressivity, ability to relate to others, needs and need systems, self-concept, etc. Thus, if one is to follow a pre- and postoperative research design, one must be very specific regarding the sample used, the time of observation, as well as the sources and tools of observation. Measurement per se is not invariably more useful than description. Descriptions have a very definite place; however, if descriptions are to be useful, they must elucidate v e r y clearly and specifically that which is being observed. I t is only in this way that one investigator can directly compare his :findings with that of another investigator. A great deal of emphasis has been placed in this discussion on tile importance of indicating tile time when particular observations are made. Research upon the hemispherectomized
MEDICAL PROGRESS
patient necessarily entails a longitudinal kind of research design. Unless the time factor is clearly specified, cross validation becomes especially difficult. One can certainly question the validity of the findings reported in this paper since the emphasis and interests of the investigators proved to be so varied. While this is true, there were nevertheless consistent findings even for so varied a group. Thus, epileptic seizures were greatly reduced in all of the studies regardless of the investigator. This was also true regarding emotional outbursts. While the findings reported here may be considered general, they nevertheless emphasize the need for more specific and systematized research. There are several ideas which come to mind at this time regarding suggestions for f u t u r e research. Since hemispherectomy has been extended to M1 age levels it would be interesting to investigate to what extent the prognosis differs from one age level to another when the presenting complaints are similar hemiplegia, intractable seizures, emotional outbursts, and mental retardation. When the present data were analyzed for three age levels (preschool, middle childhood, and adolescence), it was found that there were no differences in the consequences of hemisphereetomy in so far as status of seizures, status of hemiplegia, and status of emotional outbursts are concerned. These must be considered fairly tentative findings since tests of statistical significance could not be applied due to the m a n y zero cells and the relatively small number of cases. In like manner, consequences of sur-
483
gery can be systematically evaluated for sex differences, dominance, and the particular hemisphere removed. I t was reported that there was a greater tendency for the left hemisphere to be removed than the right. This finding in and of itself is meaningless but does raise questions which can be pursued if enough systematic data are available. To those who feel that hemis p h e r e e t o m y - g r e a t l y improved intellectual functioning, one could certainly study and evaluate the eases where this occurs and those where this does not occur. With the aid of objective measurements, one could even determine how much or how little change occurs. A study also needs to be done with regard to those cases whose hemiplegia improves and those who do not improve. Medicine has become a comprehensive field in that it is becoming more and more concerned with the treatment of the total person. Thus, the pediatrician is concerned with the illness of the patient but is also becoming concerned with the patient's reaction and adjustment to his illness. We are beginning to see more and more the need for guidance in the treatment of patients who have undergone hemispherectomy. Their entire way of life needs to be restructured, relearned, and new goals established. This can be done only with tile aid of the physician who is treating the patient. In the case of the child who is undergoing' hemispherectomy, both the pediatrician and the neurosurgeon need to understand the total consequences of surgery--social, economic, and medicM if he is to help bring about an effective adjustment for the child and his family. Research in this area will
484
THE JOURNAL OF PEDIATRICS
need to be interdisciplinary and findings pooled, since pediatrician, neurosurgeon, neurologist, psychologist, and psychiatrist eaeh contribute to the total understanding of the patient. So long as each specialty works independently, a total Gestalt will be lacking. Systematized and well-controlled interdisciplinary research will no doubt prove to be more f r u i t f u l in the end. CONCLUSIONS
W h a t information then can the pediatrician and neurosurgeon impart to the parents of hemiplegic children who are being considered for hemispherectomy? I t would seem that he could point out that research findings dealing with children tend to indicate a low surgical mortality rate, a very g o o d chance that intractible seizures will become extinct or greatly reduced in number and severity, and more adequate control of emotional outbursts. The prognosis of the hemiplegia itself is indeterminant at this time although some do improve. While mental retardation is certainly not cured by hemispherectomy, it seems reasonable to suppose that more optimal intellectual functioning will ensue if the hypertonic behavior of the individual prior to surgery interfered with the efficacy of his intellectual functioning and is absent following surgery. SUMMARY
