Neuromyelitis Optica: Historically Misdiagnosed — Now Demands Prompt Treatment

A Wealth of Treatment Choices

Patients opting for an FDA-approved treatment now have a “wildly effective” array of new drugs, said Dr. Levy, but choosing can be difficult when each has its own set of advantages and disadvantages. “I have equal numbers of patients on all the drugs, and I show all the data to my patients: efficacy, safety, logistics, cost, and then I ask ‘What are your priorities? Which of these things that I say really rings with you? Is it the infusion schedule? Is it the efficacy? Is it the safety concern? Is it the cost? What are you most concerned about?’ And then we start to have the conversation that way. It’s a shared decision-making process.”

There is definitely an art to finding the best fit for each patient, agreed Dr. Bennett, “both with the urgency of controlling the disease, the particular patient in front of you, their ability to adhere to certain therapies, their ability to have access to infusions, or to self-inject, or to get transported to an infusion center or have access to home infusion.”

Patient empowerment in the decision is very important, added Dr. Levy. “When people make the decision on their own, they’re much more likely to be compliant, rather than me telling them they have to do this. And that’s why I think we haven’t had a single relapse on the new drugs. There have been switches because of intolerance, and cost, and all those issues, but not because of a breakthrough attack.”
 

Future

Looking ahead in the field, Dr. Bennett sees the biggest potential for improvement is in the management of acute attacks, which he describes as “a major treatment gap.” Although plasma exchange is immediately effective in limiting the amount of circulating pathogenic AQP4-IgG “there are other approaches that could be even more beneficial,” he said. “A promising strategy is to use drugs that act immediately on arms of the immune response that are directly injuring brain tissue. These include serum complement and cells such as neutrophils and natural killer cells that release destructive enzymes and inflammatory mediators,” he explained. “Complement inhibitors, such as the C5 inhibitors eculizumab and ravulizumab, currently approved for NMOSD relapse prevention, act immediately to inhibit complement-mediated tissue injury. Similarly, high doses of antihistamines could be used to rapidly stop the release of the destructive enzyme elastase from neutrophils and natural killer cells, while elastase inhibitors could be given to minimize cell injury. Direct clinical studies are needed to find both the optimal treatment window and regimen.”

References

1. Hor JY et al. Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide. Front Neurol. 2020 Jun 26:11:501. doi: 10.3389/fneur.2020.00501.

2. Wingerchuk DM et al. International Consensus Diagnostic Criteria for Neuromyelitis Optica Spectrum Disorders. Neurology. 2015 Jul 14;85(2):177-89. doi: 10.1212/WNL.0000000000001729.

3. Mealy MA et al. Epidemiology of Neuromyelitis Optica in the United States: A Multicenter Analysis. Arch Neurol. 2012 Sep;69(9):1176-80. doi: 10.1001/archneurol.2012.314.

4. Contentti EC et al. Frequency of NMOSD Misdiagnosis in a Cohort From Latin America: Impact and Evaluation of Different Contributors. Mult Scler. 2023 Feb;29(2):277-286. doi: 10.1177/13524585221136259.