PSYCHIATRIC DISORDER AMONG CH I LDREN WITH TUBEROUS SCLEROSIS

Ann Hunt Jennifer Dennis

Tuberous sclerosis is a condition know to be associated with psychiatric disorder, particularly with severely socially impaired behaviour (Wing and Gould 1979). Brain lesions such as those found in tuberous sclerosis increase the likelihood of the occurrence of behaviour problems (Rutter et al. 1970). In addition to pre-existing lesions, the majority of

m children handicapped by tuberous b‘ sclerosis will have experienced infantile E spasms (Pampiglione and Moynahan m 1976), a form of epilepsy associated with N behaviour similar to infantile autism

(Kolvin et al. 197 1 , Taft and Cohen 197 1 , Thornton and Pampiglione 1979). Q’

5 The psychiatric outcome for 192 children in Finland who had experienced 2

$ earlier infantile spasms from all causes was evaluated by Riikonen and Amnell z

2 (1981). They identified 24 children who later developed autistic behaviour and d an additional 29 with hyperkinetic P

.- 2 behaviour. In a previous study of 97 children with P

2 tuberous sclerosis, using a postal questionnaire, Hunt (1983) found that

2 parents gave behaviour, including com- 5 munication problems, as the most pressing concern they had about their children. In this present, separate, study parents were questioned in detail about their children’s behaviour and epilepsy.

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Data were then also collected from the medical records of the children. The study aimed to collect as much biographical information about the children as possible to enable us to evaluate a variety of outcomes in the light of antecedent data recorded in the case-notes. Information about the behaviour of children with tuberous sclerosis who had experienced infantile spasms was then compared with that for children in the Riikonen and Amnell study. If the psychiatric disorder was produced only by infantile spasms, an incidence similar to that found by the Finnish study would be expected. If the disorder was more specific to tuberous sclerosis, a higher incidence would be found in this study.

There were also children in the study who had not had infantile spasms but who had had other forms of epilepsy. If the impaired behaviour of children with tuberous sclerosis were not simply the result of the high occurrence of infantile spasms, such behaviour should also be found in this group of children.

Method Study sample This study concerns 89 children and one young adult, all with a confirmed diagnosis of tuberous sclerosis. The sample was defined in 1980 and was

selected from families who were at that time members of the Tuberous Sclerosis Association of Great Britain. The families were selected from geographical areas within a 100-mile radius of Oxford, Manchester, Nottingham, Glasgow and Colchester. These covered 11 of the 15 Regional Health Authorities in England and Wales. The prevalence of tuberous sclerosis is estimated to be 1 in 10,OOO (Hunt and Lindenbaum 1984), so these 90 children represent at least 1 per cent of the population with tuberous sclerosis in Great Britain.

Data were derived from two sources, a family interview and medical case-notes. At the time of interview the children ranged in age from five months to 18 years.

Family interview The interviews took place during 1980 and 1981. We could not find a standardised interview schedule that adequately covered the areas with which we were concerned in a manner appropriate to these often profoundly handicapped children. We devised a 321-item interview schedule which covered family in- formation; the clinical effects of the condition; functional behaviours such as sleeping, feeding and toileting; com- munication and language skills; play and social interaction; habits and behaviour; the impact of professionals’ advice and support; and more open-ended questions about family life. This TS questionnaire followed the form of Hewett (1970) for mothers of cerebral-palsied children, and also the form of the Behavioural Screening Questionnaire of Richman and Graham (1971). In those studies questions concerning behaviour arose naturally from questions about the child’s health and abilities. The TS questionnaire focused on normal aspects of parenting and child-care as a background to the more specific questions about disability.

Interviews were carried out in the child’s own home by the first author and lasted from one to four hours. In addition, the mothers of the 12 youngest children were interviewed again by telephone during 1984 and 1985 about aspects of their children’s behaviour in order to achieve a minimum age of five years at final review.

Medical case-note review All the parents gave written permission for their children’s case-notes to be released to the authors for review. Most had been seen in more than one hospital and so had several sets of case-notes. All hospital records for each child were requested for review between 1982 and 1984, and were obtained for 85 of the 90 children. For a further four children some records were not released, but the doctors in charge completed a detailed question- naire from their own records. For only one child did the doctor in charge feel unable either to release the hospital case- notes or complete the questionnaire, but some partial information had already been obtained from another hospital’s notes.

