Recovery of locked-in syndrome following liver transplantation with calcineurin inhibitor cessation and supportive treatment

BACKGROUND: Locked-in syndrome represents the most severe form of central pontine myelinolysis (CPM) and has been associated with a dismal outcome. CASE REPORT: In this report we describe a case of severe locked-in syndrome after liver transplantation with spontaneous recovery with cessation of calcneurin inhibitor therapy and supportive treatment. A 54-year old male received deceased-donor liver transplantation and developed decreased level of consciousness with spastic quadriplegia. A diagnosis of central pontine myelinolysis with extrapontine manifestations was confirmed by magnetic resonance imaging. His immunosuppresion was modified by switching from tacrolimus to sirolimus and addition of prednisone. The patient started to recover from symptoms fourth months after transplantation. CONCLUSIONS: Tacrolimus is known to have neurotoxic side effects and it may precipitate CPM in patients who have predisposing factors. Sirolimus and steroids should be considered as safe and an effective alternative for immunosuppression in the setting of CPM after liver transplantation.