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Natural History of Ogawa Village Type Familial Amyloid Polyneuropathy in Japan

  • Masanori Shimoyama
  • Shozo Kito
  • Sadao Katayama
  • Masanori Togo
  • Yasuhiro Yamamura
  • Tomoki Nakano
Chapter

Abstract

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.

Keywords

Sudden Cardiac Death Seventh Decade Autonomic Innervation Familial Amyloid Polyneuropathy Nervous System Disorder 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 1988

Authors and Affiliations

  • Masanori Shimoyama
    • 1
  • Shozo Kito
    • 1
  • Sadao Katayama
    • 1
  • Masanori Togo
    • 1
  • Yasuhiro Yamamura
    • 1
  • Tomoki Nakano
    • 1
    • 2
  1. 1.Third Department of Internal Medicine, HiroshimaUniversity School of MedicineHiroshima 734Japan
  2. 2.Department of Internal MedicineNagano Chuo HospitalNagano 380Japan

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