Late Onset of Neuromyelitis Optica Spectrum Disorders

Neurol Ther. 2019 Dec;8(2):477-482. doi: 10.1007/s40120-019-0143-2. Epub 2019 Jul 2.

Abstract

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune demyelinating disease of the central nervous system. NMOSD starting after the age of 50 years is considered a "late onset" (LO-NMOSD) and seems to be particularly aggressive. The objective of this paper is to present a series of 37 Brazilian patients with LO-NMOSD.

Methods: Retrospective data collection from medical records of patients with LO-NMOSD seen at 14 Brazilian specialized units.

Results: The ratio of women to men in the sample was 4.3 to 1. The patients were followed up for a median period of 4 years. Sex, age at disease onset, and ethnic background were not associated with the number of relapses or disability outcomes. Extensive longitudinal myelitis affected 86% of patients, while optic neuritis affected 70%, and brainstem syndromes were present in only 16% of these patients. Six patients are currently using some type of support for walking or are wheelchair-bound. Three have died. Therapeutic options for NMOSD were particularly complicated for these elderly patients, since medications for controlling NMOSD are, in essence, immunosuppressive. Long-term use of corticosteroids can be an issue when the patients have high blood pressure, diabetes mellitus, or dyslipidemia (conditions often seen in elderly individuals).

Conclusion: This series of LO-NMOSD cases highlights the importance of prompt diagnosis and treatment for these patients.

Keywords: Disability; Elderly; Neurology; Neuromyelitis optica; Neuromyelitis optica spectrum disorders.