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Progressive multifocal leukoencephalopathy in common variable immunodeficiency: mitigated course under mirtazapine and mefloquine

Kurmann, Rebekka ; Weisstanner, Christian ; Kardas, Piotr ; Hirsch, Hans ; Wiest, Roland ; Lämmle, Bernhard ; Furrer, Hansjakob ; Du Pasquier, Renaud ; Bassetti, Claudio ; Sturzenegger, Mathias ; Krestel, Heinz

In: Journal of NeuroVirology, 2015, vol. 21, no. 6, p. 694-701

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    Titre
    • Progressive multifocal leukoencephalopathy in common variable immunodeficiency: mitigated course under mirtazapine and mefloquine
    Auteur
    • Kurmann, Rebekka. Department of Neurology, Inselspital, Bern University Hospital, Freiburgstrasse 10, 3010, Bern, Switzerland
    • Weisstanner, Christian. University Institute of Diagnostic and Interventional Neuroradiology, Inselspital, Bern University Hospital, Bern, Switzerland
    • Kardas, Piotr. Transplantation & Clinical Virology, Department Biomedicine (Haus Petersplatz), University of Basel, Basel, Switzerland
    • Hirsch, Hans. Transplantation & Clinical Virology, Department Biomedicine (Haus Petersplatz), University of Basel, Basel, Switzerland
    • Wiest, Roland. University Institute of Diagnostic and Interventional Neuroradiology, Inselspital, Bern University Hospital, Bern, Switzerland
    • Lämmle, Bernhard. Department of Hematology, Inselspital, Bern University Hospital, Bern, Switzerland
    • Furrer, Hansjakob. Department of Infectious Diseases, Inselspital, Bern University Hospital, Bern, Switzerland
    • Du Pasquier, Renaud. Department of Neurology, Lausanne University Hospital, Lausanne, Switzerland
    • Bassetti, Claudio. Department of Neurology, Inselspital, Bern University Hospital, Freiburgstrasse 10, 3010, Bern, Switzerland
    • Sturzenegger, Mathias. Department of Neurology, Inselspital, Bern University Hospital, Freiburgstrasse 10, 3010, Bern, Switzerland
    • Krestel, Heinz. Department of Neurology, Inselspital, Bern University Hospital, Freiburgstrasse 10, 3010, Bern, Switzerland
    Type de document
    • Postprint
    Langue
    • Inglese
    Publié dans
    • Journal of NeuroVirology, 2015, vol. 21, no. 6, p. 694-701. Springer US; http://www.springer-ny.com
    Autre version électronique
    Identifiant OAI-PMH
    • oai:doc.rero.ch:332422
    Summary
    Demonstration of survival and outcome of progressive multifocal leukoencephalopathy (PML) in a 56-year-old patient with common variable immunodeficiency, consisting of severe hypogammaglobulinemia and CD4+ T lymphocytopenia, during continuous treatment with mirtazapine (30mg/day) and mefloquine (250mg/week) over 23months. Regular clinical examinations including Rankin scale and Barthel index, nine-hole peg and box and block tests, Berg balance, 10-m walking tests, and Montreal Cognitive Assessment (MoCA) were done. Laboratory diagnostics included complete blood count and JC virus (JCV) concentration in cerebrospinal fluid (CSF). The noncoding control region (NCCR) of JCV, important for neurotropism and neurovirulence, was sequenced. Repetitive MRI investigated the course of brain lesions. JCV was detected in increasing concentrations (peak 2568 copies/ml CSF), and its NCCR was genetically rearranged. Under treatment, the rearrangement changed toward the archetype sequence, and later JCV DNA became undetectable. Total brain lesion volume decreased (8.54 to 3.97cm3) and atrophy increased. Barthel (60 to 100 to 80 points) and Rankin (4 to 2 to 3) scores, gait stability, and box and block (7, 35, 25 pieces) and nine-hole peg (300, 50, 300s) test performances first improved but subsequently worsened. Cognition and walking speed remained stable. Despite initial rapid deterioration, the patient survived under continuous treatment with mirtazapine and mefloquine even though he belongs to a PML subgroup that is usually fatal within a few months. This course was paralleled by JCV clones with presumably lower replication capability before JCV became undetectable. Neurological deficits were due to PML lesions and progressive brain atrophy.