[An autopsy case of rigid spine syndrome]

We reported an autopsy case of rigid spine syndrome. A 20-year-old woman developed severe respiratory failure despite well-preserved muscle strength in her extremities. Although she had mechanical ventilation during sleep, she had normal arterial blood gas values during daytime. She was found dead in a morning approximately 71/2 years after the onset of the respiratory symptoms. At autopsy, the respiratory diaphragm and intercostal muscles, and the truncal paravertebral and sternocleidomastoid muscles were more significantly affected than the limb muscles. Many corpora amylacea were found in the whole spinal cord, though the significance remains to be elucidated.