[The bent spine syndrome: a focal axial myopathy of late onset].

The bent spine is a rare syndrome appearing at an advanced age which shows a posture of anterior flexion of the trunk, which is reducible and is often hereditary. It is caused by a paresia of the extensor musculature of the trunk by a focal axial myopathy of late onset. We herein describe the case of a 72-year-old woman with a progressive bent spine initiated at the age of 55. Family questioning showed vertical transmission of the process. On exploration paresis of the paravertebral musculature and to a lesser extent of both girdles was observed. Serum CK levels were normal. Vertebral CT showed atrophy with fatty substitution of the paravertebral musculature. EMG of the scapular and paravertebral muscles demonstrated a myopathic pattern. Deltoid muscle biopsy found atrophy of type II fibers and isolated broken red fibers. This case corroborates the myopathic nature of this syndrome. A review of the nosology of the syndrome is also presented.