Disease Spotlight: NMOSD

A cross-sectional and longitudinal comparative analysis suggests a subclinical neurodegenerative process may occur in MOG antibody disease based on a distinct pattern of brain atrophy.

Results from the CIRCLES cohort demonstrated a significant association in relapse patterns and disease phenotype for patients with NMOSD who change treatment.

Test your neurology knowledge with NeurologyLive®'s weekly quiz series, featuring questions on a variety of clinical and historical neurology topics. This week's topic is neuromyelitis optica spectrum disorder, or NMOSD.

The new criteria, inclusive of both pediatric and adult patients, advocates for testing for MOG-IgG in appropriate populations, and cautions against testing patients with clinical and radiological features typical of multiple sclerosis.

Data showed that slightly more than half of the patients with NMOSD visited at least 2 neurologists before receiving full diagnosis, and less than 30% reported they were aware of at least 1 nearby specialized NMOSD center.

According to a medical records analysis, patients with late onset NMOSD experience transverse myelitis more frequently at disease onset in comparison with early onset NMOSD.

Featuring a cohort of more than 500 patients with NMOSD, data showed that those with comorbidities exhibit multiple presentations and are more likely to relapse after immunotherapy.

Results from on ongoing phase 1 trial showed a favorable safety profile with CT103A in patients with NMOSD.

A rare case of neuromyelitis optica was reported with a woman aged in her 70s from Iran who experienced neurological symptoms after COVID-19 vaccination.

A study reported a rare case of seronegative NMOSD with a middle-aged man in Nepal who did not improve in clinical status and performance after given treatment.

A rare case report illustrated an 80-year-old man with aquaporin-4 antibody-positive NMOSD following a second dose of the Pfizer-BioNTech COVID-19 mRNA BNT162b2 vaccine.

Results from a prospective study demonstrated a higher visual field defect in patients with neuromyelitis optica spectrum disorder-optic neuritis compared with those who had idiopathic optic neuritis.

As the treatment paradigm for neurologic diseases rapidly progresses, the need for more thorough biomarker tools to measure disease progression and severity has increased, and in recent years, GFAP has emerged as a valuable candidate to add to the existing panel.

Bruce Cree, MD, PhD, MAS, FAAN, offered his perspective on data from the phase 2/3 N-MOmentum trial of the recently approved inebilizumab (Uplizna; Horizon Therapeutics) and why the therapy stands out from other NMOSD treatments.

A meta-analysis suggests that 100mg/w rituximab for 3 consecutive weeks may be the appropriate dosage for patients of NMOSD, demonstrated by a low risk of adverse events.

The founder and executive director of the Sumaira Foundation discussed her experience living with NMOSD and the global organization she created to help patients with the same condition. [WATCH TIME: 6 minutes]

The senior consultant for the department of neurology at the National Neuroscience Institute discussed a report on two patients who developed an uncommon adverse event of late-onset neurotropenia after anti-CD20 therapy treatment. [WATCH TIME: 8 minutes]

The clinical research director of the UCSF Multiple Sclerosis Center provided perspective on several analyses from the N-MOmentum trial that highlight the clinical use of biomarkers in NMO and NMOSD.

The clinical research director of the UCSF Multiple Sclerosis Center provided perspective on the importance of serum neurofilament light and how it can be used in the management of patients with NMOSD. [WATCH TIME: 3 minutes]

The associate professor of neurology at the University of California, San Francisco, discussed the key points from an oral presentation at ECTRIMS 2022 on understanding postpartum for patients with MS and NMOSD. [WATCH TIME: 4 minutes]

The clinical research director of the UCSF Multiple Sclerosis Center discussed the need for measures of progression in neuromyelitis optica and the next steps in treatment. [WATCH TIME: 4 minutes]

The associate professor of neurology at the University of California, San Francisco, discussed takeaways from an oral presentation at ECTRIMS 2022 on the use of anti-CD20 therapies in postpartum for patients with MS and NMOSD. [WATCH TIME: 5 minutes]

The clinical research director of the UCSF Multiple Sclerosis Center provided insight on an abstract from ECTRIMS 2022 regarding inebilizumab’s mechanism of action to prevent NMOSD attacks. [WATCH TIME: 3 minutes]

Permanent disability, found in 51% of patients with NMOSD at follow-up, was attributed mainly to age of disease onset, delay in diagnosis, and initial EDSS score.

A cross-sectional study showed that retinal measurements in patients with “potential” neuromyelitis optica spectrum disorder were similar to those with definite neuromyelitis optica spectrum disorder.

A report on two patients with late-onset neutropenia resulted in an uncommon adverse event following anti-CD20 therapies.

P100 amplitude of 15 minute checks in the NMOSD-ON group had a significant reduction at 6 months relative to the IDON group, suggesting more several axonal damage.

