Symptomatic brain involvement as the initial manifestation of neuromyelitis optica

Neuromyelitis optica (NMO) is an inflammatory demyelinating disorder that predominantly affects the optic nerve and spinal cord; however, symptomatic brain involvement is not rare and is sometimes an initial manifestation in NMO. In this study, we investigated the characteristic features of patients with NMO with symptomatic brain involvement as the initial manifestation of disease (NMO(brain)) compared with patients with NMO who presented initially with optic neuritis or myelitis (NMO(ON/myelitis)). We retrospectively reviewed 27 consecutive Korean patients with NMO with aquaporin-4 antibodies. Patients with NMO(brain) (n=9) initially presented with intractable hiccup/nausea/vomiting and/or encephalopathy at a younger age than the patients with NMO(ON/myelitis) (n=18) (p<0.01). During the disease course, the patients with NMO(brain) continued to show more frequent symptomatic involvement of the brain than the 18 patients with NMO(ON/myelitis) (p<0.05). At the final visit, the mean age was also significantly lower in patients with NMO(brain) than in patients with NMO(ON/myelitis) (p<0.01); however, the Expanded Disability Status Scale scores, used to evaluate disease progression, were not different between the two groups. Our study suggests that patients with NMO who present initially with symptomatic brain involvement may have earlier disease onset and become disabled at a younger age compared to patients with typical NMO. Additional large scale prospective studies are warranted.