Table 2 Comparison of clinical features between early-onset and late-onset ATTR-FAP
Clinical feature | Early-onset | Late-onset |
|---|---|---|
Age of onset of symptoms | • Late 20s to early 40s [79] | |
Penetrance | • High penetrance rate [16] | • Low penetrance rate [24] |
Pattern of neuropathic symptoms | • Loss of superficial sensation, including nociception and thermal sensation (i.e. sensory dissociation) [16] | • Loss of all sensory modalities rather than sensory dissociation. Impaired superficial and deep sensation, neuropathic pain, early distal motor involvement [31, 92] |
Family history of ATTR-FAP | • Common [24] | • Frequently absent [24] |
Autonomic dysfunction | • Severe, life-threatening autonomic dysfunction [16] | • Relatively mild autonomic symptoms [16] |
Other features | • Atrioventricular conduction block requiring pacemaker implantation • Weight loss • Muscle wasting [16] | • Extreme male preponderance |