Neuromyelitis Optica
Spectrum Disorder
Where we have come from, and where we have to go
Despite first being described 125 years ago, it has remained a common myth that neuromyelitis optica spectrum disorder (NMOSD) is a variant of multiple sclerosis (MS).
The science of NMOSD has come a long way
After a long debate, the breakthrough in distinguishing between NMOSD
and MS was the discovery of the aquaporin-4 (AQP4) antibody.1
AQP4 proteins play a crucial role in water transportation as well as
maintaining balance (called homeostasis) within the central nervous
system.2 The AQP4 antibodies target the AQP4 protein causing
inflammatory damage to specific cells in the brain called
astrocytes.1
Initially, it was thought that neuromyelitis optica (NMO) was limited to inflammation of the optic nerve (optic neuritis) and spinal cord (acute myelitis).1 However, as other brain syndromes also occur in NMO, the term NMO spectrum disorder (NMOSD) was proposed in 2015 to cover a broader clinical spectrum – as a result, measures for diagnosis have been clarified over the past few years.1
The evolution of NMOSD diagnosis1
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the timeline
Cases of NMO were first published by researcher Eugene Devic – became known as Devic’s disease5
First proposed criteria for diagnosing NMO, which included inflammation of the optic nerve (optic neuritis) and spinal cord (acute myelitis)
First report of an autoantibody (an antibody created by an individual that is directed against that person’s own proteins) unique to NMO – the next year the target was identified as the AQP4 antibody
Researchers incorporated positive testing for the AQP4 antibody (known as seropositivity) in the criteria for diagnosing NMO. Both optic neuritis and acute myelitis remained requirements
Researchers introduced the term ‘NMOSD’, to cover a broader spectrum of conditions, including brain syndromes
Publication of The International Consensus Diagnostic
Criteria of Neuromyelitis Optica Spectrum Disorders. This
proposed two sub-groups of NMOSD patients, and additional
criteria for diagnosis
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The two sub-groups of NMOSD patients included in the 2015 diagnostic criteria were:
- Those with AQP4 antibodies in their blood (called AQP4 antibody seropositive NMOSD)
- Those without AQP4 antibodies in their blood (called AQP4 antibody seronegative NMOSD)
What science is still trying to uncover about NMOSD
Despite the considerable success in understanding NMOSD more deeply, researchers and scientists are continuing to work hard to reveal more about this complex condition.
Committed to advancement in NMOSD
The 21st Century has heralded a significant advancement of our understanding of NMOSD, developing in-depth knowledge and new treatments.
Recent approvals for new, effective treatment options have been welcomed by those living with NMOSD, which can have a profound impact on people.4 At Roche, we are committed to playing a significant part in this advancement, taking an innovative approach to research and development.
We never stop following the science, ensuring we’re doing now what patients need next, to help preserve what makes people who they are.
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What's next?
Learn more about the Science of NMOSD and read Chelsey’s Story, a person with NMOSD who spent 10 years misdiagnosed with MS.
References
1) Fujihara, K. Neuromyelitis optica spectrum disorders – still evolving and broadening. Curr. Opin. Neurol. 2019;32(3):385-394.
2) Yang, X; Ransom, BR and J-F Ma. The role of AQP4 in neuromyelitis optica: More answers, more questions. J. Neuroimmunol. 2016;298:63-70.
3) De Seze, J. MOG-antibody neuromyelitis optica spectrum disorder: is it a separate disease? Brain. 140;3072-3074.
4) Selmaj, K and Selmaj I. Novel emerging treatments for NMOSD. Polish Journal of Neurology and Neurosurgery. 2019;53(5):317-326.
5) Image source: Jarius, Sven & Wildemann, Brigitte. (2013). The history of neuromyelitis optica. Journal of neuroinflammation. 10. 8. 10.1186/1742-2094-10-8.