Neuromyelitis optica and neuromyelitis optica spectrum disorder: Natural history and long-term outcome, an Indian experience

Abstract

Background:Neuromyelitis optica (NMO) has evolved from devic's classical description to a broader disease spectrum, from monophasic illness to a polyphasic illness with multiple recurrences, disease confined to optic nerve and spinal cord to now brain stem, cerebrum and even endocrinopathy due to hypothalamic involvement.Objectives:To report, the epidemiological characteristics, clinical presentations, recurrence rate, treatment and response to therapy in 26 patients with NMO and NMO spectrum disorder among the Indian population.Methods:We performed observational, retrospective analysis of our prospectively maintained data base of patients with NMO, longitudinally extensive transverse myelitis during the period of January 2003–December 2012 who satisfied the national multiple sclerosis society (NMSS) task force criteria for diagnosis of NMO and NMO spectrum disorder.Results:There were 26 patients (female: male, 21:5), the mean age of onset of symptom was 27 years (range 9–58, standard deviation = 12). Twenty-one patients (80%) fulfilled NMSS criteria for NMO while rest 5 patients (20%) were considered as NMO spectrum disorder. Seven patients (27%) had a monophasic illness, 19 patients (73%) had a polyphasic illness with recurrences. The Median recurrence rate was 4/patient in the polyphasic group. 13 (50%) patient were tested for aquaporin 4 antibody, 8 (61%) were positive while 5 patients (39%) were negative. All patients received intravenous methyl prednisolone, 9 patients (35%) required further treatment for acute illness in view of unresponsiveness to steroids. Thirteen patients (50%) received disease-modifying agents for recurrences. Mean duration of follow-up was 5 years. All patients had a good outcome (modified Rankin scale, <3) except one who had poor visual recovery.Conclusion:Neuromyelitis optica/NMO spectrum disorder is demyelinating disorder with female predominance, polyphasic course, myelitis being most common event although brain stem involvement is not uncommon with NMO antibody positivity in 60% patients, confirms the literature data.

Profile of neuromyelitis optica and spectrum disorders in an Indian cohort

“…The authors confirmed a strong female preponderance for NMO/NMOSD. [ 8 ] However, women/men ratio in this study was lower compared to a recent paper on the Indian NMO/NMOSD cohort,[ 7 ] (female: male = 4.2:1 and 7.8:1, respectively), but higher in the monophasic than in the polyphasic form (6:1 and 3.75:1, respectively),[ 8 ] in contrast to previous literature data. [ 1 ]…”

“…Among 83 recurrent events, ON was found in 23%, LETM in 56.62%, both ON and LETM in 16.9% of polyphasic patients, being these data similar to those reported in a recent work on an Indian NMO/NMOSD cohort. [ 7 ] Even the median number of relapses was similar in both studies: 4 (range: 2–8) during a mean follow-up of 5 years in the former,[ 8 ] and 3 (range: 1–15) during a median follow-up of 3 years (range: 0.33–30 years) in the latter. [ 7 ] Moreover, the mean age of symptom onset of 27 years (range: 9–58) found in this study is similar to that median of 26.5 (range: 8–72) of a previous Indian study,[ 7 ] but it is lower than that reported in previous studies performed in American and Western countries,[ 1 4 ] suggesting that NMO/NMOSD cases in Indian population might tend to present at an earlier age.…”

“…In this new retrospective study,[ 8 ] the authors describe epidemiological characteristics, clinical presentations, recurrence rate, treatment and response to therapy in 21 NMO and 6 NMOSD patients among Indian population. Although the authors did not report NMO/NMOSD prevalence rate in their population, patients’ sample size is quite large and the mean follow-up period is rather long (5 years).…”

“…Among NMO-IgG tested patients only the 61% were found to be seropositive, in contrast to a higher percentage reaching the 84% reported in another Indian cohort. [ 7 ] Cerebrospinal fluid pleocytosis was noted in 33% patients with a mean of 11 leukocytes (range: 2–75/mm 3 ) and protein levels ranged 18–70 mg%,[ 8 ] whereas the previous study found a median value of 10 leukocytes (range: 2–940/mm 3 ) with a median protein level of 40.5 (range: 16–790 mg%) including likely more severe cases. [ 7 ]…”

Estudo comparativo do acometimento medular entre a mielite transversa aguda idiopática e a neuromielite óptica

“…Adicionalmente, outros pontos vêm ganhando atenção ampliando assim o espectro da caracterização dos episódios de neuromielite óptica. Notadamente, o curso monofásico da doença, a qual se atribui frequência de 5% a 10% (Wingerchuk et al, 2015), embora na Índia tenha frequência ainda superior, 27% (Jagtap et al, 2015).…”