Diagnostic criteria of neuromyelitis optica spectrum disorders (NMOSD) have expanded to include many new symptoms since the original description of Devic's disease in 1894, and the formulation of diagnostic criteria in 2006. Herein, we describe a patient with serum aquaporin positive NMOSD with the unusual presentation of recurrent regional autonomic dysfunction, with concordant MRI findings. The symptomatic treatment of the patient's causalgia required IV ketamine infusion. Besides, as the long segment myelitis occurred in the setting of extensively drug-resistant tuberculosis (XDR TB), there was the added challenge of ruling out TB myelitis and choosing an immunosuppressant that would have the least risk of reactivation of TB.
Keywords: Associated-XDR TB; NMOSD; dysautonomia.