Is intraoperative neuromonitoring (IONM) a reliable tool for scoliosis correction in patients with friedreich’s ataxia?
Muaz Alghadir, Justin Arockiaraj, Muhammad Tariq Imtiaz
Objective: Our objective is to report the role of IONM in our patients with Freidrich’s ataxia in order to create awareness among Spine surgeons. Methods: This is a retrospective analysis of three patients with Freidrich’s ataxia who required surgery for severe scoliosis were included in the study. All three of them had clinical features and neurophysiological evidence of Freidrich’s ataxia with truncal imbalance. Two of them were wheel chair bound and one had ataxic gait. Scoliosis correction was done using pedicle screw instrumentation under multi-modality neuromonitoring. Results: There was minimal response in Motor evoked potential in the child who was walking. No reproducible or replicable baseline response was present in all the modalities of IONM in spite of supramaximal stimulation in the other two patients. Post operatively, one patient developed grade 1 reduction in neurology compared to the pre-operative status. All three of them had good correction and good sitting balance after surgical correction. Conclusion: Intra-operative neuromonitoring in patients with Freidreich’s ataxia remains a challenge. The good old ‘Stagnara wake-up’ test will be a better alternative to assess integrity of the spinal cord in these patients. Awareness among Spine surgeons is needed regarding this rare disease.
Muaz Alghadir, Justin Arockiaraj, Muhammad Tariq Imtiaz. Is intraoperative neuromonitoring (IONM) a reliable tool for scoliosis correction in patients with friedreich’s ataxia?. International Journal of Orthopaedics Research, Volume 2, Issue 4, 2020, Pages 57-61