Concept: Neuromyelitis optica
OBJECTIVE To report first experiences with interleukin 6 receptor inhibition in therapy-resistant neuromyelitis optica (NMO). DESIGN Retrospective case series. SETTING Neurology department at a tertiary referral center. PATIENTS Patients with an aggressive course of NMO switched to tocilizumab after failure of anti-CD20 therapy. MAIN OUTCOME MEASURES Annualized relapse rate and disability progression measured by the Expanded Disability Status Scale. RESULTS We report 3 female patients with a median age of 39 years (range, 26-40 years) and aquaporin 4-positive NMO. All patients had been treated with different immunosuppressive and immunomodulating agents, followed by 1 to 3 cycles of rituximab. Despite complete CD20-cell depletion during rituximab therapy, the median annualized relapse rate was 3.0 (range, 2.3-3.0) and the median Expanded Disability Status Scale score increased from 5.0 (range, 4.5-7.0) to 6.5 (range, 5.0-7.0). After the switch to tocilizumab (median duration of therapy, 18 months), the median annualized relapse rate decreased to 0.6 (range, 0-1.3). A total of 2 relapses occurred; however, they were mild and there were no changes in clinical disability. CONCLUSIONS Interleukin 6 receptor-blocking therapy can be effective in therapy-resistant cases of NMO. Larger controlled studies are needed to confirm the efficacy of tocilizumab.
When studying a rare or orphan disease, we hope to shed light on more prevalent syndromes. Neuromyelitis optica (NMO), also known as Devic’s disease, is a rare disease with a prevalence of about 4 in 100,000. Since 2005 when the anti-Aquaporin 4 (AQP4) NMO autoantibody was discovered by Lennon’s group at the Mayo clinic, an enormous amount of data have been acquired on the pathogenesis of the disease. A review of the literature showed 47 relevant publications in 2004, compared with 353 in 2013. The auto-antigen AQP4 is expressed on the astrocytic foot processes suggesting a role for astrocytes in the pathogenesis of the disease. However, the astrocytes might play a more active role than has previously been suggested in the immune cascade of NMO pathology. Here we will review epidemiological, clinical diagnostic and therapeutic aspects of NMO and highlight the possible role of astrocytes as major direct and indirect players in the pathogenesis of NMO and related CNS inflammatory diseases.
- Journal of neurology, neurosurgery, and psychiatry
- Published over 1 year ago
To analyse predictors for relapses and number of attacks under different immunotherapies in patients with neuromyelitis optica spectrum disorder (NMOSD).
Neuromyelitis optica (NMO) attacks are often severe, difficult to treat, and leave residual deficits. Here, we analyzed the frequency, sequence, and efficacy of therapies used for NMO attacks.
To examine anti-KIR4.1 antibodies by 2 different assays in Japanese patients with multiple sclerosis (MS) or neuromyelitis optica (NMO).
We tested whether brain gray matter (GM) imaging measures can differentiate between multiple sclerosis (MS) and neuromyelitis optica (NMO) using random-forest classification.
The purpose of this study was to review cases of neuromyelitis optica spectrum disorder (NMOSD) relapses and pseudorelapses to identify early features that differentiate between them at onset of symptoms.
To assess volumes and microstructural integrity of deep gray matter structures in a homogeneous cohort of patients with neuromyelitis optica spectrum disorder (NMOSD).
To study rituximab in pediatric neuromyelitis optica (NMO)/NMO spectrum disorders (NMOSD) and the relationship between rituximab, B cell repopulation, and relapses in order to improve rituximab monitoring and redosing.
We investigated the sleep structure of patients with neuromyelitis optica spectrum disorder (NMOSD) and the association of abnormalities with brain lesions.