Severe Chin-on-Chest Cervical Spine Deformity in the Setting of Stiff-Person Syndrome: A Case Report.

Authors :: Stephan SR
Ju DG
Ross LB
Naruse RT
Kim TT
Johnson JP
Source :: JBJS case connector [JBJS Case Connect] 2020 Dec 10; Vol. 10 (4), pp. e20.00378. Date of Electronic Publication: 2020 Dec 10.
Publication Year :: 2020
Abstract: 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.<br />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.<br />Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/B326).<br /> (Copyright © 2020 by The Journal of Bone and Joint Surgery, Incorporated.)

Subjects :: Cervical Vertebrae diagnostic imaging
Humans
Kyphosis diagnostic imaging
Kyphosis surgery
Laminectomy
Male
Middle Aged
Osteotomy
Spondylolisthesis diagnostic imaging
Spondylolisthesis surgery
Stiff-Person Syndrome diagnostic imaging
Stiff-Person Syndrome therapy
Tomography, X-Ray Computed
Cervical Vertebrae surgery
Kyphosis etiology
Spondylolisthesis etiology
Stiff-Person Syndrome complications

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