BACKGROUND: There have been few epidemiologic studies on neuromyelitis optica (NMO) and none used the recent 2006 diagnostic criteria. Here we describe the clinical, laboratory, MRI, and disability course of NMO in a French cohort of 125 patients. METHODS: We performed an observational, retrospective, multicenter study. Data were collected from September 2007 through August 2008, corresponding to the endpoint of the study. We identified 125 patients fulfilling the 2006 NMO criteria. Selection was made using hospital files and a specific clinical questionnaire for NMO. RESULTS: Mean age at onset was 34.5 years (range 4-66) with a mean disease duration of 10 +/- 7.8 years at the endpoint. The patients were mainly (87%) Caucasian, with a female:male ratio of 3:1. In 90% of cases, the association of optic neuritis, longitudinal extensive myelitis, and a Paty-negative initial brain MRI was sufficient to fulfill the supportive criteria. Eighty-eight percent of patients were treated with immunosuppressive therapies. Median delay from onset to Expanded Disability Status Scale (EDSS) score 4 was 7 years; score 6, 10 years; and score 7, 21 years. The first episode of myelitis was immediately followed by an EDSS score > or = 4 in 37.3% of cases, and a severe residual visual loss was observed in 22% of patients after the first episode of optic neuritis. Multivariate analysis did not reveal any predictors of a poor evolution other than a high number of MRI brain lesions at diagnosis, which were predictive of a residual visual acuity < or = 1/10. CONCLUSIONS: Our demographic data provide new data on disability in patients with neuromyelitis optica, most of whom were receiving treatment.
BACKGROUND: There have been few epidemiologic studies on neuromyelitis optica (NMO) and none used the recent 2006 diagnostic criteria. Here we describe the clinical, laboratory, MRI, and disability course of NMO in a French cohort of 125 patients. METHODS: We performed an observational, retrospective, multicenter study. Data were collected from September 2007 through August 2008, corresponding to the endpoint of the study. We identified 125 patients fulfilling the 2006 NMO criteria. Selection was made using hospital files and a specific clinical questionnaire for NMO. RESULTS: Mean age at onset was 34.5 years (range 4-66) with a mean disease duration of 10 +/- 7.8 years at the endpoint. The patients were mainly (87%) Caucasian, with a female:male ratio of 3:1. In 90% of cases, the association of optic neuritis, longitudinal extensive myelitis, and a Paty-negative initial brain MRI was sufficient to fulfill the supportive criteria. Eighty-eight percent of patients were treated with immunosuppressive therapies. Median delay from onset to Expanded Disability Status Scale (EDSS) score 4 was 7 years; score 6, 10 years; and score 7, 21 years. The first episode of myelitis was immediately followed by an EDSS score > or = 4 in 37.3% of cases, and a severe residual visual loss was observed in 22% of patients after the first episode of optic neuritis. Multivariate analysis did not reveal any predictors of a poor evolution other than a high number of MRI brain lesions at diagnosis, which were predictive of a residual visual acuity < or = 1/10. CONCLUSIONS: Our demographic data provide new data on disability in patients with neuromyelitis optica, most of whom were receiving treatment.
Authors: Amy M L Quek; Andrew McKeon; Vanda A Lennon; Jayawant N Mandrekar; Raffaele Iorio; Yujuan Jiao; Chiara Costanzi; Brian G Weinshenker; Dean M Wingerchuk; Claudia F Lucchinetti; Elizabeth A Shuster; Sean J Pittock Journal: Arch Neurol Date: 2012-08
Authors: Yujuan Jiao; James P Fryer; Vanda A Lennon; Sarah M Jenkins; Amy M L Quek; Carin Y Smith; Andrew McKeon; Chiara Costanzi; Raffaele Iorio; Brian G Weinshenker; Dean M Wingerchuk; Elizabeth A Shuster; Claudia F Lucchinetti; Sean J Pittock Journal: Neurology Date: 2013-08-30 Impact factor: 9.910
Authors: Su-Hyun Kim; Maureen A Mealy; Michael Levy; Felix Schmidt; Klemens Ruprecht; Friedemann Paul; Marius Ringelstein; Orhan Aktas; Hans-Peter Hartung; Nasrin Asgari; Jessica Li Tsz-Ching; Sasitorn Siritho; Naraporn Prayoonwiwat; Hyun-June Shin; Jae-Won Hyun; Mira Han; Maria Isabel Leite; Jacqueline Palace; Ho Jin Kim Journal: Neurology Date: 2018-10-26 Impact factor: 9.910