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Devic's syndrome
Devic’s syndrome, more commonly known as Neuromyelitis Optica Spectrum Disorder (NMOSD), is a rare autoimmune inflammatory disease of the central nervous system.
Prevalence
1 / 100 000
1–4 per 100,000
US Estimated
0.5–2 per 100,000
Europe Estimated
Age of Onset
Adulthood
ICD-10
G36.0
Inheritance Pattern
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
Rare
X-linked recessive
5 Facts you should know
FACT
Devic's Syndrome, also known as Neuromyelitis Optica (NMO), is a rare autoimmune disorder primarily affecting the optic nerves and spinal cord, causing inflammation and demyelination
FACT
It is characterized by recurrent attacks of optic neuritis, leading to visual impairment or blindness, and transverse myelitis, resulting in weakness, sensory loss, and sometimes paralysis of the limbs
FACT
NMO is associated with autoantibodies against aquaporin-4 (AQP4), found abundantly in the optic nerves and spinal cord, contributing to immune-mediated damage
FACT
Differential diagnosis involves distinguishing NMO from multiple sclerosis (MS) due to similarities in symptoms, but NMO typically presents with more severe optic neuritis and longitudinally extensive transverse myelitis
FACT
Treatment includes immunosuppressive therapies such as corticosteroids, plasma exchange, and targeted biologics like rituximab or eculizumab to manage attacks and prevent relapses
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Common signs & symptoms
Optic neuritis with vision loss or eye pain
Transverse myelitis causing limb weakness or paralysis
Sensory disturbances
Bladder and bowel dysfunction
Severe nausea, vomiting, or hiccups (area postrema syndrome)
Recurrent attacks with incomplete recovery
Current treatments
Acute attack management:
- High-dose intravenous corticosteroids
- Plasma exchange (PLEX) for steroid-refractory attacks
Long-term disease-modifying therapies:
- Immunosuppressive agents (e.g., azathioprine, mycophenolate mofetil)
- Targeted biologic therapies approved for NMOSD, including complement and B-cell pathway inhibitors
References:
Wingerchuk DM, Banwell B, Bennett JL, et al. International Panel for NMO Diagnosis. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. <i>Neurology.</i> 2015;85(2):177–189. doi:10.1212/WNL.0000000000001729 Pittock SJ, Lucchinetti CF, Lennon VA, et al. Efficacy and safety of eculizumab in neuromyelitis optica spectrum disorder. <i>N Engl J Med.</i> 2019;381(7):614–625. doi:10.1056/NEJMoa1900866 Jarius S, Ruprecht K, Kleiter I, et al. Neuromyelitis optica spectrum disorders: update on diagnosis, pathophysiology, and treatment. <i>Curr Opin Neurol.</i> 2016;29(3):276–283. doi:10.1097/WCO.0000000000000324 Huda S, Whittam DH, Bhojak M, et al. Neuromyelitis optica spectrum disorders: review of clinical features, diagnostic criteria and treatment options. <i>Clin Exp Immunol.</i> 2019;195(2):149–164. doi:10.1111/cei.13295 Pittock SJ, Weinshenker BG, Lucchinetti CF, et al. Neuromyelitis optica and non organ‐specific autoimmunity. <i>Arch Neurol.</i> 2004;61(2):166–171. doi:10.1001/archneur.61.2.166