BEHAVIORAL
PEDIATRICS
Ricli:irdW. Oh,,stcd, edi,o;
Behavioral consequences of congenital rubella Psychiatric and behavioral consequences o f congenital rubella are reported for 243 children studied during the preschool period, and for 205 of these who were re-examined at ages 8 to 9. A t preschool 37% were retarded, with the skew toward severe and profound; 15% had reactive behavior disorder and 7% had atttism. At school age retardation diminished to 25%, but neurotic problems and behavioral pathology due to neurologic damage both increased. There were two remissions and three new instances o f autisnt.
Steila Chess, M.D., Paulina Fernandez, Ph.D., and Sam Korn, Ph.D., New
TIlE
Y o r k , N . Y.
ORIGINALLY
DEFINED
CONSEQUENCES
of
congenital rubella, identified by Gregg t in 1941, have been gradually widened as more organ defects have been identified, especially as the children affected in 1964 were examined in a systematic manner in several centers. 2-' These physical consequences included such effects as fetal loss, neonatal death, failures of immune mechanisms, and cardiac, neurologic, audiologic, and visual defects. As the survivors grew older, behavioral deviations were also identified? Although the expected epidemic, which without an immunization program should have appeared between 1970 and 1974, was apparently prevented,".' there have been a number of rubella epidemics in localized populations of high school age within the past few years. It is uncertain whether these have occurred entirely in those just above the age of the vaccination campaign, or whether some may have received vaccine. If the latter, the questions remain whether the vaccine was always administered properly, or whether it provides only time-limited protection. In addition, a rare sequela similar to Dawson sclerosing panencephalitis of measles has now been identified during adolescence in children with congenital rubella, and recovery of the rubella virus from pathologic tissue has been reported:. 9
From the New York University-Bellevue Hospital Medical Center and lltmter College-City University o f New York. *Reprint address: Department of Ps)'chiatr), New York University,School of Medicine, 550 First Ave., New York, N Y 10016.
0022-3476/78/100699+05500.50/0 9 1978 The C. V. Mosby Co.
MATERIALS
AND METHODS
Our behavioral study of a sample of 243 children with congenital rubella, of whom 205 were seen in both the preschool period (ages 289 to 5) and also at early school age (ages 8 to 9), provides both prevalence and longitudinal data. The children's physical status had been determined in the Rubella Birth Defect Evaluation Project initiated at New York University-Bellevue ltospital Medical Center under Drs. Saul Krugman and Louis Cooper; many of these patients had been followed since birth. The sample included both infants with defects identified at birth and infants without physical defects whose mothers had been reported by obstetricians to have contracted rubella during pregnancy.
See related article, p. 584.
[
As background for the findings on behavioral deviations, the prevalence of physical defect of the 243 children is given as at the time of the initial study at ages 289 to 5 years. The defects counted for the purpose of this study were those which.might have direct influence on behavioral adaptation, namely cardiac, neurologic, audiologic, and visual. Cardiac defects occurred in 79 or 32.5%, neurologic defects in 79 or 32.5%, hearing loss in 177 or 72.8%, and visual problems in 80 or 32.9%. These defects appeared either singly or in combination as follows: no defect in 50 children or 20.6%, one defect area in 72 or 29.6%, two defect areas in 47 or 19.3%, and four defect areas in 27 children or 11.1%. Of those with a single The Journal o f P E D I A T R I C S Vol. 93, No. 4, pp. 699-703
699
700
Chess, Fernandez, and Korn
The Journal of Pediatrics October 1978
Table I. Psychiatric diagnoses of children with congenital rubella ages 289 to 5 years (N = 243)
I N* I %
Diagnosis No psychiatric disorder (normal) Reactive behavior disorder Neurotic behavior disorder Behavorial disorder/neurologic damage Mental retardation Autism
!!8 37 0 8 91 18
49.0 15.0 0.0 3.3 37.0 7.4
"The total N is greater than 243 because of the occurrenceof multiple diagnoses in some cases.
