71

Educational Attendance of Handicapped Chil­dren in the Suburban Area of Tokyo Megumu Tohjo, MD, Masa Ohtsuka, MSW and Matao Nagase,MD Division of Child Neurology, National Musashi Re­search Institute for Mental and Nervous Diseases, Tokyo (MT, MO); School Health Service Center, Tokyo Gakugei University, Tokyo (MN)

Education of handicapped children is an important matter for their parents or guardians. In order to provide educational counselling for handicapped children, especially the mentally handicapped, it is necessary to obtain detailed information about their educational environment and tuition states.

Method Questionnaires were sent to the parents or guardians of handicapped children in the suburban area of Tokyo. Thirty-six replies were received. Thirty-two of the handicapped children were diagnosed as being mentally retarded; 13 had simple mental retardation, 5 were autistic, 2 had Down syndrome and 2 were unspecified. Also 2 children were severely mentally handicapped, 6 had cerebral palsy and 8 had other problems. The questionnaires, specifically included the following points; parent's ideas about the educa­tional requirements of their children, the teacher's attitude to handicapped children in normal-grade schools, and whether any changes in the children's emotional or mental states had been discerned.

Results and Discussion In most cases the school administrator had advised the parents to place their handicapped children in special schools regardless of the parent's ideas or wishes. Thirty-three handicapped children (92%) had been progressing harmoniously with normal children in regular classes. The teachers in the regular classes showed a warm encouraging attitude to these children's education. The mentally handicapped children became adjusted to the regular classes with their friends and improved mentally and socially. Twenty-four parents felt that satisfactory educational progress was made. But three expressed concern about the poor understanding of the teachers of the prob­lems of mentally handicapped children. Judging from these results, we suggest that mentally handicapped children should be placed in regular school classes after careful assessment of their medical, educational and social needs. And a satisfactory educational environment should be created in accordance with medical and educational staff, and parents.

Key words: Handicapped children, mental retardation, normalization, mainstreaming.

182 Brain & Development, Vol 6, No 2, 1984

72

Early Signs of Spastic Diplegia: Comparative Analysis on Video Recording Eiji Kitazumi, MD, Shouko Enomoto, MD, Akiko Yokochi, MD, Kenji Yokochi, MD, Tomoko Uchida, MD and Kazuo Kodama, MD Department of Pediatrics, National Rehabilitation Center for Disabled Children (Seishiryougoen) , Tokyo

Early characteristic signs of spastic diplegia were analyzed. Eleven cases of spastic diplegia who first visited our clinic under the corrected age of eight months were studied in comparison with 15 control cases. Control cases were those who showed signs indicating spastic diplegia such as an extending tenden­cy of lower limbs, hypertonus of lower limbs, plantar flexion of feet and so on in early infancy, but did not develop spastic diplegia. The video recordings of spastic diplegia cases and control cases at under the age of eight months were comparatively reviewed, and the distinctive differences between the diplegia and control groups were analyzed. The results were as follows.

(A) The differential signs of spastic diplegia can be summarized as follows;

1) Lack or marked poverty in segmental inde­pendent movement and posture of hip, knee and ankle joints isolated from each other in the same lower limb; for example, dorsiflexion of the ankle with the hip and knee kept in the extended posture, or flexion-extension of the knee independent of the posture of the hip. Such isolated segmental motor patterns could be seen at early months in the control group, but were completely absent or markedly poor in the spastic diplegia group.

2) Lack of variable motor pattern in hip joints. In the spastic diplegia group, hip joints could hardly take a posture other than either flexion in abduction or extension in adduction . Transitional movement of thighs from abduction to adduction or vice versa with the hip kept in the flexed position could be observed even at early months in the control group, but could not be seen in the diplegia group. In the supine pos­ture, control cases could lift their legs and pull their feet up over the plane made with the thigh and trunk, but in the diplegia group such a feet pulling up pattern could hardly be observed, which seems to be due to the inability of rotation of the thigh with the hip kept in the flexed position.

(B) No significant difference between the diplegia and control groups was noted in the following signs; extending tendency of lower limbs, poverty of move­ment of lower limbs, hypertonus of lower limbs, plantiflexion of feet, hyperreflexia, ankle clonus and arm fisting. The average abnormal score of Vojta's seven postural reactions was lower in the diplegia group than in the control group, and · over half of the diplegia cases did not fulfil Vojta's criteria for early treatment in contrast with that over half of the con­trol cases fulfilled the criteria.

Key words: Cerebral palsy, spastic diplegia, early diagnosis, Vojta method.

