Table 1.

Particularities of early and late onset familial amyloid polyneuropathy and its course.

	Early onset met30 TTR FAP [Coutinho et al. 1980; Koike et al. 2002]	Late onset met30 TTR FAP [Koike et al. 2002 ; Koike et al. 2012]
Age of onset	<50 years	>50 years
Country	Portugal, Japan, Brazil	Sweden, France, UK, Italy, Japan
Positive family history	94%	48%
Initial clinical presentation
Peripheral neuropathy	57%	81%
Autonomic neuropathy	48%	10%
Weight loss	5%	0
Mean delay for aid for walking	>5.6 years	3 years
Cardiac	Progressive conduction disorders	Restrictive cardiomyopathy Cardiac insufficiency
Median survival	11 years	7.3 years
Causes of death	Cachexia, infections	Cardiac insufficiency Sudden death Cachexia or secondary infection

FAP, familial amyloid polyneuropathy; TTR, transthyretin.