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Table 2 Comparison of clinical features between early-onset and late-onset ATTR-FAP

From: Diagnosis and management of transthyretin familial amyloid polyneuropathy in Japan: red-flag symptom clusters and treatment algorithm

Clinical feature Early-onset Late-onset
Age of onset of symptoms • Late 20s to early 40s [79] • ≥50 years [16, 20, 24]
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
• Frequent presence of cardiomegaly [92, 157]
  1. ATTR-FAP Transthyretin familial amyloid polyneuropathy