Cervical Myelopathy Due to Atlantoaxial Instability Associated with Down Syndrome: A Case Report

Abstract

Spinal cord compression secondary to atlantoaxial instability in Down syndrome is relatively uncommon in young children. We report a 4-year-old boy with Down syndrome and myelopathy caused by atlantoaxial instability (AAI). At presentation he had right limb hemiparesis, ataxia, and bilateral hyper-reflexia for 2 weeks. There was no obvious history of trauma except for frequent upper respiratory tract infection. Cervical spine x-ray showed increased distance between the atlas and the dental process, which fulfilled the criteria for atlantoaxial instability, and cervical MRI showed spinal cord compression and myelomalacia. His neurologic signs gradually improved after surgical treatment. This report emphasized the importance of early recognition and diagnostic approach for prevention of the potential cervical spinal cord injury secondary to AAI in very young children with Down syndrome.