Hereditary Systemic Primary Amyloidosis
The purpose of this report is to review for the Neurochemistry Commission of the World Federation of Neurology the results, previously published1–7, of an extensive investigation of a pedigree in the U.S.A. Certain individuals in this pedigree were afflicted with hereditary systemic primary amyloidosis. This selected review will bring out the clinical and pathological similarities and differences between Portuguese Paramyloidosis8–12 and hereditary systemic primary amyloidosis1–7. Furthermore, an atypical electrophoretic peak (alpha 2′) has been found in hereditary systemic primary amyloidosis2. This biochemical abnormality has not been reported in patients with Portuguese Paramyloidosis. And finally, we would like to raise the following speculation: Could it be that alpha 2′ is a preamyloid substance?
KeywordsPeripheral Neuropathy Carpal Tunnel Syndrome Lipoprotein Fraction Globulin Fraction Vitreous Opacity
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