Devic's disease (neuromyelitis optica)

Introduction

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
• 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
• recovery may be complete or partial

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
• see ARUP consult

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

Complications

• cervical myelitis can extend into the brainstem, resulting in
  • intractable hiccups
  • nausea/vomiting,
  • vertigo
  • bulbar symptoms
  • neurogenic respiratory failure

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)
• monthly pulse cyclophosphamide, mitoxantrone, mycophenolate, & methotrexate (2nd line)
• cyclosporine A has been used
• interferon & natalizumab are not effective & may worsen disease
• satralizumab 70% effective in preventing relapse & 90% effective in preventing severe relapse in AQP4(+) neuromyelitis optica for > 3.5 years

More general terms

• multiple sclerosis variants

Patient information

Devic's disease (neuromyelitis optica) patient information