Limited atlantooccipital and cervical range of motion in patients with familial dysautonomia.

Abstract

Familial dysautonomia (FD) is a rare neurological disease with autosomal recessive inheritance and is associated with severe kyphoscoliosis. Investigators have reported subjective observation of decreased cervical motion and high rates of proximal instrumentation failure in this population. A radiographic study of sagittal plane cervical spine motion was performed with 15 patients with FD. Measurements were compared with normal values. Patients with FD had decreased sagittal motion at all cervical levels (P<0.05). Intervertebral translation was also decreased significantly at C3-C6. FD is associated with decreased sagittal motion in the cervical spine.