Huntington´s disease (HD) is a neurodegenerative disorder first reported from Sweden in 1936 observed in a small river valley population. This population has recently been reported to be carrying a number of recessive disorders, such as, hereditary haemochromatosis (HH), Wilson´s Disease (WD), the long QT syndrome and autosomal recessive deafness (ARD). Whether the two neurodegenerative disorders, HD and WD, have a common origin is unclear. It was traced to a family born between 1579-1580, most probably succeeded by effective family size (EFS). HD haplotype studies have shown an ancestor born around 1610 in this family.

Aims:  To test whether HD and WD had a common origin and whether their evolution was influenced by the EFS in the founder family around 1579-1580. 

Methods: The surnames and date of birth were available for 40 HD families including 88 family members reported in 1936. Pedigrees were given in Cyrillic.

Results: 35 of 40 HD families came from the pedigree of a founder couple in late 17^th century and all but one individual had origin in a known WD/HH founder family born around 1620. The EFS had a strong impact on the HD/WD founder pedigree. The ARD-, HH- and LQTS-alleles were also present in these families.

Conclusions:  Extensive genealogical studies of a small Swedish river valley population showed a common origin with a higher frequency of HD and WD, possibly because of EFS of the 1579-1580 founder family that also included HH, LQTS and ARD alleles. The epidemiological studies suggested that this gene pool migrated from the British islands possibly transmitted by the Vikings.