What is the clinical course of patients with osmotic demyelination syndrome?

Updated: Jun 17, 2019
  • Author: Eric E Simon, MD; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Answer

The clinical course of these patients features initial encephalopathy secondary to hyponatremia, then improvement as the plasma Na concentration increases, and finally deterioration several days later. The disorder can resolve completely or result in permanent disability or death. This typical clinical course has been called the osmotic demyelination syndrome (ODS). The clinical neurologic picture may be confusing, as it may include a variety of findings from psychiatric, behavioral, and movement disorders, such as dysphagia and flaccid or spastic quadriparesis, depending on the involvement of extrapontine or central pontine myelinolysis. Disruption of the blood-brain barrier is presumed to play an important role in the pathogenesis of osmotic demyelination.

An increased susceptibility to osmotic demyelination is also observed in cirrhotic patients. In this setting, myoinositol, the most abundant organic osmolyte, is depleted because of glutamine- and hyponatremia-induced brain cell swelling. CPM is a common and often fatal complication of orthotopic liver transplantation, affecting up to 10% of patients who were hyponatremic prior to transplant. [42]


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