Table 1.

Characteristics of Val30Met early- and late-onset ATTR amyloidosis at the time of diagnosis and clinical course

	Early-onset Val30Met [7, 19]	Late-onset Val30Met [8, 19, 20]
Age at onset, years	< 50	≥ 50
Country	Portugal, Japana, Brazil, Swedenb	Swedenb, France, UK, Italy, Japan, USA
Positive family history, %	94	48
Peripheral neuropathy, %	57	81
Autonomic neuropathy, %	48	10
Weight loss, %	5	0
Disease course
Mean delay in need for aid in walking, years	> 5.6	3
Mean delay for wheelchair bound, years	10	6
Cardiac events	Progressive conduction disorders	Restrictive cardiomyopathy Cardiac insufficiency Progressive conduction disorders
Median survival, years	11	7.3
Cause of death	Cachexia Infection	Cardiac insufficiency Sudden death Cachexia or secondary infection

Reprinted with permission from Adams [18]

ATTR amyloid transthyretin

^aEndemic areas, Nagano and Arao Kumamoto, Japan

^bPatients with early- and late-onset disease are found in the endemic area in Sweden; all are believed to have a common Swedish founder [21]