Table
II. Correlation between physical defect and psychiatric disorder in children ages 2 89 to 5 years (N = 243) Number of defect areas Psychiatric diagnosis
,,oelOnelTwolhreelourI ota,
(N) (50) (72)(47) (47) (27) (243) 40 41 24 12 I 118 No psychiatric disorder Reactive behavior disorder 8 14 4 3 1 30 Neurotic Behavior disorder 0 0 0 0 0 0 3 1 4 0 8 Behavior disorder/neurologic 0 damage 2 15 18 31 25 91 Mental retardation 0 5 4 6 3 18 Autism Note: Due to multiple diagnoses,total is greater than 243 subjects. defect, hearing loss, which occurred in 65 youngsters, was the largest category. 5 Psychiatric diagnosis was determined by utilizing all of the behaviorai data available. These included clinical psychiatric history, psychiatric and neurologie examination, psychologic study, and parent and teacher interviews. Behavior disorder was categorized in two ways: (1) psychiatric Classification and (2) number and severity of behavioral symptoms. The psychiatric classification utilized has been described in detail elsewhere. In brief, it is as follows: No psychiatric disorder: Behavior is within expectancy for chronologic age, both developmentally and adaptively. Reactive behavior disorder: Developmental level is age appropriate but behavior problems exist. These can be directly related to such stresses as inappropriate parental handling, other environmental trauma, or both. Expectation is that, were such stresses alleviated, behavioral problems would abate. Neurotic behavior disorder: Developmental level is age appropriate. "Maladaptive behaviors are expressive of
fixed inappropriate attitudes, which require more extensive intervention than altered management in order to achieve rapid improvement. Behavior disorder due to neurologic dantage: (1) Without mental retardation-developmental findings are age appropriate. Symptoms, such as perseveration, correlate with central nervous system damage. Physical findings must include a minimum of one focal or two nonfocal (soft) signs of neurologie damage. (2) With mental retardation-the behavioral findings include cognitive and adaptive retardation but are not entirely explained by this factor. Additional symptoms correlate with central nervous system damage, and physical findings include a minimum of one focal or two soft neurologic signs. Mental retardation: The total adaptive findings provide the basis for this assignment. When feasible, depending upon type and degree of sensory loss, formal testing is included as part of the assessment. The categorization of the American Association on Mental Deficiency provides the subclassification. II Autism: The criteria are those given by Leo Kanner in his original definition of the syndrome." These include failure of affective relaiedness toward people; stereotyped actions; echolalic language or other specified language deviations, such as absence of or peculiar word usage; odd repetitive actions; and unusually strong attachments to objects. "Especially prominent is great distress when changes in the physical environment occur, such as rearrangement of furniture. As can be seen from Table I, during the preschool period approximately half of the children had one or more types of behavior disorder. Fifteen percent had reactive behavior disorder. Fully 37% of the study children were mentally retarded. In contrast to the usual distribution of mental retardation, in which about 95% can be expected to be in the mild category, here the severely retarded comprised the largest proportion. The combination of those who were either severely or profoundly retarded included 43 children, or almost half of the 91 retarded subjects. A very high number of 18, or 7.4%, fell into the category of autism, whereas the expected rate is 0.7 per 10,000.13 The relationship between psychiatric disorder and physical disorder was correlated, as shown in Table II. Forty of the 50 (80%) children who had no diagnosed physical defect had no psychiatric disorder. Eight, an expected prevalence, had reactive behavior disorder. Two were retarded. Of the 72 children with a single physical defect, 41 or 57% were without psychiatric disorder. One child had the single diagnosis of behavior disorder due to neurologic
Volume 93 Number 4
damage; another had the single diagnosis of autism. Fifteen (20%) had mental retardation as a sole diagnosis or in combination with reactive behavior disorder, behavior disorder due to neurologic damage, or autism. In the group of 47 children with two physical defects, about half had no psychiatric disorder. Many of the mentally retarded children were in the moderately or profoundly retarded classification. The 47 subjects with three physical defects had a considerable higher degree of psychiatric pathology. Only one-fourth were free of psychiatric pathology, and only 6% had the mildest type, reactive behavior disorder. The remaining youngsters, with the exception of one autistic child, were retarded, usually to a moderate or profound degree. Several of these had additional symptoms, either of behavior disorder due to neurologic damage (4) or autism (5). With four areas of physical damage, only one child was free of psychiatric pathology out of the total of 27. All others were retarded, most commonly to a severe o r profound degree; three of these had autism as well. In sum, increase in the number of t~hysical defects in congenital rubella brings greater risk of both presence and severity of psychiatric pathology. However, this risk is not a certainty; each group, fro m that with no physical defect to that with four defects, included one or more children with intellectual and behavioral normality. ~ In general, however, the pediatrician who is presented with a child with physical defect resulting from congenital rubella must consider the possibility of accompanying psychiatric casualty. The greater the number of defects, the greater is this possibility. The sample of children was once again examined behaviorally at early school age, 8 to 9 years. It was possible to locate 205 of the original group, and an additional five children of the same age with congenital nabella were added. A few parent s of the original sample refused to have their children rerexamined. Without exception these latter patients were at the two extremes: Parents of those without physical or behavioral consequences preferred to put behind them the thought Of the risk that had occurred during pregnancy, and parents of those with Severe handicaps felt that no help or study would change their burden. Table III shows the prevalence of psychiatric disorder at ages 8 to 9 years. Some important changes occurred after the preschool period. Although the proportion without behavior problems and with reactive behavior disorder had not altered appreciably, there were now 5 or 2.4% whose problem behaviors reflected neurotically fixed inappropriate attitudes (neurotic I~ehavior disorder). This change is devel-
