Highlights
- •2015 NMOSD criteria increase the rate of diagnosis.
- •Higher EDSS at onset is an independent predictor of reaching severe disability.
- •Severe visual deficit is reached earlier after optic neuritis or opticospinal onset.
- •Longer time from NMOSD onset to maintenance treatment predicts severe disability.
- •NMOSD studies should utilize OSIS as an outcome disability measure.
Abstract
Background
Neuromyelitis optica spectrum disorder (NMOSD) most commonly, although not exclusively,
targets optic nerves and spinal cord. Untreated, early and severe disability is common.
We evaluated the long-term outcome in NMOSD patients diagnosed according to the 2015
criteria.
Methods
We retrospectively analyzed 74 patients from the hospital-based NMOSD cohort at the
Clinic of Neurology, Belgrade, Serbia, who fulfilled the 2015 NMOSD criteria. We identified
patients based on 2015 criteria; 51.4% of whom would not have fulfilled 2006 criteria.
Median follow-up was 6.9 years. Aquaporin-4 (AQP4) IgG was tested in all patients
using a cell-based indirect immunofluorescence assay. The level of neurological disability
was assessed by the Expanded Disability Status Scale (EDSS) score, and by Opticospinal
Impairment Scale (OSIS), visual acuity (VA) and motor function subscores.
Results
The disease course was monophasic in 17.6% patients and relapsing in the remainder;
none developed progressive disease. AQP4-IgG was detected in 89.2% of patients. 45
of 74 patients were treated with immunosuppressants, 40 with azathioprine, 3 with
mycophenolate mofetil, 1 with cyclophosphamide, 1 with mitoxantrone, and 2 patients
with rituximab. The median intervals from onset to EDSS 4.0, 6.0 and 7.0 were 6.5
years, 11.9, and 22.0 years, respectively. Higher baseline EDSS was associated with
risk of attaining EDSS 4.0, 6.0 and 7.0; a shorter first inter-attack interval for
reaching EDSS 4.0 and 6.0; longer time to the start of treatment for reaching EDSS
7.0. Worse visual acuity at the disease onset predicted faster assignment of OSIS
VA = 6 and VA = 8. Severe visual deficit (OSIS VA 6) was reached earlier after optic neuritis (median time, 10.0 years) or combined
opticospinal onset (median time, 11.4 years) than after myelitis onset (median time,
18.0 years) (p = 0.002).
Conclusion
Our results support the benefits of early diagnosis and treatment of NMOSD, especially
in persons with severe optic and spinal disability at onset.
Keywords
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References
- Neuromyelitis optica in Brazil: a study on clinical and prognostic factors.Mult. Scler. 2009; 15: 613-619
- Relapsing neuromyelitis optica: long term history and clinical predictors of death.J. Neurol. Neurosurg. Psychiatry. 2009; 80: 1162-1164
- Application of the 2015 diagnostic criteria for neuromyelitis optica spectrum disorders in a cohort of Latin American patients.Mult. Scler. Relat. Disord. 2018; 20: 109-114
- Neuromyelitis optica in France: a multicenter study of 125 patients.Neurology. 2010; 74: 736-742
- Clinical characteristics of 153 Brazilian patients with neuromyelitis optica spectrum disorder (NMOSD).Mult. Scler. Relat. Disord. 2019; 27: 392-396
- Clinical characteristics, course and prognosis of relapsing Devic's Neuromyelitis Optica.J. Neurol. 2004; 251: 47-52
- The impact of 2015 neuromyelitis optica spectrum disorders criteria on diagnostic rates.Mult. Scler. 2017; 23: 228-233
- Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan.Brain. 2012; 135: 1834-1849
- Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS).Neurology. 1983; 33: 1444-1452
- A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis.Lancet. 2004; 364: 2106-2112
- Understanding the antibody repertoire in neuropsychiatric systemic lupus erythematosus and neuromyelitis optica spectrum disorder: do they share common targets?.Arthritis. Rheumatol. 2018; 70: 277-286
- Eculizumab in AQP4-IgG-positive relapsing neuromyelitis optica spectrum disorders: an open-label pilot study.Lancet Neurol. 2013; 12: 554-562
- Characteristics of Devic's disease (neuromyelitis optica) in Mexico.J. Neurol. 2008; 255: 710-715
- Area postrema syndrome: frequency, criteria, and severity in AQP4-IgG-positive NMOSD.Neurology. 2018; 91: e1642-e1651
- International consensus diagnostic criteria for neuromyelitis optica spectrum disorders.Neurology. 2015; 85: 177-189
- The clinical course of neuromyelitis optica (Devic's syndrome).Neurology. 1999; 53: 1107-1114
- The spectrum of neuromyelitis optica.Lancet Neurol. 2007; 6: 805-815
- A secondary progressive clinical course is uncommon in neuromyelitis optica.Neurology. 2007; 68: 603-605
- Neuromyelitis optica: clinical predictors of a relapsing course and survival.Neurology. 2003; 60: 848-853
Article info
Publication history
Published online: September 27, 2019
Accepted:
September 25,
2019
Received in revised form:
September 22,
2019
Received:
August 16,
2019
Identification
Copyright
© 2019 Elsevier B.V. All rights reserved.
