Table 1

Backgrounds and clinical characteristics

FeaturesLate-onset FAP ATTR Val30Met from non-endemic areas
n=50
Men/women43/7
Age of onset (years)64.5±6.5
Age at diagnosis (years)67.3±5.9
Initial symptom of amyloidosis
 Neuropathic symptoms*40 (80)
 Autonomic symptoms5 (10)
 Ocular symptoms3 (6)
 Cardiac symptoms§2 (4)
Presence of sensory dissociation13 (26)
Presence of pain34 (68)
Pacemaker implantation6 (12)
  • Values are expressed as numbers of patients with percentages in parentheses or as the mean±SD.

  • * Paresthesia, pain or weakness in distal portion of the extremities.

  • Diarrhoea/constipation or impotence.

  • Blurred vision.

  • § Oedema in the lower extremities or syncope due to atrioventricular conduction block.

  • Predominant loss of superficial sensation including nociception and thermal sensation.