Annualized relapse rates were elevated compared with the prepregnancy period, especially during the initial 3 months after delivery, whereas EDSS scores worsened during pregnancy and the postpartum period.

A study using patient data from John Hopkins showed that rituximab treatment was associated with reduced annualized relapse rates in AQP4-IgG seropositive NMOSD and MOGAD.

Using magnetic resonance spectroscopy, investigators observed significantly different levels of total NAA across MS and NMOSD subtypes, and a negative correlation between total NAA and disability score.

Among a cohort of more than 300 patients in China, those with APQ4 positive NMOSD reported a significantly lower risk of relapse among those treated with rituximab compared with the other groups.

The founder and executive director of the Sumaira Foundation offered her perspective on the patient journey in NMOSD and the vital need to increase awareness [WATCH TIME: 9 minutes]

CXCL2 and CXCL12, known to induce B-cell proliferation and differentiation, were significantly higher in those with acute and remitted cases of NMOSD compared with controls.

Data assessing samples of deep cervical lymph node aspirates and blood suggest that germinal center activity was associated with clinical relapses in individuals with neuromyelitis optica spectrum disorder.

Data suggest that long-term treatment with immunosuppression therapies, such as rituximab, is generally appropriate for patients with neuromyelitis optica, though transverse myelitis might indicate a higher risk of relapse post discontinuation.

BPCLI was observed in both NMOSD and MOGAD but was relatively rarer in AQP4 NMOSD, with meningoencephalitis-like episodes among the sources of misdiagnosis.

Test your neurology knowledge with NeurologyLive®'s weekly quiz series, featuring questions on a variety of clinical and historical neurology topics. This week's topic is neuromyelitis optica spectrum disorder.

In a cohort of patients with NMOSD, more severe depressive symptoms were associated with increased time-varying functional connectivity between the precuneus and temporal cortex.

Using rigorous cell-based assays, cerebrospinal fluid GFAP levels were significantly lower in double-seronegative NMOSD than AQP4-positive NMOSD but did not differ between those with MOGAD or other neurological diseases.

The director of the Mayo Clinic Center for Multiple Sclerosis and Autoimmune Neurology discussed the technological capabilities the center has, including the improved use of neural antibody testing. [WATCH TIME: 6 minutes]

Neuropathic pain and constipation coexisted in 43.5% of the survey participants, and approximately one-third of patients with both symptoms reported a possible link between the two.

Between AQP4-IgG-positive and negative patients with NMOSD, there were no significant differences in cognitive performance, despite having similar demographic and clinical characteristics.

All 12 of the children met diagnostic criteria for multiple sclerosis and were seropositive for MOG-IgG at baseline experienced a disease course different than typical MS through the observed time.

The director of the Mayo Clinic Center for Multiple Sclerosis and Autoimmune Neurology discussed the complexities in diagnosing autoimmune encephalitis and the approach needed to be taken. [WATCH TIME: 5 minutes]

Through dedicated research into biomarker discovery and patient care, the neuroimmunology laboratory at Mayo Clinic has paved a new way of life for those with autoimmune disorders.

Findings from a new post hoc analysis of data from the randomized-controlled period of the N-MOmentum phase 2/3 pivotal trial (NCT02200770), suggest the Horizon agent is effective in NMOSD.

An expert neurologist, advocacy director, and a pair of patients with NMOSD discuss the current landscape of the demyelinating disorder.

These findings have clinical implications for the diagnosis of AQP4-NMOSD and in helping clinicians differentiating it from other demyelinating diseases such as MOGAD.

Amy Kunchok, MD, staff neurologist at the Mellen Center for Multiple Sclerosis and Research at Cleveland Clinic, discussed a recent talk she gave that highlighted the different phenotypes of AQP-IgG NMOSD and MOGAD.

The staff neurologist at the Cleveland Clinic Mellen Center for Multiple Sclerosis outlined key findings from her talk at a recent Institutional Perspectives in Neurology: Multiple Sclerosis event. [WATCH TIME: 3 minutes]

Results from the cohort study contrast with reports in multiple sclerosis, finding that new remission silent lesions were rare in both myelin oligodendrocyte glycoprotein antibody disease and aquaporin-4 antibody neuromyelitis optica spectrum disorder.

The professor of neurology at the University of Colorado provided insight on the data that reinforced the relationship between B-cell depletion and improved outcomes using inebilizumab (Uplizna; Horizon Therapeutics). [WATCH TIME: 2 minutes]

Annualized attack rate was reduced to 0.08 attacks per year after the first administration of inebilizumab, similar to those who were not previously exposed to rituximab.

Repeated analyses using both 3-mm and 1-mm diameter macular data—narrower volumes than standard—showed no relevant outer plexiform layer or outer nuclear layer thinning in those with seropositive AQP4-IgG.