Behavioral consequences of congenital rubella
70 1
Table !!!. Psychiatric diagnoses of children with congenital rubella ages 8 to 9 years (N = 210) Diagnosis
N
%
No psychiatric disorder (normal) Reactive behavior disorder Neurotic behavior disorder Behavior disorder/neurologic damage Mental retardation Autism
98 38 5 26 54 13
46.7 18.1 2.4 12.4 25.7 6.2
opmental. In these children child-environment maladaptive interactions continued over time, and the disordered reactions became fixed and elaborated at a more sophisticated conceptual level. The augmented prevalence of behavior disorder due to neurologic damage also reflects a developmental trend. With increasing age," impulse control normally improves, and repetitive verbalizations and actions, which are appropriate to the preschool age period, should disappear. It is sometimes difficult to diagnose impulsive and repetitive language and behavior until its persistence is identified at school age. Mental retardation, on the other hand, decreased from V~to ',~ of the sample, and eight Of the retardedchildren had a lesser degree of retardation. In children with normal or above normal intelligence in the preschool evaluation, there was also a trehd toward higher intellec!ual functioning at the eight- and nine-Year assessment. Thus, there was an overall tendency for rising cognitive competence despite continuing sensory impairment, even in some children in whom there had been worsening of the hearing impairment. It is possible that in some patients the chronic viral infection was overcome by the body's immune mechanisms, making possible a reorganization of brain function. One may also postula!e that the 8- to 9Year-old children were capable of using intact or residual sensory abilities with growing competence at higher developmental levels, and with increasing cognitive awareness. In other words, they could have been using alternative pathways toward the comprehension of the World and its social patterns and expectations. Thus, the rates and sequences of intellectual development in sensorially normal children may not be an accurate yardstick for prognosticating the future capabilities of preschool children with congenital rubella. Of special interest are the changes that occurred in the children with autism, which is by far the most serious psychiatric disorder of childhood. Although there were 13 such children at ages 8 to 9, as compared to 18 in the preschool period, the longitudinal picture is not reflected in these figures. Several of.the original au!istic children were among those not seen at the older age period,
702
Chess, Fernandez, and Korn
because of parental refusal with such statements as "she cannot be helped." Although one can conjecture that the autistic findings remained unchanged, these children were not in fact evaluated and are not included in the later tabulation. Two of the originally autistic children were found at school age to be completely recovered from their autism. One had normal intelligence despite continued profound hearing loss and monocular cataract. The other continued with the same degree of mental retardation as previously (moderate), but at 8 years had compulsive behaviors that dominated his actions and which he used as the basis for affectively related social interactions. Three new instances of autism emerged; all of these children had had prior behavior disorder but had affecrive social relations at preschool age which contraindicated the diagnosis of autism. At 8 to 9 years of age, all three showed a lack of affective relations and other behavior typical of autism. DISCUSSION In an attempt to condense and select data, physical defects have been grouped as if of equivalent importance. It was in fact found that, at least at the ages studied, cardiac defect was virtually unimportant in any of the correlations examined. Most of the children with cardiac anomalies had been assigned to cardiac classification IA, either in terms of the anatomic lesion or its status after surgical correction. Pulmonic stenosis was the most common lesion reported by the Rubella Birth Defect Evaluation Project. A very small group was classifiedas IB, and in no case had limitation of physical activity been prescribed. There was no direct correlation between cardiac defect as such and the presence of behavior disorder. However, most of the children with cardiac defect had hearing impairment as well. Neurologic impairment occurred in isolation in only one child, and in only two patients was it found paired with cat'diac defect only. Thus, most of the neurologically damaged children also had sensory deprivation, with each defect compromising the child's coping methods for the other. The major motor finding was spasticity; its importance to the child and to the family was determined by whether the child was wheelchair bound or could ambulate with braces or crutches or both, since this determined his degree of participation in ordinary childhood activities. The direct behavioral consequences of brain damage required independent evaluation, inasmuch as some children with central neurologic damage did not have a .behavior disorder. With the increased proportion of such behavior disorder at the later age, the presence of neurologic damage in the preschool period should suggest the possibility that, with increasing social expectations in the
The Journal
of Pediatrics October 1978
