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Very late-onset neuromyelitis optica spectrum disorder beyond the age of 75

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Item Type:Article
Title:Very late-onset neuromyelitis optica spectrum disorder beyond the age of 75
Creators Name:Krumbholz, M. and Hofstadt-van Oy, U. and Angstwurm, K. and Kleiter, I. and Jarius, S. and Paul, F. and Aktas, O. and Buchholz, G. and Kern, P. and Straube, A. and Kümpfel, T.
Abstract:Aquaporin-4 antibody (AQP4-Ab)-positive neuromyelitis optica spectrum disorder (NMOSD) is a rare but often severe autoimmune disease with median onset around 40 years of age. We report characteristics of three very-late-onset NMOSD (including complete NMO) patients >75 years of age, in whom this diagnosis initially seemed unlikely because of their age and age-associated concomitant diseases, and briefly review the literature. All three patients, aged 79, 82 and 88 years, presented with a spinal cord syndrome as the first clinical manifestation of AQP4-Ab-positive NMOSD. They all had severe relapses unless immunosuppressive therapy was initiated, and one untreated patient died of a fatal NMOSD course. Two patients developed side effects of immunosuppression. We conclude that a first manifestation of NMOSD should be considered even in patients beyond the age of 75 years with a compatible syndrome, especially longitudinally extensive myelitis. Early diagnosis and treatment are feasible and highly relevant. Special attention is warranted in the elderly to recognize adverse effects of immunosuppressive therapies as early as possible.
Keywords:Neuromyelitis Optica (Devic Syndrome), Myelitis, Aquaporin 4 Antibodies, Very Late-Onset, Elderly/Old-Age, Therapy/Immunosuppression
Source:Journal of Neurology
Page Range:1379-1384
Date:May 2015
Official Publication:https://doi.org/10.1007/s00415-015-7766-8
PubMed:View item in PubMed

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