There was an interval from two to four years for all the children between the interviews and the case-note review. Tuberous sclerosis is a variable disorder and the outcome for any one child cannot be predicted in the first few months of life. The youngest children therefore had their case-notes recalled again during 1984 and 1985 in order to achieve a minimum age of five years at the final review.

Social impairment data The effects of a child with disturbed behaviour on a family cannot be neatly circumscribed in a questionnaire section headed ‘Behaviour’. Valuable insights are also obtained when a mother describes problems connected with such everyday events as sleeping or shopping.

Previous experience of these children with tuberous sclerosis had already shown that many had impaired social behaviour, communication problems, obsessions and hyperactivity (Hunt 1983). In their study of children in Camberwell, Wing and Gould (1 979) found that even in severely mentally handicapped children the specific pattern of childhood autism could be reliably identified among other socially impaired behaviours. Questions aimed at elucidating social impairment and autistic behaviour therefore were included in the TS questionnaire. The equivalent questions used by Riikonen and Amnell to screen for autism, i.e. the criteria proposed by Rendle-Short (Bruce 1967), are compared with the TS questions in

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Appendix 1. In both studies a score of seven out of 13 possible behaviours was taken to indicate that the child might show symptoms of autism. As in the Riikonen and Amnell study, the diagnosis was confirmed from information in case- notes. In this study we chose to evaluate outcome at a single target age-five years-by reviewing total case-note information from doctors, psychologists and teachers. Also, as in the Riikonen and Amnell study, we used the Rutter criteria to define autism (Rutter and Hersov 1977). These are a profound failure to develop social relationships, language retardation, ritualistic or compulsive behaviour and onset of such behaviour before 30 months of age.

The behaviour of the children with tuberous sclerosis therefore could be directly compared with the behaviour of the wider sample of children who had experienced infantile spasms. The two study methods are compared in Appendix 2 and it can be seen that they are very similar.

Developmental level An estimate of the childrens' mental development at five years was also made. Unfortunately very few had standardised developmental tests recorded in their case- notes, so we made general assumptions about mental development from the reports of educational placements at age five. We assumed that children in mainstream education were unlikely to be notably impaired and those with mild or severe learning difficulties would be in appropriate schools. As the majority of the children were severely mentally retarded, those who could neither walk nor talk by five years we regarded as profoundly handicapped. Parents had been asked if their children were in special-care classes within the special school. The number of children who could walk but were in such classes was taken to indicate social impairment caused by their behaviour problems.

Results Psychiatric disorder among children who had had infantile spasms Sixty-nine children with tuberous sclerosis had had infantile spasms, with onset

before 17 months of age. The diagnosis either was taken directly from the case- notes or was made retrospectively from a clear description of seizures recorded at the time. It is this group of children which can be directly compared with the 192 children in the study by Riikonen and Amnell (Appendix 2).

Thirty-seven of these 69 children at the time of interview scored seven points or above on the screening criteria for autism (Appendix 1). They also fulfilled the Rutter criteria for autism at age five. Another three children, although scoring below seven points at the time of interview when they were 14, 15 and 17 years old, had fulfilled the Rutter criteria for autism at age five. Therefore, of the children with infantile spasms, 40 (58 per cent) were considered to have autistic behaviour at five years of age.

Twelve children who had had infantile spasms were so profoundly handicapped that they were unable to walk or talk by five years. It is difficult to rate the behaviour of children who are too handicapped to interact with their surroundings, although the mother of one child described clearly how her daughter became progressively unresponsive as her seizures increased. These children all scored below seven on the TS criteria. One further child was temporarily not walking at age five following a severe bout of seizures, but is not rated as profoundly handicapped. If the 12 profoundly handicapped children are excluded because of lack of behaviour, then 4 0 of 57 children (70 per cent) had autistic behaviour at five years.

