Case: Stiff-person syndrome (SPS) presents with progressive muscle rigidity, postural instability, and periodic debilitating spasms. Reports of axial hyperextension exist, but kyphotic deformities have not been described. We surgically treated a patient with debilitating SPS and severe cervicothoracic hyperkyphosis with posterior spinal fusion and instrumentation. At 1-year follow-up, the patient displayed better upright gait and forward gaze, 18° cervical lordosis, and improved patient-reported outcome scores.
Conclusion: SPS can lead to extreme spinal deformity and disease, including hyperkyphosis of the cervicothoracic spine, and can successfully be managed with a multidisciplinary team and a posterior-only correction with spinal instrumentation and fusion.
Copyright © 2020 by The Journal of Bone and Joint Surgery, Incorporated.