A 32-year-old lady presented with acute paroxysmal facial pain in the V1-V2 distribution, lower cranial nerve dysfunction, and mild long-tract neurological signs. Paroxysmal facial pain with features of trigeminal autonomic cephalgia (TAC) and trigeminal neuralgia (TN) (Tic) was the unique presentation in this case, explained by involvement of the somatotopically arranged spinal trigeminal nucleus. She had two attacks of area postrema syndrome (APS) preceding the current symptoms, evaluated as a gastroenterological disorder, causing a delay in imaging and AQP4-seropositive diagnosis of neuromyelitis optica spectrum disorder (NMOSD). She recovered from brainstem symptoms with immunosuppression but continued to have painful tonic spasms and neuropathic pain as immediate sequelae. The untreated first attack, AQP4 positivity, and relapse within the 1st year are all risk factors for further relapses and long-term morbidity, as was the case with our patient.
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Varghese et al. (2024) studied this question.
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