Background: Locked-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.
Methods: We report a 10-year-old boy who developed a severe case of locked-in syndrome after heart transplantation.
Results: Patient 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.
Conclusion: Physicians 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.
Keywords: central pontine myelinolysis; heart transplant.
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