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ALL / Neurology

Neuromyelitis optica

Neuromyelitis optica

Neuromyelitis optica (NMO) is a monophasic or relapsing-remitting demyelinating CNS disorder Although previously thought to be a variant of multiple sclerosis, it is now recognised to be a distinct disease, particularly prevalent in Asian populations. It typically involves the optic nerves and cervical spine, with imaging of the brain frequently normal. Vomiting is also a common presenting complaint.

Possible presenting features
  • optic neuritis: often bilateral or severe, leading to sudden visual loss, eye pain, and reduced colour vision.
  • transverse myelitis: longitudinally extensive spinal cord inflammation causing motor weakness, sensory loss, and sphincter dysfunction.
  • area postrema syndrome: intractable hiccups, nausea, or vomiting (due to medullary involvement).

Diagnosis requires bilateral optic neuritis, myelitis and 2 of the following 3 criteria:
  • 1. Spinal cord lesion involving 3 or more spinal levels
  • 2. Initially normal MRI brain
  • 3. Aquaporin 4 positive serum antibody

Management
  • treatment of acute attacks
    • high-dose IV methylprednisolone
  • maintenance therapy for attack prevention
    • long-term monoclonal antibody therapy, e.g. eculizumab, ravulizumab (antibody target = complement C5), inebilizumab (antibody target = CD19 on B cells) or satralizumab (antibody target = IL-6 receptor)
+PassMedicine Notes

Neuromyelitis optica (NMO) is a monophasic or relapsing-remitting demyelinating CNS disorder Although previously thought to be a variant of multiple sclerosis, it is now recognised to be a distinct disease, particularly prevalent in Asian populations. It typically involves the optic nerves and cervical spine, with imaging of the brain frequently normal. Vomiting is also a common presenting complaint.

Possible presenting features
  • optic neuritis: often bilateral or severe, leading to sudden visual loss, eye pain, and reduced colour vision.
  • transverse myelitis: longitudinally extensive spinal cord inflammation causing motor weakness, sensory loss, and sphincter dysfunction.
  • area postrema syndrome: intractable hiccups, nausea, or vomiting (due to medullary involvement).
Diagnosis requires bilateral optic neuritis, myelitis and 2 of the following 3 criteria:
  • 1. Spinal cord lesion involving 3 or more spinal levels
  • 2. Initially normal MRI brain
  • 3. Aquaporin 4 positive serum antibody
Management
  • treatment of acute attacks
    • high-dose IV methylprednisolone
  • maintenance therapy for attack prevention
    • long-term monoclonal antibody therapy, e.g. eculizumab, ravulizumab (antibody target = complement C5), inebilizumab (antibody target = CD19 on B cells) or satralizumab (antibody target = IL-6 receptor)


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