Cerebral palsy and handicapped

63 Dendritic Arborization of Cortical Neurons in Severely Handicapped Children Shigeru Ohta, MD, Tsunekazu Yamano, MD, Yoshiaki Abe, MD, and Morimi Shimada, MD Department of Pediatrics, Shiga University of Medical Science, Ohtsu, Shiga This study was undertaken to elucidate the maturation and plasticity of the cortical neurons in severely mentally and physically handicapped children.

Materials and Methods The precentral gyrus of six autopsied severely handicapped children, 4 , 6, 7, 8, 12 and 16 years old, were collected. Five of them were considered as having had brain damage during gestation and/or the perinatal period, and one was Hunter syndrome. Small tissues were stained either by the Golgi-Cox or rapid Golgi method, and were cut serially into 150}Jm sections. The ramification and extension of the pyramidal cells in layers III and V were observed and carefully traced on plastic film using a projection microscope. The precentral cortex of children who had died of various diseases other than neurological disorders were used as a normal control. Results Ramification and extension of dendrites of the pyramidal cells in layer III of handicapped children were significantly impaired as compared with those of controls. Decreases in dendritic arborization of the pyramidal cells in layer V were not so remarkable compared with those of layer III. However, total ramification and extension of the pyramidal cell dendrites in layer V were significantly decreased in the handicapped children. Discussion The dendritic arborization of the pyramidal cells in layer III were significantly involved in handicapped children. As the cause of these involvement, the laminar necrosis subsequent to neonatal asphyxia, defect of reciprocal neuronal induction and/or secondary nutritional impairment were considered . Key words: Dendritic arborization, handicapped children, Golgi-Cox method.

64 The Electroencephalogram (EEG) and Auditory Evoked Response (AER) of Double Handicapped Infants and Adults

Kazuo Matsuzawa, MD, Shoji Seki, MD, Hideki Fujita, MD, and Yoshi Yano, MD Department of Neuropsychiatry, School of Medicine, Gunma University, Maebashi, Gunma (KM, SS); Department of Neuropsychiatry, Takasaki National Hospital, Takasaki, Gunma (HF); Kibonoie Ryoiku Hospital, Omama, Gunma (YY)

We examined auditory brain stem responses (ABR) and slow vertex responses (SYR) of 27 double handicapped patients, recording polygraphs for them simultaneously, and tried to understand their physiopathological function in the brain. The polygraphs showed 13 paroxysmal discharges, 7 abnormalities of sleep spindles on the electroencephalogram, and 2 respiratory disorders on the polygraph. We checked the latencies and the amplitudes of each component of ABR and tried to determine whether the abnormalities of ABR were caused by hearing disorders or by neurological disorders, considering the threshold of wave V in ABR and the threshold of SVR to the minimal sound stimuli. We divided the abnormalities of ABR into three groups with consideration of the latency and the amplitude as follows: 1) A-group; both the latency and the amplitude were normal, 2) B-group; both the latency and the amplitude were abnormal , or no ABR appeared at 95 dB, and 3) C-group; either the latency or the amplitude alone was abnormal, or the latency of wave V was prolonged although that of wave I was normal. In conclusion, A-group was regarded as a normal hearing group due to both the normal threshold of wave V in ABR and of SVR, B-group was considered to have hearing disorders because of both the high threshold of wave V in ABR and of SVR, and C-group as having neurological disorders of the brain stem because of the normal threshold of SVR although with some abnormalities of latencies or amplitudes of ABR.

Key words: Auditory evoked response, auditory brain stem response, slow vertex response, brain stem lesion.

