Cervical Spine Fusion for the Treatment of Refractory Idiopathic Cervical Dystonia

Stelios Koutsoumbelis, MD; Federico P. Girardi, MD

Disclosures

March 15, 2017

Case Report

A 41-year-old female presented with a 2.5-year history of neck pain, discomfort in her shoulders and severe neck dystonia with fixed kyphosis. The symptoms began after an atraumatic neck muscle strain–she felt a pulling in the neck that was followed by awaking the following morning with her neck in a rigid forward flexed position. The patient has been unable to correct the neck position since then. She was seen by multiple physicians and had undergone unsuccessful Botox injections and manipulations under anesthesia.

The patient complained primarily of neck pain, but also had pain that radiated to both shoulders and scapulae. She also experienced thoracic spine pain and recurrent headaches. Her neck was flexed forward and rotated toward the right shoulder and she was unable to hold her head in a functional position. She reported her pain to be 7/10. The patient was able to sit up in a chair, but could not lie in the supine position. Her symptoms were aggravated by prolonged sitting, walking, bending and pushing objects–persistent pain and disability that made activities of daily living intolerable. The patient denied any history of trauma to the neck, head, or shoulders. She had also never been treated for psychiatric disorder, nor had she ever taken any psychotropic medications or illicit drugs.

On physical exam, the patient stood with a significant cervical, chin on chest, deformity. She was unable to lift her chin unassisted. Her gait was normal and she had intact balance and proprioception. The patient was neurologically intact with no focal motor or sensory deficits. Long track signs, including Babinski's and Hoffman's, Spurling's and Lhermitte's, were negative.

Radiographs revealed rigid cervical kyphosis from C2 to C7 with significant angulation involving the occipitocervical area (Figure 1). Full length standing radiographs demonstrated the chin on chest deformity and CT imaging showed the fixed C1-C2 deformity (Figure 2). MR imaging and myelogram were performed and revealed no abnormalities or significant cord signal changes.

Figure 1

Lateral (a) and extension (b) cervical spine X-rays demonstrating fixed cervical kyphosis measuring 77 and 74 degrees respectively. Anteriorposterior x-ray (c) shows fixed rotatory occipital cervical deformity.

Figure 2

Lateral scoliosis x-ray demonstrating chin-on-chest deformity (a). CT coronal imaging (b) and a 3D reconstruction (c), viewed from behind, illustrating right C1-C2 fixed deformity and atlantoaxial rotatory instability.

The patient underwent an anterior cervical decompression and fusion (ACDF) from C3 to T2, followed by a posterior spinal fusion from the occiput to T2 with use of iliac crest bone autograft. This surgery was done in one sitting. During the procedure, the patient also underwent a tenotomy of the right sternocleidomastoid. Postoperatively she was placed in a rigid cervical orthosis and transitioned to a soft collar after two weeks. At 3 months the patient reported that her overall preoperative symptoms had improved by 60% and at 6 months her neck pain had improved by 90% and overall alignment by 95%. Postoperative x-rays show successful fusion with restored neck alignment (Figure 3).

Figure 3

Anterior-posterior (a) and lateral (b) radiographs after anterior-posterior spinal fusion and right sternocleidomastoid tenotomy.

Discussion

Cervical dystonia (CD), also called spasmodic torticollis, is the most common of the focal dystonias.[1] In CD, the muscles in the neck that control the position of the head are affected, causing the head to turn to one side or be pulled forward or backward.[12] Cervical dystonia can occur at any age, although most individuals first experience symptoms in middle age.[12] Females are 1.5 times more likely to develop spasmodic torticollis than males.[1] Worldwide, the incidence rate of CD is at least 1.2 per 100,000 person years, at a prevalence rate of 57 per 1 million.[3] It often begins slowly and usually reaches a plateau over a few months or years. About 10 percent of those with torticollis may experience a transient, spontaneous remission, but this usually does not last and there is recurrence.[23] Trauma to the neck, head, or shoulder can also precede the onset of CD; none of which were reported by this patient. Treatment with certain anti-psychotic medications and the abuse of illicit psychotropic agents can also induce CD, again not reported in this case. Treatment for CD can range from stretching, Botox injection and Deep Brain stimulation. Surgical correction is usually indicated to treat rigid deformities, including cervical kyphosis and atlantoaxial rotatory instability.[45] Presented here is a case of idiopathic cervical dystonia that led to muscular torticollis, rotatory instability, and chronic fixed cervical deformity.

This case illustrates the multifaceted approach necessary to treat refractory cervical dystonia with concomitant rigid cervical kyphosis, in the face of neuromuscular pathology. Proper patient selection, consultation with pain management and neurology, and realistic patient expectations are crucial to successful and sustained correction of CD with rigid cervical deformity.

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