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

The term 'long term neurological cripple' is an unattractive and yet an all embracing one, covering a wide spectrum of disorders from spina bifida or cerebral palsy with or without associated epilepsy and behavioural and learning problems, through muscular dystrophy, multiple sclerosis, and motor neurone disease, to the effects of head injury, cerebrovascular lesions and the degenerative disorders of later life such as Parkinson's disease and the senile and presenile dementias. Whilst many of the problems are common to several of these entities, each has its own particular aspects.
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PMID:The support of the long term neurological cripple. 10 16

A total of 600 handicapped patients had dental rehabilitation under general anesthesia during an eight-year period. Handicaps included mental retardation, cerebral palsy, Down syndrome, seizure disorders, autism, cystic fibrosis, osteogenesis imperfecta, and muscular dystrophy. No significant complications developed in the majority of patients. This is attributed to thorough preoperative evaluation, appropriate anesthetic management, and vigilant postoperative observation.
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PMID:Complications related to the administration of general anesthesia in 600 developmentally disabled dental patients. 15 47

A clinical locomotion laboratory has been developed to provide quantitative information in the management of gait disorders. The biomedical engineering development of this system identified two major clinical constraints: (a) the need for instrumentation that would not alter the natural gait of the patient and (b) the need for data-processing techniques that would permit analysis and correlation of the large volume of electromyographic (EMg) and kinematic information. The net result has been a unit that incorporates a multichannel telemetry system to capture the EMG and foot-switch information and a television computer system to handle the kinematic information. Gait studies on children with hemiparesis, muscular dystrophy and cerebral palsy have yielded quantitative EMG and kinematic information on the pathomechanics of ambulation in these disorders. Because the information obtained is quantitative, an accurate measure of improvement (or lack of it) after treatment can be documented. Therefore, the locomotion laboratory may have an important role in the preoperative and postoperative evaluation of children whose abnormal gait may require surgical corrective procedures or rehabilitative treatment including the use of prostheses or orthoses.
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PMID:Locomotion studies as an aid in clinical assessment of childhood gait. 116 37

The sport of snow skiing by the physically disabled, which originated in Europe in 1935 and first received attention in the United States in the 1940s, is reviewed in terms of opportunities available, instructions, adaptive equipment necessary, and benefits provided. Persons with a wide variety of disabilities (such as cerebral palsy, multiple sclerosis, spinal cord injury, hemiplegia, amputation, blindness, spina bifida, and muscular dystrophy) can participate. Accordingly, a wide range of adaptive equipment is available--including outrigger skis, flip-skis, canting wedges, ski bras, "toe spreaders," sit-skis, and mono-skis--to allow safe enjoyment of the sport. Programs for instruction of the disabled skier are increasing in number and popularity, and numerous opportunities are available to enter competitive events sponsored by National Handicapped Sports. Both the participants and the instructors relate the numerous physical and psychologic benefits that can be derived from skiing; the sport provides an almost universal enjoyment of the sense of freedom and independence. Snow skiing is an enjoyable, beneficial, outdoor cold-weather activity that the disabled population can safely learn with proper instruction.
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PMID:Snow skiing for the physically disabled. 182 30

The child that is slow to walk causes concern. When cerebral palsy, mental retardation and muscular dystrophy have been excluded, what remains? Thirty five children (19 boys and 16 girls) with hypermobile joints, blue sclera and bat ears (the 'lax ligament syndrome') were referred by general practitioners to a general paediatric outpatient clinic over two years. Three were referred in the first three months of life because of clicking hips; 14 children aged one to two years, had delayed milestones of motor development and exhibited bottom shuffling; 10 children aged four to five years presented with 'growing pains' or 'funny gait' and eight older children had multiple minor complaints. The lax ligament syndrome is a comparatively common mild collagenopathy. It may well come to light on routine surveillance in general practice. It is dominantly inherited and improves with time; management is therefore expectant and symptomatic. A firm and reassuring diagnosis can be given which saves both anxiety and investigations.
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PMID:Lax ligament syndrome in children associated with blue sclera and bat ears. 211 44

A rehabilitation program for a patient with a neuromuscular disease can be developed only after an accurate diagnosis has been established. The diagnosis and its ramifications should suggest a natural course of disease which, it is hoped, can be improved upon with a rational and realistic program. The program is best developed by an interdisciplinary team, including a pediatric neurologist, who should have the greatest understanding of the patient's problem and should ultimately be responsible for the implementation and monitoring of the program. A child with cerebral palsy commonly requires the services of physical and occupational therapists as well as knowledgeable orthopedists. Is the program appropriate? Does it consider the child's potential as well as his limitations? A child with a traumatic brain injury requires, in addition to the above, psychological intervention and an intensive educational program. Will the child and family need help from mental health professionals? A child with a motor unit disease such as Duchenne's muscular dystrophy requires, in addition to the above services, a "philosophy" of care. Will the child ever ambulate independently? If so, at what cost? What will be necessary for the child to reach this potential, including items such as orthoses and adaptive equipment? Will respirator care become necessary? What issues must be addressed for this form of care to be established? There is no one program for all children. The programs must be individualized to meet the needs of the patient and the family. This point cannot be overemphasized.
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PMID:Rehabilitation of the pediatric patient with a neuromuscular disease. 214 6

