Complement biomarkers reflect the pathological status of neuromyelitis optica spectrum disorders.
We retrospectively analyzed 21 patients with NMOSD (19 females and 2 males) and 25 patients with GBS (14 females and 11 males) (Table 1). The mean age at the time of blood collection in the acute phase of NMOSD was 48.0 years, mean duration of illness was 5.1 years, and mean expanded disability status scale (EDSS) was 5.3. Mean EDSS during NMOSD remission was 4.5. The mean age at GBS onset was 50.8 years, and the mean severity of illness was Hughes functional grade scale 3.4. Mean Hughes functional grade scale during the remission phase of GBS (at discharge) was 1.7. Anti-AQP4 and anti-glycolipid autoantibodies were positive in 81% patients with NMOSD and 88% patients with GBS, respectively.
Overlapping Autoimmune Neurological Syndrome: A Case Report of Triple-Positive Antibody
In this case report, we present a middle-aged patient displaying the clinical manifestations of these three rare neurological disorders (NMOSD, MG, and anti-NMDAR encephalitis). By presenting this unusual case, we hope to help clinicians be more vigilant about the possibility of overlapping neurological syndromes that will help avoid delays in the diagnosis and treatment of these conditions.
Burden and cost of comorbidities in patients with neuromyelitis optica spectrum disorder
A total of 162 patients with NMOSD and 810 non-NMOSD controls were evaluated. A significantly higher proportion of NMOSD patients had comorbidities than non-NMOSD controls.
Results: A total of 162 patients with NMOSD and 810 non-NMOSD controls were evaluated. A significantly higher proportion of NMOSD patients had comorbidities than non-NMOSD controls (66.7% vs 41.5%; P < 0.001). Concomitant autoimmune disease occurred in 19.1% vs 4.9% (P < 0.001) of patients with NMOSD vs non-NMOSD controls. NMOSD patients incurred significantly higher total median (interquartile range) healthcare costs per patient ($68,386.48 [$23,373.54-$160,862.70]) than matched non-NMOSD controls with autoimmune disease ($17,215.13 [$6715.48-$31,441.93]; P < 0.001) or patients with NMOSD without autoimmune comorbidity ($23,905.42 [$8632.82-$67,251.54]; P = 0.022). Similarly, patients with NMOSD and non-autoimmune comorbidities incurred higher median healthcare costs than matched controls.
Impact of psychiatric distress and physical disability on quality of life in neuromyelitis optica spectrum disorder and chronic autoimmune demyelinating polyneuropathies
Therapy Approved for Rare Neurological Autoimmune Disorder
Satralizumab-mwge, marketed as Enspryng, was approved for adults with NMOSD who have antibodies against the aquaporin 4 (AQP4) protein. Binding of the antibodies with AQP4 activates the immune system and results in elevated, inflammation-promoting levels of interleukin 6 in cerebrospinal fluid. Satralizumab-mwge blocks interleukin 6 signaling pathways.
Application of 2015 Seronegative Neuromyelitis Optica Spectrum Disorder Diagnostic Criteria for Patients With Myelin Oligodendrocyte Glycoprotein IgG–Associated Disorders
Elevated serum interleukin-39 levels in patients with neuromyelitis optica spectrum disorders correlated with disease severity
Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide
Accessibility to AQP4-antibody and MOG-antibody testing, which is currently limited in many regions, is a challenge to overcome.
Altered Resting-State Functional Connectivity Density in Patients With Neuromyelitis Optica-Spectrum Disorders
Optic neuritis associated with anti-NMDA receptor antibody in the remission phase of anti-NMDA receptor encephalitis
Dramatic efficacy of ofatumumab in refractory pediatric-onset AQP4-IgG neuromyelitis optica spectrum disorder
EC Clears Eculizumab (Soliris) for Neuromyelitis Optica
Update on the diagnosis and treatment of neuromyelitis optica spectrum disorders (NMOSD) – revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part II: Attack therapy and long-term management
Recent insights into the pathogenesis of NMOSD have led to the development of novel targeted and highly effective therapies for patients with AQP4-IgG-positive NMOSD. These therapies provide a more personalized approach to treatment, considering factors, such as disease activity, age, comorbidities, family planning, side effects, route of administration, patient choice, availability, and costs. They also allow for switching between therapies in case of side effects or insufficient treatment response. Unmet needs in NMOSD therapy include understanding the long-term disease course, determining optimal immunotherapy durations, developing strategies for treatment cessation and de-escalation, and searching for biomarkers indicating attack risk.
New Treatment Perspectives for Acute Relapses in Neuromyelitis Optica Spectrum Disorder
Targeting B Cells to Modify MS, NMOSD, and MOGAD Part 1
This review more closely examines the clinical development of CD20 depletion and the pioneering contribution of rituximab. Phase 3 and the recently published postmarketing studies will be highlighted to better understand the relevant efficacy data and safety aspects of long-term B-cell depletion.
Connexins in neuromyelitis optica: a link between astrocytopathy and demyelination
Overall, our results suggest that dysregulation of connexins would play a pathogenetic role in neuromyelitis optica. The further identification of mechanisms leading to connexin dysfunction and soluble factors implicated, would provide interesting therapeutic strategies for demyelinating disorders.
Isolated opsoclonus heralding neuromyelitis optica spectrum disorder
High-throughput investigation of molecular and cellular biomarkers in NMOSD
Results NMOSD samples had a lower frequency of CD16+CD56+ NK cells. Both serum cohorts and multivariable logistic regression revealed increased levels of B-cell activating factor associated with NMOSD. Interleukin 6, CCL22, and CCL3 were also elevated in 1 NMOSD cohort of the 2 analyzed. Multivariable linear regression of serum analyte levels revealed a correlation between CX3CL1 (fractalkine) levels and the number of days since most recent disease relapse.
Conclusions Integrative analyses of cytokines, chemokines, and immune cells in participants with NMOSD and HCs provide congruence with previously identified biomarkers of NMOSD and highlight CD16+CD56+ NK cells and CX3CL1 as potential novel biomarker candidates.