A clinical scoring system for neurological assessment of high cervical myelopathy: measurements in pediatric patients with congenital atlantoaxial dislocations.

OBJECTIVE: The assessment of response to treatment in pediatric patients with congenital atlantoaxial dislocation (AAD) is performed using a disability grading system but may be better determined by a score based on clinical parameters. This study proposes a scoring system based on a comprehensive neurological examination to assess surgical outcome in these patients.
METHODS: Sixty-seven patients with congenital AAD aged 14 years or younger were included and analyzed prospectively. A scoring system based on six factors (motor power, gait, sensory involvement, sphincteric involvement, spasticity, and respiratory difficulty) was designed at the beginning of the study and all patients were assessed using this score as well as the Di Lorenzo's grade preoperatively, postoperatively, and at the time of each follow-up visit.
RESULTS: There was a very high incidence of occipitalized arch of atlas and fusion of the second and third cervical vertebrae in the irreducible variety. Most patients were classified in poor grades preoperatively; however, the changes in score were seen more often when using the scoring system we developed compared with the Di Lorenzo's grade. Our score also corroborated better with the clinical improvement.
CONCLUSION: The clinical profiles of pediatric patients with AAD are similar with a higher incidence of atlas arch anomalies in patients with irreducible AAD. A scoring system based on clinical parameters is proposed for clinical evaluation of such patients. This system is easy to use and interpret and is more sensitive to the changes in the neurological status of patients.