This paper reviews the British and American literature regarding the application of hemispherectomy to children suffering from infantile hemiplegia, intractable seizures, emotional outbursts, and mental retardation. While investigators differ in the amount and kind of information they
report, certain trends are evident when the 29 cases are evaluated in terms of consequences of surgery. There seems to be a low surgical mortality rate, decrease or extinction of seizures, more effective control of emotional outbursts, and, in some cases, improvement in the hemiplegia itself. Consideration is given to ways in which research in this area can be made more systematic and more adequately eormnunicated. Suggestions for f u t u r e research are also presented. The author wishes to express his gratitude to Dr. Robert B. King, assistant professor of neurosurgery, Washington University School of Medicine--now professor and head of the department of neurosurgery, Syracuse Upstate Medical C e n t e r - - f o r introducing him to this area of study. REFERENCES 1. Dandy, W.: Removal of Right Cerebral I:lemisphere for Certain Tumors, J. A. M. A. 90: 823, 1928. 2. Mensh, I v a n N., Schwartz, H. G., Matarazzo, Ruth, and Matazarro, ft.: Psychological Functioning Following Cerebral Hemisphereetomy in Man, A . M . A . Arch. Neurol. & Psychiat. 67: 787, 1952. 3. Bell, E., and Kernosh, L.: Cerebral Hemispherectomy: A Case 10 Years A f t e r Operation, J. Neurosurg. 6: 285, 1949. 4. Cabieses, F., Raul, J., and Landa, R. : F a t a l Brain-Stem Shift Following Hemispherectomy, ft. Neurosurg. 14: 74, 1957. 5. Gardner, W. J . : Removal of the Right Cerebral Itemisphere for Infiltrating Glioma, Arch. Neurol. & Psychiat. 28: 470, 1932. 6. Gardner, W. J . : Removal of the Right Cerebral Hemisphere for Infiltrating Glioma, J. A. M. A. 1" 823, 1933. 7. Hillier, W.: Case Report: Total Left Hemispherectomy for Malignant Glioma, Neurology 4: 718, 1954. 8. Karnosh, L., and Gardner, W.: The Physical and Mental Capacity A f t e r Removal of the Right Cerebral Hemisphere, Dis. Nerv. System 1: 343, 1940. 9. Lhermitte, ft.: L ' a b l a t i o n compl6te de l 'h6misph6re droit duns les cas de tumeur c6r6brale localis6e eompliqu6e d 'h6mipl6gic, Enc4phale 23: 314, ]928. 10. O'Brien, J. D.: F u r t h e r Report on Case of Removal of R i g h t Cerebral Hemisphere, J. A. M. A. 107" 657, 1936.
MEDICAL PROGRESS 11. Rowe, S. : Mental Changes Following the Removal of the Right Cerebral Hemisphere for Brain Tumor, Am. 3-. Psychiat. 94: 605, 1937. ]2. Zollinger, R.: Removal of the Left Cerebral Hemisphere, Arch. Neurol. & Psychiat. 34: 1055, 1935. 13. Cairns, Sir Hugh, and Davidson, M.: Hemispherectomy in the Treatment of Infantile Hemip]egia, Lancet. 2: 411, 1951. 14. Fleischacker, H.: Hemispherectomy, J. Ment. Se. 100: 66, 1954. 15. Ferey, D.: I_ln cas d'h6mipl4glc Infantile Trait6 par Hdmisph~reetomie, M6m. Acad. chir. 79: 621, 1953. 16. French, L., Johnson, D., Brown, I. A., and VanBergen, F. : Cerebral Hemisphereetomy for Control of Intractable Convulsive Seizures, 3-. Neurosurg. 12: 154, ]955. 17. Goldstein, R., Goodman, A., and King, R.: Hearing and Speech in Infantile Hemiplegia Before and After Left Hemispherectomy, Neurology 6: 869, 1956. ]8. Gros, B., Vlahovitch, B., and Enjalbert, J . M . : Observations d~h~misph~rectomle pour h6miatrophie c6r~brale avee h6miplSgie, 6pilepsie et troubles caract~riels graves, Arch. fran~, p6diat. 11: 205, 1954. 19. Krynauw, Rowland A. : Infantile Hemiplegia Treated by Removing One Cerebral Hemisphere, 3-. Neurol., Neurosurg. & Psyehiat. 13: 243, 1950. 20. Laine, E., Fontan, M., Delandtsheer, and Desfontaines: ]~tude d'une s6rie d'h~mispherectomies pour h~mipl6gie infantile, Rev. neurol. 86: 344, 1952. 21. Mason, E., and Shapiro, I.: Hemlsphereetomy for Convulsions in Infantile Hemiplegia, N. Y. 3-. Med. 53: 449, 1953. 22. Munz, A., and Tolor, A. : Psychological Effects of Major Cerebral Excision: In-
23. 24.
25.
26.
27.
28. 29. 30. 31. 32.
33. 34.
485
tellectual and Emotional Changes Following Hemispherectomy, 5. Nerv. & Ment. Dis. 121: 438, 1955. MeKissock, Wylie: Infantile IIemlplegia, Prec. Roy. Soc. Me/[. 46: 431, 1953. Obrador, Alcalde S.: Hemispherectomy as Treatment of Convulsions Due to Cerebral Hemistrophy in Infantile Henriplegia, A. M. A. Arch. Neurol. & Psyehiat. 68" 722, 1952. Ueckerm, A. E., French, L. A., and Johnson, D.: Psychological Studies of Seven Epileptic Hemipareties Before and After tIemispherectomy, A. M. A. Arch. Neurol. & Psychiat. 72: 555, 1954. Walsh, L. (for MeKissoek, W . ) : Infantile Hemiplegia Treated by Hemispherectomy, Prec. Roy. See. Med. 44: 335, 1951. Ford, Frank l~.: Diseases of the Nervous System in Infancy, Childhood, and Adolescence, Springfield, Ill., 1952, Charles G Thomas. Howells, J. G., and Kay, E. I.: Cerebral Hemispherectomy, Lancet 2: 683, 1951. Terman, L. M., and Merrill, M. A.: Measuring" Intelligence, Boston, 1937, Houghton ~V[ifl]in. Wechs]er, D.: Wechsler Intelligence Scale for Children, N. Y., 1949, Psychological Corporation. Doll, E.: Vineland Social Maturity Scale, Minneapolis, ]947, Educational Test Bureau. Klopfer, B., Ainsworthy, Mary D., and K]opfer, W.: Developments in the Rorschach Technique, u I, New York, 1954, World Book Co. Beck, S. J.: Rorschach's Test, New York, 1944, Grune &Stratton. Murray, H. A.: Explorations in Personality, New York, 1938, Oxford University Press.
