Acquired amyloid neuropathy in a Portuguese patient after domino liver transplantation

Authors

  • Isabel Conceição MD,

    Corresponding author
    1. Department of Neurosciences, Centro Hospitalar Lisboa Norte, Hospital de Santa Maria, Avenida Prof. Egas Moniz, Lisbon 1649-028, Portugal
    2. Neuromuscular Unit, Instituto de Medicina Molecular, Faculty of Medicine, University of Lisbon, Lisbon, Portugal
    • Department of Neurosciences, Centro Hospitalar Lisboa Norte, Hospital de Santa Maria, Avenida Prof. Egas Moniz, Lisbon 1649-028, Portugal

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  • Teresinha Evangelista MD,

    1. Department of Neurosciences, Centro Hospitalar Lisboa Norte, Hospital de Santa Maria, Avenida Prof. Egas Moniz, Lisbon 1649-028, Portugal
    2. Neuromuscular Unit, Instituto de Medicina Molecular, Faculty of Medicine, University of Lisbon, Lisbon, Portugal
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  • José Castro Tech,

    1. Department of Neurosciences, Centro Hospitalar Lisboa Norte, Hospital de Santa Maria, Avenida Prof. Egas Moniz, Lisbon 1649-028, Portugal
    2. Neuromuscular Unit, Instituto de Medicina Molecular, Faculty of Medicine, University of Lisbon, Lisbon, Portugal
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  • Pedro Pereira Tech,

    1. Department of Neurosciences, Centro Hospitalar Lisboa Norte, Hospital de Santa Maria, Avenida Prof. Egas Moniz, Lisbon 1649-028, Portugal
    2. Neuromuscular Unit, Instituto de Medicina Molecular, Faculty of Medicine, University of Lisbon, Lisbon, Portugal
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  • Ana Silvestre Tech,

    1. Department of Neurosciences, Centro Hospitalar Lisboa Norte, Hospital de Santa Maria, Avenida Prof. Egas Moniz, Lisbon 1649-028, Portugal
    2. Neuromuscular Unit, Instituto de Medicina Molecular, Faculty of Medicine, University of Lisbon, Lisbon, Portugal
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  • Conceição A Coutinho PhD,

    1. Neuromuscular Unit, Instituto de Medicina Molecular, Faculty of Medicine, University of Lisbon, Lisbon, Portugal
    2. Department of Cardiology Centro Hospitalar Lisboa Norte, Hospital de Santa Maria, Lisbon, Portugal
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  • Mamede de Carvalho PhD

    1. Department of Neurosciences, Centro Hospitalar Lisboa Norte, Hospital de Santa Maria, Avenida Prof. Egas Moniz, Lisbon 1649-028, Portugal
    2. Neuromuscular Unit, Instituto de Medicina Molecular, Faculty of Medicine, University of Lisbon, Lisbon, Portugal
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Abstract

Familial amyloid polyneuropathy (FAP) is a progressive neuropathy with autonomic dysfunction. Domino liver transplantation (DLT), in which the liver of an FAP patient is transplanted into another patient, is routinely applied to compensate for the shortage of available organs. We report a patient who developed a clinical picture of FAP 9 years after a DLT from an FAP donor. Electrophysiological, neuropathological, and autonomic tests were administered. The patient presented with typical clinical features of FAP. Electrophysiological investigation confirmed a moderate sensorimotor axonal and autonomic neuropathy. Sural nerve biopsy confirmed the presence of amyloid deposits in the endoneurium. Skin biopsy at the ankle showed reduced intraepidermal nerve fiber density. Our report shows that FAP can develop in a recipient of an FAP liver. This suggests that careful longitudinal study is required to evaluate the risk of FAP polyneuropathy in patients who undergo domino liver transplantation. Muscle Nerve, 2010

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