Features | Late-onset FAP ATTR Val30Met from non-endemic areas |
n=50 | |
Men/women | 43/7 |
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 symptoms† | 5 (10) |
Ocular symptoms‡ | 3 (6) |
Cardiac symptoms§ | 2 (4) |
Presence of sensory dissociation¶ | 13 (26) |
Presence of pain | 34 (68) |
Pacemaker implantation | 6 (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.