INTRODUCTION
OTH the pediatric practitioner and the neurosurgeon are frequently confronted by parents who raise very practical, important, and difficult questions. To the parent who must make a decision regarding such drastic surgery as a hemispherectomy, consequences are of p r i m a r y importance. As u n h a p p y as parents are with a child's hemiplcgia, endless convulsions, and incorrigible behavior, the thought of surgery evokes certain anxieties and fears which must be dealt with by the pediatrician a n d / o r neurosurgeon. The probability of surgical survival is always uppermost in the minds of parents whose children undergo such major surgery. The next question is usually, " W h a t effect will such surgery have on my child's behavior ? " The question of surgical survival can be answered objectively in terms of probability based upon statistical data. The question regarding consequences of surgery has until recently been answered within the subjective, personal, and anecdotal realm. A definitive study dealing with the consequences
B
THE APPLICATION OF HEMISPHERECTOMY TO INFANTILE HEMIPLEGIA IIemispherectomy is a relatively new procedure. The first cerebral hemispherectomy was done by Dandy, in 1923, at the Johns Hopkins Hospital, and this case first appeared in the literature in 1928~--just 30 years ago. Since that time, numerous case reports have appeared. Mensh and his associates ~ listed 32 publications between 1928 and 1951. These represented 41 hemispherectomies. While a preponderance of this surgery was applied to the adult suffering from infiltrating tumors of the subdominant hemisphere, 1-12 hemispherectomy has of late been utilized and applied to a specific g r o u p - - t h e infantile hemiplegic. 13-26
F r o m t h e D i v i s i o n of M e d i c a l P s y c h o l o g y , Department of P s y c h i a t r y and Neurology, W a s h i n g t o n U n i v e r s i t y S c h o o l of :Medicine. 476
MEDICAL PROGRESS
F o r d 27 classifies this condition as one of either acute infantile hemiplegia of obscure etiology or congenital hemiplegia. Regarding the acute type, F o r d states : I n the e a r l y s t a g e s of t h e illness t h e affected h e m i s p h e r e m a y be so swollen t h a t ventrieulographie study may suggest brain tumor. L a t e r , of course, a t r o p h y becomes evident. Later the hemiplegia extremities become s p a s t i c a n d t h e u s u a l d e f o r m i t i e s develop. T h e a f f e c t e d l i m b s do n o t develop as well as t h e n o r m a l l i m b s a n d y e a r s l a t e r m a y be very s m a l l a n d distorted. T r e m o r or athetosis may appear. I n other cases, t h e f o c a l s i g n s m a y l a r g e l y or c o m p l e t e l y disappear. U n f o r t u n a t e l y , in a l m o s t h a l f of all cases convulsive seizures r e c u r f r o m t i m e to time.
The prognosis as presented by F o r d is one of low m o r t a l i t y and is unfavorable in outlook for recovery. E p i l e p s y is said to follow in about half of all cases, and mental retardation is to be expected. This type of hemiplegia is said to result f r o m a single gross vascular lesion of the brain. The congenital f o r m of hemiplegia is said to result from a birth injury. I n t r a u t e r i n e i n j u r y and disease are other explanations offered. F o r d describes the clinical features of the congenital t y p e as follows: F l a c c i d i t y of the p a r a l y z e d l i m b s is relatively more f r e q u e n t in c o n g e n i t a l hemiplegias t h a n a m o n g those o c c u r r i n g l a t e r in life. I n such cases, we find s t r i k i n g u n d e r development of t h e h a n d a n d less evident u n d e r d e v e l o p m e n t of the a r m a n d leg. T h e h a n d is of childish a p p e a r a n c e , b u t it is n o t in a n y w a y d e f o r m e d . D u e to t h e shorteni n g of t h e leg t h e pelvis m a y be tilted a n d t h e spine m a y show a p o s t u r a l curve. T h e t e n d o n reflexes a r e u s u a l l y s o m e w h a t increased on t h e affected side, b u t t h i s is never so s t r i k i n g as in s p a s t i c p a r a l y s i s .
477
I n other cases, the p a r a l y t i c l i m b s are very s p a s t i c since t h e muscle s p a s m develops w h e n the bones a r e plastic, e x t r e m e d e f o r m ity of t h e h a n d is produced. I n a l m o s t all cases, t h e r e is m e n t a l def i c i e n c y - c o n v u l s i v e seizures occur in Mmost 40 or 50 per cent of all cases. E v e n severe h e m i p l e g i a s of c o n g e n i t a l origin m a y not be a p p a r e n t u n t i l t h e child is several m o n t h s of age.
