[Familial portuguese type amyloid polyneuropathy. Attempt at a pathogenetic interpretation]

The pathogenesis of familial portuguese amyloid polyneuropathy, a form of hereditary peripheral neuropathy is at present uncertain. Our hypothesis is that there may be a genetic modification in the metabolism of the Schwann cell. Normally this cell produces myelin, however with genetic modification perhaps it could switch its production to the protein precursor for amyloid. Therefore, the abnormality would reside primarily in the nervous tissue with the amyloid deposits being secondary but contributing to the axonal degeneration.