PLEX has been shown to be effective in adults with TM and other inflammatory disorders of the CNS. 40 PLEX is believed to work in autoimmune CNS diseases through the removal of specific or nonspecific soluble factors likely to mediate, be responsible for, or contribute to inflammatory-mediated organ damage. 19 It is often given as five treatments, each with exchanges of 1.1 to 1.5 plasma volumes, every other day for 10 days. PLEX is often initiated in individuals with motor impairment 36 or who show little clinical improvement after intravenous steroid treatment, 37-39 but may also be initiated at first presentation for those with significant deficits. 19 The decision to offer continued steroids or to add a new treatment is often based on the clinical course and MRI appearance at the end of 5 days of steroids. The standard of care includes intravenous methylprednisolone (30 mg/kg up to 1000 mg daily) or dexamethasone (120 to 200 mg daily for adults) for 3 to 5 days unless there are compelling reasons to avoid this therapy. Though there is no randomized double-blind placebo-controlled study that supports this approach, evidence from related disorders and clinical experience support this treatment. Corticosteroids have multiple mechanisms of action including anti-inflammatory activity, immunosuppressive properties, and antiproliferative actions.

Intravenous steroid treatment is the first line of therapy often used in acute TM. The acute therapies most frequently used to treat an inflammatory attack include: high dose intravenous steroids (methylprednisolone), Plasmapheresis (Plasma Exchange or PLEX), Immunoglobulin Therapy (IVIG), and cyclophosphamide. Treatment in the acute or early stages involves quieting down the immune system as quickly as possible, before damage is done. It is extremely important to begin treatments as soon as possible after a rare neuroimmune diagnosis.

2022 Rare Neuroimmune Disorders Symposium.

COVID-19 and Rare Neuroimmune Disorders.

Plex treatment