Heterogeneity of clinical symptoms in patients with familial amyloidotic polyneuropathy (FAP TTR Met30)

Patients with familial amyloidotic polyneuropathy (FAP TTR Met30) manifest clinical findings, such as autonomic dysfunction, sensori-motor polyneuropathy, and visceral al gan impairment with the progression of the disease. To clarify the clinical features of each patient and to determine the accurate clinical stages of FAP: patients were given clinical scores and divided into 4 different groups by their clinical symptoms. Radar charts of the scores revealed that the shapes of the plotted scares between 2 different time examinations were similar until the bedridden stage of FAP suggesting that the clinical type of FAP had been determined at the onset of the disease and did Mot change as the disease progressed Retrospective study revealed that most of the patients were near death when the total scare became about 70. This evaluating method for FAP TTR Met30 may be useful in systematically evaluating the heterogeneity and the stage of each FAP patient, and also in elucidating the effect of therapies on the patients.