school-age period, behavioral pathology due to neurologic damage may become manifest. In this sample of children with congenital rubella, visual defect in the absence of hearing impairment occurred only in a small proportio n. The addition of visual difficulties of severe degree to a hearing loss immediately reduced the Options of home management, of educational placement, and of socialization opportunities. Cataract in one eye, while creating in effect monocular vision whether removed or not, did not seem to hinder the child behaviorally. Bilateral cataract, in contrast, made it necessary to place the child in the deaf-blind category and to tap the resources open to such children. Comparisons of rates of autism in other syndromes involving central nervous system damage, deafness, blindness, and mental retardation would be of interest, but data are scant. Rutter, 1~ surveying the total population of children 5 to 14 years on the Isle of Wight, found an overall prevalenc e of behavior disorder of 6.6%, but a 34.3% rate of psychiatric disorder in the brain-damaged group; autism was not separately reported. Rutter '~ also reported that tuberous sclerosis and cerebral lipidosis have been associated with atypical autism. Patients with retrolental fibroplasia have a high prevalence of autistic symptoms, according to Chase, 's although rigorous criteria for this diagnosis are not given. Of a group of 100 consecutively referred mentally retarded children seen in a psychiatric clinic, Philips and Williams ~' found 38 to be psychotically disturbed; autism as such was not separately reported. Although there i s g e n e r a l agreement, as reporte d by Vernon, Is that a higher prevalence of maladjustment exisis in children as a result of congenital rubella, as opposed to those whose deafness is genetic or consequent to Rh incompatibility, precise comparison is not available. Finally, Jan et al ~9 report that, other than children blind as a result of rubella, they have not found impressive numbers with psychosis or autism, and are convinced that such rePorts reflect examiners' inexperience with blind children. A multihandicapped child, as in our congenital rubella group, is often seen b y m a n y specialists. Not infrequently, the parents are bewildered by a host of recommendations which appear (or may in fact be) contradictory. It remains the task of the.pediatrician, as the primary physician, to judge the ~,alidity and relative importance of the different recommendations for management and interventions. The pediatrician can also take into account the total family picture, including the parents' emotional and financial abilities, as well as the needs of the other children. The pediatrician is also in the best position to follow the handicapped child's development over the years and to iden!ify changing capacities. Re-evaluation
Volume 93 Number 4
Behavioral consequences of congenital rubella
of defects and of cognitive abilities at each new age-period is essential for flexibility in m a n a g e m e n t and treatment to conform to the changing status of the child. REFERENCES 1..Gregg N: Congenital cataract following German measles in the mother, Trans Ophthalmol Soc Aust 3:35, 1941. 2. Cooper LZ, Green RH, Krugman S, Giles JP, and Mirick GS: Neonatal thrombocy!openic purpura and other manifestations of rubella contracted in utero, Am J Dis Child 110:416, 1965. 3. Krugman S: Rubella-new light on an old disease, J PEDIArR 67:159, 1965. 4. Lundstr6m R: Rubella during pregnancy: A follow-up study of children born after an epidemic of rubella in Sweden, 1951, and additional investigations on prophylaxis and treatment of maternal rubella, Acta Paediatr 5hSupplement 133, 1962. 5. Chess S, Korn S, and Fernandez P: Psychiatric disorders of children with congenital rubella, New York, 1971, Brunner/ Mazel. 6. Krugman S: Present status of measles and rubella immuniration in the United States: A medical progress report, J PEDIATR90:!, 1977. 7. American Academy of Pediatrics; Committee on Infectious Diseases: Measles and rubella immunization, Postgrad Med 61:269, 1977. 8. Townsend J J, Baringer R, Wolinsky JS, Malamud N, Mednick JP, Panitch HS, Scott RAT, Oshiro LS, and Cremer NE: Progressive rubella panencephalitis: Late onset after congenital rubella, N Engl J Med 292:990, 1975.
703
9. Well NIL, ltabashi HH, Cremer NE, Oshiro LS, Lennette EII, and Carnay L: Chronic progressive panencephalitis due to rubella virus simulating subacute sclerosing panencephalitis, N Engl J Med 292:994, 1975. 10. Chess S: Introduction to child psychiatry, ed 2, New York, 1969, Grune & Stratton. I1. Grossman HJ: (1973) Manual on terminology and classification in mental retardation, Washington, DC, 1973, American Association on Mental Deficiency. 12. Kanner L: Autistic disiurbances in affective contact, Nervous Child 2:217, 1943. 13. Treffen DA: Epidemiology of infantile autism, Arch Gen Psychiatry 22:431, 1970. 14. Rutter M: Brain damage syndromes in childhood: Concepts -,ad findings, J Child Psychol Psychiatry 18:1, 1977. 15. Rutter M: Language, cognition, and autism, paper presented at the meeting of The Association for Research in Nervous and Mental Disease, Dec. 3, 1977, New York City. 16. 9 Chase JB: Retrolental fibroplasia and autistic symptomatology, New York, 1972, American Foundation for The Blind. 17. Philips J, and Williams N: Psychopathology and mental retardation: A study of 100 mentally retarded children, Am J Psychiatry 132:!265, 1975. 18. Vernon M: Multiply handicapped deaf children: Medical, educational, and psychological considerations, Washington, D.C., 1969, The Council for Exceptional Children, lnc, p 75. 19. Jan JL, Freeman RD, and Scott EP: Visual impairment in children and adolescents, New York, 1977, Grune & Stratton, Inc.
Information for authors
Most of the provisions of the Copyright Act of 1976 became effective on January 1, 1978. Therefore, all manuscripts must be accompanied by the following statement, signed by each author: "The undersigned author(s) transfers all copyright o',vnership of the manuscript entitled (title of article) to The C. V. Mosby Company in the event the work is published. The author(s) warrants that the article is original, is not under consideration by another journal, and has not been previously published." Authors will be consulted, when possible, regarding republication of their material.
