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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The total population of 11,865 children of compulsory school age resident on the Isle of Wight was studied to determine the prevalence of epilepsy, cerebral palsy, and other neurological disorders. With the use of reliable methods, children selected from screening of the total population were individually studied by means of parental interviews and questionaries, neurological examination and psychiatric assessment of each child, information from school teachers, and perusal of the records of hospitals and other agencies. The association between organic brain dysfunction and psychiatric disorder was studied by comparing the findings in the children with epilepsy or with lesions above the brain stem (cerebral palsy and similar disorders) with those in (1) a random sample of the general population, (2) children with lesions below the brain stem (for example, muscular dystrophy or paralyses following poliomyelitis), and (3) children with other chronic physical handicaps not involving the nervous system (for example, asthma, heart disease, or diabetes).Psychiatric disorders in children with neuro-epileptic conditions were five times as common as in the general population and three times as common as in children with chronic physical handicaps not involving the brain. It was concluded, on the basis of a study of factors associated with psychiatric disorder, that the high rate of psychiatric disorder in the neuro-epileptic children was due to the presence of organic brain dysfunction rather than just the existence of a physical handicap (though this also played a part). However, organic brain dysfunction was not associated with any specific type of disorder. Within the neuro-epileptic group the neurological features and the type of fit, intellectual/educational factors, and socio-familial factors all interacted in the development of psychiatric disorder.
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PMID:Organic brain dysfunction and child psychiatric disorder. 423 74

The vaccination status of 98 physically handicapped children was examined to identify factors associated with an inadequate vaccination status. Of the 98 children, 57 had cerebral palsy, 14 had myelomeningocele, 3 had muscular dystrophy and 24 had myelomeningocele, 3 had muscular dystrophy and 24 had other motor disabilities. According to the available vaccination records, only 17 children had received all the recommended injections on schedule; 26 had missed at least one injection, and 3 of them had never been vaccinated. The overall rate of vaccination in our study group (63%) was lower than expected. The children with moderate to severe limitation of function due to cerebral palsy were significantly less likely (P less than 0.05) than those with less severe limitations to have received a basic series of vaccinations. Health departments must ensure that physically handicapped children receive the preventive health measures viewed normal and appropriate for other children.
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PMID:Vaccinations and the physically handicapped child. 621 4

A survey was made of the clinical characteristics of wheelchair-users at the Children's Rehabilitation Centre in Saskatoon, Saskatchewan. 261 patients were studied, of whom 214 had cerebral palsy, 34 had myelomeningocele and 13 had Duchenne muscular dystrophy. Among the patients with cerebral palsy it was found that the attributes likely to define a wheelchair-user were spastic quadriplegia, functional level III and IV, seizures and severe sensory handicap; among patients with myelomeningocele, wheelchair use ws related to level of lesion; and in the muscular dystrophy group it ws related to age. These results have been of help to rehabilitation centres and related service agencies in formulating long-term plans to meet the needs of wheelchair-users.
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PMID:Wheelchair users at a children's rehabilitation center: attributes and management. 710 4

Conventional bracing places constraints on the determinants of gait, which in the borderline patient, may derange gait as much or more than the postural deformity being treated. A small number of children with cerebral palsy, spina bifida, and muscular dystrophy were treated with below-knee orthoses (BKO) along with elastic bracing of the remainder of the limb. The elastics were used to augment muscle power in weakness, oppose spastic muscles, supply antagonism to unopposed muscle, and improve function. The key elements in this system include proximal attachment of elastics to the below-knee orthosis resultin in effective knee action independent of foot control; variable 2-joint function of elastics; and emphasis on a BKO-shoe combination, which positions the knee in front of the ankle at midstance. Because a small number of components are employed to produce a variety of actions, an understanding of the mechanics fo gait is needed for the rational application of this method of treatment as well as for the selection of suitable patients.
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PMID:Dynamic bracing: elastics for patients with cerebral palsy, muscular dystrophy and myelodysplasia. 737 5

Although developmental disabilities are among the major chronic health problems affecting children in the United States, the contribution of developmental disabilities to childhood mortality is unknown. To investigate the magnitude of this contribution, multiple cause-of-death data were examined for US children, aged 1-19 years, for 1980 and 1983-1989. The following conditions were included as developmental disabilities: autism, attention deficit disorder, learning disorders, mental retardation, cerebral palsy, epilepsy, muscular dystrophy, blindness and deafness. Based on underlying cause only, it was found that developmental disabilities were the fifth leading cause of nontraumatic death for children between 1 and 14 years of age and the third leading cause of non-traumatic death for children between 15 and 19 years. When a multiple cause approach was used to define developmental disability-related deaths (i.e. when contributing as well as underlying cause was considered), the number of such deaths nearly doubled. On the basis of both underlying- and multiple-cause analyses, cerebral palsy was the developmental disability most frequently cited as a cause of death. Mental retardation ranked second according to the multiple-cause approach but only fourth according to the underlying-cause approach. The least frequent causes of death (autism, attention deficit disorder, learning disorders, blindness, and deafness) were the ones most likely to be coded as contributing rather than underlying causes. Developmental disability-related mortality rates were highest among children aged 1-4 and 15-19 years, highest among blacks and lowest among racial groups other than blacks and whites, and higher among males than females. Although results of multiple-cause-of-death analyses more accurately reflect the proportion of deaths related to developmental disabilities, even this approach may underestimate the degree to which mortality is associated with a developmental disability.
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PMID:Contribution of developmental disabilities to childhood mortality in the United States: a multiple-cause-of-death analysis. 753 59

