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Intrathecal Baclofen Therapy After Liver Transplant in a Patient With Crigler‐Najjar Syndrome
Author(s) -
Pratini Napala R.,
Neufeld Jacob A.
Publication year - 2014
Publication title -
pmandr
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.617
H-Index - 66
eISSN - 1934-1563
pISSN - 1934-1482
DOI - 10.1016/j.pmrj.2013.09.004
Subject(s) - medicine , spasticity , baclofen , dystonia , intrathecal , anesthesia , phenobarbital , surgery , agonist , receptor , psychiatry
Crigler‐Najjar syndrome (CNS) is described as severe infantile, nonhemolytic, unconjugated hyperbilirubinemia and is divided into type I and type II according to the patient's response to phenobarbital treatment. Patients with type I CNS usually require a liver transplant. These patients often have spasticity and dystonia, both of which can be treated with intrathecal baclofen therapy. We present the case of a patient with CNS type I who underwent a liver transplant followed years later by intrathecal baclofen therapy. To our knowledge, this article provides the first report of a patient with CNS being treated for dystonia with an intrathecal baclofen pump. Despite his complicated history, this patient has remained medically stable after both interventions.