The prognosis is said to depend upon the amount of i n j u r y to the nervous system. " L i t t l e i m p r o v e m e n t is to be expected. The presence of convulsions makes the outlook unfavorable, for deterioration often occurs in such eases." I t would certainly seem f r o m the above that the prognosis of infantile hemiplegia accompanied by convulsive seizures is indeed gloomy. I t is with this in mind t h a t surgical intervention occurred. K r y n a u w is worked primarily with children and carried out twelve hemisphereetomies over a fiveyear period. I n all cases, hemiplegia per se was not a sufficient condition for surgery. Convulsive seizures and behavioral changes (uncontrolled emotional outbursts) were also evident. Thus, a triad of symptoms were considered before hemisphereetomy was undertaken--hemiplegia, convulsive seizures, and the presence of lowered intellectual functioning a n d / o r uncontrolled emotional outbursts as behavioral manifestations. Occasionally, surgery was u n d e r t a k e n in the absence of seizures or in the absence of emotional outbursts while the other symptoms were present. Thus, it was only a little over seven years ago that a n y specific formulation developed in the application of henfispherectomy to children.
478
T H E J O U R N A L OF P E D I A T R I C S
FINDINGS
A survey of the American and British literature pertaining to hemispherectomies in children (ages from birth to 16 years) yielded 29 cases. Table I summarizes the data reported by each investigator. I t is interesting to note that investigators differ in the amount and kind of information reported. F o r example, one investL gator ~9 relied on parental report and subjective evaluation of change in intellectual functioning. Another investigator 25 focused on the social adjustment of the child and reported no TABLE I .
EFFECTS OF CEREBRAL HEMISPHERECTOMIES
HEIViI ~ S AGE P H E R EACT-
AUTHOR
Krynauw
(1950)
IIENIISPHERE
TOMY
SEX
9 7 3 ]0 2 9
F F I~ M M N[
L L L L R L
2 15
F F
L L
14 I3
M
R
F
L L
7 too.
F F
4 II 4 4
M l~ M M
E L R L
13
M
R
12 4 8 9
3/[ F M F
Fleischacker (1954)
12 ].3 12
Ueeker (1954)
McK:issoek (1953)
Munz & Tolor (1955)
IN
CIrIILDREN
II~TELLECCONVULSIVE
REMOVED
7
Cairns (1951)
data regarding the particular hemisphere removed, the effect on the child's seizures, and hemiplegia. Still another investigator 2~ concluded that intellectual functioning increases following hemispherectomy, yet gave no specific data to support his conclusions. While systematic and quantitative measures arc at times lacking and like the blind men, each investigator touches a different part of the huge elephant, the impressions and findings that are reported do seem to indicate specific trends. Tables I I - V I I I present these trends.
SEIZURES
HEMIPLEGIA T U A L F U N G TIONING
Gone Gone Gone Gone
Improved No change Improved Improved Died None before No change surgery Not reported Improved Gone No change
Improved Improved Improved Improved
EMOTIONAL
OUTBURSTS
Goue Improved Improved Improved
Not reported Gone Improved Improved
Improved None before surgery
Not reported Not reported Not reported Improved Not reported Not reported Improved
Improved
Improved
Improved
L L L R
Gone Improved Gone Gone Gone Gone Improved Gone Gone Gone
No change Worse Improved No change No change No change Worse No change No change No change
Not Not Not Not Not Not Not Not Not Not
Gone Gone Gone Gone Gone Gone No change Gone Gone Gone
M M 3/[
L R L
Improved Gone Improved
Worse Improved No change
Not reported Improved Not reported Improved Not reported No change
13 13 15
F M M
? ?
Not reported Not reported No change Improved Not reported No change Gone Not reported No change
Improved Improved Not reported
14
F
L
Gone
Gone
No change
reported reported reported reported reported reported reported reported reported reported
No change
479
MEDICAL PROGRESS
Of the 29 hemispherectomized children, one or 3 per cent died. This occurred to a 2-year-old male and is reported b y K r y n a u w . 19 His condition is said to have given no cause for alarm d u r i n g surgery. He became v e r y restless while r e t u r n i n g to the w a r d and suddenly collapsed and died. Thus, the m o r t a l i t y rate as a function of surgery is seemingly low. TABLE I I . AGE ])ISTRIBUTION OF ALL CASES REPORTED IN THE LITEBATURE
AdE DISTRIBUTION < 1 2 3 4 5 6 7 8 9 10 11 ]2 ]3 14 15 16 No
year years years years years years years years years years years years years years years years age reported
I
N 1 2 1 4 0 0 1 1 3 1 1 3 5 2 2 0 1
I
% 4 7 4 14 0 0 4 4 10 4 4 10 17 7 7 0 4
Table I I indicates t h a t the eases reported in the literature are d r a w n f r o m all age groups except years 5 and 6. W h e n the sample is categorized into preschool (ages f r o m birth to 6 years), middle childhood (ages 7 to 12 years) and adolescence (ages 13 to 16 years) we find little difference between the n u m b e r included in each developmental category. F o r 27 reported ages, 30 per cent come f r o m the preschool group, 37 per cent f r o m the middle childhood group, and 33 per cent f r o m the early to middle adolescent period. According to Tables I I I and IV, boys underwent hemispherectomy more often t h a n girls, and the left hemi-
sphere was r e p o r t e d l y removed more frequently than the right one. The sex differences m a y not necessarily be significant since these differences are relatively small. There does, however, a p p e a r to be a greater proclivity for the left hemisphere to be removed. More t h a n half of the cases consisted of the removal of the ]eft hemisphere. Of the 26 cases in which the p a r t i c u l a r hemisphere removed is indicated, 19 or 65 per cent r e f e r r e d to the removal of the left hemisphere. Ten per cent failed to indicate which hemisphere was removed. TABLE I I I . SEX DISTRIBUTION OF ALL CASES REPORTED IN THE LITERATURE
1
N I
Boys Girls TABLE :IV.