Clinical forms of deforming ++ muscular dystrophy in children are described with their features of clinical course. Differential diagnosis of these with hepatocerebral dystrophy, familial spastic Strumpel paraplegia and children cerebral palsy was performed. Recommendations are offered for treatment of the condition.
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PMID:[Problems of diagnosis and treatment of deforming muscular dystrophy in children]. 217 16

In a Nigerian town with a stable population of 20,000, a door-to-door survey was conducted, using a questionnaire involving a complete census and a simple neurological evaluation which had previously showed a 95% sensitivity and an 80% specificity for detecting neurological disease. Positive responders were evaluated and categorised, using agreed criteria for diagnoses. Nearly 100% cooperation was obtained. Life prevalence ratio for at least one episode of headache was 51/1000. Crude point prevalence ratio for migrainous headache was 5.3/100, and peak age-specific ratio was in the first decade. Prevalence ratio for epilepsy was 533/100,000 and peak age-specific prevalence ratio occurred in the 5-14 years age groups. The prevalence ratio for peripheral nerve disorders was 268/100,000, and age-specific prevalence ratio for tropical neuropathy increased with age. Prevalence ratio for stroke was rather low at 58/100,000, but was probably due to the people's attitude to the disabled elderly and high mortality of stroke which showed annual mortality rate of 70/100,000 which increased with age to 1519/100,000 per year in the eighth decade. Crude prevalence ratios (cases per 100,000) for others are 112 for neurological complications (including sciatica) of spondylosis, 15 each for poliomyelitis, motor neurone disease, development speech disorders, 10 each for syncope, hereditary neuropathies. Parkinson's disease, benign essential tremor, primary cerebellar degeneration, cerebral palsy, mental retardation, organic psychosis (probable intracranial tumor) and 5 each for muscular dystrophy, pyomyositis, spina bifida occulta, alcohol dependence and cerebral malaria. The implications of the findings are important for development of community neurological services in the developing countries.
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PMID:Neurological disorders in Nigerian Africans: a community-based study. 303 73

While deficient exercise performance of sick children results from hypoactivity and detraining, it can also be caused by specific pathophysiological factors. These can affect one or more components of physical fitness. A low maximal aerobic power will result from a low maximal stroke volume, as in aortic stenosis or cardiomyopathy; a low maximal heart rate, as in congenital complete heart block or intake of beta-blockers; a low O2 content of the arterial blood, as in anemia or advanced cystic fibrosis; and a high O2 content of mixed-venous blood, as in muscle atrophy or severe malnutrition. A high O2 cost of locomotion, as in advanced obesity or cerebral palsy, will cause the patient to exert at a high percentage of his maximal aerobic power and thus fatigue easily. A subnormal muscle strength, as in progressive muscular dystrophy or juvenile rheumatoid arthritis, is sometimes the primary factor that limits the walking ability or other daily functions. Recent data suggest that local muscle endurance, as assessed by the Wingate anaerobic test, is particularly deficient in some neuromuscular diseases. Examples are muscular dystrophies and spastic cerebral palsy. The ratio of peak anaerobic power to peak aerobic power seems lower in such patients than in able-bodied controls.
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PMID:Pathophysiological factors which limit the exercise capacity of the sick child. 372 7

Patients who cannot walk, particularly those with orthopaedic deformities, need more than a simple wheelchair; they require a special seating system. Since the early 1970's many different systems have been introduced to the field of orthotics and prescribed through "Seating Clinics" in many centres in North America and around the world. Our experience in the Seating Clinic at the University of Kansas Medical centre is presented. Most patients were children (70%) with 69% male and 31% female. Various seating systems were prescribed, including the Safety Travel Chair, the Moulded Plastic Insert, the Orthopaedic Body Support (foam on plywood), the Spinal Support System, the Moulded Seat Total Contact Shell (sitting support orthosis) and the DESEMO support system. The majority of patients had cerebral palsy (58%) and muscular dystrophy (33%). Other conditions included spinal cord injury, spina bifida and multiple orthopaedic deformities. Sixty five percent of the custom made seating systems prescribed were the Orthopaedic Body Support. Unilateral dislocation of the hip presents a major problem in seating because of pelvic obliquity.
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PMID:Experience of a seating clinic. 391 40


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