Central pontine myelinolysis (CPM) and
extrapontine myelinolysis (EPM) are syndromes of osmotic
demyelination attributed to the rapid correction of hyponatraemia. Isolated EPM is a rare clinical entity which poses a significant diagnostic challenge especially in the absence of a rapid rise in
sodium. Typical MRI findings aid in the diagnosis. Treatment for established osmotic
demyelination syndrome (ODS) is nonstandardized and the prognosis is considered poor. Therefore, different strategies including
plasmapheresis (TPE),
immunoglobulins (
IVIG), and
steroids have been used. We present our findings from a series of successfully treated patients at a high-volume tertiary care center in Sri Lanka, with an appraisal of available literature. A total of 21 patients with established ODS are analyzed here, including 5 cases of EPM managed by the authors over a 2-year period. Thirteen (40.2%) patients were treated with
plasmapheresis alone, 6 (28.5%) received dual
therapy (TPE +
IVIG or
steroids) and 2 (9.5%) received triple
therapy (TPE +
IVIG +
steroids). There was complete or near complete response in 18 (85.7%) and complete response in 10 (47.6%) patients. We conclude that although the management of CPM/EPM is largely symptomatic, patients may show a significant response to
immunomodulatory therapy. The marked improvement in motor, cognitive, and functional domains supports an immune basis for osmotic
demyelination.
Plasmapheresis, in particular, leads to favorable outcomes in ODS which is supported by previously published case reports. We propose its utility as standard treatment.