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

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...

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Bibliographic Details
Published in:Journal of neurology 2015-05, Vol.262 (5), p.1379-1384
Main Authors: Krumbholz, Markus, Hofstadt-van Oy, Ulrich, Angstwurm, Klemens, Kleiter, Ingo, Jarius, Sven, Paul, Friedemann, Aktas, Orhan, Buchholz, Grete, Kern, Peter, Straube, Andreas, Kümpfel, Tania
Format: Article
Language:English
Subjects:
Age
Aged
Aged, 80 and over
Antibodies - blood
Aquaporin 4 - immunology
Aquaporins
Autoimmune diseases
Hospitals
Humans
Immunoglobulins
Late Onset Disorders - blood
Late Onset Disorders - diagnosis
Magnetic resonance imaging
Medicine
Medicine & Public Health
Neurology
Neuromyelitis Optica - blood
Neuromyelitis Optica - diagnosis
Neuroradiology
Neurosciences
Short
Short Commentary
Spinal cord
Vertebrae
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Summary: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.
ISSN:0340-5354
1432-1459
DOI:10.1007/s00415-015-7766-8