Among the remaining 17 children who were mobile but not autistic at five years, three later developed behaviour problems. One boy was obsessional about knives and two others had become increasingly violent as they grew older. One had been excluded from his special school because he was aggressive, destructive and hyperactive. He required a period of inpatient treatment in a psychiatric hospital to modify his outbursts. The other, at 11 years, was reported by a psychiatrist to physically attack his family, urinate against the walls indoors and verbally abuse strangers. He pulled down drips and attacked injured children

TABLE 1 Children with autistic or hyperkinetic behaviour after infantile spasms

Autistic and Survey Autistic Hyperkinetic hyperkinetic

N N % N % N %

Present study 69 40 58.0 41 59.0 32 46.0

Riikonen and Amnell (all causes) 192 24 13.0 29 15.0 16 8.0

TABLE I I Psychiatric disturbance among children with tuberous sclerosis

Psychotic, No behaviour Psychotic and Hyperkinetic hyperactive

problems Psychosis Hyperkinetic hyperkinetic Aggressive and aggressive and aggressive ~~

Infantile spasms (N = 69) Mobile (N = 57) 8 40 41 32 7 7 4 Non-mobile ( N = 12) 12 - - - - - -

Seizures other than infantile spasm (N = 17)

Mobile (N = 15) 4 6 I 1 5 5 5 3 - - - - - 1 Non-mobile (N = 2) I

Mobile (N =4) 4 No epilepsy (N = 4)

Total (N = 90) 29 (32%) 46 (51%) 53 (59%) 37 (41%) 12 (13%) 12 (13%) 7 (8%)

- - - - - -

when he was on a general hospital ward. In addition to autistic behaviour, 41

children (59 per cent) had descriptions in their case-notes of hyperkinetic behaviour. Among those who were mobile, 72 per cent had hyperkinetic behaviour.

Thirty-two of the children (46 per cent) were described as both autistic and hyperactive. These results for behaviour are compared with Riikonen and Amnell’s sample in Table I. The incidence of autistic behaviour among children with tuberous sclerosis is very much higher than would be expected simply as a sequel to infantile spasms.

Aggressive behaviour was reported in seven children who had had infantile spasms. All were hyperactive and four were also autistic. Psychiatric disturbance found in the whole sample is shown in Table 11.

All 40 children with autistic behaviour at five years were attending schools for

children with severe learning difficulties. 24 of them were in special-care units of their schools, as were the 12 profoundly handicapped children. In contrast, only eight of the 17 mobile non-autistic children were in such schools and only one was in a special-care class. Two children attended schools for those with moderate learning difficulties and seven started at ordinary schools, one changing later to a special school. The percentage of children in ordinary schools (9 per cent) is similar to those estimated in the Riikonen study to have an IQ > 85 (12 per cent).

It might be assumed that children with tuberous sclerosis would have developmental delay even before infantile spasms, since there are known neurological abnormalities associated with the condition. They are usually included in the symptomatic group of children in studies of infantile spasms (Lombroso 1983). In the present study 29 of the 40 autistic children (73 per cent) 193

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had normal development, as reported by mothers when they first presented to doctors, and for some this development was recorded for several months afterwards. In the mobile, non-autistic group, 16 of the 17 children (94 per cent) were reported to have normal development before the onset of infantile spasms. We noted that social withdrawal was often an important feature recorded in the notes around the time of onset of infantile spasms. As one mother said about her son, ‘he was no longer being an entertaining baby’.

Psychiatric disorder among children who had not had infantile spasms Four of the 90 children had never had seizures. They were all of normal intelligence and showed no psychiatric disorder.

Psychiatric disorder, including autistic behaviour, was not confined to the children in the survey who had had infantile spasms. 17 children had had other types of seizures, and for six of these psychotic behaviour was clearly described. They scored above seven on the TS Questionnaire criteria for autism. Five of them also fulfilled Rutter’s criteria for autistic behaviour at five years of age.

One boy had been treated for four years in a unit for autistic children before tuberous sclerosis was diagnosed. His case-notes described generalised tonic- clonic seizures and frequent staring absences. He used echolalic speech throughout the taped interview with his mother, and at 13 years old was obsessed with shadow patterns in the garden. Two other children had clear descriptions of autistic behaviour and both had partial seizures. Another severely retarded 12-year-old was hyperactive and wandered about the house during the night. Over the years he had only infrequent episodes of febrile or partial status. He had echolalic and perservative speech and tapping stereotypies. He was also aggressive to his mother, who avoided being alone in the house with him.

