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

Recommendations for exercise programs in patients with muscular dystrophy are controversial. These programs are designed to keep a patient ambulatory or functional for as long as possible. There have been no reports of a patient with muscular dystrophy who incurred a spinal cord injury. This report details the course of recovery and rehabilitation of a man with facioscapulohumeral muscular dystrophy with quadriplegia from a traumatic spinal cord injury. The patient was admitted to a spinal cord injury unit. After prolonged bedrest, he participated in physical and occupational therapy that was designed to exercise him just short of fatigue. Except for one incident, when the patient reinjured his spinal cord, he did not lose function that he had already attained. The rehabilitation of this patient demonstrates that it is possible for a patient with facioscapulohumeral muscular dystrophy who sustains a traumatic quadriplegia to enter a rehabilitation program with expectations for a good outcome despite a double disability.
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PMID:Quadriplegia from spinal cord injury in muscular dystrophy. 276 95

Intermittent positive pressure ventilation (IPPB) is reported to improve lung compliance and decrease the work of breathing in subjects with kyphoscoliosis. These results suggest that IPPB may improve chest wall and lung compliance in patients with neuromuscular disease. We studied the short-term effects of IPPB on total respiratory system compliance in 14 subjects with neuromuscular disease. Seven were quadriplegics, and seven had muscular dystrophy. Vital capacity was reduced to 38 +/- 14 percent of the predicted normal values. Baseline measurements of total respiratory system compliance were 57 +/- 18 percent when compared to normal control values. After a 20 minute treatment of IPPB delivered with inspiratory pressures of 20 to 25 cm H2O that more than tripled resting tidal volume, there was no significant change in total respiratory system compliance in either group of patients. These findings indicate that patients with quadriplegia or muscular dystrophy do not derive immediate improvement in ventilatory mechanics from IPPB treatments.
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PMID:Intermittent positive pressure breathing in patients with respiratory muscle weakness. Alterations in total respiratory system compliance. 353 Jun 48

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

Severe degenerative features of the nervous system of a hitherto unknown kind, associated with a neuromuscular disorder with histopathological features of congenital muscular dystrophy, are reported in two female siblings. The clinical profile was characterized by generalized hypotonia followed by spastic tetraplegia, contractures, polyneuropathy, lack of cognitive development and progressive microcephaly. There as no involvement of the eyes. Neuropathological examination of the brain of one sibling, who died at the age of 30 months, revealed subtotal loss of neurons in the cerebral and cerebellar cortex and in the ventral pons, and secondary loss of myelin in the cerebral and cerebellar subcortical white matter. Sural nerve biopsy in the other sibling, who had a similar neurological affection, showed a lack of large myelinated fibers.
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PMID:Congenital muscular dystrophy and severe central nervous system atrophy in two siblings. 861 88

Sleep has a physiological influence on respiration, which can have major adverse effects on gas exchange in patients with respiratory insufficiency. These effects relate largely to a reduction in various stimulant inputs to the brainstem respiratory centre. Conditions that may be associated with sleep-related respiratory insufficiency range from pulmonary disorders (such as chronic obstructive pulmonary disease (COPD)), to central respiratory insufficiency (such as central alveolar hypoventilation), neurological and neuromuscular disorders (such as polio and muscular dystrophy), and thoracic cage disorders (such as kyphoscoliosis). All these conditions have in common the finding of hypoxaemia and hypercapnia, which become more pronounced during sleep. The relative hypoventilation, which is common to each condition, is due to varying combinations of an inadequate respiratory drive and an increase in the work of breathing. Management of respiratory insufficiency during sleep should be directed first at optimizing the underlying disorder, then at correcting hypoxaemia with controlled low-flow supplemental oxygen. Pharmacological therapy may be effective in some instances, but the choice of agent varies with the underlying disorder. Assisted ventilation is an important part of the management of advanced cases, and the recent development of intermittent positive pressure ventilation by nasal mask (NIPPV) has been an important advance in this area. Use of NIPPV during the night is associated with beneficial effects during the day, particularly improved awake gas exchange and respiratory muscle strength, in addition to less dyspnoea and improved quality of life. Electrophrenic pacing of the diaphragm is helpful in highly selected cases, particularly patients with central respiratory insufficiency and high quadriplegia, but is frequently complicated by the development of obstructive sleep apnoea.
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PMID:Impact of sleep in respiratory failure. 915 Mar 36

Alveolar hypoventilation associated with neuromuscular disease can occur in acute and chronic forms. In the acute form, progressive weakness of respiratory muscles leads to rapid reduction in vital capacity followed by respiratory failure with hypoxemia and hypercarbia. Symptoms are those of acute respiratory failure, including dyspnea, tachypnea, and tachycardia. In the chronic form, impairment of the respiratory muscles affects mechanical properties of the lungs and chest wall, decreases the ability to clear secretions, and eventually may alter the function of the central respiratory centers. Symptoms include orthopnea, fatigue, disturbed sleep, and hypersomnolence. Treatment and outcome of the disease's chronic form are dependent on the underlying clinical cause of the alveolar hypoventilation. For chronic but stable diseases such as old polio, quadriplegia, or kyposcoliosis, mechanical support of minute ventilation can reverse symptoms. For chronic and progressive disease such as muscular dystrophy and amyotrophic lateral sclerosis, mechanical support of minute ventilation provides only symptomatic relief and is usually associated with deterioration to the point of complete ventilator dependency for survival. For the chronic progressive forms of alveolar hypoventilation, there is currently a need for quality randomized controlled clinical trials to define physiologic indicators and appropriate timing for mechanical support of minute ventilation.
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PMID:Neuromuscular disease and hypoventilation. 1057 Jul 36

The school nurse plays a vital role in providing care and meeting the health needs of students in the school setting. Students attend school with chronic conditions and complex medical problems such as quadriplegia, cerebral palsy, spina bifida, and muscular dystrophy. It is the responsibility of the school nurse to provide appropriate assessment, early intervention, and care for children in the school environment. The purpose of this article is to review and discuss the knowledge and skills the school nurse needs to care for students with central venous lines, gastrostomy tubes, altered urinary elimination, and tracheostomies.
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PMID:A Review of Four Health Procedures That School Nurses May Encounter. 2945 55