Brain & Development, Vol 4, No 3-4,1982 237

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The Results of Three-Month-Old Babies Screening Test by Vojta's Method (IV)

Trial Screening for Infants with Risk of Cerebral Motor Disturbance by the Visual Following Test

Hiroyuki Hatanaka, MD, Koichi Koyama, MD, Hiroshi Tanaka, MD, and Masato Imamura, MD National Sanatorium Minami-Kyushu Hospital, Kagoshima

Since 1977 we participated in the screening program for the health of 3-month-old babies in this area which covered 8,700 cases of new subjects every year, and we attempted to promote the very early diagnosis of cerebral palsy. The effect of the treatment was compared between the cases of the very early treatment (treatment started within 6 months after birth) and the other cases (the start was 6 months or later after birth). Thirteen babies were confirmed as suffering from CP among the babies born in 1977 . Likewise, 9 and 6 CP patients were found in 1978 an<;t 1979, respectively . Thus, the incidence of CP was ·calculated as 1.5, 1.0 and 0.6 per 1,000 live born for 1977, 1978 and 1979, respectively. On the other hand, closer cooperation between local medical facilities besides our participation in the screening activity has led to a new situation where a very early treatment was delivered to the greater number of at risk cases. An investigation was performed on the treatment effect in 123 cases which have been treated for more than 6 months up to the present. Motor development was compared between 58 cases of spastic quadriplegia, 24 spastic diplegia, 18 spastic hemiplegia and 23 athetoid type. The results were as follows. In spastic quadriplegia, 8 out of 21 cases with the very early treatment were able to walk, and 7 out of 37 cases without it were able to do so. In spastic diplegia, 7 out of 11 cases with the very early treatment and 6 out of 13 cases without it were able to walk. Hemiplegia was the type which responds the best to the treatment, while athetoid type showed a rather poor response.

Key words: Cerebral palsy, diagnosis, coordination.

Yuriko Yamori, MD, Junko Suzuki, MD, Toy oko Kanda, MD, and Hiroshi Fukase, MD St. Joseph Hospital for the Handicapped, Kyoto

According to our observations, infants with cerebral motor disturbance show discoordinated movements of trunk and extremities responded to head rotation during visual following. In the present study we tried to differentiate between two groups, one of many pathologic infants and the other of many normal infants in order to obtain check points for screening infants with cerebral motor disturbance.

Materials and Methods The subjects induded 57 infants who visited St. Joseph Hospital at 3 or 4 months of age (group A) and 91 infants who visited the public health center for group examination at 3 months of age (group B). Movies were taken from a position above the head of infants laying in th e supine position in order to investigate the motion pattern, laterality of the center of gravity, etc which appeared on head rotation during visual following. Results 1) Habitual turned head: A, 57/57; B, 67/91. 2) Direction of head turn : A, right 43/57 , left, 14/57; B, righ t 25/67, left 42/67 . 3) Motion pattern during visual following: (a) Asymmetrical pattern (positioned turned head, half head rotation & quick reversal rotation): A, 35; B, 9. (b) Throwing back movement: A, 7; B, 1. (c) Insufficient head rotation: A, 11; B, 29. (d) Sufficient head rotation : A, 3; B, 47. (e) Lateral turn: A, 0; B, 4 . 4) Laterality of the center of gravity: A, 50/57 ; B, 15/91. 5) Relationship between motion pattern and laterality of the center of gravity during visual following: In the cases with the asymmetrical pattern, 32 cases of group A and 3 cases of group B showed laterality of the center of gravity. In the cases with sufficient rotation, 2 cases of group A and 3 cases of group B showed laterality of the center of gravity. 6) During visual following, motion patterns noticed for extremities were, for example, elevation of legs, foot-foot coordination, asymmetrical tonic neck reflex posture, etc. These were not different between the two groups. Conclusion In the visual following test the two groups showed statistically significant differentiation in the following three points : (1) Laterality of head turn and the direction ; (2) motion pattern; and (3) laterality of the center of gravity . A follow-up study should be carried out. Key words: Cerebral motor disturbance, visual following test.

238 Brain & Development, Vol 4, No 3-4,1982

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CT Findings and Laterality of Spastic Diplegia Junko Suzuki, MD, Yuriko Yamori, MD. Toyoko Kanda, MD, and Hiroshi Fukase. MD St. Joseph Hospital for the Handicapped, Kyoto

CT Findings of Spastic Diplegia with Special

We reported the correlation between CT findings and laterality of handedness in spastic diplegia patients at the 21th Annual Meeting of the Japanese Society of Child Neurology. We examined further cases with spastic diplegia and report on them herein.