One child who scored for autistic behaviour had a history which was in many ways atypical. She had a febrile convulsion at IS months and two further

seizures at age six, but no continuing seizures. Early development was within normal limits and by 19 months old she was using small phrases of speech. However, by 29 months she was reported to be very overactive and miserable, screaming for hours, and with regression in her speech. She was referred to a specialist as ‘mildly autistic’ at 39 months, when she was hitting and biting people and unable to communicate. She began day care in a local mental handicap hospital when the special school could not manage her behaviour, and was admitted to residential care in the hospital at six years. Within the next year she began to smear and eat faeces, pull her hair out and flick her eyes when reprimanded. At eight years old she was transferred to the special-care class in a school for severely handicapped children. At her final assessment recorded at 11 years, interpersonal interactions were sporadic and seldom prolonged. She operated at a 2- to 2Vi-year level, her speech was mainly echolalic and she showed ritualistic, obsessional behaviours and hyperkinesis.

The final child who scored above seven on the TS Questionnaire criteria did not fulfil Rutter’s criteria for autism at age five. She was the only child in the survey for whom we could obtain only partial information. It was recorded that she had begun to have seizures at age six, when her IQ was 92 and she was attending normal school. She developed frank psychotic behaviour at age nine and entered special education. She was referred to a psychiatrist at age 11, when her IQ had dropped to 69. She had extremely negative and bizarre behaviour, and was experiencing distortions of perception and hallucinations. She received no schooling after the age of 12 years, and treatment in an adolescent psychiatric day-unit was stopped when they could not modify her unpredictable and violent behaviour.

The remaining 11 in this group of children did not have psychotic behaviour described in the case-notes and did not score above seven on the screening questions for autistic behaviour. Two of the 11 were non-mobile and profoundly retarded. Six children were described as hyperkinetic (Table 11). Proportionately

more mobile children (33 per cent) showed marked aggression than children in the infantile spasms group (12 per cent).

Psychiatric disorder among the total sample Over-all, 45 of the 90 children (50 per cent) in this study showed autistic behaviour by five years of age. This increases to 59 per cent if only the mobile children are considered. A further child developed psychotic behaviour after the age of five (Table 11).

As shown in Table 11, 53 children were impaired by hyperkinetic behaviour and 37 were both psychotic and hyperkinetic. The amount of severe aggression reported in case-notes was much smaller, but the 12 aggressive children were also hyper- kinetic. Only seven children were reported to be psychotic, hyperactive and aggressive.

There were 29 children who did not have severe behaviour problems, but 13 of these were profoundly mentally handi- capped. Altogether, 62 children (69 per cent) had psychotic, aggressive or hyperkinetic behaviour severe enough to seriously disrupt family life.

Discussion I t is not possible to collect a large sample of children with tuberous sclerosis within any one area of the country. The problems of using families from a patient's association are well-known, since often only those with a severely affected child join such groups. However, this does not apply in the same way to groups representing rare conditions because people often join to get access to information that they cannot obtain otherwise. This produces a membership with a wide variety of concerns, especially in a disease as variably manifested as tuberous sclerosis. There is no absolute marker for the condition, so a true population sample cannot be derived. Our sample, in common with those in all other studies of tuberous sclerosis, is subject to bias.

Half the children in this study developed psychotic behaviour which satisfied agreed criteria for infantile autism. Two-thirds had severe 'behaviour problems which impaired social

m 0' interaction. At five years of age all the

autistic children were in special education ki for the severely mentally retarded. Over half were in the special-care units in those

behaviour further depressed their level of 5

found that 13 per cent of severely retarded mobile children in Camberwell were ; autistic. Over 80 per cent of equivalently 2

2 severely retarded mobile children with tuberous sclerosis in our study have Li autistic behaviour. 2

It is now accepted that autistic children Q2

have a basic cognitive deficit (Rutter

2 1983). Like the term 'epilepsy', 'autism'

cluster of behaviours which may arise c from a variety of causes. Although

implicated in some forms of autistic behaviour, the incidence of such behaviour found in this study (58 per cent) seems far higher than can be attributed to spasms alone. In addition, autistic behaviour was also found among children who had not had infantile spasms, but who had other forms of seizures. However, the social withdrawal which so often occurs at the onset of spasms, and the fact that many of the psychotic children were developmentally normal before their onset, suggests that infantile spasms are in some way implicated in the development of psychotic behaviour of some of the children.

