Type I familial amyloid polyneuropathy--report of a family in Taiwan.

We report three cases of histologically verified systemic amyloidosis with polyneuropathy. Common to them were early onset progressive peripheral sensorimotor disturbance starting in the legs and prominent autonomic dysfunctions such as postural hypotension, anhidrosis, and loss of pupillary light reflexes. Other characteristic features included vomiting, alternating diarrhea and constipation, opacities of the vitreous bodies, and congestive heart failure. All these clinical manifestations resemble type I familial amyloid polyneuropathy described by Andrade in 1952 from Portugal. Sural nerve biopsy stained with Congo red showed typical green birefringence under polarized light microscope. Histologically verified familial cases of this form of amyloid polyneuropathy have not been reported from Taiwan before.