This paper reports a familial amyloid polyneuropathy (FAP) family in China. This family being investigated had 69 members of five generations. From the third generation
there have been 16 patients. The age of onset was about 3 to 5 decades. The initial symptoms were autonomic nerve symptoms
such as impotence
dyspepsia and diarrhoea
associated with the sensory loss of lower extremities. As the disease progressed
the upper extremities and motor ability were also involved. The duration of disease course was about 8-10 years
most patients died of infection and cachexia. Sural biopsy in 3 patients had showed positive Congo red staining. From the clinical view
this FAP family is similar to FAP I found in Japan. The true classification