Aggression featured in only 13 per cent of the sample and never occurred in isolation. All the aggressive children were hyperkinetic and most were also psychotic. It is interesting that these children cluster in the non-spasm group. It must be emphasised, however, that the majority of children with tuberous sclerosis are not aggressive.

Many children with tuberous sclerosis have calcified lesions in the periventricular region of the brain, which is involved in the limbic system. These lesions are present from birth and it remains to be resolved whether they are implicated in the cognitive defects which lead to psychotic behaviour.

All the children with autistic behaviour in this study are mentally retarded, but it

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cannot be known to what extent their development has been limited by their avoidance of social contact. A common observation made by parents was that their children could not be taught or cajoled, they only learnt through their obsessions. Although these children are mentally retarded, their behaviour is not that of the majority of retarded children. Many such children, handicapped by other conditions, preserve a vital interest in social interactions, even though profoundly mentally and physically handicapped.

One mother described her feelings about her daughter age five: ‘To me, if I looked through my daughter’s eyes I would think it was like one of those old picture movies, things that flicker around. That must be how their life goes, things flick through and they’ve forgotten it. They can appear to be concentrating on something but all the time there’s a silent movie flickering around in their minds at the same time’. Most paediatricians will have noticed this behaviour in their patients with tuberous sclerosis, but the condition is relatively rare, no one doctor will treat a large group of such patients. As with many infrequent conditions,

APPENDIX 1 Equivalent criteria for diagnosis of autism

TS Questionnaire criteria

1. Does X sometimes seem to dislike physical contact? Screams if cuddled? Yes= 1 No=O

Yes= 1 No=O 2. Does X stay aloof in a group?

3. How does X get on with other children? Ignores/worried bylwrecks games = 1 Plays with = 0

4. Eye contact was coded either from the description of the child by the interviewer at the time of interview, or from information in case-notes. Minimal or no eye contact = 1 No comment, or eye contact = 0

5 . Would you say X is generally under- activehormally active/overactive for age? (Cross-referenced with information on sleeplessness.) Overactive = 1 Underactivehormally active = 0

variations can be assumed to be specific to the child, particularly variations in behaviour, which can be so influenced by family and environment. However, this study shows that the severely disordered behaviour of many children with tuberous sclerosis is a result of the disease. The behaviour problems are far more serious than those that can be attributed to faulty upbringing. The behaviour of the majority of children in this study seriously disrupted family life and their parents needed continuing advice on management problems. Often these problems outweighed those to do with the children’s seizures. Both autistic and hyperkinetic children are recognised to need very specialised educational techniques which differ from those for other children with severe learning difficulties (Rutter 1985, Taylor 1986).

Any baby or small child diagnosed with tuberous sclerosis should be given special educational help as soon as possible to minimise the effects that autistic and hyperkinetic behaviours can have upon their development. Their parents should be given support and guidance to help reduce the shattering impact of such behaviours on family life.

Riikonen and Amnell (Rendle-Short) criteria

I . Not cuddly: either holds himself stiff or clings limply.

2. Aloof manner: little appropriate com- munication with other people: uses them as objects rather than people.

other children. 3. Great difficulty in mixing and playing with

4. No eye contact: persistently looks past or turns away from people.

5 . Marked physical overactivity: child may wake and play for hours during the night, yet be full of energy next day.

6. Do you sometimes wonder if X is deaf?

7. What happens if X wants something?

Yes=l No=O

Leads by hand/stares/points = I Asks normally/pesters/gets for self = 0

What? Yes= 1 (with interpretation as to whether appropriate) No=O

8. Is X very attached to one object?

9. Is there one thing X likes doing over and over again rather than anything else? Does X play with toys as you’d expect or do unexpected things? Over and over again + unexpected play = I Neither criteria occurs = 0

10. Does X have any odd playing habits-such as round and round or finger-flickering? (Cross- referenced with: Is there anything X likes doing over and over again?) Yes= I N o = O

11. Do you have to introduce new things/places to X gradually or does X willingly accept change? Gradually = 1 Accepts change = 0

co-operate or strongly resist? Resist = 1 Co-operates = 0

etc.? Yes=O N o = I

12. When you want to teach X something does X

13. Does X have an idea of danger-traffic, water,

Total score 7t regarded as autistic behaviour.

