title: Carpal tunnel syndrome: a common initial symptom of systemic wild-type ATTR (ATTRwt) amyloidosis.
abstract: BACKGROUND
  Systemic wild-type ATTR (ATTRwt) amyloidosis is a prevalent aging-related disorder. However, a limited number of systemic ATTRwt amyloidosis patients have been diagnosed antemortem, and therefore, the prevalence of ATTRwt is underestimated. Here, we investigated clinical findings of a series of systemic ATTRwt amyloidosis patients with antemortem diagnosis.
  
  
  METHODS
  Thirty-one consecutive patients diagnosed with systemic ATTRwt amyloidosis at Shinshu University Hospital were included in this study. Systemic ATTRwt amyloidosis was diagnosed based on proven ATTR amyloid deposition in biopsy specimens and confirmation of wild-type TTR genotype.
  
  
  RESULTS
  The systemic ATTRwt amyloidosis patients consisted of 24 men and seven women, and mean age of onset was 69.8 ± 9.0 years. The most common initial symptom was carpal tunnel syndrome (CTS, 17 patients), followed by heart failure symptoms (14 patients). The mean age at diagnosis was 74.5 ± 8.3 years and the duration of illness from onset to diagnosis was 5.4 ± 4.4 years. Cardiogenic embolism and renal dysfunction are also frequently seen during the course of the disease.
  
  
  CONCLUSIONS
  CTS is the most common initial symptom of systemic ATTRwt amyloidosis. Our results suggest the possibility of systemic ATTRwt amyloidosis diagnosis at an early stage by carefully examining patients with CTS.
authors: a Department of Medicine (Neurology and Rheumatology) .
authors: Michitaka
authors: M
authors: Nakagawa
authors: a Department of Medicine (Neurology and Rheumatology) .|b Institute for Biomedical Sciences .
authors: Yoshiki
authors: Y
authors: Sekijima
authors: a Department of Medicine (Neurology and Rheumatology) .|b Institute for Biomedical Sciences .
authors: Masahide
authors: M
authors: Yazaki
authors: a Department of Medicine (Neurology and Rheumatology) .
authors: Kana
authors: K
authors: Tojo
authors: a Department of Medicine (Neurology and Rheumatology) .
authors: Tsuneaki
authors: T
authors: Yoshinaga
authors: a Department of Medicine (Neurology and Rheumatology) .
authors: Tadashi
authors: T
authors: Doden
authors: c Department of Cardiovascular Medicine , and.
authors: Jun
authors: J
authors: Koyama
authors: d Department of Radiology , Shinshu University School of Medicine , 3-1-1 Asahi, Matsumoto , Japan.
authors: Shin
authors: S
authors: Yanagisawa
authors: a Department of Medicine (Neurology and Rheumatology) .|b Institute for Biomedical Sciences .
authors: Shu-Ichi
authors: S
authors: Ikeda
mesh_terms: D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000453:epidemiology / Q000378:metabolism*; D002349:Carpal Tunnel Syndrome / Q000453:epidemiology / Q000378:metabolism*; D005260:Female; D006801:Humans; D000075363:Immunoglobulin Light-chain Amyloidosis; D008297:Male; D011228:Prealbumin / Q000235:genetics / Q000378:metabolism*; D012744:Sex Ratio
chemical_list: D011228:Prealbumin
keywords: Amyloid; carpal tunnel syndrome; senile systemic amyloidosis; transthyretin; wild-type ATTR (ATTRwt) amyloidosis