Background and Objectives: Atlantoaxial tuberculosis (TB) is rare, and its diagnosis is difficult. Herein, we present a rare case with a challenging diagnostic journey of atlantoaxial TB spanning over two years. Materials and Methods: A 70-year-old immunocompetent female patient presented with a four-week history of nuchal pain, stiffness, and headache. She did not have any TB-associated constitutional symptoms. The result of the initial biopsy indicated only a nonfermenting Gram-negative bacillus and the histopathological report revealed concurrent acute and chronic inflammation. Posterior fusion with bilateral C1 lateral mass and C2 transpedicular screw fixation was performed after a five-week course of antibiotics. Results: However, the atlantoaxial abscess progressed and led to myelopathy two years later. Tuberculous spondylitis was not confirmed until the second biopsy. We chose the transoral approach for prompt abscess evacuation and to prevent unnecessary damage to the nearby vital neurovascular structures. The sputum culture and chest radiograph did not reveal concurrent pulmonary TB. Conclusions: Spinal TB has a greater likelihood of presenting with a cold abscess without the typical constitutional symptoms of pulmonary TB. Distinctive magnetic resonance imaging (MRI) features, such as a thin and smooth abscess wall, subligamentous spread, severe vertebral body destruction, and heterogenous vertebral wall enhancement, might help to differentiate between tuberculous and pyogenic spondylitis. We hope to offer meaningful insights to clinicians facing similar intricate scenarios, including subtle clues that may lead to a quicker diagnosis and the considerations we made while designing a treatment plan.
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