Cases with familiar amyloid neuropathy starting of the upper limbs and having hepatic disorder

Here we are reporting two cases consisting of a male patient and his elder sister from Kagashima in Gifu City and both suffering from polyneuropathy of dissociation type and skin amyloidosis. In the former, the presence of amyloid was demonstrated not only in the skin, but also in the stomach, liver and gums. He was also diagnosed suffering from chronic hepatitis of inactive type. He responded to DMSO and Cepharanthin. In the patients, no urinary Bence Jones protein nor blood M component was detected and the amyloid exhibited resistance to potassium permanganate treatment. The neuropathy of the patients were slightly different from that of the Portuguese type which starts on lower extremities as well as those conventionally have been reported in Japan.