Natural History of Ogawa Village Type Familial Amyloid Polyneuropathy in Japan
Clinical studies were made on 118 cases of familial amyloid polyneuropathy (FAP) originated from Ogawa Village, Japan. The age of onset ranged from 17 to 72 years, and the mean age of onset was 32.1 years in males and 38.4 years in females. One family was unique in that ages of onset of 6 affected members were in their seventh decade. There were 3 exceptional cases in which the disease remained stationarily for more than 10 years with normal activities of daily living (ADL) functions. Developement of the disease was progressive. The progression was not steadily, but stepwise. Clinically presumed causes of death were various including sudden cardiac death, renal failure and sepsis. The average duration of the illness was 10 years. In general, clinical pictures of Ogawa Village cases showed a broader spectrum than those from other areas of the world. For a therapeutic purpose, DMSO was administered to 40 patients orally and/or cutaneously. DMSO administration alleviated gastrointestinal, sensory disturbances and dysuria. When DMSO was discontinued, the phenomena of the “rebound” progression were sometimes observed.
KeywordsSudden Cardiac Death Seventh Decade Autonomic Innervation Familial Amyloid Polyneuropathy Nervous System Disorder
Unable to display preview. Download preview PDF.
- Andrade, C., 1952. Peculiar form of peripheral neuropathy, familial atypical generalized amyloidosis with special involvement of the peripheral nerve, Brain., 75: 407.Google Scholar
- Isobe, T., and Osserman, E. F., 1976, Effect of dimethyl sulfoxide (DMSO) on Bence Jones proteins, amyloid fibrils and casein-induced amyloidosis, in: “Amyloidosis,” O. Wegelius., and A. Pasternack, eds. Academic Press, London, New York, San Francisco.Google Scholar
- Yamamura, Y., Kito, S., Shimoyama, M., Abe, A., Matsubayashi, H., Anzai, N., and Nakano, T, 1986, Cardiac disorders and autonomic nervous system involvement in familial amyloidosis, in: “Amyloidosis,” G. G. Glenner., E. F. Osserman., N. P. Benditt., E. Calkins., A. S. Cohen., and D. Zucker-Franklin, eds. Plenum Press, New York and London.Google Scholar