Rigid spine syndrome
نویسندگان
چکیده
منابع مشابه
Rigid spine syndrome.
A patient is described with distinctive clinical features including childhood onset of non-progressive muscle weakness, limitation of flexion of the neck and spine, scoliosis, and joint contracture. Muscle biopsy and electromyography showed findings suggestive of a myopathic disorder. The combination of these distinctive clinical features is termed the rigid spine syndrome. Only six cases have ...
متن کاملThe rigid spine syndrome.
Four patients are reported, 3 females and 1 male, with (as a prominent symptom of muscle disease) limitation of flexion of cervical and dorsolumbar spine. The nosological classification of these cases is discussed. In two patients there was evidence of an inclusion body myositis. At necropsy one of these patients had a remarkable distribution of muscle changes.
متن کاملRigid spine syndrome and nocturnal alveolar hypoventilation.
A 17-year-old Japanese woman with rigid spine syndrome (RSS) presented with respiratory failure leading to CO2 narcosis. The clinical symptoms were drowsiness, asterixis and cardiac arrhythmias. Tracheostomy and temporary ventilatory support abolished these symptoms. However, polygraphic sleep studies without a ventilator revealed Cheyne-Stokes respiration and profound arterial oxygen desaturat...
متن کاملRigid spine syndrome with respiratory failure.
The pathogenesis and therapy of respiratory failure in the rigid spine syndrome are discussed in two cases who improved with respiratory assistance. In both cases, the partial pressures of oxygen and carbon dioxide were reversed in arterial blood gas analysis and %VC was less than 30%. Remission from respiratory failure has been obtained by the use of a ventilator during the night. The cause of...
متن کاملThe rigid spine syndrome--a myopathy of uncertain nosological position.
Four patients meeting the clinical criteria of the rigid spine syndrome are presented; they are one girl with a positive family history and three boys. Clinical and histological findings are discussed in relation to the 14 cases of rigid spine syndrome reported in the literature. The delineations of the syndrome from other benign myopathies with early contractures are discussed suggesting that ...
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ژورنال
عنوان ژورنال: Journal of Neurology, Neurosurgery & Psychiatry
سال: 1979
ISSN: 0022-3050
DOI: 10.1136/jnnp.42.3.276