Three forms of dominant amyloid neuropathy

While a common name and similar pathogenic features unite the genetic amyloidoses, these disorders generally can be distinguished by distinctive clinical patterns. This is particularly apparent when large kindreds are compared. The authors present instructive examples of members from kindreds of the three most clearly defined autosomal dominant types of amyloid neuropathy. Salient features, important for diagnosis, prognosis, and management, distinguish each type. Growing understanding of the biochemical changes in the amyloidoses aids appreciation of these disorders even as it provides an opportunity to study mechanisms which may be important in normal protein metabolism.