Clinical manifestations of NMO

Abstract Neuromyelitis optica (NMO), also known as Devic’s disease, is an idiopathic inflammatory demyelinating disease of the central nervous system (CNS) that preferentially targets the optic nerve and the spinal cord. Due to the significant risk of permanent visual and neurological impairment in NMO, rapid diagnosis and the prompt initiation of the most appropriate treatment are essential The current diagnostic criteria require a clinical history of optic neuritis and transverse myelitis accompanied by at least two of the following three supportive criteria: (i) brain MRI not diagnostic of multiple sclerosis (MS); (ii) spinal cord MRI with contiguous lesion over three or more vertebral segments; and (iii) IgG seropositivity against the aquaporin‐4 (AQP4) water channel. The development of optic neuritis and transverse myelitis can be simultaneous or sequential. In this presentation, the classical clinical features of NMO will be reviewed and put in the context of other demyelinating CNS disorders. Recent clinicopathological studies have revealed striking differences between NMO and prototypic MS, establishing them as distinct entities, and with important practical implications for the management of these two disorders.