73

Clinical Features for Developing Cerebral Palsy Toyoko Kanda, MD, Mariko Yuge, MD, Junko Suzuki, MD, Yuriko Yamori, MD and Hiroshi Fukase, MD Department of Neuropediatrics, St Joseph Hospital for the Handicapped, Kyoto (TK, MY, YY); Depart­ment of Neuropediatrics, Pediatric Orthopedic Center, Shiga (JS); Department of Orthopedics, St Joseph Hospital for the Handicapped, Kyoto (HF)

Up to now cerebral palsy could not be diagnosed before 6 months of age according to Bobath. How­ever, in 1974, Vojta presented "Disturbance of Central Coordination" (CCD) for the purpose of checking high risk infants prone to developing cerebral palsy before 6 months of age. He also presented each characteristic regarding the development of the spastic or athetotic types of cerebral palsy in order to clarify for what reasons babies should start treatment after 3 months of age.

We examined the characteristic signs of developing cerebral palsy proposed by Vojta at the first examina­tion and then studied their agreement with the con­dition at present. Also we examined head circum­ference and CT findings .

Materials and Methods We selected 300 cases at random out of those who were checked for the first time before 1 year of age and showed more than 6 abnormal postural reac­tions out of 7 postural reactions proposed by Vojta. The 300 cases were divided into 3 groups according to their present condition as follows; cerebral palsy group (CP group) (100 cases), trained group (100 cases), and normalized group (100 cases) . The CP group included those who had cerebral palsy at that time with or without training, the trained group in­cluded those who did not develop cerebral palsy with training for more than 6 months, and the normalized group included those who were normalized without treatment or with treatment for less than 6 months.

Based on the clinical signs at the first check the patients were divided into 5 groups. One group showed spastic signs, the second group athetotic signs; the third group, the mixed type and both signs were strongly positive, the fourth group ; the premature type, had both signs only slightly and it could not be decided which type they belonged to; and lastly, the fifth group showed no significant sign so they could not be placed in any type. Head circumference (HC), at the first check, was corrected in premature babies and divided into 4 groups: (1) HC < mean - 2SD, (2) mean - 2SD ~ HC < mean, (3) mean ~ HC ~ mean + 2SD, and (4) HC > mean + 2SD. CT findings were divided into 4 groups; none, slight, moderate and severe. CT was performed after 3 months of age (corrected) as soon as possible after the first check.

Results 1) In the CP group 93 cases (93 %) showed characteris­

tic signs for developing cerebral palsy, in the trained group 23 cases (23%) showed them and in the normalized group none showed them. In the CP group all 7 cases who showed the premature

type of clinical signs were checked between 0 and 2 months of age.

2) In the CP group 35 cases (36.5%) showed micro­cephalus and in the other 2 groups none showed it.

3) In the CP group 24 cases (26.1 %) showed severe abnormality in CT findings, in the trained group 1 case (1.1 %) showed such CT findings and in the normalized group none showed them.

Conclusion The clinical characteristics for developing cerebral palsy proposed by Vojta were useful criteria for deciding the reason for starting treatment. Head circumference and the degree of abnormality in CT were also useful.

Key words: Cerebral palsy, Vojta.

74

An Investigation of Cerebral-Palsied Children in Regular Classrooms Hideki Gakuhari, MA and Kazuo Kodama, MD National Rehabilitation Center for Disabled Children (Seishi-Ryogoen) , Tokyo

This study intended to clarify actual states and prob­lems of Cerebral-Palsied children who were enrolled in regular classes of primary schools, and to facilitate the development of integration of disabled children.

Methods SUbjects: 50 CP children (aged 6-12 years). Methods: Visiting their schools; gathering informa­

tion by means of questionnaires given to the teachers in charge and the parents.

Results 1. The children mainly had spastic diplegia or hemi­plegia. Most of the cases were able to use at least one hand and had no serious problems in writing and com­munication. Their mobility was not considered. 2. Their parents wanted their children to study in able-bodied children groups, and recognized the great value of their children's social development. 3. As for schools, there were several requirements for admission of CP children, such as aides. Eighty-four percent of the schools took care of and devised their equipment, such as bathroom reconstruction to west­ern style, installation of handrails, etc. 4. The teachers in charge judged that the CP children made progress in their moral education. The teachers made great efforts, but most of them did not grasp the situation. 5. The regular schools valued the children's achieve­ments. If the children showed high achievement, the schools thought that they could cope with the ADL.

Discussion: The problems pointed out in this study were as follows: 1) TIle integration of disabled children depends not

only on the children's disability but also the phys­ical condition and attitudes at the schools.

