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Neuromyelitis optica spectrum disorders: profile of a cohort according to the 2015 diagnostic criteria

R. Uribe-San Martín, E. Ciampi, A. Galilea, P. Sandoval, H. Miranda, P. Mellado, J.P. Cruz, I. Huete, C. Cárcamo [REV NEUROL 2017;65:193-202] PMID: 28849860 DOI: https://doi.org/10.33588/rn.6505.2017037

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Volumen 65 | Number 05 | Nº of views of the article 11.879 | Nº of PDF downloads 1.233 | Article publication date 01/09/2017

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ABSTRACT

INTRODUCTION The new 2015 criteria for neuromyelitis optica spectrum disorders (NMOSD) have been recently incorporated in the study of different international cohorts.

AIM To describe clinical-radiological characteristics and prognostic factors in patients with NMOSD according to the 2015 criteria.

PATIENTS AND METHODS Retrospective analysis of 36 patients diagnosed with NMOSD according to serologic AQP4 status (positive, negative, unknown and negative + unknown). Clinical and radiological characteristics were compared and possible disability prognostic factors were evaluated.

RESULTS AQP4 were positive in 7 patients, negative in 12 and unknown in 17. Age of presentation was 36.6 ± 16 years, with higher female proportion (4:1). Mean disease duration was 7.4 ± 7.6 years. Most frequent presenting symptoms were acute myelitis (61%), optic neuritis (33%) and area postrema syndrome (11%). Most frequent MRI lesion was longitudinally extensive transverse myelitis (75%). All patients received acute treatment during attacks, and preventive treatment was used in 81% (azathioprine and rituximab mostly prescribed). Median EDSS was 2.0 at the end of follow-up. No differences were observed in any of the variables comparing serologic status. Age of first attack was prognostic, with direct correlation with EDSS. First attack in < 30 years was protective, meanwhile > 50 years old patients had increased risk of disability.

CONCLUSIONS The 2015 criteria allow the description and classification of NMOSD patients within different cohorts. Age of first attack seems to be a prognostic factor for developing disability. KeywordsAnti-aquaporin 4 antibodies Devics disease Diagnostic criteria Myelitis Neuromyelitis optica Optic neuritis CategoriesTécnicas exploratorias

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Original

Neuromyelitis optica spectrum disorders: profile of a cohort according to the 2015 diagnostic criteria