Atlantoaxial rotatory subluxation associated with cervical dystonia

Case report.

To document an interesting case of cervical dystonia associated with atlantoaxial rotatory subluxation and compressive myelopathy.

Dystonia is an involuntary disorder characterized by twisting, pulling, and sustained contractions, which may be extremely powerful and painful. Cervical dystonia can contribute to the development of cervical spondylosis. However, cervical dystonia with atlantoaxial rotatory subluxation is very rare. Only 2 cases of primary dystonia with atlantoaxial subluxation have been reported previously.

We present the case of a 53-year-old man who had dystonic movements involving the neck. The head tilted to the left and rotated to the right. The subluxation was reduced by a sensory trick. Involuntary movements of the neck worsened. After 2 years, neck pain and difficulty in walking developed.

Cervical MRI showed atlantoaxial subluxation with compression of the spinal cord at C1-C2. Computed axial tomography of the cervical spine demonstrated rotatory subluxation of the atlas on the axis. Surgical intervention combined with globus pallidus internus-deep-brain stimulation and atlantoaxial fixation successfully controlled the cervical dystonia and atlantoaxial rotatory subluxation.

Although this case is extremely rare, clinicians should be keep in mind that cervical dystonia may carry a risk of atlantoaxial subluxation, potentially progressing to myelopathy. Neurosurgery combined with globus pallidus internus-deep-brain stimulation and atlantoaxial fixation is one treatment option for cervical dystonia with atlantoaxial rotatory subluxation.