2) We must approach the integration of handicapped children from these two sides.

Key words: Cerebral palsy, integration, education.

Brain & Development, Vol 6, No 2, 1984 183

75

A Study on the Pathophysiology of Sudden Death in Severely-Handicapped Children Chikahiko Kono, MD, Eiji Oka, MD, Hideo Inoue, MD, Susumu Miyake, MD, Kuniaki Iyoda, MD and Shunsuke Ohtahara, MD Department of Developmental Neuroscience and Child Neurology, Institute for Neurobiology, Okayama University Medical School, Okayama

Sudden death is the main cause of death in severely­handicapped children. Noticeably, among 18 deaths experienced during 4 years in an institution for severely-handicapped, all 4 cases of sudden death oc­curred during sleep in the most severely-handicapped group of children with tube feeding.

To clarify the pathophysiology of sudden death, a neurophysiological study was conducted on cases with a comparable degree of disability.

Subjects and Methods All-night polygraphy, auditory brainstem response (ABR), photoevoked eyelid microvibration (MV) and CT were examined in 14 severely-handicapped chil­dren; 6 males and 8 females aged from 4 to 14 years.

Results 1) In 2 cases, apneas of over 10 seconds' duration

were observed more than 30 times during 7 hours of EEG recording. Apneas observed in these 2 cases were mainly of the central type. These cases comprise the apneic group.

2) No correlation was noted between the apnea and the degree of developmental differentiation of sleep stages.

3) Percentage stage REM (%REM) was decreased in 13 cases including the 2 apneic cases.

4) Decreases in respiration and pulse rates during stage REM, and in the REM density were char­acteristic of the apneic group.

5) Normal ABR responses were seen in 7 cases and only a delay in the latency of wave I in 3 cases of the 12 non-apneic cases, while the disappearance of wave V was noted in one and no response in the other apneic case.

6) No consistent response was noted in MV in both apneic and non-apneic cases; there was a normal response in 11 cases including one apneic case and no response in 3 cases.

7) Among the 14 cases, CT revealed brainstem ab-normality in 7 including both apneic cases.

Conclusion In the 2 apneic cases various findings suggested organic and functional abnormalities in the brainstem. Among severely-handicapped children, apneic cases probably have a specific brainstem lesion.

Key words: Sudden death, severely-handicapped chil­dren, brainstem, sleep, AER.

184 Brain & Development, Vol 6, No 2, 1984

76

Possible Risk Factors and Signs of Mental Retardation: Comparative Study of Mentally Retarded Children and Controls Toyojiro Matsu ish i, MD, Kouko Yoshimura, MD, Akira Yoshida, MD, Fumio Yamashita, MD and Yuko Shiotsuki, MD Department of Pediatrics, Kurume University Medical Center, Kurume, Fukuoka (TM, FY, YS); Kurume Health Center, Kurume, Fukuoka (KY); Department of Public Health, Kurume University Medical Center, Kurume, Fukuoka (A Y)

A retrospective survey was performed to compile information useful for the early detection, intervEa.· tion and prevention of mental retardation (MR) in children.

Methods Based on the prevalence rate of MR in Kurume City, with a popUlation of 220,000, which was reported in our preliminary report, the results of a question­naire for MR children (N = 171), which were com­pared with those for control children without MR (twice the number of MR children matched for age and sex, N = 342) to determine risk factors and signs of MR. The questionnaire consisted of 57 items. Mother or caretakers of MR children were asked to fill out the questionnaire. MR children were classified by the clinical diagnosis into 4 groups: Group A (etiology unknown, N = 74); Group B (with cerebral palsy, N = 15); Group C (Down syndrome, N = 26); and Group D (autistic children, N = 28). These groups were compared with the control group. The x 2 test was utilized for statistical analysis, and Fisher's test was used for a small number (N < 10). Thirty-three percent of the questionnaires were returned.

Results Significant difference between MR children and control children in the incidences of items were as follows in order of frequency: (1) Asphyxia, (2) birth weight of less than 2,500 g, (3) poor sucking, (4) poor activity, (5) dull responses to a human voice, (6) dif­ficulties in eye to eye contact, (7) delayed head control (more than 5 months after birth), (8) delayed walking with support (more than 10 months after birth), (9) delayed walking alone (more than 1.5 yars old), and (10) delayed onset of saying significant words (more than 1.5 years old) (p < 0.005). In addi­tion, the following items showed significant higher incidences in the MR compared with controls: (1) Mothers aged over 30 years, (2) infants who used an incubator, (3) babies given oxygen inhalation, (4) prolonged delivery, (5) pregnant mothers with X-ray irradiation, (6) "too quiet babies", (7) babies hyper­sensitive to sounds, (8) hypertonus, and (9) frequent crying without reason (0.01 < p < 0.05).

