A 65-year-old woman, a case of cranio-vertebral junction anomaly, presented with a sudden onset of quadriparesis, dysphagia, and breathing difficulty and required emergency surgery. She underwent image-guided endoscopic transnasal odontoidectomy followed by occipito-cervical decompression and stabilization. She recovered well, with immediate improvement of respiratory function. Burning paresthesias disappeared and the upper limbs function normalized.
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