A process that includes proximal muscle weakness scoliosis, joint contractures, and spinal rigidity.
Several metabolic and myopathic abnormalities may be responsible for this disease.
Reduced vital capacity and progressive respiratory failure uniformly present.
Muscle weakness can affect the diaphragm and accessory muscles.
Thoracic cage compliance is decreased by scoliosis and spinal rigidity.
Joint contractures at the costovertebral joints develop.