Key words: Mental retardation, risk factor, risk sign.

77

The Prevalence Rate and an Etiological Survey of Mental Retardation (MR) in Japanese School Children Yuko Shiotsuki, MD, Toyojiro Matsuishi, MD, Kohko Yoshimura, MD, Fumio Yamashita , MD, Kazuo Yano and Hiroshi Tokimasa Department of Pediatrics, Kurume University Medical Center, Kurume, Fukuoka (YS, TM, FY); Kurume Health Center, Kurume, Fukuoka (KY); Th e Institute of Education for Preschool Children, Kurume, Fuku­oka (KY, HT)

For the purpose of early detection and interventiop in mentally handicapped children, a committee w~s organized in 1981 and sponsored by Kurume City. The committee performed the survey to ascertain the prevalence rate and etiology of MR children who werf born between 1969 to 1974 in Kurume City . '

Methods In this study the designation MR was based on the measured IQ, following the recommendations of thje WHO in 1968: MR was defined here as being assoc~­ated with an IQ below 70 by the WISC or Binet test. IQs below 50 were all classified as severe MR, and IQs between 50 to 70 classified as mild or moderate MR. The study population comprised all children born iin 1969-1974, i.e., 7-12 years of age, living in Kururrie City, amounting to 21,622 children . Kurume City is a middle sized city in Japan with about 230,000 inhabitants. The etiology of MR was classified by t~e MR monitoring research committee sponsored by the Japanese Ministry of Health and Welfare (1981). The prevalence rate of MR is expressed as the ratio to 1,000 (1,000 children without MR, living in the ciW in the corresponding year). '

Results 1) 154 MR children were born between 1969 and

1974 in the city, and the prevalence rates ranged from 5.7/1,000 to 8.3/1,000 in each year group, with an average of 7.1/1,000.

2) Among the 154 MR children, 109 were male (9.8/ 1,000) and 45 females (4.3/1,000), giving a male excess ratio of 2.4 : 1. This sex difference was significant (p < 0.05) .

3) The causes were classified as follows ; prenatal (12%); perinatal (3.2%); complicated LFD and asphyxia, etc (22.7%); postnatal (occurred later than 5 weeks after birth) (11 %); infantile autism (13 .6%); indeterminate factors (familial, etc) (9 .1%); unknown factors (18 .8%); and uncertain information (9.7%).

Key words: Mental retardation, prevalence rate, etiology.

78

A Statistical Study on Resident Deaths at Institutions for the Profoundly Retarded Hiroshi Nakamura, MD, Tadanori Honda, MD, Isamu Uemura, MD, Yoshio Nanba, MD, Katsumi Mita, PhD and Kitoku Okada, MD Department of Pediatrics, National Ashikaga Hospital, Ashikaga, Tochigi, (HN); Murasaki Aiikuen, Tokyo (TH); Nogiku Ryoikuen, Naka-cho, Hyogo (IU); Hiro­shima Prefectural Colony, Higashi-hiroshima, Hiroshima (YN); Aichi Prefectural Colony, Aichi (KM, KO)

The profoundly retarded are defined in Japan as those who have double handicaps of severe mental retarda­tion and severe motor disturbance.

Materials and Methods The authors asked 48 institutions which have been provided by law for the profoundly retarded to reply with regard to resident deaths, and thus obtained various information from 46 institutions concerning 1,424 cases who had died from 1962 to 1982.

Results The mortality per year of residents had been 3.5-4 .6 percent until 1969. Slight decreases (approximately 2 percent) were noted in recent years after 1970. The highest mortality rate was in the 7-9 years age group, followed by the 10-12-year olds. Among the cases, 50 and 90 percent had died by the ages of 12 and 24 years, respectively . Half of the cases died within 3 years after admission . Many cases survived for only a short time after signs of abnormality were detected and many cases died suddenly . Disease of the respi­ratory system was suspected to be the commonest cause among the etiological events which resulted in death, and that of the circulation system the second. With regard to basic diagnosis of handicaps and level of retardation, 60 percent of the total cases had cerebral palsy , and no less than 70 percent of the dead cases had been assumed to be in Group 1 (the most profoundly retarded) according to the classification of K Ohshima.