Materials and Methods Seventy-four patients with spastic diplegia who could crawl or walk were examined. The ages were 1 year and 8 months to 14 years. We assessed the asymmetry of ventricular dilatation (A VD) and cerebral asymmetry (CA) (obvious asymmetrical curvature of parietooccipital region) in CT. Handedness was determined as follows: (a) the side more frequently used, (b) the side of easier external rotation of the wrist joint and the side of easier upward extension of the arm. Results 1) AVD was found in 23 cases (31 %). Eighteen cases whose left ventricles were dilated included 10 lefthanders and 8 right-handers, and the rest of them whose right ventricles were dilated were all righthanders, that is, the side of handedness correlated with that of the smaller ventricle in two-thirds of this group. 2) AVD was not found in 51 cases but 45 cases showed CA. In 33 cases the right parieto-occipital region was flat. Twenty-three cases were right-handers and 10 were left-handers. In 12 cases the left parietooccipital region was flat. Six of them were right-handers and 6 were left-handers. The side of handedness correlated with that of the larger parieto-occipital region in two-thirds of this group but not in others. Summary 1) Laterality of handedness in patients with spastic diplegia correlated with the side of the less dilated ventricle or with the side of the larger parieto-occipital region of the cerebrum in two-thirds of the cases. 2) Left ventricles were more frequently dilated in cases of AYD. Key words: CT, cerebral palsy, spastic diplegia, handedness, laterality.

Reference to Grade of Motor Disturbance Toyoko Kanda, MD, Junko Suzuki, MD, Yuriko Yamori, MD, and Hiroshi Fukase, MD Department of Pediatrics, St. Joseph Hospital for the Handicapped, Kyoto

We report herein the CT findings of spastic diplegia and the correlation between the grade of lateral ventricular dilatation and the walking prognosis as the grade of motor disturbance (of spastic diplegia).

Materials and Methods We examined 66 patients with spastic diplegia who had started training after 6 months of age. The age distribution of these cases was from 1 year 6 months to 14 years old. Distribution of the lateral ventricular dilatation on CT was divided into three parts; frontal horn, body and occipital horn. The grade of lateral ventricular dilatation was divided into four grades; severe, moderate, slight and none. The subjects were divided into two groups according to the possibility of walking at 3 years 6 months of age. The group of those able to walk included 21 cases. The other group, those unable to walk, included 25 cases. There were 17 cases (25.8%) with complications of mental retardation (lQ < 75). Results In the group able to walk there was 1 (6 .2%) among the 16 cases whose grade of ventricular dilatation was moderate or severe. Among the 30 cases whose grade of ventricular dilatation was slight or none there were 20 (66.7 %). Among the 16 cases whose grade of ventricular dilatation was moderate or severe, the IQ was over 75 for 10 of them (62.5%). For the 30 cases whose grade of ventricular dilatation was slight or none the number was 25 cases (83.3 %). The CT findings of the 66 cases were as follows; normal 17 cases (25.8%), atypical ventricular dilatation 37 (56.1 %), atypical cortical atrophy 22 (33.3%) and others 15 (22.7%): arrested hydrocephalus 3, porencephaly 2, corpus callosum agenesis 1, lipoma 1, and midline anomaly 10. The distribution of the lateral ventricular dilatation of CT was more often at the body, 28 cases (75.6%), than at the anterior horn, 4 cases (10.8%). The 4 cases whose lateral ventricle was enlarged at the frontal horn belonged to the group unable to walk. Conclusion The grade of lateral ventricular dilatation is well correlated with the grade of motor disturbance of spastic diplegia. Key words: Spastic diplegia, CT findings, lateral ventricular dilatation, walking prognosis.