PEDIATRICS
Ricli:irdW. Oh,,stcd, edi,o;
Behavioral consequences of congenital rubella Psychiatric and behavioral consequences o f congenital rubella are reported for 243 children studied during the preschool period, and for 205 of these who were re-examined at ages 8 to 9. A t preschool 37% were retarded, with the skew toward severe and profound; 15% had reactive behavior disorder and 7% had atttism. At school age retardation diminished to 25%, but neurotic problems and behavioral pathology due to neurologic damage both increased. There were two remissions and three new instances o f autisnt.
Steila Chess, M.D., Paulina Fernandez, Ph.D., and Sam Korn, Ph.D., New
TIlE
Y o r k , N . Y.
ORIGINALLY
DEFINED
CONSEQUENCES
of
congenital rubella, identified by Gregg t in 1941, have been gradually widened as more organ defects have been identified, especially as the children affected in 1964 were examined in a systematic manner in several centers. 2-' These physical consequences included such effects as fetal loss, neonatal death, failures of immune mechanisms, and cardiac, neurologic, audiologic, and visual defects. As the survivors grew older, behavioral deviations were also identified? Although the expected epidemic, which without an immunization program should have appeared between 1970 and 1974, was apparently prevented,".' there have been a number of rubella epidemics in localized populations of high school age within the past few years. It is uncertain whether these have occurred entirely in those just above the age of the vaccination campaign, or whether some may have received vaccine. If the latter, the questions remain whether the vaccine was always administered properly, or whether it provides only time-limited protection. In addition, a rare sequela similar to Dawson sclerosing panencephalitis of measles has now been identified during adolescence in children with congenital rubella, and recovery of the rubella virus from pathologic tissue has been reported:. 9
From the New York University-Bellevue Hospital Medical Center and lltmter College-City University o f New York. *Reprint address: Department of Ps)'chiatr), New York University,School of Medicine, 550 First Ave., New York, N Y 10016.
0022-3476/78/100699+05500.50/0 9 1978 The C. V. Mosby Co.
MATERIALS
AND METHODS
Our behavioral study of a sample of 243 children with congenital rubella, of whom 205 were seen in both the preschool period (ages 289 to 5) and also at early school age (ages 8 to 9), provides both prevalence and longitudinal data. The children's physical status had been determined in the Rubella Birth Defect Evaluation Project initiated at New York University-Bellevue ltospital Medical Center under Drs. Saul Krugman and Louis Cooper; many of these patients had been followed since birth. The sample included both infants with defects identified at birth and infants without physical defects whose mothers had been reported by obstetricians to have contracted rubella during pregnancy.
See related article, p. 584.
[
As background for the findings on behavioral deviations, the prevalence of physical defect of the 243 children is given as at the time of the initial study at ages 289 to 5 years. The defects counted for the purpose of this study were those which.might have direct influence on behavioral adaptation, namely cardiac, neurologic, audiologic, and visual. Cardiac defects occurred in 79 or 32.5%, neurologic defects in 79 or 32.5%, hearing loss in 177 or 72.8%, and visual problems in 80 or 32.9%. These defects appeared either singly or in combination as follows: no defect in 50 children or 20.6%, one defect area in 72 or 29.6%, two defect areas in 47 or 19.3%, and four defect areas in 27 children or 11.1%. Of those with a single The Journal o f P E D I A T R I C S Vol. 93, No. 4, pp. 699-703
699
700
Chess, Fernandez, and Korn
The Journal of Pediatrics October 1978
Table I. Psychiatric diagnoses of children with congenital rubella ages 289 to 5 years (N = 243)
I N* I %
Diagnosis No psychiatric disorder (normal) Reactive behavior disorder Neurotic behavior disorder Behavorial disorder/neurologic damage Mental retardation Autism
!!8 37 0 8 91 18
49.0 15.0 0.0 3.3 37.0 7.4
"The total N is greater than 243 because of the occurrenceof multiple diagnoses in some cases.