59 41
~IEMISPHERE RE1V[OVED
I
(% oF REPORTED
I-IE]~KISPttERE REIV[0VED
N
Left Right N o t reported
17 9 3
TABLE
V.
%
17 12
THE
CASES) N ~ 26
%. 58 32 10
65 35
STATUS OF
CONVULSIVE
SEIZURES FOLL0WIN~ SURgEry (% OE STATUS OF CONVULSIVE SEIZURES Completely gone Improved l~lo change Absent before surgery No d a t a reported Expired
N
%
15 5 0
52.0 17.0 0.0
1 7 1
3.5 24.0 3.5
I~EPORTED CASES) N ~ 20 75 25
Table V indicates that a m a j o r consequence of hemispherectomy is the absence or reduction of convulsions. While close to one fourth of the reported cases did not deal with the preand poststatus of convulsions, of the
480
THE JOURNAL OF PEDIATRICS
20 eases which did deal with this variable, 75 per cent no longer experience convulsions, and 25 per cent are improved in t h a t the frequency a n d / o r intensity of the convulsions have diminished. This is one v e r y positive finding. Of the three presenting complaints usually considered as the criteria for hemispherectonly, one m a y v e r y well be able to predict t h a t convulsions will become extinct or at least become g r e a t l y reduced in severity in all or at least in the m a j o r i t y of eases. A second criteria for hemispherectomy, hemiplegia, is more difficult to assess. Investigators focus o n different aspects and with more or less specificity. W h e n the findings are categorized in a general w a y according to improved, no change in status, or condition worse folIowing hemisphereetomy, the results are less f o r t u n a t e t h a n is the ease with convulsions. Table V I indicates that of the 23 TABLE u
THE STATUS OF THE I-IE~IPLEGIA FOLLOWING SURGERY
STATUS OF HE~IPLEGIA Improved No change Condition worse No data reported Expired
% 8 12 3 5 1 29
28 41 10 17 4
( % OF REPORTED CASES) N ~ 23 35 52 13
cases in which this variable is considered, 35 per cent showed some form of improvement. This varied behaviora]ly f r o m improved balance to decreased spasticity of the affected limbs. F i f t y - t w o per cent did not show a n y change, and 13 per cent becanle worse. The hemiplegia became worse in two cases reported by Me-
Kissock. 2a He states, " [Cases Nos, 2 and 7] suffered as a result of operation: both of these were errors of j u d g m e n t on m y part. One developed an increase in hemiplegia which has f o r t u n a t e l y been offset by disappearance of the severe behavior disorder, whilst the other has acquired an increased speech defect. This latter ease should have been subjected only to a local excision of abnormal parietal cortex r a t h e r than to ablation of the h e m i s p h e r e . " Thus, while occasionally one can expect worsening of the hemiplegia, in 87 per cent of the cases the patients were no worse off and some (35 per cent) improved. The t h i r d criteria, emotional outbursts, also diminish to a great extent. Table V I I indicates that of 24 cases in TABLE VIIi.
