It is extremely important to begin treatments as soon as possible after a rare neuroimmune diagnosis. Treatment in the acute or early stages involves quieting down the immune system as quickly as possible, before damage is done. Time is critical. While not all individuals present alike, the following are possible treatments in the management of an acute event.

Intravenous Steroids

Although there are no clinical trials that support a unique approach to treat patients experiencing Transverse Myelitis (TM) or Optic Neuritis (ON), it is well recognized as a standard of care to give high-dose intravenous methylprednisolone for suspected acute myelitis, generally for 5 days, unless there are compelling reasons not to. The decision to offer continued steroids or add a new treatment is often based on the clinical course and MRI appearance at the end of 5 days of steroids.

Plasma Exchange (PLEX)

PLEX is often recommended for moderate to aggressive forms of TM and ON, as is very often the case with NMOSD, if there is not much improvement after being treated with intravenous steroids. If presenting symptoms are severe, PLEX may be initiated concurrently with steroids. There have been no prospective clinical trials that prove PLEX’s effectiveness in NMOSD, but retrospective studies of TM treated with IV steroids followed by PLEX have shown a beneficial outcome. PLEX also has been shown to be effective in other autoimmune or inflammatory central nervous system disorders. Early treatment is beneficial – PLEX is typically started within days of administering steroids, very often before the course of steroids has finished. Particular benefit has been shown if started within the acute or sub-acute stage of the myelitis or if there is continued active inflammation on MRI.

Other Acute Treatments

In cases of no response to either steroids or PLEX therapy and continued presence of active inflammation in the spinal cord, other forms of immune-based interventions may be required. The use of immunosuppressants or immunomodulatory agents may be considered in some cases. One of those approaches is the use of intravenous cyclophosphamide (a chemotherapy drug often used for lymphomas or leukemia). Initial presentation with aggressive forms of myelitis, or if particularly refractory to treatment with steroids and/or PLEX, aggressive immunosuppression with cyclophosphamide is considered. Individuals should be monitored carefully as potential complications may arise from immunosuppression. As with all medications, risks versus benefits of aggressive immunosuppression need to be considered and discussed with the clinical care team.

The use of IV immunoglobulin (IVIG) has not been tested and its use in the management of acute or sub-acute NMOSD is not supported.