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

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.

journal_name

J Neurol

journal_title

Journal of neurology

authors

Krumbholz M,Hofstadt-van Oy U,Angstwurm K,Kleiter I,Jarius S,Paul F,Aktas O,Buchholz G,Kern P,Straube A,Kümpfel T

doi

10.1007/s00415-015-7766-8

subject

Has Abstract

pub_date

2015-05-01 00:00:00

pages

1379-84

issue

5

eissn

0340-5354

issn

1432-1459

journal_volume

262

pub_type

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