Abstract
Atlantoaxial instability (AAI) occurs as a result of trauma, congenital conditions such as os odontoideum, neoplasm, infection and degenerative connective tissue disorders such as rheumatoid arthritis, genetic conditions such as HOX-D3 and Down syndrome, and heritable connective tissue disorders, emblematic of which are the Ehlers Danlos syndromes (EDS). Prototypical of disorders in which AAI is diagnosed, is rheumatoid arthritis (RA). Prior to the development of effective disease-modifying pharmacotherapies, 88% of RA patients exhibited radiographic evidence of C1-C2 involvement, in whom 49% were symptomatic and 20% myelopathic; ultimately, 10% may have suffered atlantoaxial dislocation and death.
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