Resources

Neuromyelitis Optica (NMO) spectrum disorder is an autoimmune disease of the central nervous system that commonly affects the spinal cord (paralysis), brainstem (intractable hiccups), and optic nerves (blindness).

Rapid treatment for acute relapses is essential to prevent permanent disability.

  • For a moderate or life-threatening attack, prescribe methylprednisilone, one gram IV daily for three to five days. 
  • For a severe attack that does not respond to steroids, transfer to an Apheresis centre (VGH in BC).

NMO Instant Ally Resources (PDF)

NMO ally and clinician resources

NMO pathology

NMO spectrum disorders involve antibody-mediated destruction of astrocytes with a serologic biomarker, aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) which are highly specific to NMO. Patients can be diagnosed with NMO whether antibody positive or negative.

Identifying relapses/attacks

NMO relapses are typically more severe and disabling than in multiple sclerosis. Relapse commonly involves:

  • Optic neuritis (ON): marked by eye pain on movement visual loss, colour desaturation. Bilateral or very severe ON is more suggestive of NMO than MS.
  • Transverse myelitis: marked by weakness, numbness/parasthesia, bowel or bladder changes. Longitudinally extensive transverse myelitis where ≥3 vertebral segments are involved is more suggestive of NMO than multiple sclerosis.
  • Area postrema syndrome: marked by persistent hiccups, nausea or vomiting.

Less commonly, relapse can involve:

  • Acute brainstem syndrome: marked by nystagmus, opthalmoplegia, dysarthria, dyshphagia, ataxia.
  • Narcolepsy or acute diencephalic clinical syndrome (i.e., hypothalamus/thalamus): marked by SIADH, hypothermia, hypersomnia, hypotension, hyperthyroidism, hyperprolactinernia, amenorrhea, galactorrhea, behavioural changes.
  • Cerebral syndrome: e.g., encephalopathy, hemparesis, visual field defects.
Acute treatment

Early and aggressive treatment may reduce the severity and duration of the relapse. Please contact the UBC NMO team for discussion and advice:

  • MS/NMO Program Relapse Nurse: 604-822-0753
  • UBC Hospital Paging Service: 604-822-7121 (ask for Dr. Traboulsee or Dr. Carruthers to be paged)
  • MS/NMO Program Fax: 604-297-9685
Long-term treatment

Treatment consists of three aims:

  1. Acute treatment of relapses
  2. Preventing relapses
  3. Symptomatic management of residual symptoms, including neuropathic pain, stiffness, muscle spasms, bowel/bladder/sexual dysfunction.

Immunosuppressive agents are recommended for prevention of relapses which is key to preventing accumulation of disability. These include either azathioprine, mycophenolate mofetil or rituximabm sometimes combined with corticosteroids. There are Phase 3 trials underway investigating the efficacy of monoclonal antibodies against CD19, IL-6, and C5, all targeting the immune pathway of NMO.

Neuropathic pain

Severe pain syndromes are a common complication of NMO, and result from spinal cord damage. They can represent an acute relapse when presenting abruptly, or residual neuropathic pain when there is a history of waxing and waning of chronic symptoms. Acute pain can be treated as a relapse.

Persistent neuropathic pain can be managed with one or more of the following medications in titrating doses: amitriptyline/nortiptyline, gabapentin, pregabalin, duloxetine, carbamazepine (for trigeminal neuralgia). If initial treatment is not effective or not tolerated, consider switching medications. Avoid opiods for chronic neuropathic pain.

Contacts

UBC NMO program medical leads:

  • MS/NMO Program Relapse Nurse: 604-822-0753
  • UBC Hospital Paging Service: 604-822-7121 (ask for Dr. Traboulsee or Dr. Carruthers to be paged)
  • MS/NMO Program Fax: 604-297-9685