Author Of 2 Presentations
P0129 - Pattern of brain attack occurrence in neuromyelitis optica spectrum disorder: effect of treatment (ID 1626)
Abstract
Background
Brain involvement in neuromyelitis optia spcectrum disorder (NMOSD) is well recognized and some characteristic magnetic resonance imaging (MRI) abnormalities are incorporated into the recently proposed diagnostic criteria for NMOSD. However, little is known about clinical implication of brain attacks in NMOSD.
Objectives
To analyze patterns of frequency and timing of brain attacks with regard to treatment compared to those of optic neuritis or myelitis in patients with NMOSD.
Methods
This study retrospectively reviewed 260 consecutive aquaporin-4 immunoglobulin G (AQP4-IgG) seropositive NMOSD patients who were on immunosuppressive therapies (IST) more than 6 months between October, 2005 and April, 2020 at National Cancer Center, Korea. Brain attack was defined as newly developed acute neurological symptoms lasting ≥24 hours with corresponding acute NMOSD-characteristic brain lesion(s). The following demographic and clinical variables were collected: sex, onset age, disease duration, types (optic neuritis [ON], myelitis, brain attack and area postrema syndrome [APS]) and date of each attack, and treatment history.
Results
A total of 1738 attacks occurred in 260 patients during median 143 months (interquartile range 84-195 months) of observation period: 1377 and 361attacks before and after initiation of IST, respectively. Brain attacks were observed in 95 (37%) patients: 147 attacks in 93 (36%) patients before IST and 13 attacks in 11 (4%) patients after IST. APS was observed in 25 (9.6%) patients before IST especially at the disease onset, but no one experienced APS after IST. Before IST, ON, myelitis and brain attack were observed at 36.8, 78.7 and 14.1 per 100 patient-years, respectively, while decreased to 4.5, 12.1 and 0.6 per 100 patient-years after IST, respectively. The reduction rates of each attack frequency after IST were significantly greater in brain attack than in ON or myelitis (mean 78% vs. 95%, p=0.014). Patients with brain attacks despite IST showed higher mean annualized relapse rate (ARR) compared to those without brain attacks (p=0.029).
Conclusions
Impact of IST appeared to be greater on the reduction of brain attack than that of ON and myelitis in AQP4-IgG seropositive NMOSD, resulting in rare occurrence of brain attacks after IST. ARR was higher in patients with brain attack than in those without brain attack particularly after IST. These results suggest brain attack may allude high disease activity in NMOSD.
P0709 - Discontinuation of therapy may lead to an increased risk of relapse in patients with neuromyelitis optica even after 5 years of remission (ID 1617)
Abstract
Background
Background: Current treatment guidelines recommend early immunosuppressive therapy (IST) to prevent additional relapses in neuromyelitis optica spectrum disorder (NMOSD). The feasibility of IST discontinuation regarding safety, adverse events, and cost, after long-term remission is achieved is a commonly encountered question in clinical practice.
Objectives
We aimed to evaluate the outcomes of IST discontinuation in patients with anti-aquaporin-4 antibody-positive NMOSD an after a sustained remission period.
Methods
We retrospectively reviewed medical records of 17 patients with anti-aquaporin-4 antibody-positive NMOSD who discontinued IST after a relapse-free period ≥3 years. Anti-aquarporin-4 antibodies were meausred once a year during IST and after IST discontinuation.
Results
IST was discontinued at a median age of 40 years (interquartile range [IQR], 32–51) after a median relapse-free period of 62 months (IQR, 52–73). Among 17 patients, 14 (82%) relapsed after a median duration of 6 months (IQR, 4–34) after IST discontinuation, three (18%) of which had severe attacks: all three patients had a severe attack history before IST. The three patients received steroids followed by plasma exchange for acute treatment, but two showed poor recovery and significant Expanded Disability Status Scale worsening after 6 months of the attack. Six (35%) patients showed seroconversion (from seropositive to seronegative) of anti-aquaporin-4 antibodies at least once during IST, and 5 (29%) were seronegative for anti-aquaporin-4 antibodies at the time of discontinuation. However, 4 (80%) of the 5 patients had seroreversion (from seronegative to seropositive) again after IST discontinuation.
Conclusions
IST discontinuation may increase relapse risk in seropositive NMOSD patients even after 5 years of remission. Given the potential devastating consequence of a single attack of NMOSD, IST discontinuation requires more caution in patients with severe attack prior to IST.
Presenter Of 1 Presentation
P0129 - Pattern of brain attack occurrence in neuromyelitis optica spectrum disorder: effect of treatment (ID 1626)
Abstract
Background
Brain involvement in neuromyelitis optia spcectrum disorder (NMOSD) is well recognized and some characteristic magnetic resonance imaging (MRI) abnormalities are incorporated into the recently proposed diagnostic criteria for NMOSD. However, little is known about clinical implication of brain attacks in NMOSD.
Objectives
To analyze patterns of frequency and timing of brain attacks with regard to treatment compared to those of optic neuritis or myelitis in patients with NMOSD.
Methods
This study retrospectively reviewed 260 consecutive aquaporin-4 immunoglobulin G (AQP4-IgG) seropositive NMOSD patients who were on immunosuppressive therapies (IST) more than 6 months between October, 2005 and April, 2020 at National Cancer Center, Korea. Brain attack was defined as newly developed acute neurological symptoms lasting ≥24 hours with corresponding acute NMOSD-characteristic brain lesion(s). The following demographic and clinical variables were collected: sex, onset age, disease duration, types (optic neuritis [ON], myelitis, brain attack and area postrema syndrome [APS]) and date of each attack, and treatment history.
Results
A total of 1738 attacks occurred in 260 patients during median 143 months (interquartile range 84-195 months) of observation period: 1377 and 361attacks before and after initiation of IST, respectively. Brain attacks were observed in 95 (37%) patients: 147 attacks in 93 (36%) patients before IST and 13 attacks in 11 (4%) patients after IST. APS was observed in 25 (9.6%) patients before IST especially at the disease onset, but no one experienced APS after IST. Before IST, ON, myelitis and brain attack were observed at 36.8, 78.7 and 14.1 per 100 patient-years, respectively, while decreased to 4.5, 12.1 and 0.6 per 100 patient-years after IST, respectively. The reduction rates of each attack frequency after IST were significantly greater in brain attack than in ON or myelitis (mean 78% vs. 95%, p=0.014). Patients with brain attacks despite IST showed higher mean annualized relapse rate (ARR) compared to those without brain attacks (p=0.029).
Conclusions
Impact of IST appeared to be greater on the reduction of brain attack than that of ON and myelitis in AQP4-IgG seropositive NMOSD, resulting in rare occurrence of brain attacks after IST. ARR was higher in patients with brain attack than in those without brain attack particularly after IST. These results suggest brain attack may allude high disease activity in NMOSD.