Relapsing Inflammatory Opticneuritis: Is It Neuromyelitis Optica?

Isolated, relapsing and progressive demyelinating diseases of the central nervous system

“…Some of these patients will develop NMO [35,48,51] , and here seropositivity for NMO-IgG is of prognostic relevance [35]. The percentage of RION patients who were positive for NMO-IgG was 20-25 % in two reports from the Mayo Clinic [30,35], 19 %-25 % in two French studies [5,34] and 14 % in a collaborative Spanish-Italian study [65]. Our own experience is closer to the 6 % mark (unpublished).…”

Pathogenesis, diagnosis and treatment of neuromyelitis optica: Changing concept of an old disease

“…54 However, considering the natural course of NMO spectrum, anti-AQP4 seropositivity suggests a high risk for relapses and NMO conversion. 15,56,58,59,62 In addition, seropositive cases of myelitis and definite NMO were similar in regard to annual relapse rate, pathology and CSF findings, suggesting similar pathogenesis. 57 Initiation of immunosuppressive therapy was suggested in all cases of LETM, RION with NMOIgG, and severe cases of relapsing ON regardless of antibody testing.…”

“…54 However, because pathological and immunological profiles are similar to NMO, the risk for severe relapses and conversion rate to NMO is high, early therapy should be considered in certain clinical situations (Table 3). [57][58][59][60][61][62] Besides RION ⁄ BION and LETM, some other less defined spectrum diseases have been also suggested: atypical cases, Japanese ''opticospinal MS'', an association of ON or LETM with co-morbidities especially systemic autoimmune diseases and with specific Figure 2 Diagnostic steps in the case of relapsing ⁄ bilateral optic neuritis. Brain magnetic resonance imaging (MRI) and screening for anti-aquaporin 4 (AQP4) antibodies can help in differentiating neuromyelitis optica (NMO) spectrum from clinically isolated syndrome (CIS) or multiple sclerosis (MS).…”

“…Although titers in spatially limited forms are not different from NMO, seropositivity is less frequent; depending on the method, only 14-55% of LETM and 5-27% of RION is seropositive. 6,26,56,[58][59][60][61][62]135,143,145 Nevertheless, screening can help in differentiating from other demyelinating diseases: (i) in contrast to NMO spectrum, sera of patients with MS, acute disseminated encephalomyelitis, monophasic ON and short transverse myelitis were negative; 7,146 (ii) in a prospective study of ON, seropositivity was 56% for NMO, but just 5% for single isolated ON, relapsing ON, chronic relapsing inflammatory ON, and 0% for ON associated with MS.…”

“…56,58,59,62 In RION, seropositivity predicts a worse prognosis and more severe residual signs; more than 50% convert to NMO in 5 years in contrast to 10% of seronegative RION patients in 25 years. 58 In the case of LETM, seropositivity predicts relapses and 65% might experience another myelitis or ON within a year.…”