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

Krumbholz, Markus and Hofstadt-van Oy, Ulrich and Angstwurm, Klemens and Kleiter, Ingo and Jarius, Sven and Paul, Friedemann and Aktas, Orhan and Buchholz, Grete and Kern, Peter and Straube, Andreas and Kuempfel, Tania (2015) Very late-onset neuromyelitis optica spectrum disorder beyond the age of 75. JOURNAL OF NEUROLOGY, 262 (5). pp. 1379-1384. ISSN 0340-5354, 1432-1459

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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.

Item Type: Article
Uncontrolled Keywords: MULTIPLE-SCLEROSIS; MULTICENTER; DISEASE; Neuromyelitis optica (Devic syndrome); Myelitis; Aquaporin 4 antibodies; Very late-onset; Elderly/old-age; Therapy/immunosuppression
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Neurologie
Depositing User: Dr. Gernot Deinzer
Date Deposited: 18 Jul 2019 11:18
Last Modified: 18 Jul 2019 11:18
URI: https://pred.uni-regensburg.de/id/eprint/5550

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