To discuss the management of a patient with Extrapontine Myelinolysis (EPM) treated with a quadruple-style approach who displayed significant clinical improvement following intervention. 

Central Pontine Myelinolysis (CPM) and EPM are Osmotic Demyelination Syndromes (ODS) that traditionally have a bleak prognosis with high mortality rates.  Numerous published case studies have used experimental treatments of ODS, yet no clinical trials have been performed to identify an effective approach to management.  Intravenous immunoglobulin (IVIG), steroids, thyrotropin releasing hormone and reintroduction of hyponatremia have displayed improved outcomes in patients, yet no clinical trials have been published identifying a definitive treatment plan.  IVIG and plasmapheresis are two commonly documented therapeutic measures with treatment efficacy ranging from “moderate neurologic recovery” to “complete motor recovery”.

Case report

We present a case of a 52-year-old Caucasian female who was hospitalized for correction of electrolyte abnormalities, including hyponatremia.  Hospitalization was uncomplicated and with no evidence of neurologic deficit.  Patient returned to the hospital eight days after discharge with a constellation of symptoms suggesting dysfunction of the pons including spastic dysarthria, confusion, generalized weakness and emotional lability. MRI brain confirmed presence of CPM.  The patient’s clinical status progressively deteriorated to include ataxia, vertigo and dysphagia which ultimately progressed to a “locked-in” state with MRI brain displaying EPM.  Patient was treated with plasmapheresis, re-introduction of hyponatremia, high-dose steroids, and IVIG therapy, respectively.  She was discharged to acute rehabilitation.  The only persistent deficit four months later was mild hand grip weakness.

This case illustrates the efficacy of utilizing multiple interventions to manage ODS with re-introduction of hyponatremia, plasmapheresis, IVIG, and steroids.  These interventions may be aimed at targeting at decreasing the permeability of the blood-brain barrier that occurs with insult of rapid correction of hyponatremia.