Abstract
Background Neuromyelitis optica (NMO) is a rare relapsing inflammatory disorder of the central nervous system, characterised by immune-mediated demyelination, predominately affecting the spinal cord and optic nerves. It is associated with autoantibodies to aquaporin-4 (AQP4) water channels.
Methods We describe the case of a 77 year old female who was diagnosed with neuromyelitis optica after presenting with acute myelitis.
Results T2-weighted magnetic resonance imaging (MRI) of the whole spine revealed a longitudinally extensive intramedullary spinal cord lesion extending from T1-T12, involving the central cord grey matter. Serum and cerebrospinal fluid (CSF) AQP4 antibody testing were both positive. The CSF was notable for elevated protein and a monocyte predominant pleocytosis. CSF oligoclonal bands were positive. MRI brain revealed numerous subcortical T2-FLAIR hyperintensities. Fundoscopy and MRI orbits did not reveal any evidence of optic neuritis.
Conclusion Knowledge of NMO is important as it is a differential for patients presenting with an acute myelitis; whatever the age. Although the disease typically affects patients of middle-age (median age of onset is 39 years) it is important to remember that 15–20% of patients may present to paediatricians (<16 years) or elderly care physicians (>65 years). It should be suspected when the MRI spine demonstrates a longitudinally extensive spinal cord lesion, extending for three or more vertebral segments, primarily involving the central cord gray matter. The syndrome is rare therefore there are no randomised controlled trials to guide treatment. Relapses are severely disabling, therefore prevention with immunosuppression is the most important aspect of management.
