The Natural History of Recurrent Optic Neuritis

Department of Neurology, Mayo Medical School, 200 First Street SW, Rochester, MN 55905, USA.
JAMA Neurology (Impact Factor: 7.42). 10/2004; 61(9):1401-5. DOI: 10.1001/archneur.61.9.1401
Source: PubMed


Optic neuritis (ON) may occur in isolation or may herald multiple sclerosis (MS) or neuromyelitis optica (NMO). Occasionally, ON may recur many times without intervening evidence of dissemination in space.
To define the clinical course and prognosis of patients with recurrent ON.
Retrospective medical record review and telephone follow-up survey.
Clinic-based practice in a large tertiary referral institution.
Survival analysis of conversion to MS and NMO and final visual impairment. We studied the association of clinical and demographic factors, the presence of brain lesions on magnetic resonance images, and the use of corticosteroid treatment at the time of the first ON occurrence with conversion to MS and NMO.
We identified 1274 patients with ON between 1994 and 2000 and selected 72 (5.7%) with recurrent ON without intervening symptoms of a disseminated demyelinating condition for further analysis. The 5-year conversion rate to NMO was 12.5% and to MS, 14.4%. Among 5 patients with 2 or more lesions consistent with MS on brain magnetic resonance images, 2 (40.0%) converted to MS and none to NMO, while among 11 patients without such lesions, none converted to MS and 2 (18.2%) converted to NMO (P =.16). Conversion to MS occurred in 7 (19.4%) of 36 individuals treated for their first ON episode with corticosteroids vs 4 (44.4%) of 9 untreated individuals (P =.19). There was no difference in the conversion rate to MS between those treated with intravenous steroids (4 [16.7%] of 24) vs oral steroids (3 [25.0%] of 12) (P =.33). Conversion to NMO occurred earlier than conversion to MS (2.3 +/- 1.6 vs 5.3 +/- 4.3 years, respectively; P =.01). Women tended to convert to NMO more frequently than men (female-male ratio for NMO converters, 7:1; MS converters, 2:1; nonconverters, 2:1; P =.56), as did those with a higher annual frequency of ON episodes (NMO converters, 2.0 +/- 1.3; MS converters, 1.0 +/- 1.0; nonconverters, 0.6 +/- 0.5; P =.04). The number of ON events in the first 2 years following the first ON episode was higher in the NMO group (NMO converters, 2.4 +/- 0.9; MS converters, 1.9 +/- 1.1; nonconverters, 1.7 +/- 0.7; P =.04). The final visual impairment was greatest in the NMO group (P =.02).
Patients with rapid succession of severe ON events are more likely to develop a generalized demyelinating disease. Patients with NMO had a worse visual outcome.

