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Mult Scler. 2014 Jul;20(8):1086-94. doi: 10.1177/1352458513515085. Epub 2013 Dec 9.

Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset.

Author information

1
Department of Neurology, Hautepierre Hospital, University of Strasbourg, France nicolas.collongues@chru-strasbourg.fr.
2
Department of Neurology, Pierre Wertheimer Hospital, University of Lyon, France.
3
The Walton Centre for Neurology and Neurosurgery, The Walton Centre Foundation Trust, Liverpool, UK.
4
Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, UK.
5
Department of Neurology, Istanbul University, Turkey.
6
Neurocure, Charité University Medicine Berlin, Germany.
7
Department of Neurology, Robert Salengro Hospital, University of Lille Nord de France, France.
8
Division of Molecular Neuroimmunology, University of Heidelberg, Germany.
9
Department of Neurology, Groupe Hospitalier Pitié Salpétrière, Paris, France.
10
Department of Neurology, Hacettepe University, Ankara, Turkey.
11
Department of Neurology, Heinrich-Heine-University of Düsseldorf, Germany.
12
Department of Neurology, Bakýrkoy Hospital for Psychiatric and Neurological Diseases, Istanbul, Turkey.
13
Department of Neurology, Hautepierre Hospital, University of Strasbourg, France.

Abstract

BACKGROUND:

Few data are available for patients with a late onset (≥ 50 years) of neuromyelitis optica (LONMO) or neuromyelitis optica spectrum disease (LONMOSD), defined by an optic neuritis/longitudinally extensive transverse myelitis with aquaporin-4 antibodies (AQP4-Ab).

OBJECTIVE:

To characterize LONMO and LONMOSD, and to analyze their predictive factors of disability and death.

METHODS:

We identified 430 patients from four cohorts of NMO/NMOSD in France, Germany, Turkey and UK. We extracted the late onset patients and analyzed them for predictive factors of disability and death, using the Cox proportional model.

RESULTS:

We followed up on 63 patients with LONMO and 45 with LONMOSD during a mean of 4.6 years. This LONMO/LONMOSD cohort was mainly of Caucasian origin (93%), women (80%), seropositive for AQP4-Ab (85%) and from 50 to 82.5 years of age at onset. No progressive course was noted. At last follow-up, the median Expanded Disability Status Scale (EDSS) scores were 5.5 and 6 in the LONMO and LONMOSD groups, respectively. Outcome was mainly characterized by motor disability and relatively good visual function. At last follow-up, 14 patients had died, including seven (50%) due to acute myelitis and six (43%) because of opportunistic infections. The EDSS 4 score was independently predicted by an older age at onset, as a continuous variable after 50 years of age. Death was predicted by two independent factors: an older age at onset and a high annualized relapse rate.

CONCLUSION:

LONMO/LONMOSD is particularly severe, with a high rate of motor impairment and death.

KEYWORDS:

Aging; aquaporin antibody; aquaporin-4; late onset; morbidity; mortality; neuromyelitis optica; prognosis; spectrum disorders

PMID:
24323817
DOI:
10.1177/1352458513515085
[Indexed for MEDLINE]

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