CONTIgOLOF EI~IOTIONAL OUTBURSTS
STATUS
N
%
Improved No change Worse None present p r i o r to surgery No d a t a reported Expired
21 3 0
72 10 0
1 3 1
4 10 4
( % OF REPORTED CASES) N--__24 87 13
which severe emotional outbursts are reported prior to surgery, 21 or 87 per cent show i m p r o v e m e n t in this area. Only 13 per cent of the reported eases indicate no change in behavior. I n no ease did this behavior become worse. I t would seem then that along with control of convulsions, one can anticipate effective control of emotionnl outbursts. Little systematic attention has been given to the actual measurement of
48l
MEDICAL PROGRESS
intellectual functions. While the consensus of opinion seems to be that intellectual functioning improves as a result of hemisphereetomy, few studies ~3, ==, ~ have actually reported objective measurements. Table V I I I indiTABLE
VIII. THE FUNCTIONING
STATUS OF INTELLECTUAL FOLLOWING SURGERY
t STATUS OF INTELLECTUAL
EUNCTIONING Improved No change Worse No data reported Expired
N 7 4 0
% 24 14 0
17 1
58 4
NUMBER BASED NUMBER UPON O~BASED ,JECTIVE UPON IM- 3gEASUaF,PaXSSlON MENTS 6 1 2 2 0 0
cates t h a t no data were reported with respect to intellectual functioning for 58 p e r cent of the cases. While close to one fourth report improvement, 86 p e r cent of these are based upon subjective evaluations. Of the 14 per cent who report no change in intellectual functioning, only 50 per cent are based upon subjective evaluat i o n s - t h e other 50 per cent being based upon objective measurements. Assessing this variable is extremely complex. No one can measure intelligence per se but only the functions of intel/igenee as manifested in behavior. W h a t is being measured is intellectual functioning and not native intelligence. The latter is i n f e r r e d ; the former is observed and ascertained through standardized and objective measures. The appraisal of intellectual functioning is f u r t h e r complicated b y the numerous factors which alter one's level of functioning'. Some o f the more i m p o r t a n t factors are: the ability to see and h e a r ; level of motivation including' the desire to co-
operate and do as well as one is able; and adequate control of impulses. Howells and K a y 2s state: I n our experience in testing eases of infantile hemiplegia before operation we have become aware of the difficulties involved in ascertaining reliable and stable indices of tile intellectual ability. In particular, we have noted that as well as the child's physical handicap in completing performance tests, his frequent distractibility, memory defects, short lapses of attentim b lack of confldence~ and perhaps Mso his often eonsideralfle gap in formal education, have lowered his test results to an undetermined extent.
In view of these m a n y variables, one needs to be cautioned against conclusions which infer t h a t native intelligence r a t h e r than intellectual functioning becomes or does not become elevated. K r y n a u w a~ indicates : Improvement in the mental sphere presupposes and is dependent upon~ the integrity of the remaining hemisphere and its ability to function normally once it has been released front abnormal iitfluences from the pathological side.
I t would seem then that once the pathology is removed and the motor "drivencss" of the individual restored to a more stable level, intellectual functioning as measured by standardized tests, is able to become more adequately maximized. DISCUSSION
A review such as this brings to the fore m a n y questions and problems for the researcher in this area. While investigators are idiosyncratic in their interests and necessarily focus on different aspects of the problem, there is a need for greater systematization in reporting clinical case accounts if a complete understanding of the hemisphereetomized patient is to be
482
T H E J O U R N A L OF P E D I A T R I C S
achieved. This discussion will consider the ways in which investigators can communicate and make their findings more meaningful to others as well as present suggestions for f u t u r e research. It would seem that all investigators should report their face sheet data, i.e., a patient's age, color, religion, socioeconomic level, etc. Furthermore, one would need to consider dominance, presenting symptoms, the specific time in the total course of study when certain observations are made, and the particular hemisphere removed. These data are available to all and should be reported if systematic comparisons are to be made between investigators. I f a follow-up study is done one would also need to indicate the sources from which particular data stem and the time factor in relation to the date of hemispherectomy. Thus, it is important to indicate whether the investigator is reporting his own observations based on specific tests or is reporting anecdotal material related by the patient's family: One would also need to indicate whether the observations are made while the patient still resides in the hospital or as an outpatient. There is also a tendency to speak collectively of drugs r a t h e r than to specify particu]ar drugs and dosage. I t is insufficient to indicate that a patient no longer has seizures following hemispherectomy. One also needs to spell out to what extent this is evident when the patient is maintained on anticonvulsive drugs and to what extent this is so when the patient is free of drugs. There are also investigators who describe ~in a general manner that a child has emotional outbursts, but there are no indications as to what
situations instigate the outbursts and just what the outbursts entail. Also, quantitative measurements should be utilized whenever possible. It was pointed out earlier that subjective evaluations arc made regarding intellectual functioning and also regarding the appraisal of social and emotional development and adjustment. This is u n f o r t u n a t e since there are tools available29, 3o which can quantitatively measure changes in specific intellectual functions such as general f u n d of information, comprehension, arithmetical reasoning, immediate recall, vocabulary, planning ability, perceptual skills, visual-motor coordination and integration, and ability to handle abstract ideas. I n like manner, there are scales which can measure social development, sl and projective techniques32, 3a, 34 which can measure specific personality variables such as hostility and aggressivity, ability to relate to others, needs and need systems, self-concept, etc. Thus, if one is to follow a pre- and postoperative research design, one must be very specific regarding the sample used, the time of observation, as well as the sources and tools of observation. Measurement per se is not invariably more useful than description. Descriptions have a very definite place; however, if descriptions are to be useful, they must elucidate v e r y clearly and specifically that which is being observed. I t is only in this way that one investigator can directly compare his :findings with that of another investigator. A great deal of emphasis has been placed in this discussion on tile importance of indicating tile time when particular observations are made. Research upon the hemispherectomized
