Acute-Onset Severe Occipital Neuralgia Associated With High Cervical Lesion in Patients With Neuromyelitis Optica Spectrum Disorder

Objective.-To address occipital neuralgia in patients with neuromyelitis optica spectrum disorder (NMOSD). Background.-NMOSD is an inflammatory demyelinating disease that commonly presents with pain; however, headache symptoms have received little attention. Methods.-We presented three cases of NMOSD in which the patients experienced acute-onset, severe, and steroid-responsive occipital neuralgia. All patients provided consent to use their demographic and imaging data retrospectively. Results.-In all three cases, MRI revealed a new high-intensity area in the cervical cord at the C1-C3 level of the spine, which was diminished in two of the three cases after corticosteroid pulse therapy. Conclusion.-Our cases support the recognition of NMOSD as a cause of secondary headache. As patients with NMOSD experience severe occipital neuralgia, a relapse should be considered and a cervical MRI should be performed.