The associate director of the Neuromyelitis Optica Clinic and Research Unit at Mass General spoke about some of the unmet needs and unanswered questions surrounding NMO management.

Data presented at CMSC 2021 suggest worse COVID-19 outcomes were associated with patients with neuromyelitis optica spectrum disorder and other comorbidities.

Rates of infection or serious infection with satralizumab in the overall treatment period were not higher than those on placebo in the double-blind period and did not increase over time.

Following the approval of 3 treatments for the disease, the clinical research director of the UCSF Multiple Sclerosis Center commented on the implications for the clinical development pipeline. [WATCH TIME: 4 minutes]

Jeffrey Bennett, MD, PhD, professor of neurology, University of Colorado, discussed his presentation at ECTRIMS involving the relationship of B-cell depletion and improved outcomes in patients treated with inebilizumab.

An indirect comparison study evaluated relative treatment effects of eculizumab (Soliris; Alexion), inebilizumab (Uplizna; Horizon), and satralizumab (Enspryng; Genentech), the 3 FDA-approved options for NMOSD.

The clinical research director of the USCF Multiple Sclerosis Center discussed the importance of improving knowledge and education for patients with NMOSD, as well as strategies to do so. [WATCH TIME: 3 minutes]

Discussing the 3 FDA-approved treatments for NMOSD, the clinical research director of the UCSF Multiple Sclerosis Center commented on the positive impact, as well as resultant barriers to care. [WATCH TIME: 4 minutes]

The neurologist at Wayne State University provided background on why there needs to be a continued focus on including and studying African American patients with NMOSD in trials. [WATCH TIME 3 minutes]

Neuromyelitis optica patient Doug highlights the types of treatments he has received since his diagnosis and provides suggestions to patients on navigating therapy, especially during the COVID-19 pandemic.

Kim, a patient with neuromyelitis optica, describes what it has been like receiving rituximab infusions as preventive therapy, and shares advice for others who are about to embark on a similar treatment journey.

The clinical research director of the UCSF Multiple Sclerosis Center provided an overview on the state of care for NMOSD, specifically the development of inebilizumab, satralizumab, and eculizumab. [WATCH TIME: 6 minutes]

Investigators suggest recognition of predictors can aid in directing future clinical trials, as well as inform early therapeutic decisions in this patient population.

Evanthia Bernitsas, MD, neurologist, Wayne State University, discussed a recently conducted analysis, which confirmed inebilizumab’s ability to treat African Americans with NMOSD.

Most attacks occurred within the first year of inebilizumab treatment, and nearly all participants were attack free in subsequent years following treatment initiation.

The neurologist at Wayne State University School of Medicine discussed recently published data describing inebilizumab’s role in producing rapid and sustained B-cell depletion in African Americans with NMOSD [WATCH TIME: 4 minutes]

What to know about treating patients with neuromyelitis optica spectrum disorders throughout the COVID-19 pandemic.

An overview of newer preventive therapies approved for acute attacks of neuromyelitis optica spectrum disorders, and variables that impact treatment selection.

The study is the first to provide data from all Latin American countries, further identifying geographic-resultant limitations of access to technology and therapy for NMOSD.

Types of testing that can help neurologists distinguish between acute attacks of neuromyelitis optica spectrum disorders (NMOSD) and multiple sclerosis, as well as therapies that can be used to help manage patients with NMOSD.

Jacinta M. Behne, MA, describes how the Guthy-Jackson Charitable Foundation supports patients with neuromyelitis optica spectrum disorders.

Between PREVENT baseline and end, mean EDSS and Hauser Ambulation Index scores improved with eculizumab (Soliris; Alexion) monotherapy and deteriorated with placebo alone.

The findings identified Th2-related cytokines as characterizing for the prognosis of acute episodes of NMOSD at 1 month and found serum NfL to be a likely biomarker of disease severity at attack.

More than 80% of those with NMOSD experienced disease relapse 6 months after discontinuation of immunosuppressive therapy.

Doug and Kim, patients with neuromyelitis optica spectrum disorders, describe the types of symptoms that prompted each of them to seek an evaluation by a neurologist for an eventual diagnosis of a neuromyelitis optica spectrum disorder.

Mayo Clinic’s Brian G. Weinshenker, MD, compares multiple sclerosis with neuromyelitis optica spectrum disorders (NMOSD) and explains why community neurologists need to receive more education on NMOSD risk factors and symptoms.

Get the latest news in neuromyelitis optica spectrum disorder, with data updates and expert insights, all in one place from the NeurologyLive team.

The registry aims to enroll approximately 800 patients with NMOSD to evaluate the efficacy of new medications and treatments.

The director of the Mayo Clinic Center for Multiple Sclerosis and Autoimmune Neurology discussed unmet needs of care for patients with neuromyelitis optica spectrum disorder.