MEDICAL PROGRESS
patient necessarily entails a longitudinal kind of research design. Unless the time factor is clearly specified, cross validation becomes especially difficult. One can certainly question the validity of the findings reported in this paper since the emphasis and interests of the investigators proved to be so varied. While this is true, there were nevertheless consistent findings even for so varied a group. Thus, epileptic seizures were greatly reduced in all of the studies regardless of the investigator. This was also true regarding emotional outbursts. While the findings reported here may be considered general, they nevertheless emphasize the need for more specific and systematized research. There are several ideas which come to mind at this time regarding suggestions for f u t u r e research. Since hemispherectomy has been extended to M1 age levels it would be interesting to investigate to what extent the prognosis differs from one age level to another when the presenting complaints are similar hemiplegia, intractable seizures, emotional outbursts, and mental retardation. When the present data were analyzed for three age levels (preschool, middle childhood, and adolescence), it was found that there were no differences in the consequences of hemisphereetomy in so far as status of seizures, status of hemiplegia, and status of emotional outbursts are concerned. These must be considered fairly tentative findings since tests of statistical significance could not be applied due to the m a n y zero cells and the relatively small number of cases. In like manner, consequences of sur-
483
gery can be systematically evaluated for sex differences, dominance, and the particular hemisphere removed. I t was reported that there was a greater tendency for the left hemisphere to be removed than the right. This finding in and of itself is meaningless but does raise questions which can be pursued if enough systematic data are available. To those who feel that hemis p h e r e e t o m y - g r e a t l y improved intellectual functioning, one could certainly study and evaluate the eases where this occurs and those where this does not occur. With the aid of objective measurements, one could even determine how much or how little change occurs. A study also needs to be done with regard to those cases whose hemiplegia improves and those who do not improve. Medicine has become a comprehensive field in that it is becoming more and more concerned with the treatment of the total person. Thus, the pediatrician is concerned with the illness of the patient but is also becoming concerned with the patient's reaction and adjustment to his illness. We are beginning to see more and more the need for guidance in the treatment of patients who have undergone hemispherectomy. Their entire way of life needs to be restructured, relearned, and new goals established. This can be done only with tile aid of the physician who is treating the patient. In the case of the child who is undergoing' hemispherectomy, both the pediatrician and the neurosurgeon need to understand the total consequences of surgery--social, economic, and medicM if he is to help bring about an effective adjustment for the child and his family. Research in this area will
484
THE JOURNAL OF PEDIATRICS
need to be interdisciplinary and findings pooled, since pediatrician, neurosurgeon, neurologist, psychologist, and psychiatrist eaeh contribute to the total understanding of the patient. So long as each specialty works independently, a total Gestalt will be lacking. Systematized and well-controlled interdisciplinary research will no doubt prove to be more f r u i t f u l in the end. CONCLUSIONS
W h a t information then can the pediatrician and neurosurgeon impart to the parents of hemiplegic children who are being considered for hemispherectomy? I t would seem that he could point out that research findings dealing with children tend to indicate a low surgical mortality rate, a very g o o d chance that intractible seizures will become extinct or greatly reduced in number and severity, and more adequate control of emotional outbursts. The prognosis of the hemiplegia itself is indeterminant at this time although some do improve. While mental retardation is certainly not cured by hemispherectomy, it seems reasonable to suppose that more optimal intellectual functioning will ensue if the hypertonic behavior of the individual prior to surgery interfered with the efficacy of his intellectual functioning and is absent following surgery. SUMMARY
This paper reviews the British and American literature regarding the application of hemispherectomy to children suffering from infantile hemiplegia, intractable seizures, emotional outbursts, and mental retardation. While investigators differ in the amount and kind of information they
report, certain trends are evident when the 29 cases are evaluated in terms of consequences of surgery. There seems to be a low surgical mortality rate, decrease or extinction of seizures, more effective control of emotional outbursts, and, in some cases, improvement in the hemiplegia itself. Consideration is given to ways in which research in this area can be made more systematic and more adequately eormnunicated. Suggestions for f u t u r e research are also presented. The author wishes to express his gratitude to Dr. Robert B. King, assistant professor of neurosurgery, Washington University School of Medicine--now professor and head of the department of neurosurgery, Syracuse Upstate Medical C e n t e r - - f o r introducing him to this area of study. REFERENCES 1. Dandy, W.: Removal of Right Cerebral I:lemisphere for Certain Tumors, J. A. M. A. 90: 823, 1928. 2. Mensh, I v a n N., Schwartz, H. G., Matarazzo, Ruth, and Matazarro, ft.: Psychological Functioning Following Cerebral Hemisphereetomy in Man, A . M . A . Arch. Neurol. & Psychiat. 67: 787, 1952. 3. Bell, E., and Kernosh, L.: Cerebral Hemispherectomy: A Case 10 Years A f t e r Operation, J. Neurosurg. 6: 285, 1949. 4. Cabieses, F., Raul, J., and Landa, R. : F a t a l Brain-Stem Shift Following Hemispherectomy, ft. Neurosurg. 14: 74, 1957. 5. Gardner, W. J . : Removal of the Right Cerebral Itemisphere for Infiltrating Glioma, Arch. Neurol. & Psychiat. 28: 470, 1932. 6. Gardner, W. J . : Removal of the Right Cerebral Hemisphere for Infiltrating Glioma, J. A. M. A. 1" 823, 1933. 7. Hillier, W.: Case Report: Total Left Hemispherectomy for Malignant Glioma, Neurology 4: 718, 1954. 8. Karnosh, L., and Gardner, W.: The Physical and Mental Capacity A f t e r Removal of the Right Cerebral Hemisphere, Dis. Nerv. System 1: 343, 1940. 9. Lhermitte, ft.: L ' a b l a t i o n compl6te de l 'h6misph6re droit duns les cas de tumeur c6r6brale localis6e eompliqu6e d 'h6mipl6gic, Enc4phale 23: 314, ]928. 10. O'Brien, J. D.: F u r t h e r Report on Case of Removal of R i g h t Cerebral Hemisphere, J. A. M. A. 107" 657, 1936.
