Syringomyelia with irreducible atlantoaxial dislocation, basilar invagination and Chiari I malformation

[摘要] A 27-year-old woman presented with a 5-year history of progressive weakness and numbness that began in her right extremities. Her symptoms progressed rapidly for the first year and eventually her entire right side became involved. When admitted, she complained of an unsteady gait requiring an assistive device to ambulate, and lacked coordination in her hands (impossible to button up her shirt). Neurologic examination of the lower extremities revealed normal sensation to light touch, pain and temperature, while sensory dysfunction of the upper limbs was present. Symmetrical hyperreflexia, as well as positive Hoffman’s and Babinski signs were present bilaterally. Weakness was present on both sides, with the right side being more severe. Her gait was wide-based, spastic, and unsteady. She also had limited range of motion on cervical in both flexion and extension. No other significant medical history was present.

[学科分类] 骨科学

[关键词] Syringomyelia;Irreducible atlantoaxial dislocation;Basilar invagination;Surgery;Transoral release