Carbon Dioxide Narcosis in Association With Muscular Dystrophy

Neustadt, Jerome E.; Levy, Robert C.; Spiegel, I. Joshua

doi:10.1001/jama.1964.03060210066022

Cited by 14 publications

(2 citation statements)

References 8 publications

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“…Bilateral diaphragmatic paralysis and myotonic dystrophy are asso ciated with arterial desaturation induced by apnea or hy poventilation during sleep. Neustadt et al [11] reported a patient with limb-girdle dystrophy who, after becoming comatose due to probable sleep hypoventilation, was later able to resume his previous life pattern after nocturnal mechanical ventilation. Our patient initially showed marked sleep-associated hypoventilation, but nocturnal mechanical ventilation completely restored normal arte rial saturation in the morning as well as during the night.…”

Section: Discussionmentioning

confidence: 99%

Rigid Spine Syndrome with Selective Respiratory Muscle Weakness

Akiyama

Aimoto²,

Nishimura³

et al. 1992

Respiration

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Rigid spine syndrome is a rare myopathy characterized by mild axial and proximal muscle weakness, limitation of neck and trunk flexion, scoliosis and mild joint contractures. We describe a 20-year-old woman with this syndrome who developed severe respiratory muscle failure disproportionate to the well-preserved extremity muscles. Nocturnal home care ventilation has completely restored her arterial oxygen desaturation during a 5-year follow-up.

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Section: Discussionmentioning

confidence: 99%

Rigid Spine Syndrome with Selective Respiratory Muscle Weakness

Akiyama

Aimoto²,

Nishimura³

et al. 1992

Respiration

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“…Sixty patients with well established limb-girdle dystrophy of 11-15 years duration had minimal changes in respiratory function measured spirometrically, and no evidence of respiratory hypoventilation, even in those who were not ambulatory [6]. Mild pulmonary function abnormalities were found in several other patients with limb-girdle dystrophy, but significant abnormality of ventilation was evident on blood gas determination in only 3 patients who were already wheelchair-bound [7,8]. Two additional patients with limb-girdle dystrophy whose motor functional status was not reported had spirometric pulmonary function abnormalities, CO 2 retention, and weakness of diaphragm muscles measured by transdiaphragmatic pressure change [9].…”

Section: Discussionmentioning

confidence: 97%

Chronic respiratory failure in limb-girdle muscular dystrophy: Successful long-term therapy with nasal bilevel positive airway pressure

Robertson

Roloff

1994

Pediatric Neurology

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Chronic respiratory failure is a major factor contributing to mortality in progressive neuromuscular disorders. Among the muscular dystrophies, respiratory failure most commonly occurs with Duchenne dystrophy, while in Becker, limb-girdle, and facioscapulohumeral dystrophies, respiratory failure is infrequent and generally occurs in the more severe cases that have progressed to a nonambulatory, advanced functional stage. We report two brothers with a myopathic disease in which the distribution of weakness, initial clinical course, heredity, and muscle pathology most closely resembled a limb-girdle type of dystrophy. Both brothers, however, presented with chronic alveolar hypoventilation and respiratory failure when their locomotor disabilities were still mild. Respiratory failure was reversed, and satisfactory ventilation has been maintained for more than a year using a type of noninvasive intermittent positive pressure ventilation, with a bilevel positive airway pressure device (Bi-PAP), administered through a nasal mask during sleeping hours. These cases demonstrate an unusual presentation of limb-girdle dystrophy, and document that nocturnal, nasal administration of continuous airway pressure using the Bi-PAP device may be sufficient to maintain adequate long-term ventilation in some patients with neuromuscular causes of respiratory failure, and thus significantly improve quality of life and delay the need for more complex or invasive forms of assisted ventilation.

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Respiratory Aspects of Pseudohypertrophic Muscular Dystrophy

Burke¹

1971

Arch Pediatr Adolesc Med

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Carbon Dioxide Narcosis in Association With Muscular Dystrophy

Cited by 14 publications

References 8 publications

Rigid Spine Syndrome with Selective Respiratory Muscle Weakness

Rigid Spine Syndrome with Selective Respiratory Muscle Weakness

Chronic respiratory failure in limb-girdle muscular dystrophy: Successful long-term therapy with nasal bilevel positive airway pressure

Respiratory Aspects of Pseudohypertrophic Muscular Dystrophy

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