The authors analyzed lateral deviation, anteroposterior deformity, and rotation of the spinal column of 11 patients with scoliosis associated with cerebral palsy (CP) and 11 patients with progressive muscular dystrophy (PMD). There was a correlation between the magnitude of Cobb angle and that of the vertebral rotation in scoliosis associated with both CP and PMD, but the ratio of spinal rotation to Cobb angle in the former was lower than that in the latter. The magnitudes of thoracic kyphosis and of lumbar lordosis were not correlated with Cobb angle in either group, but the sagittal plane deformity in the CP patients was less severe than that in the PMD patients, and the latter demonstrated kyphosis of the lumbar spine and lordosis of the thoracic spine.
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PMID:Three-dimensional spinal deformity in scoliosis associated with cerebral palsy and with progressive muscular dystrophy. 827 49

A retrospective analysis was conducted of 397 children, ranging in age from 2 to 19 years, attending the Regency Park Centre for Young Disabled in Adelaide. The disorders represented included cerebral palsy, head injury, spina bifida, severe speech and/or language disorders, muscular dystrophy and a number of less common conditions. The incidence of significant ophthalmic abnormalities in this population was 51%, with the highest incidence being 69 and 62% among children with head injury and cerebral palsy, respectively. Children with severe speech and language disorders had an incidence of 24%. Overall, routine examinations revealed previously unsuspected significant eye abnormalities in 31% of the children. A complete ophthalmic examination should be part of the evaluation of all physically disabled children to ensure early identification and treatment of abnormalities, and to optimize rehabilitation.
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PMID:Ophthalmic assessment of physically disabled children attending a rehabilitation centre. 848 93

It has been reported that serum levels of ketone bodies often elevate in patients with Duchenne muscular dystrophy (DMD). The cause of this abnormal metabolism, however, has not been elucidated. In this study, serum levels of ketone bodies were measured in the early morning in patients with DMD, congenital muscular dystrophy and cerebral palsy. Serum free fatty acids (FFA), glucose, and thickness of subcutaneous fat were also measured. Ketone bodies elevated in most DMD patients, but not elevated in patients with the other disorders. Hyperketonemia in the DMD patients was accompanied by elevation of the FFA level, but no correlation was seen between hyperketonemia and the creatine kinase level or the severity of muscle involvement. No differences were found between DMD patients and others in the levels of FFA, glucose and thickness of subcutaneous fat. These results indicate that DMD patients are more prone to ketosis than the others. The effect of biotin administration (2 mg/day) on hyperketonemia also was investigated in 11 patients with DMD. The levels of total ketone bodies decreased by biotin administration for 2 weeks. These data suggest that the utilization of FFA and ketone bodies may be impaired in DMD patients. Biotin treatment will be useful for improving the abnormal metabolism of ketone bodies in DMD patients.
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PMID:[Abnormal metabolism of fatty acids and ketone bodies in Duchenne muscular dystrophy, and the effect of biotin on these abnormalities]. 898 91

This study examined the impact of computer and assistive device use on the employment status and vocational modes of people with physical disabilities in Australia. A survey was distributed to people over 15 years in age with physical disabilities living in the Brisbane area. Responses were received from 82 people, including those with spinal cord injuries, cerebral palsy and muscular dystrophy. Of respondents 46 were employed, 22 were unemployed, and 12 were either students or undertaking voluntary work. Three-quarters of respondents used a computer in their occupations, while 15 used assistive devices. Using logistic regression analysis it was found that gender, education, level of computer skill and computer training were significant predictors of employment outcomes. Neither the age of respondent nor use of assistive software were significant predictors. From information obtained in this study guidelines for a training programme designed to maximize the employability of people with physical disabilities were developed.
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PMID:Relationship between use of technology and employment rates for people with physical disabilities in Australia: implications for education and training programmes. 927 89

Neuromuscular scoliosis can be a problem in children with underlying neuromuscular conditions such as cerebral palsy, spina bifida, and muscular dystrophy. A comprehensive preoperative assessment is essential to provide comprehensive postoperative care. Surgical procedures to correct neuromuscular scoliosis include anterior spinal fusion, posterior spinal fusion, or a combined anterior-posterior spinal fusion. Postoperative problems can include respiratory failure, hemodynamic instability, neurovascular compromise, and pain control. With an understanding of the developmental status of these patients, pediatric patients can be safely managed in an adult ICU.
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PMID:Neuromuscular scoliosis: a case of the pediatric patient in the adult ICU. 973 30


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