MEDICAL PROGRESS 11. Rowe, S. : Mental Changes Following the Removal of the Right Cerebral Hemisphere for Brain Tumor, Am. 3-. Psychiat. 94: 605, 1937. ]2. Zollinger, R.: Removal of the Left Cerebral Hemisphere, Arch. Neurol. & Psychiat. 34: 1055, 1935. 13. Cairns, Sir Hugh, and Davidson, M.: Hemispherectomy in the Treatment of Infantile Hemip]egia, Lancet. 2: 411, 1951. 14. Fleischacker, H.: Hemispherectomy, J. Ment. Se. 100: 66, 1954. 15. Ferey, D.: I_ln cas d'h6mipl4glc Infantile Trait6 par Hdmisph~reetomie, M6m. Acad. chir. 79: 621, 1953. 16. French, L., Johnson, D., Brown, I. A., and VanBergen, F. : Cerebral Hemisphereetomy for Control of Intractable Convulsive Seizures, 3-. Neurosurg. 12: 154, ]955. 17. Goldstein, R., Goodman, A., and King, R.: Hearing and Speech in Infantile Hemiplegia Before and After Left Hemispherectomy, Neurology 6: 869, 1956. ]8. Gros, B., Vlahovitch, B., and Enjalbert, J . M . : Observations d~h~misph~rectomle pour h6miatrophie c6r~brale avee h6miplSgie, 6pilepsie et troubles caract~riels graves, Arch. fran~, p6diat. 11: 205, 1954. 19. Krynauw, Rowland A. : Infantile Hemiplegia Treated by Removing One Cerebral Hemisphere, 3-. Neurol., Neurosurg. & Psyehiat. 13: 243, 1950. 20. Laine, E., Fontan, M., Delandtsheer, and Desfontaines: ]~tude d'une s6rie d'h~mispherectomies pour h~mipl6gie infantile, Rev. neurol. 86: 344, 1952. 21. Mason, E., and Shapiro, I.: Hemlsphereetomy for Convulsions in Infantile Hemiplegia, N. Y. 3-. Med. 53: 449, 1953. 22. Munz, A., and Tolor, A. : Psychological Effects of Major Cerebral Excision: In-
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25.
26.
27.
28. 29. 30. 31. 32.
33. 34.
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tellectual and Emotional Changes Following Hemispherectomy, 5. Nerv. & Ment. Dis. 121: 438, 1955. MeKissock, Wylie: Infantile IIemlplegia, Prec. Roy. Soc. Me/[. 46: 431, 1953. Obrador, Alcalde S.: Hemispherectomy as Treatment of Convulsions Due to Cerebral Hemistrophy in Infantile Henriplegia, A. M. A. Arch. Neurol. & Psyehiat. 68" 722, 1952. Ueckerm, A. E., French, L. A., and Johnson, D.: Psychological Studies of Seven Epileptic Hemipareties Before and After tIemispherectomy, A. M. A. Arch. Neurol. & Psychiat. 72: 555, 1954. Walsh, L. (for MeKissoek, W . ) : Infantile Hemiplegia Treated by Hemispherectomy, Prec. Roy. See. Med. 44: 335, 1951. Ford, Frank l~.: Diseases of the Nervous System in Infancy, Childhood, and Adolescence, Springfield, Ill., 1952, Charles G Thomas. Howells, J. G., and Kay, E. I.: Cerebral Hemispherectomy, Lancet 2: 683, 1951. Terman, L. M., and Merrill, M. A.: Measuring" Intelligence, Boston, 1937, Houghton ~V[ifl]in. Wechs]er, D.: Wechsler Intelligence Scale for Children, N. Y., 1949, Psychological Corporation. Doll, E.: Vineland Social Maturity Scale, Minneapolis, ]947, Educational Test Bureau. Klopfer, B., Ainsworthy, Mary D., and K]opfer, W.: Developments in the Rorschach Technique, u I, New York, 1954, World Book Co. Beck, S. J.: Rorschach's Test, New York, 1944, Grune &Stratton. Murray, H. A.: Explorations in Personality, New York, 1938, Oxford University Press.