Table
II. Correlation between physical defect and psychiatric disorder in children ages 2 89 to 5 years (N = 243) Number of defect areas Psychiatric diagnosis
,,oelOnelTwolhreelourI ota,
(N) (50) (72)(47) (47) (27) (243) 40 41 24 12 I 118 No psychiatric disorder Reactive behavior disorder 8 14 4 3 1 30 Neurotic Behavior disorder 0 0 0 0 0 0 3 1 4 0 8 Behavior disorder/neurologic 0 damage 2 15 18 31 25 91 Mental retardation 0 5 4 6 3 18 Autism Note: Due to multiple diagnoses,total is greater than 243 subjects. defect, hearing loss, which occurred in 65 youngsters, was the largest category. 5 Psychiatric diagnosis was determined by utilizing all of the behaviorai data available. These included clinical psychiatric history, psychiatric and neurologie examination, psychologic study, and parent and teacher interviews. Behavior disorder was categorized in two ways: (1) psychiatric Classification and (2) number and severity of behavioral symptoms. The psychiatric classification utilized has been described in detail elsewhere. In brief, it is as follows: No psychiatric disorder: Behavior is within expectancy for chronologic age, both developmentally and adaptively. Reactive behavior disorder: Developmental level is age appropriate but behavior problems exist. These can be directly related to such stresses as inappropriate parental handling, other environmental trauma, or both. Expectation is that, were such stresses alleviated, behavioral problems would abate. Neurotic behavior disorder: Developmental level is age appropriate. "Maladaptive behaviors are expressive of
fixed inappropriate attitudes, which require more extensive intervention than altered management in order to achieve rapid improvement. Behavior disorder due to neurologic dantage: (1) Without mental retardation-developmental findings are age appropriate. Symptoms, such as perseveration, correlate with central nervous system damage. Physical findings must include a minimum of one focal or two nonfocal (soft) signs of neurologie damage. (2) With mental retardation-the behavioral findings include cognitive and adaptive retardation but are not entirely explained by this factor. Additional symptoms correlate with central nervous system damage, and physical findings include a minimum of one focal or two soft neurologic signs. Mental retardation: The total adaptive findings provide the basis for this assignment. When feasible, depending upon type and degree of sensory loss, formal testing is included as part of the assessment. The categorization of the American Association on Mental Deficiency provides the subclassification. II Autism: The criteria are those given by Leo Kanner in his original definition of the syndrome." These include failure of affective relaiedness toward people; stereotyped actions; echolalic language or other specified language deviations, such as absence of or peculiar word usage; odd repetitive actions; and unusually strong attachments to objects. "Especially prominent is great distress when changes in the physical environment occur, such as rearrangement of furniture. As can be seen from Table I, during the preschool period approximately half of the children had one or more types of behavior disorder. Fifteen percent had reactive behavior disorder. Fully 37% of the study children were mentally retarded. In contrast to the usual distribution of mental retardation, in which about 95% can be expected to be in the mild category, here the severely retarded comprised the largest proportion. The combination of those who were either severely or profoundly retarded included 43 children, or almost half of the 91 retarded subjects. A very high number of 18, or 7.4%, fell into the category of autism, whereas the expected rate is 0.7 per 10,000.13 The relationship between psychiatric disorder and physical disorder was correlated, as shown in Table II. Forty of the 50 (80%) children who had no diagnosed physical defect had no psychiatric disorder. Eight, an expected prevalence, had reactive behavior disorder. Two were retarded. Of the 72 children with a single physical defect, 41 or 57% were without psychiatric disorder. One child had the single diagnosis of behavior disorder due to neurologic
Volume 93 Number 4
damage; another had the single diagnosis of autism. Fifteen (20%) had mental retardation as a sole diagnosis or in combination with reactive behavior disorder, behavior disorder due to neurologic damage, or autism. In the group of 47 children with two physical defects, about half had no psychiatric disorder. Many of the mentally retarded children were in the moderately or profoundly retarded classification. The 47 subjects with three physical defects had a considerable higher degree of psychiatric pathology. Only one-fourth were free of psychiatric pathology, and only 6% had the mildest type, reactive behavior disorder. The remaining youngsters, with the exception of one autistic child, were retarded, usually to a moderate or profound degree. Several of these had additional symptoms, either of behavior disorder due to neurologic damage (4) or autism (5). With four areas of physical damage, only one child was free of psychiatric pathology out of the total of 27. All others were retarded, most commonly to a severe o r profound degree; three of these had autism as well. In sum, increase in the number of t~hysical defects in congenital rubella brings greater risk of both presence and severity of psychiatric pathology. However, this risk is not a certainty; each group, fro m that with no physical defect to that with four defects, included one or more children with intellectual and behavioral normality. ~ In general, however, the pediatrician who is presented with a child with physical defect resulting from congenital rubella must consider the possibility of accompanying psychiatric casualty. The greater the number of defects, the greater is this possibility. The sample of children was once again examined behaviorally at early school age, 8 to 9 years. It was possible to locate 205 of the original group, and an additional five children of the same age with congenital nabella were added. A few parent s of the original sample refused to have their children rerexamined. Without exception these latter patients were at the two extremes: Parents of those without physical or behavioral consequences preferred to put behind them the thought Of the risk that had occurred during pregnancy, and parents of those with Severe handicaps felt that no help or study would change their burden. Table III shows the prevalence of psychiatric disorder at ages 8 to 9 years. Some important changes occurred after the preschool period. Although the proportion without behavior problems and with reactive behavior disorder had not altered appreciably, there were now 5 or 2.4% whose problem behaviors reflected neurotically fixed inappropriate attitudes (neurotic I~ehavior disorder). This change is devel-