Brain & Development, Vol 4, No 3-4, 1982 239

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A Study of Brain-Damaged Children Weighing under 2,500 gm at Birth

Gastroesophageal Reflux in Severely Handicapped Children

Yasumasa Suga, MD, Yukikatsu Ochiai, MD, Yuki Yamazaki, MD, Shigenobu Tsuzura, MD, and Kihei Maekawa, MD Tokyo Metropolitan Kitaryoikuen Hospital for Crippled Children, Tokyo (YS, YO, YY, ST); Department of Pediatrics, The likei University School of Medicine, Tokyo (KM) Neonatal asphyxia, premature birth and neonatal jaundice are said to be three factors involved in the occurrence of brain damage such as cerebral palsy (CP). Countermeasures against neonatal asphyxia and neonatal jaundice have undergone considerable development in Japan since the 1970s owing to the improvement of neonatal medical treatment. In such circumstances we consider it very meaningful for the prevention of brain damage to consider problems related to low birth weight. We have recently examined cases of infants of low birth weight with subsequent brain damage, concentrating mainly on neonatal neurological details and report some results herein.

Materials and Methods Examinations were carried ou t on 258 cases of lowbirth-weight infants which were diagnosed as so-called "brain damaged infants," among the first outpatients at our hospital during 1965, 1968, 1970, 1973 and 1975. Since our hospital deals mostly with crippled children, we analyzed charts for infants whose CP was mainly examined. Results and Conclusions 1) Cases of infants of low birth weight with CP showed a gradual yearly decrease. 2) Children with complex handicaps and accompanying damage such as epilepsy or mental retardation (IQ below 67) showed yearly increases. 3) For mental retardation, the athetoid type showed relatively slight damage, but the spastic type showed a tendency of yearly increasing damage. 4) For movement damage, infants with relatively advanced damage were recognized in the group with birth weights below 1,500 g. Thus, low birth weight is a significant cause of the occurrence of CP, which suggests its importance in the consideration of the prevention of brain damage. Key words: Neonatal brain damage, cerebral palsy, mental retardation, low·birth-weight infant.

Michiko Hayashi, MD, Masaru Tatsuno, MD, Hiroko Iwamoto , MD, Kuniomi Yokota, MD, and Kunio Odagiri, MD Department of Neurology (MH, MT, HI), Institution for Severely Mentally and Physically Handicapped Children (MH, KY) , Kanagawa Children's Medical Center, Yokohama, Kanagawa; Department of Radi· ology, Yokohama City University Hospital. Yoko· hama, Kanagawa (KO)

1) We undertook 24-hour pH monitoring on 20 normal children, lo oking for the tim e % 24 hours of gastroesophageal reflux (GER) when the pH was below 4 and the reflux number of GER of more than five minutes. On the basis of the results, the pH score was decided as follows: pH score = (+) SD of pH below 4 time % 24 hours + (+) SD of the reflux number of GER of more than five minutes. The pH scores of normal children were all below 4. 2) We studied the upper GI series and 24-hours pH monitoring in 17 severely handicapped children. We divided the patient into three groups. Group 1, pH score < 5 (9 cases), Group 2, pH score 5-13 (4 cases) and Group 3, pH score > 14 (4 cases) . There were no age differences among the three groups. The GER for the upper GI series and combination with hernia hiatus of the esophagi (sliding type) were found at a higher rate in Groups 2 and 3 than in Group 1. 3) Clinical feature of gastroesophageal reflux was shown in Table.

Head control Tube feeding Vomiting (coffee-like) Asthma Scoliosis Anemia (HB < 8 g/dl) Recurrent pneumonia

pH pH pH score < 5 score 5-13 score> 14 (n = 4) (n = 4) (n = 9) 1 (25 %) 0 0 7 (77.8%) 2 (50%) 3 (75 %) 1 (11.1%)

3 (75 %)

2 (25 %)

1 (11.1%) 7 (77.8%)

0 3 (75 %)

0 4 (50%)

1 (11.1 %)

0

0

4 (44.4%)

1 (25 %)

1 (25 %)

Coffee-like vomiting was significant in Groups 2 and 3 as opposed to Group 1 (p = 0.01). Two patients were successfully treated by a surgical procedure. The clinical manifestation of Case No 1 was aspiration pneumonia due to GER and that of Case No 2 was esophagitis due to GER. In both cases, the pneumonia, anemia and vomiting were improved postoperatively.