APPENDIX 2 Samples

Riikonen and Amnell (N = 192)

All children in Helsinki diagnosed with infantile spasms.

Birth 1960-1976.

Age at follow-up for case-notes 3-19 yrs.

Questionnaire 1978-79 Postal questionnaire to parents, using Rendle-Short criteria for autism.

Retrospective case-note study.

Interview with parents of 33 children assessed to have autism.

All information on 33 children assessed by Rutter criteria for autism identifies 26 children who were autistic at any age.

No constant age-sample chosen.

Transient autistic behaviour reported in 14/24 children up to age 19 yrs.

6. Acts deaf: no reaction to speech or noise.

7. Prefers to indicate desires by gestures: speech may or may not occur.

8. Inappropriate attachment to particular objects and preoccupation with details or characteristics of them, without regard to their accepted function.

9. Repetitive and sustained odd play with objects, e.g. flicking pieces of string, rattling stones in tin, teasing paper. (This is taken to refer to perseverative non-constructive play and nor to spinning, twirling or other voluntary movements of objects or self.)

10. Spins objects, especially round ones: can become totally absorbed in this activity and distressed if interrupted. (This is interpreted as including all rotational stereotypies, whether of objects or self, and finger-flickering.)

11. Resists change in routine: change in the smallest thing may precipitate acute, excessive or seemingly illogical anxiety; e.g. child rejects new or all but a few foods.

12. Strong resistance to any learning, either new behaviour or new skills.

13. Lack of fear about realistic dangers, e.g. may play with fire, climb dangerous heights, run into busy road.

Total score of 7+ regarded as autistic behaviour.

Present study (N = 90)

Children with tuberous sclerosis from membership of Tuberous Sclerosis Association.

Birth 1956-1981.

Age at follow up for case-notes 2-26 yrs. (repeat at 5 yrs. for infants).

Questionnaire 1980-81 Interview with parents, including questions on equivalent criteria for autism (Appendix I) .

Retrospective case-note study.

Part of original interview.

All information in 90 children assessed by Rutter criteria for autism identifies 40 children who were autistic at 5 yrs.

Outcome at 5 yrs. chosen.

Transient autistic behaviour reported in 3/40 children after 5 yrs. and up to 26 yrs.

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Accepted for publication 8th May 1986. Authors’ Appointments *Ann Hunt, B.A., Research Worker;

Ackno wledgement Jennifer Dennis, D.M., D.C.H., Associate We thank all the doctors who kindly let us see their Specialist in Childhood Mental Handicap; Human hospital records and the parents who allowed us to Development Research Unit, Park Hospital for seek the information from the start. The study was Children, Old Road, Headington, Oxford OX3 supported by grants from the Simon Yudkin Trust 7LQ. and the Tuberous Sclerosis Association of Great Britain. *Correspondence to first author.

SUMMARY The psychiatric status of 90 children with tuberous sclerosis was investigated. Over 50 per cent of the sample showed psychotic behaviour, 59 per cent were hyperkinetic and 13 per cent severely aggressive. 57 per cent of the children who had infantile spasms were autistic, as opposed to 13 per cent in a Finnish study of children who had infantile spasms from all causes. The very high incidence of psychiatric disturbance has implications for the advice doctors should give on the management of children diagnosed to have tuberous sclerosis and for the special educational methods that may be needed.

RLSUME Troubles psychiatriques chez les en fants prdsentant une scldrose tubdreuse L’etat psychiatrique de 90 enfants presentant une sclkrose tubereuse a 6th Ctudie. Plus de 50 pour cent de I’Cchantillon presentait un comportement psychotique, 59 pour cent prtsentait une instabilite psychomotrice et 13 pour cent une agressivitk severe. 57 pour cent des enfants ayant eu des spasmes infantiles etaient autistiques alors qu’une etude finlandaise d’enfants ayant eu des spasmes infantiles de causes diverses ne montraient que 13 pour cent d’autisme. L’incidence tres Clevte de troubles psychiatriques a des implications sur les conseils que les mtdecins peuvent donner pour I’approche des enfants chez qui un diagnostic de sclkrose tubereuse est porte et pour les methodes educatives specialisees qui peuvent Stre ntcessaires.

