Familial amyloidotic polyneuropathy.

The hereditary amyloidotic polyneuropathies are a heterogeneous collection of familial diseases with the systemic accumulation of amyloid fibrils in the peripheral nerves and other organs. At the present time, our knowledge concerning the biochemical nature of the amyloid fibril proteins in the hereditary syndromes is still limited. Consequently, the only current classification is based on the 3 prototypic clinical forms as follows: Type I with onset in the lower limbs (Portuguese, Japanese, Swedish and Jewish families), Type II with onset in the upper limbs (Swiss-German families), and Type III with cranial neuropathy plus lattice corneal dystrophy (Finnish families) (1) (Table I).