Key words: Gastroesophageal reflux, severely handi· capped children, esophagitis, anemia in handicapped children.

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A Therapeutic Trial of Intermuscular TRH Injections for Patients with Nonprogressive Ataxic Syndromes (NPA) Yukiharu Une, MD, Hiroyuki Haraguchi, MD, Atsuko Shionaga, MD, and Minoru Hata, PhD Division of Pediatrics, The Center of Developmental Medicine and Education in Kitakyushu City, Fukuoka

Survey on Disabled Children Attending Normal Schools Keiichi Tamura, MSW National Habilitation Center for Disabled Children (Seishiryogoen), Tokyo

The effect of TRH injections on patients with cerebellar ataxia has been generally recognized in adult neurology. So we tried TRH injection for children with NPA. The ages of the patients ranged from 2 to 14 years (3 males, 8 females). All patients were evaluated from the neurological findings, their motor developmental history and were also examined radiologically by CT. They were divided into 4 cases of dysequilibrium syndrome (DES), 5 cases of congenital cerebellar ataxia (CCA), and 2 cases remain to be classified. Three cases of DES had abnormalities in the vermis of the cerebellum, but all cases of CCA showed no abnormalities in the vermis on CT. Three of the 5 cases of CCA, who were not able to walk, got to walk a few steps, and another case, who stayed at the sitting level, got to stand up alone, and the other case, who was able to walk a few steps, got to walk over 20 steps after TRH injections. On the other hand, TRH injections were not effective for the 4 cases of DES. The metabolites of catecholamine in CSF were detected in all patients, before treatment and one hour after the TRH injection on the last day of treatment. Before treatment, the homovanillic acid (HV A) level of the patients with NPA was higher than the control. After TRH injection, HVA levels significantly decreased, but remained higher than the control. Before treatment, HVA levels of the patients with CCA, who improved with the TRH injections, were higher than the levels of the patients with DES. The decreasing ratio of HV A between pre and post TRH injection, was not characteristic for both CCA and DES.

Key words: Congenital cerebellar ataxia, dysequilibrium, TRH.

It was legislated that all disabled children regardless of

the severity of their handicap are to attend school from 1979. (In Tokyo it has been required since 1974.) It is difficult in many cases to reach a common decision as to which school, a normal school, a special class in a normal school or a school for the handicapped, is the most appropriate for a child, among parents and the staff who participate in consultation on the child's school attendance. It is a matter of difficulty that we are confronted with every year as medical social workers working in institutions for the physically handicapped and nursery schools for the handicapped. With the cooperation of medical social workers of 8 facilities in the Tokyo area (the Tokyo Metropolitan Kitaryoikuen Hospital for Crippled Children, its Jyonan and Jyohoku annexes, the Tokyo Metropolitan Tama Habilitation Clinic, Habilitation National Center for Disabled Children, Central Guidance Center for Crippled Children with Outpatients Clinic, Tokyo Rehabilitation Hospital for Children, and Treatment and Training Center for Crippled Children "Seiikuen"), we conducted a survey on 165 children who had been institutionalized or attended one of the above 8 facilities, enrolled in normal schools between 1974 and 1979, and have been attending until present. The results of the survey were analyzed from the following four standpoints: 1) the severity of the handicap, 2) consultation before enrollment, 3) parents opinion on the child's attending the school, and 4) the actual situation when each child was put in a school. Although the severity of a child's mental handicap seems to be a serious problem in many cases, some children with rather severe multiple handicaps successfully attend schools. The results suggest that various ways of attending normal schools seem possible when the child, parents and school reach a consensus on the child's school education. The details of the survey will be reported. We hope that the results of the survey will be a helpftll guide for future consultations on disabled children's enrollment in schools.

Key words: Habilitation, schooling for disabled person, deinstitutionalization.

Brain & Development, Vol 4, No 3-4, 1982 241