Central pontine myelinolysis and locked‐IN syndrome associated with tacrolimus after pediatric heart transplantation

Abstract

AbstractBackgroundLocked‐in syndrome represents the most severe form of central pontine myelinolysis and varies in presentation from asymptomatic to fully developed locked‐in‐syndrome characterized by the combination of quadriplegia, loss of the ability to communicate except through the use of the eyes, and an inability to follow commands.MethodsWe report a 10‐year‐old boy who developed a severe case of locked‐in syndrome after heart transplantation.ResultsPatient had a spontaneous recovery, treated with supportive treatment and the improvement was detected with cessation of calcineurin inhibitor therapy by substituting with an mTOR inhibitor (sirolimus). No cases of locked‐in syndrome post‐heart transplant in pediatrics cases have been documented in the literature.ConclusionPhysicians should recognize a rapid progression of central pontine myelinolysis and locked‐in syndrome in the context of heart transplant and although several factors likely contributed to this outcome, adjustment of immunosuppression including by substituting tacrolimus with sirolimus could be effective.