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Devic's disease (neuromyelitis optica)

optic nerve involvement in multiple sclerosis vs distinct entity Pathology: - CNS inflammatory demyelinating disease - inflammation mediated by aquaporin-4 (AQP4) autoantibodies or less commonly results in demyelination of the optic nerve & spinal cord [4] - inflammatory demyelination in the optic nerves & spinal cord - lack of significant brain involvement - large & longitudinally extensive spinal cord lesions - profound CSF leukocytosis. Clinical manifestations: - typically, a fulminant disease - visual impairment due to unilateral or bilateral optic neuritis - paresthesias, dysethesias - symmetric paresis (quadraparesis) - multiphasic or relapsing demyelinating disease - episodes of optic neuritis & longitudinally extensive myelitis usually occur sequentially rather than simultaneously, but may follow in quick succession or be separated by years or even decades [4,5] - recovery may be complete or partial [5] Laboratory: - serology: - autoantibody neuromyelitis optica IgG (NMO-IgG) may be aquaporin 4 receptor Ab in serum/plasma - 64%, 0% of multiple sclerosis patients - also aquaporin-4 IgG Ab in CSF - CSF analysis: - CSF leukocytosis, neutrophil predominance - oligoclonal banding in 15-30% of patients [5] - see ARUP consult [2] Radiology: - MRI of the cervical spinal cord - presence of extensive longitudinal lesions in spinal cord suggests neuromyelitis optica - MRI of the brain in generally normal except for gadolinium enhancement of the optic nerve [5] Complications: - cervical myelitis can extend into the brainstem, resulting in - intractable hiccups - nausea/vomiting, - vertigo - bulbar symptoms - neurogenic respiratory failure [5] Differential diagnosis: - multiple sclerosis (MS) - neutrophils in CSF unlikely with MS - spinal cord lesions in MS may be segmental rather than longitudinal - optic neuritis associated with MS more likely to be unilateral - aquaporin 4 receptor Ab in serum/plasma distinguishes - brain involvement suggests multiple sclerosis Management: - glucocorticoid-refractory, but seems to be standard of care for initial treatment of acute attacks - plasmapheresis for glucocorticoid-refractory disease - azathioprine, with or without prednisolone, & rituximab (1st line) [5] - monthly pulse cyclophosphamide, mitoxantrone, mycophenolate, & methotrexate (2nd line) - cyclosporine A has been used - interferon & natalizumab are not effective & may worsen disease [5] - satralizumab 70% effective in preventing relapse & 90% effective in preventing severe relapse in AQP4(+) neuromyelitis optica for > 3.5 years [6]

Interactions

disease interactions

General

multiple sclerosis variants

References

  1. Magana SM et al Neuromyelitis optica IgG serostatus in fulminant central nervous system inflammatory demyelinating disease. Arch Neurol 2009 Aug; 66:964 PMID: 19667216
  2. ARUP Consult: Neuromyelitis Optica - NMO The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/neuromyelitis-optica - ARUP Consult: Neuromyelitis Optica https://arupconsult.com/ati/neuromyelitis-optica
  3. Wingerchuk DM. Diagnosis and treatment of neuromyelitis optica. Neurologist. 2007 Jan;13(1):2-11. PMID: 17215722
  4. Medical Knowledge Self Assessment Program (MKSAP) 17, 19. American College of Physicians, Philadelphia 2015, 2021 - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  5. Nabi S, Ahmed S, Tariq M Medscape, Case Challenges. November 10, 2015 Limb Weakness and Vision Loss in a 30-Year-Old Man http://reference.medscape.com/viewarticle/854135
  6. Kleiter I, Traboulsee A, Palace J et al Long-term Efficacy of Satralizumab in AQP4-IgG-Seropositive Neuromyelitis Optica Spectrum Disorder From SAkuraSky and SAkuraStar. Neurology Neuroimmunology & Neuroimflammation. 2022. Dec 8. PMID: 36724181 PMCID: PMC9756307 Free PMC article https://nn.neurology.org/content/10/1/e200071
  7. NINDS Neuromyelitis Optica Information Page hhttps://www.ninds.nih.gov/Disorders/All-Disorders/Neuromyelitis-Optica-Information-Page