Koti Neeraja1, Shweta Prasad2, Bharath Kumar Surisetti1, Vikram Venkappayya Holla1, Dinesh Sharma3, Nitish Kamble1, Karthik Kulanthaivelu3, Srinivas Dwarakanth4, Nupur Pruthi4, Pramod Kumar Pal1, Ravi Yadav5. 1. Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India. 2. Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India; Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences, Bengaluru, India. 3. Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Bengaluru, India. 4. Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru, India. 5. Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India. Electronic address: docravi20@yahoo.com.
Abstract
OBJECTIVE: Atlantoaxial 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. METHODS: Retrospective analysis of 4 cases of longstanding CD with complications of myelopathy or radiculopathy. RESULTS: The 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. CONCLUSIONS: Early-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.
OBJECTIVE: Atlantoaxial 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. METHODS: Retrospective analysis of 4 cases of longstanding CD with complications of myelopathy or radiculopathy. RESULTS: The 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. CONCLUSIONS: Early-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.