ZUSAMMENFASSUNG Psychiatrische Erkrankungen bei Kindern mit tuberoser Hirnsklerose 90 Kinder mit tuberoser Hirnsklerose wurden psychiatrisch untersucht. Uber 50 Prozent hatten psychotische Verhaltensweisen, 59 Prozent waren hyperkinetisch und 13 Prozent waren hochgradig agressiv. 57 Prozent der Kinder, die Neugeborenenkrampfe hatten, waren autistisch. im Gegensatz zu nur 13 Prozent in einer finischen Studie uber Kinder mit Neugeborenenkrampfen verschiedener Ursache. Durch das sehr haufige Auftreten von Verhaltensstorungen mussen die Arzte entsprechende Empfehlungen fur die Betreuung von Kindern mit tuberoser Hirnsklerose und fur die speziellen Unterrichtsmethoden, die eventuell erforderlich sind, geben.

References Bruce, A. (1967) ‘Infantile autism.’ New Zealand

Medical Journal, 66, 895-897. Hewett, S. (1970) The Family and the Handicapped

Chi/d. London: George Allen Kt Unwin. Hunt, A. (1983) ‘Tuberous sclerosis: a survey of 97

cases.’ Developmental Medicine and Child Neurology, 25, 346-357.

- Lindenbaum, R. H. (1984) ‘Tuberous sclerosis: a new estimate of prevalence within the Oxford region.’ Journal of Medical Genetics, 21,

Kolvin, I., Ounsted, C., Roth, M. (1971) ‘Studies in the childhood psychoses. V; Cerebral dysfunction and childhood psychoses. British Journal of Psychiatry, 118, 407-414.

Lombroso, C. T. (1983) ‘A prospective study of infantile spasms: clinical and therapeutic correlations.’ Epilepsia, 24, 135-1 58.

Pampiglione, G., Moynahan, E. J. (1970) ‘The tuberous sclerosis syndrome: clinical and EEG studies in 100 children.’ Journal of Neurology, Neurosurgery and Psychiatry, 39, 666-613.

Richman, N., Graham, P. J. (1971) ‘A Behavioural Screening Questionnaire for 3-year-old children.’ Journal of Child Psychology and Psychiatry, 12, 5-33.

Riikonen, R.. Amnell, G. (1981) ‘Psychiatric disorder in children with earlier infantile spasms.’ Developmental Medicine and Child Neurology,

272-277.

23, 747-760. Rutter, M. (1983) ‘Cognitive deficits in the

pathogenesis of autism.’ Journal of Child Psychology and Psychiatry, 24, 513-53 I .

- (1985) ‘The treatment of autistic children.’ Journal of Child Psychology and Psychiatry, 26,

- Hersov, L. (1977) Child Psychiatry: Modern Approaches. Oxford: Blackwell Scientific.

- Graham, P., Yule, W. (1970) A Neuropsychiatric Study in Childhood. Clinics in Developmental Medicine Nos. 35/36. London: S.I.M.P. with Heinemann Medical: Philadelohia:

193-2 14.

Lippincott. pp. 7-8. Taft, L. T., Cohen, H. J. (1971) ‘Hypsarrhythmia

and infantile autism: a clinical report.’ Journal of Autism and Childhood Schizophrenia, 1,

Taylor, E. A. (Ed.) (1986) The Overactive Child. Clinics in Developmental Medicine No. 95. London: S.I.M.P. with Blackwell Scientific; Philadelphia: Lippincott .

Thornton, E. M., Pampiglione, G. (1979) ‘Psychiatric disorders following infantile spasms.’

327-336.

Lancet, 1, 1297. Winn. L.. Gould. J. (1979) ‘Severe impairments of

social interaction and associated abnormalities i; children: epidemiology and classification. Journal of Autism and Developmental Disorders, 9, 11-29.

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