Disabilities in 830 severely retarded children

Patterns of disabilities in 830 severely retarded children aged 6-14 years identified through the prevalence study in Tokyo were analyzed. Severely retarded children were defined as those who neither capable to walk nor have their IQs over 35. Sixty-four percent of the total children belonged to class 1 of Ooshima's classification (bedridden and IQs less than 20) and most of the others belonged to class 2 of Ooshima's classification (those who able to sit alone and IQs less than 20). The percentages of the complications were as follows; epilepsy 84%, visual disturbance 40%, respiratory distress 30%, and feeding difficulty 46%. Sixteen percent of the total children were institutionalized. We conclude that the proportion of the most severe retardation has become larger than before and that the day care service for such children at their homes will be more important.     

Prevalence rate of severely retarded children in Tokyo

Prevalence rate of severely retarded children aged 6-14 years was studied in Tokyo. The definition of severely retarded children was those who neither walk nor have their IQs over 35. We visited total of 60 institutions and schools for retarded children in and around Tokyo. Because of the school system in Tokyo that enrolls all children regardless of their disabilities, case finding was exhaustive. The number of severely retarded identified was 830. The prevalence rate was 0.68/1,000 on April 1, 1988. We conclude that the prevalence rate of severe retardation is modestly lower than the rates previously reported from other areas of Japan.     

Radiological studies of feeding disorders in severely retarded children

Feeding difficulty is a major problem in severely retarded children. Fourteen patients aged from 5 to 36 years with physical and intellectual impairments who had feeding difficulties were investigated by videofluoroscopy. Video-fluoroscopic findings showed that the liquid was easier to be aspirated than the paste, and that in the sitting position aspirations were more frequent than in the supine. The predisposing factors of feeding difficulties were thought to be poor bolus formation by the tongue, delayed swallowing reflex, incomplete closure of the larynx and poor pharyngeal pressure. Of ten cases that showed markedly gulped air, three of them had gastro-esophageal reflux, which was very likely caused by the increased intragastric pressure. Seven patients fed by nasogastric tube feeding were examined their swallowing ability by dropping contrast medium into the oral cavity. Three patients aspirated contrast media into bronchi. This suggests that they also generally aspirated saliva which resulted in recurrent respiratory infection. It is therefore important to maintain the swallowing function in patients fed by nasogastric tube. Low osmolality water soluble contrast medium was used in this study. There were no complications. This medium is thought to be safer than the standard media of barium solutions or other high osmolality contrast media, especially for the patients who suffer from aspiration.     

Tourette syndrome in severely behavior disordered mentally retarded children

Although generally overlooked in mentally retarded individuals, the occurrence of Tourette Syndrome (TS) in seven such children is documented here. These subjects also shared a very early and persistent severe behavior disorder consisting of hyperactivity, agitation, aggressivity, and noncompliance. Correct diagnosis of TS is important to appropriate treatment, rather than assuming that motor and phonic tics and other associated TS symptoms are necessarily a function of a more pervasive developmental disorder in a disturbed mentally retarded person.     

Neurological symptoms and etiology of severely retarded children

I identified forty-three patients with severe retardation aged 6-14 years in a defined community. More than half of the patients (23/43) had spastic tetraplegia and 9 had spastic diplegia. Only one case showed abnormal increase of muscle tone of dyskinetic type. The period when they had got underlying causes of their handicaps was as follows : congenital (acquired intrauterine, including intractable cases) 25, perinatal 6, and after the neonatal period 12. An increased proportion of congenital cases and a decreased proportion of perinatal cases are noted when the data were compared with previous reports. About a half of the total cases could be considered to be caused by preventable diseases such as perinatal anoxia, purulent meningitis, or intracranial hemorrhage due to vitamin K deficiency.     

Nutritional assessment of severely retarded children and adults

The nutritional status was assessed with physical and biochemical parameters on 39 severely retarded children and adults, 17 males and 22 females, ranging from 6 to 40 years of age. In the anthropometric measurements, a majority of the cases showed short stature, light body weight, decreased triceps skinfold thickness and decreased mid-upper arm muscle circumference. The analysis of serum amino acids showed decreases of the sum of essential amino acids, the sum of branched chain amino acids, the ratio of the sum of essential amino acids to the sum of nonessential amino acid, and the ratio of the [methionine + tryptophan] to [valine + lysine + threonine]. The levels of prealbumin and transferrin in the serum were within normal limits. Other biochemical parameters were lower limits, including total protein, albumin, total cholesterol, triglyceride, phospholipid and total lipids in the serum. In addition, the level of fasting blood glucose tended to be low, and levels of serum free fatty acid were markedly different between before and after breakfast. These results suggest the presence of nutritional disturbances in most of severely retarded children and adults.     

Neuropsychological dysfunctions in amyotrophic lateral sclerosis: relation to motor disabilities

A study was undertaken to elucidate the existence of cognitive and memory impairments in patients with amyotrophic lateral sclerosis (ALS) by comparing a group of ALS patients with both nonneurological medical controls (MC) and healthy controls (HC) on neuropsychological tests. We also examined the relationship between the severity of motor disabilities and intellectual impairment. Twenty-two ALS patients, 18 MCs and 17 HCs participated. The tests used were the Mini-mental state examination (MMS) and the immediate and delayed memory tests. The mean MMS score of the ALS patients was lower than the mean scores of both control groups. In the memory tests, there were significant differences between the ALS group and the two control groups. Correlation analyses of several motor symptoms and neuropsychological results revealed that there was a significant negative correlation between upper motor neuron symptoms and MMS, as well as memory tests. The evidence for multisystem degeneration in ALS has prompted speculation that more sensitive neuropsychological measurements might reveal cerebral dysfunction in ALS patients who are not presenting evident dementia.     

Surgical management of gastroesophageal reflux in severely mentally retarded children

ABSTRACT. Thirty-three severely mentally retarded children with profond malnutrition and aspiration pneumonitis were treated by gastrostomy and Nissen fundoplication. Early and late complications were 27.3% and 16.6% respectively. Mortality was 9.4%. All surviving patients gained weight. Time required for feeding was greatly, reduced. This study supports earlier operative intervention for the mentally retarded child with gastroesophageal reflux, malnutrition and aspiration pneumonitis.     

Mortality patterns of non-institutionalized severely retarded children in Tokyo]

Mortality patterns of severely retarded children who were cared at their homes in Tokyo were investigated. We identified sixty-seven physically handicapped children who died in 1988 and 1989 at 15 schools for such children. 42 children out of 67 were severely retarded (children who neither walk nor have IQs over 35). Annual case-fatality rate of severely retarded children was 2.5%. Respiratory tract infections accounted for 38% of the causes of severely retarded children and other respiratory tract diseases such as aspiration and complications of tracheotomy accounted for 29%.