Jasmin Naumann

This manuscript presents practical recommendations for managing acute attacks and implementing preventive immunotherapies for neuromyelitis optica spectrum disorders (NMOSD), a rare autoimmune disease that causes severe inflammation in the central nervous system (CNS), primarily affecting the optic nerves, spinal cord, and brainstem. The pillars of NMOSD therapy are attack treatment and attack prevention to minimize the accrual of neurological disability. Aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) are a diagnostic marker of the disease and play a significant role in its pathogenicity. Recent advances in understanding NMOSD have led to the development of new therapies and the completion of randomized controlled trials. Four preventive immunotherapies have now been approved for AQP4-IgG-positive NMOSD in many regions of the world: eculizumab, ravulizumab - most recently-, inebilizumab, and satralizumab. These new drugs may potentially substitute rituximab and classical immunosuppressive therapies, which were as yet the mainstay of treatment for both, AQP4-IgG-positive and -negative NMOSD. Here, the Neuromyelitis Optica Study Group (NEMOS) provides an overview of the current state of knowledge on NMOSD treatments and offers statements and practical recommendations on the therapy management and use of all available immunotherapies for this disease. Unmet needs and AQP4-IgG-negative NMOSD are also discussed. The recommendations were developed using a Delphi-based consensus method among the core author group and at expert discussions at NEMOS meetings.

Viele blinde Flecken zur Geographiedidaktik, wie sie eine Sitzung auf dem DKG 2015 thematisiert, scheinen genau da zu liegen, wo ein emanzipatorischer Bildungsbegriff angebracht werden kann, der sich u. a. durch Kritik, Reflexivität und Innovativität im emanzipatorischen Sinne auszeichnet. Zugleich muss dieser sorgsam von Tendenzen der Bildung hin zu einem „ökonomischen Selbst“ abgegrenzt werden. In dieser theoretischen Abwägung werden Ansätze der Geographiedidaktik auf ihre Passung zur Aneignung eines emanzipatorischen Bildungsziels überprüft.

BACKGROUND: B-cell-depleting therapies are increasingly being used to treat autoimmune diseases. Although thousands of patients are and have been treated with these agents, the data are not yet strong enough to identify rare side effects with certainty. METHODS: We report the case of a patient undergoing ocrelizumab therapy for relapsing multiple sclerosis who developed a severe limbic syndrome. RESULTS: Autoimmune pathogenesis was initially suspected, with worsening on immunosuppressive therapy. Later, after diagnosis of an enterovirus infection, treatment with ribavirin and favipiravir in combination with intravenous immunoglobulins was initiated. After 4 weeks of therapy, the patient's clinical condition had stabilized with residual cognitive deficits. CONCLUSION: Diagnosis and treatment of enterovirus infections remain challenging, especially in patients receiving immunosuppressive therapy.

INTRODUCTION: Rituximab is effective and widely used as long-term treatment in aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (AQP4-IgG+ NMOSD). However, infections remain a significant concern during rituximab treatment. METHODS: We conducted a retrospective multicenter cohort study within the NMO Study Group (NEMOS) in Germany, analyzing demographic and clinical data from people with AQP4-IgG+ NMOSD receiving rituximab or azathioprine by retrospective chart, and compared infection occurrence and severity. For rituximab-treated patients, we collected laboratory data (blood lymphocytes, B-cell counts, serum IgG, IgM, and IgA levels), assessed risk factors for infections, and determined the probability of infection within a 3-month window before and after the laboratory assessment. RESULTS: In 92/170 rituximab and in 12/33 azathioprine treatment episodes, one or more infections were documented. Rituximab and azathioprine showed comparable types and risk of infection (HR = 1.24, 95% CI: 0.68-2.25). Rituximab-treated individuals older than 60 years had a higher risk of infection (HR = 1.62, 95% CI: 1.02-2.57). Hypogammaglobulinemia (IgG < 6.0 g/L: OR = 2.27, 95% CI: 1.15-4.48; IgM < 0.3 g/L: OR = 2.08, 95% CI: 1.05-4.09) predicted infections and the occurrence of both low IgG and IgM serum levels further increased the risk of infection (OR = 2.77, 95% CI: 1.10-6.98) during rituximab treatment. Low IgG and IgA serum levels as well as lymphopenia predicted infection-related hospitalizations. CONCLUSION: Age > 60 years and immunoglobulin serum levels during rituximab treatment may serve as predictors for infection and help to individualize treatment decisions in NMOSD.