The neuro-ophthalmologist at UT Southwestern further discussed the high cost of treatment for patients with neuromyelitis optica spectrum disorder and the need to improve access to care.

The neuro-ophthalmologist at UT Southwestern Medical Center discussed optical coherence tomography and antibody detection technology used to diagnose optic neuritis associated with NMOSD.

The director of Mayo Clinic Center for Multiple Sclerosis and Autoimmune Neurology provided context on challenges within the NMOSD space and what’s next following the first-approved therapies.

Discussing NMOSD’s relation to AQP4 and pattern enhancement on MRIs, the neuro-ophthalmologist at UT Southwestern Medical Center further commented on the importance of early diagnosis.

Bruce Cree, MD, PhD, clinical research director of the UCSF Multiple Sclerosis Center, provided context on the status of patient education efforts and clinical development for neuromyelitis optica spectrum disorder.

The neuro-ophthalmologist from UT Southwestern Medical Center provided her opinion on the state of care for patients with NMOSD and associated optic neuritis technology.

The director of Mayo Clinic Center for Multiple Sclerosis and Autoimmune Neurology discussed the most notable recent advances that has propelled care for patients with NMOSD.

The clinical research director of the UCSF Multiple Sclerosis Center shared his perspective on the history of clinical care for neuromyelitis optica spectrum disorder in light of 3 recent FDA approvals.

Investigators found that frequency of MOG-Ab associated NMOSD was not significantly different between Asian and Caucasian populations despite the prevalence of NMOSD in Asian regions.

Lesions in the canalicular and intracranial optic nerve, area postrema, medulla, and cervical spinal cord were prominent in patients with aquaporin-4–NMOSD.

The professor of neurology at the Mayo Clinic College of Medicine highlighted the importance of the diagnostic process and implementing an early treatment regimen for patients with NMOSD.

The professor of neurology at Mayo Clinic College of Medicine discussed which biomarkers have shown the most relevancy in NMOSD and which deserve more attention.

The professor of neurology at Mayo Clinic College of Medicine discussed several areas within the neuromyelitis optica spectrum disorder that are of interest for ongoing and new research efforts.

Patients with AQP4+NMOSD with long cord lesion showed higher annualized atrophy rate of normalized grey matter volume compared with those without long cord lesion.

The professor of neurology at Mayo Clinic College of Medicine discussed the 3 currently FDA-approved agents for NMOSD and other agents currently within the clinical pipeline.

The professor of neurology at Mayo Clinic College of Medicine discussed lesser-known facets of NMOSD and how the disease evolves over time.

The professor of neurology at the Mayo Clinic College of Medicine detailed the strides made in recognizing NMOSD as its own disease, along with specific biomarkers that identify it.

Among independent variables including DMT group, time from COVID-19 infection, total IgG level, age, sex, obesity, and COVID-19 severity, only the anti-CD20 group was associated with decreased odds of positive serology.

Complete resolution of the index T2-lesion and resolution of all T2 lesions occurred most often in patients with MOGAD rather than AQP4-IgG-NMOSD or MS.

Investigators evaluated the occurrence of multiple sclerosis and neuromyelitis optica spectrum disorder in Australian and New Zealand Indigenous populations, comparing data with ancestral data.

The associate professor of neurology at Mayo Clinic Rochester discusses the importance of alternative diagnoses and rarer presentations of MS, in order to avoid misdiagnoses and inappropriate treatment for patients.

These study results highlight the interest in immunosuppressive therapy taken before or during pregnancy and to reduce the risk of relapse during pregnancy and 1 year postpartum.

Serum GFAP levels showed consistent correlations with disease activity and the largest area under the ROC curve to differentiate attacks from remissions in individuals with NMOSD.

The basis for the approval was data from the phase 3 SAkuraSky and SAkuraStar studies, which combined included more than 170 patients who were treated with either satralizumab or placebo.

NeuroMetrix will be evaluating the effectiveness of the transcutaneous electrical nerve stimulation platform in pain relief for patients with neuromyelitis optica spectrum disorder.

Although existing research has shown structural differences between multiple sclerosis and neuromyelitis optica spectrum disorder in following optic neuritis, the new report also covers non–optic neuritis eyes.

The clinical research director at the UCSF Multiple Sclerosis Center shared some of the takeaways from his research for the clinical community of specialists treating patients with NMOSD.

NMOSD is associated with elevated levels of the pleiotropic cytokine interleukin-6, making the cytokine an apt therapeutic target.

The clinical research director at the UCSF Multiple Sclerosis Center discussed the findings of the long-term open-label assessment of inebilizumab in neuromyelitis optica spectrum disorder.

Patients in the randomized controlled trial and the open-label extension treated with inebilizumab experienced similar rates of being attack-free, extending as far as 4 years.

The findings were consistent with the established safety profile for eculizumab in other non-neurologic indications, according to study authors.