• Koti Neeraja, Shweta Prasad, Bharath Kumar Surisetti, Vikram Venkappayya Holla, Dinesh Sharma, Nitish Kamble, Karthik Kulanthaivelu, Srinivas Dwarakanth, Nupur Pruthi, Pramod Kumar Pal, and Ravi Yadav.
• Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
• World Neurosurg. 2021 Feb 1; 146: e1287-e1292.

ObjectiveAtlantoaxial instability, although rarely reported in the literature, can be associated with cervical dystonia (CD) and may lead to compression of the cord at the craniovertebral junction. We present a case series of 4 patients of longstanding CD with neurologic complications. Treatment strategies and challenges are discussed.MethodsRetrospective analysis of 4 cases of longstanding CD with complications of myelopathy or radiculopathy.ResultsThe average age at onset of complications was 28 years (range, 17-37). The average duration of CD was 23.75 years. Narrowing of the craniovertebral junction was seen in 3 patients, of which 2 had os odontoideum, and 1 had rotational malalignment at the atlantoaxial joint. One patient had disc desiccation with bulge and intramedullary signal changes in the cord at C3-4 level. Medical treatment was not satisfactory, but botulinum toxin was partly useful in all. One patient had sequelae of myelopathy and did recover partially after deep brain stimulation. Of the 2 patients who underwent surgical fixation with a fusion of the spine, one improved, and the other had no improvement due to irreversible cord damage. The overall outcome was satisfactory only in 2 patients.ConclusionsEarly-onset CD can lead to cord complications at a young age and at higher levels of the cervical spine and at the cervicovertebral junction. Comprehensive management by a multidisciplinary team is crucial to prevent complications early.Copyright © 2020 Elsevier Inc. All rights reserved.

41 keywords...

hide…