Conclusion Characteristics of the death of the institutionalized profoundly retarded are as follows :

The younger and the more severe (particularly in motor disturbance) residents are, the earlier in age they are apt to die, occasionally within a short period after abnormal signs are observed:

Key words: The profoundly retarded, resident deaths, mortality at institutions.

Brain & Development, Vol 6, No 2, 1984 185

79

On the Development of Mental Activity in the Monozygotic Twins with Early Infantile Brain Injury Youko Hasegawa, MPH, Ryoho Okada , MD and Keizo Tachi,MD Department of Pediatrics, Kanazawa Medical Univer­sity, Ishikawa

We studied mental activity on 21 paired infants of 13 monozygotic twins and 8 dizygotic twins with rigospasticity and opisthotonic posture by using MCC test, and conducted follow-up for two or three years.

1) Almost all cases of monozygotic twins with cerebral symptoms showed low mental age (DQ of 50 to 60) in early stage of infantile period.

2) As a result of physical training (V ojta method) or medication, each pair of the monozygotic twins with handicap displayed their abilities. And the level of mental activity also was elevated and reached normal mental age by the treatment in most twins.

3) No difference in the mental activity between the each monozygotic twin could be observed lately with some exception.

4) The monozygotic twins with extreme low birth weight of under 1,500 g and neonatal hypocalcemia, hypoglycemia and hyperbilirubinemia showed marked delay of mental development. The difference of mental activity levels between the each twin was changed remarkably by aging, and the IQ difference was increased over the value of 30 at the age of three years.

5) The different mental activities between the each monozygotic twin in the all cases delivered before 34 fetal weeks showed remarkably than the twin born after the 35th week.

Key words: Monozygotic twin, MCC test, premature infant.

186 Brain & Development, Vol 6, No 2, 1984

80

Etiological Aspects of Epilepsy and Febrile ConVUlsions-Pregnancy and Perinatal Factors Yukihiko Katafuchi, MD, Nobuyuki Aoki, MD, Toyo­jiro Matsuishi, MD and Fumio Yamashita, MD Department of Pediatrics, Kurume University School of Medicine, Kurume, Fukuoka

The possible significance of abnormalities during pregnancy, labour and delivery, and the neonatal period which could have caused brain damage was studied as to their being high risk factors for children with epilepsy and febrile convulsions.

A comparison was made between 91 children with epilepsy and 52 with febrile convulsions who visited Kurume University Medical Center as out-patients in 1980, and 67 children as controls who had neither neurological abnormalities nor convulsive disorders. Results of cranial computed tomography were also compared between the children with epilepsy or febrile convulsions and controls.

Detailed histories during pregnancies and pe"rinatal periods were obtained from each mother with refer­ence to a maternity passbook or obstetrical record. The factors were abnormality during pregnancy, gesta­tional ages of < 37 weeks and > 43 weeks, birth weights of < 2,500 g and > 4,000 g, light for date infants, abnormal delivery, asphyxia, severe jaundice, neonatal feeding difficulties, neonatal seizures, other neonatal abnormal signs (Le. vomiting, irritability), a maternal age of > 30 years, consanguinity and family history of a convulsive disorder. These factors were examined statistically by the X 2 test.

High risk factors for epilepsy (p < 0.05) were six of the above items, especially abnormal delivery (20 .9%), family history of a convulsive disorder (15.4%) and asphyxia (13 .2%) showed a high inci­dence. Seventeen of the 91 epileptic children with mental retardation showed seven significant high risk factors (p < 0.05); asphyxia (37.5 %), abnormality during pregnancy (23.5%), neonatal abnormal signs (17 .6%) and a gestational age of < 37 weeks (12.5%).

High risk factors for febrile convulsions (p < 0.05) were six e.g. a birth weight of < 2,500 g (11.5%), family history of a convulsive disorder (15.8%), a gestational age of < 37 weeks (7.7 %) and asphyxia (7 .7%). Although divided into two categories (Fuku­yama 1963): A. simple febrile convulsions, 21 cases (40.4%) and B. complicated, 31 cases (59.6%); Group A had only two high risk factors and Group B had five .

Of the 62 epileptic children who were examined by computed tomography, 13 cases (21%) showed abnormal findings (cortical atrophy and/or ventricular dilatation). Also 2 cases of febrile convulsions (13.3%) had abnormalities. In epileptic children, the incidence of abnormalities was statistically higher compared with controls (p < 0.05).

In conclusion, pregnancy and perinatal risk factors which could have caused brain damage were closely related with the etiology of convulsive disorders.

Key words: Epilepsy, febrile convulsion, etiological aspects, brain damage, pregnancy and perinatal risk factors.