title: Progressive multifocal leukoencephalopathy after liver transplantation can have favorable or unfavorable outcome.
abstract: Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by the JC polyomavirus (JCPyV) in immunocompromised patients, including solid organ transplant recipients. We report 2 cases of PML late after liver transplantation (144 and 204 months) and review the few other published cases. The clinical course of PML is characterized by a rapid progressive neurological decline coinciding with the presence of white matter lesions on magnetic resonance images. No direct antiviral therapy is available against the JCPyV. The prognosis is therefore extremely poor. Restoration of the immune response achieved by tapering or ending the immunosuppressive therapy is the basis of treatment in transplanted patients. One of our patients is alive 3 years after diagnosis after total withdrawal of immunosuppressive therapy. The other presented severe rejection when tapering immunosuppression and died 26 months after diagnosis.
authors.affiliation: Fédération des Spécialités Digestives, Hospices civils de Lyon, Hôpital Edouard Herriot, Lyon, France.|Université Claude Bernard Lyon 1, Lyon, France.
authors.forename: J
authors.initials: J
authors.lastname: Dumortier
authors.affiliation: Fédération des Spécialités Digestives, Hospices civils de Lyon, Hôpital Edouard Herriot, Lyon, France.
authors.forename: O
authors.initials: O
authors.lastname: Guillaud
authors.affiliation: Fédération des Spécialités Digestives, Hospices civils de Lyon, Hôpital Edouard Herriot, Lyon, France.|Université Claude Bernard Lyon 1, Lyon, France.
authors.forename: A
authors.initials: A
authors.lastname: Bosch
authors.affiliation: Service de Médecine Interne, Hospices civils de Lyon, Hôpital Edouard Herriot, Lyon, France.
authors.forename: B
authors.initials: B
authors.lastname: Coppéré
authors.affiliation: Service d'Explorations Fonctionnelles Neurologiques, Hospices civils de Lyon, Hôpital de la Croix-Rousse, Lyon, France.
authors.forename: P
authors.initials: P
authors.lastname: Petiot
authors.affiliation: Université Claude Bernard Lyon 1, Lyon, France.|Service de Neurologie Unité 102, Hospices civils de Lyon, Hôpital Neurologique Pierre Wertheimer, Lyon, France.
authors.forename: S
authors.initials: S
authors.lastname: Roggerone
authors.affiliation: Université Claude Bernard Lyon 1, Lyon, France.|Service de Neurologie Unité 102, Hospices civils de Lyon, Hôpital Neurologique Pierre Wertheimer, Lyon, France.
authors.forename: S
authors.initials: S
authors.lastname: Vukusic
authors.affiliation: Fédération des Spécialités Digestives, Hospices civils de Lyon, Hôpital Edouard Herriot, Lyon, France.|Université Claude Bernard Lyon 1, Lyon, France.
authors.forename: O
authors.initials: O
authors.lastname: Boillot
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D000328:Adult; D002540:Cerebral Cortex / Q000000981:diagnostic imaging; D017809:Fatal Outcome; D005260:Female; D006084:Graft Rejection / Q000188:drug therapy*; D006801:Humans; D016867:Immunocompromised Host; D007165:Immunosuppression Therapy / Q000009:adverse effects* / Q000379:methods; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D007577:JC Virus / Q000302:isolation & purification*; D007968:Leukoencephalopathy, Progressive Multifocal / Q000134:cerebrospinal fluid / Q000000981:diagnostic imaging / Q000821:virology; D016031:Liver Transplantation / Q000009:adverse effects*; D008279:Magnetic Resonance Imaging; D008297:Male; D011379:Prognosis; D028761:Withholding Treatment
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents
keywords: JC virus; liver transplantation; polyomavirus; prognosis; progressive multifocal leukoencephalopathy; treatment