Behavioral consequences of congenital rubella
70 1
Table !!!. Psychiatric diagnoses of children with congenital rubella ages 8 to 9 years (N = 210) Diagnosis
N
%
No psychiatric disorder (normal) Reactive behavior disorder Neurotic behavior disorder Behavior disorder/neurologic damage Mental retardation Autism
98 38 5 26 54 13
46.7 18.1 2.4 12.4 25.7 6.2
opmental. In these children child-environment maladaptive interactions continued over time, and the disordered reactions became fixed and elaborated at a more sophisticated conceptual level. The augmented prevalence of behavior disorder due to neurologic damage also reflects a developmental trend. With increasing age," impulse control normally improves, and repetitive verbalizations and actions, which are appropriate to the preschool age period, should disappear. It is sometimes difficult to diagnose impulsive and repetitive language and behavior until its persistence is identified at school age. Mental retardation, on the other hand, decreased from V~to ',~ of the sample, and eight Of the retardedchildren had a lesser degree of retardation. In children with normal or above normal intelligence in the preschool evaluation, there was also a trehd toward higher intellec!ual functioning at the eight- and nine-Year assessment. Thus, there was an overall tendency for rising cognitive competence despite continuing sensory impairment, even in some children in whom there had been worsening of the hearing impairment. It is possible that in some patients the chronic viral infection was overcome by the body's immune mechanisms, making possible a reorganization of brain function. One may also postula!e that the 8- to 9Year-old children were capable of using intact or residual sensory abilities with growing competence at higher developmental levels, and with increasing cognitive awareness. In other words, they could have been using alternative pathways toward the comprehension of the World and its social patterns and expectations. Thus, the rates and sequences of intellectual development in sensorially normal children may not be an accurate yardstick for prognosticating the future capabilities of preschool children with congenital rubella. Of special interest are the changes that occurred in the children with autism, which is by far the most serious psychiatric disorder of childhood. Although there were 13 such children at ages 8 to 9, as compared to 18 in the preschool period, the longitudinal picture is not reflected in these figures. Several of.the original au!istic children were among those not seen at the older age period,
702
Chess, Fernandez, and Korn
because of parental refusal with such statements as "she cannot be helped." Although one can conjecture that the autistic findings remained unchanged, these children were not in fact evaluated and are not included in the later tabulation. Two of the originally autistic children were found at school age to be completely recovered from their autism. One had normal intelligence despite continued profound hearing loss and monocular cataract. The other continued with the same degree of mental retardation as previously (moderate), but at 8 years had compulsive behaviors that dominated his actions and which he used as the basis for affectively related social interactions. Three new instances of autism emerged; all of these children had had prior behavior disorder but had affecrive social relations at preschool age which contraindicated the diagnosis of autism. At 8 to 9 years of age, all three showed a lack of affective relations and other behavior typical of autism. DISCUSSION In an attempt to condense and select data, physical defects have been grouped as if of equivalent importance. It was in fact found that, at least at the ages studied, cardiac defect was virtually unimportant in any of the correlations examined. Most of the children with cardiac anomalies had been assigned to cardiac classification IA, either in terms of the anatomic lesion or its status after surgical correction. Pulmonic stenosis was the most common lesion reported by the Rubella Birth Defect Evaluation Project. A very small group was classifiedas IB, and in no case had limitation of physical activity been prescribed. There was no direct correlation between cardiac defect as such and the presence of behavior disorder. However, most of the children with cardiac defect had hearing impairment as well. Neurologic impairment occurred in isolation in only one child, and in only two patients was it found paired with cat'diac defect only. Thus, most of the neurologically damaged children also had sensory deprivation, with each defect compromising the child's coping methods for the other. The major motor finding was spasticity; its importance to the child and to the family was determined by whether the child was wheelchair bound or could ambulate with braces or crutches or both, since this determined his degree of participation in ordinary childhood activities. The direct behavioral consequences of brain damage required independent evaluation, inasmuch as some children with central neurologic damage did not have a .behavior disorder. With the increased proportion of such behavior disorder at the later age, the presence of neurologic damage in the preschool period should suggest the possibility that, with increasing social expectations in the
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of Pediatrics October 1978