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Available from: Lori Blauwet, Jan 12, 2016
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    • "In our series, during a short follow-up, the conversion rate to MS and NMO was estimated to be 7.3 and 4.9%, respectively, in a SION cohort, which is comparable with another report from the Mayo Clinic, in which the one-year conversion rate to MS and NMO was estimated to be about 2.8 and 5.6%, respectively.[13] "
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    ABSTRACT: Optic neuritis (ON) can be the first presentation of multiple sclerosis (MS) or neuromyelitis optica (NMO). Anti-aquaporin-4 IgG (AQP4 IgG) is a highly specific and moderately sensitive biomarker for NMO. This study was designed to assess the rate of seropositivity for AQP4 IgG, and the short-term outcome of patients presenting with single isolated ON (SION). A cohort of 41 consecutive patients experiencing severe (< 20 / 200) SION (not fulfilling the diagnostic criteria for MS or NMO), was prospectively recruited. Blood sampling was carried out immediately after the diagnosis of ON, and AQP4 IgG was tested qualitatively, using an indirect immunofluorescence kit. After clinical and paraclinical investigations, all the patients were followed up for a short-term period of at least 18 months. The seroprevalence among the initial ON patients was 9.7% (4 / 41). The short-term conversion rate to MS and NMO was estimated to be about 7.3 and 4.9%, respectively. The conversion rate to NMO in initially seropositive patients was greater than that for the whole cohort [2 / 4 (50%) vs. 2 / 41 (4.9%); P = 0.035; Odds ratio: 19.5, 95% confidence interval: 1.73 to 219.50]. AQP4 IgG seropositive SION patients were more likely to develop NMO in comparison to the total SION population. Further studies, with a longer follow-up period and larger sample sizes are warranted to assess the clinical and prognostic value of assessing AQP4 IgG in SION.
    Full-text · Article · Sep 2012 · International journal of preventive medicine
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    • "Although NMO is often fulminant and has a more negative outcome than MS [32], NMO responds to glucocorticoids, immunosuppressive agents, or plasmapheresis. Since monosymptomatic ON is often seen as being the first indication of an attack of NMO and MS, ophthalmoscopic examination, especially the visual field test, is helpful for diagnosis of NMO, and anti-AQP4 antibody should be checked to decide the most effective treatment [33]. "
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    ABSTRACT: Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that predominantly affects the optic nerves and the spinal cord, and is possibly mediated by an immune mechanism distinct from that of multiple sclerosis (MS). Central scotoma is recognized as a characteristic visual field defect pattern of optic neuritis (ON), however, the differing pathogenic mechanisms of NMO and MS may result in different patterns of visual field defects for ON. Medical records of 15 patients with NMO and 20 patients with MS having ON were retrospectively analyzed. A thorough systemic and neurological examination was performed for evaluating ON. The total number of relapses of ON and visual fields was investigated. Visual fields were obtained by Goldmann perimeter with each ON relapse. All MS patients experienced central scotoma, with 90% of them showing central scotoma with every ON relapse. However, 53% of NMO patients showed central scotoma with every ON relapse (p = 0.022), and the remaining 47% of patients experienced non-central scotoma (altitudinal, quadrant, three quadrant, hemianopia, and bitemporal hemianopia). Thirteen percent of NMO patients did not experience central scotoma during their disease course. Altitudinal hemianopia was the most frequent non-central scotoma pattern in NMO. NMO patients showed higher incidence of non-central scotoma than MS, and altitudinal hemianopia may be characteristic of ON occurring in NMO. As altitudinal hemianopia is highly characteristic of ischemic optic neuropathy, we suggest that an ischemic mechanism mediated by anti-aquaporin-4 antibody may play a role in ON in NMO patients.
    Full-text · Article · Jun 2010 · BMC Neurology
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    • "This study indicates that a subpopulation of patients with ON may have an immunological pathogenesis similar to that seen with NMO. A recent large study (Pirko et al. 2004) revealed that 12.5% of cases of recurrent ON evolved to NMO. It is important to test for the presence of AQP4-Ab in patients with ON, because AQP4-Ab positive patients may have greater potential for developing severe transverse myelitis in the future. "
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    ABSTRACT: It has recently been reported that the anti-aquaporin-4 antibody (AQP4-Ab) can be a specific marker of neuromyelitis optica. We present three cases of optic neuritis (ON) where the patients tested positive for AQP4-Ab, but showed no neurological signs. Sera were obtained from 32 Japanese patients with ON and no other neurological abnormalities (mean age 46 +/- 20 years). AQP4-Ab was detected by indirect immunofluorescence staining using human-AQP4-transfected HEK 293 cells. AQP4-Ab was positive in three female patients (aged 9, 64 and 82 years). Their illness was characterized by bilateral severe optic nerve involvement, insufficient visual recovery, and autoimmune abnormalities (such as positive antinuclear antibody). Two of these patients experienced recurrent episodes of ON. In at least two episodes, the intracranial portion of the optic nerve showed significant inflammation on magnetic resonance imaging. These cases indicate that some ON patients have an immunological pathogenesis similar to that seen in neuromyelitis optica. In addition, examination for AQP4-Ab positivity in the initial phase of ON is important in predicting the prognosis, including the possibility of the development of transverse myelitis.
    Full-text · Article · Dec 2008 · Acta ophthalmologica
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