school-age period, behavioral pathology due to neurologic damage may become manifest. In this sample of children with congenital rubella, visual defect in the absence of hearing impairment occurred only in a small proportio n. The addition of visual difficulties of severe degree to a hearing loss immediately reduced the Options of home management, of educational placement, and of socialization opportunities. Cataract in one eye, while creating in effect monocular vision whether removed or not, did not seem to hinder the child behaviorally. Bilateral cataract, in contrast, made it necessary to place the child in the deaf-blind category and to tap the resources open to such children. Comparisons of rates of autism in other syndromes involving central nervous system damage, deafness, blindness, and mental retardation would be of interest, but data are scant. Rutter, 1~ surveying the total population of children 5 to 14 years on the Isle of Wight, found an overall prevalenc e of behavior disorder of 6.6%, but a 34.3% rate of psychiatric disorder in the brain-damaged group; autism was not separately reported. Rutter '~ also reported that tuberous sclerosis and cerebral lipidosis have been associated with atypical autism. Patients with retrolental fibroplasia have a high prevalence of autistic symptoms, according to Chase, 's although rigorous criteria for this diagnosis are not given. Of a group of 100 consecutively referred mentally retarded children seen in a psychiatric clinic, Philips and Williams ~' found 38 to be psychotically disturbed; autism as such was not separately reported. Although there i s g e n e r a l agreement, as reporte d by Vernon, Is that a higher prevalence of maladjustment exisis in children as a result of congenital rubella, as opposed to those whose deafness is genetic or consequent to Rh incompatibility, precise comparison is not available. Finally, Jan et al ~9 report that, other than children blind as a result of rubella, they have not found impressive numbers with psychosis or autism, and are convinced that such rePorts reflect examiners' inexperience with blind children. A multihandicapped child, as in our congenital rubella group, is often seen b y m a n y specialists. Not infrequently, the parents are bewildered by a host of recommendations which appear (or may in fact be) contradictory. It remains the task of the.pediatrician, as the primary physician, to judge the ~,alidity and relative importance of the different recommendations for management and interventions. The pediatrician can also take into account the total family picture, including the parents' emotional and financial abilities, as well as the needs of the other children. The pediatrician is also in the best position to follow the handicapped child's development over the years and to iden!ify changing capacities. Re-evaluation
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Behavioral consequences of congenital rubella
of defects and of cognitive abilities at each new age-period is essential for flexibility in m a n a g e m e n t and treatment to conform to the changing status of the child. REFERENCES 1..Gregg N: Congenital cataract following German measles in the mother, Trans Ophthalmol Soc Aust 3:35, 1941. 2. Cooper LZ, Green RH, Krugman S, Giles JP, and Mirick GS: Neonatal thrombocy!openic purpura and other manifestations of rubella contracted in utero, Am J Dis Child 110:416, 1965. 3. Krugman S: Rubella-new light on an old disease, J PEDIArR 67:159, 1965. 4. Lundstr6m R: Rubella during pregnancy: A follow-up study of children born after an epidemic of rubella in Sweden, 1951, and additional investigations on prophylaxis and treatment of maternal rubella, Acta Paediatr 5hSupplement 133, 1962. 5. Chess S, Korn S, and Fernandez P: Psychiatric disorders of children with congenital rubella, New York, 1971, Brunner/ Mazel. 6. Krugman S: Present status of measles and rubella immuniration in the United States: A medical progress report, J PEDIATR90:!, 1977. 7. American Academy of Pediatrics; Committee on Infectious Diseases: Measles and rubella immunization, Postgrad Med 61:269, 1977. 8. Townsend J J, Baringer R, Wolinsky JS, Malamud N, Mednick JP, Panitch HS, Scott RAT, Oshiro LS, and Cremer NE: Progressive rubella panencephalitis: Late onset after congenital rubella, N Engl J Med 292:990, 1975.
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9. Well NIL, ltabashi HH, Cremer NE, Oshiro LS, Lennette EII, and Carnay L: Chronic progressive panencephalitis due to rubella virus simulating subacute sclerosing panencephalitis, N Engl J Med 292:994, 1975. 10. Chess S: Introduction to child psychiatry, ed 2, New York, 1969, Grune & Stratton. I1. Grossman HJ: (1973) Manual on terminology and classification in mental retardation, Washington, DC, 1973, American Association on Mental Deficiency. 12. Kanner L: Autistic disiurbances in affective contact, Nervous Child 2:217, 1943. 13. Treffen DA: Epidemiology of infantile autism, Arch Gen Psychiatry 22:431, 1970. 14. Rutter M: Brain damage syndromes in childhood: Concepts -,ad findings, J Child Psychol Psychiatry 18:1, 1977. 15. Rutter M: Language, cognition, and autism, paper presented at the meeting of The Association for Research in Nervous and Mental Disease, Dec. 3, 1977, New York City. 16. 9 Chase JB: Retrolental fibroplasia and autistic symptomatology, New York, 1972, American Foundation for The Blind. 17. Philips J, and Williams N: Psychopathology and mental retardation: A study of 100 mentally retarded children, Am J Psychiatry 132:!265, 1975. 18. Vernon M: Multiply handicapped deaf children: Medical, educational, and psychological considerations, Washington, D.C., 1969, The Council for Exceptional Children, lnc, p 75. 19. Jan JL, Freeman RD, and Scott EP: Visual impairment in children and adolescents, New York, 1977, Grune & Stratton, Inc.
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Most of the provisions of the Copyright Act of 1976 became effective on January 1, 1978. Therefore, all manuscripts must be accompanied by the following statement, signed by each author: "The undersigned author(s) transfers all copyright o',vnership of the manuscript entitled (title of article) to The C. V. Mosby Company in the event the work is published. The author(s) warrants that the article is original, is not under consideration by another journal, and has not been previously published." Authors will be consulted, when possible, regarding republication of their material.
