Note: the below is a port of an archived report preserved for the purpose of publication links.
Huntington disease (HD) is a progressive neurodegenerative disorder that is dominantly inherited and results from a mutation that expands the polymorphic trinucleotide (CAG) tract in the huntingtin gene. The average CAG-tract size in the general population is 16–20 repeats. However, in HD patients the CAG-tract has expanded to 36 repeats or greater.
Although HD occurs worldwide, there are large geographic differences in its prevalence, a measure of the total number of people in a population who have the disease at a given time. Overall, the prevalence of HD is much higher in European populations than in East Asia. Currently, the Maracaibo region of Venezuela has the highest reported worldwide prevalence at 700 cases per 100,000 people.
The Hayden laboratory at the CMMT is investigating genetic signatures called ‘haplotypes’ in the huntingtin gene that might contribute to differences in HD prevalence in different populations. If you have information to add to this compilation of prevalence reports, please email Dr. Michael Hayden.
| POPULATION | DATE OF SURVEY | PREVALENCE (per 100,000) | REFERENCE |
| Australia (New South Wales) | 1996 | 6.3 | (McCusker et al 2000) |
| Australia (Queensland) | 1976 | 5.8 | (Wallace & Parker 1979) |
| Australia (Queensland) | 1969 | 6.3 | (Wallace & Parker 1973) |
| Australia (Tasmania) | 1990 | 12.1 | (Pridmore 1990) |
| Australia (Victoria) | 1963 | 4.6 | (Brothers 1964) |
| Austria | 1993-1997 | 10.0 | (Laccone et al 1999) |
| Azerbaijan | 1985 | 23.8 | (Al-Jader et al 2001) |
| Belgium (Liege, Limbourg, Namur, Luxembourg) | 1970 | 1.6 | (Husquinet 1973) |
| Canada (Manitoba & Saskatchewan) | 1975 | 8.4 | (Shokeir 1975) |
| China (Hong Kong) | 1984-1991 | 0.4 | (Leung et al 1992) |
| China (Hong Kong) | 1984-1991 | 0.4 | (Chang et al 1994) |
| Croatia | 2002 | 1.0 | (Hecimovic et al 2002) |
| Egypt (Assiut) | 1988-1990 | 21.0 | (Kandil et al 1994) |
| Finland | 1986 | 0.5 | (Palo et al 1987) |
| France (North-West) | 5.0 | (Petit & Salomez 1985) | |
| Germany (Kassel) | 1950 | 2.6 | (Al-Jader et al 2001) |
| Germany (Rhineland) | 1933 | 3.2 | (Al-Jader et al 2001) |
| Germany (West, without Berlin & Saarland) | 1950 | 2.2 | (Al-Jader et al 2001) |
| Germany (West, without Berlin & Saarland) | 1939 | 2.2 | (Al-Jader et al 2001) |
| Germany | 1993-1997 | 10.0 | (Laccone et al 1999) |
| Guam (Chamorros) | 1967 | 0.0 | (Chen et al 1968) |
| Iceland | 1963 | 2.7 | (Gudmundsson 1969) |
| India (Pakistan, Punjab and Gujerat) | 1990 | 1.7 | (Shiwach & Lindenbaum 1990) |
| Italy (Aosta) | 1982-1991 | 5.0 | (Al-Jader et al 2001) |
| Italy (Emilia & Parma) | 1980 | 4.8 | (Al-Jader et al 2001) |
| Italy (Ferrara province) | 1987 | 1.9 | (Govoni et al 1988) |
| Italy (Florence) | 1970-1979 | 4.1 | (Groppi et al 1986) |
| Italy (Frogiuone) | 1981 | 2.6 | (Frontali et al 1990) |
| Italy (Genoa Region) | 1930-1977 | 28.0 | (Roccatagliata et al 1979) |
| Italy (Genoa-Savona) | 1973 | 4.5 | (Roccatagliata & Albano 1976) |
| Italy (Latima) | 1981 | 3.0 | (Frontali et al 1990) |
| Italy (Lazio) | 1981 | 2.6 | (Frontali et al 1990) |
| Italy (Puglia) | 1980 | 2.9 | (Al-Jader et al 2001) |
| Italy (Rieti) | 1981 | 5.6 | (Frontali et al 1990) |
| Italy (Rome) | 1981 | 2.5 | (Frontali et al 1990) |
| Italy (Tuscany) | 1978 | 2.3 | (Al-Jader et al 2001) |
| Italy (Viterbo) | 1981 | 1.5 | (Frontali et al 1990) |
| Japan (Aichi) | 1959 | 0.4 | (Kishimoto et al 1957) |
| Japan (Ibaraki) | 1982 | 0.1 | (Kanazawa et al 1990) |
| Japan (San-in) | 1993 | 0.7 | (Nakashima et al 1996) |
| Japan (San-in) | 1997 | 0.7 | (Adachi & Nakashima 1999) |
| Malta | 1994 | 11.8 | (Gassivaro Gallo et al 1999) |
| Malta | 7.8 | (Al-Jader et al 2001) | |
| Mauritius (European descent) | 1977 | 46.2 | (Hayden et al 1981) |
| Mexico | 2008 | 4.0 | (Alonso et al 2009) |
| New Zealand | 5.7 | (Harper 1996) | |
| Nigeria (Ibadan) | 0.2 | (Aiyesimoju et al 1984) | |
| Norway | 1930 | 6.9 | (Saugstad & Odegård 1986) |
| Norway | 1940 | 6.7 | (Saugstad & Odegård 1986) |
| Norway | 1950 | 5.8 | (Saugstad & Odegård 1986) |
| Poland (Pruszkow) | 1960 | 4.8 | (Al-Jader et al 2001) |
| Russia (6 populations in Central Asia) | 1.3 | (Kirilenko et al 2004) | |
| Russia (Volgograd and Volzhsky) | 0.6 | (Kirilenko et al 2004) | |
| Russia (Vladimir Oblast) | 1.9 | (Kirilenko et al 2004) | |
| Slovenia | 2006 | 5.2 | (Peterlin et al 2008) |
| South Africa | 1979 | 0.6 | (Hayden et al 1982) |
| South Africa (Blacks) | 1979 | 0.01 | (Hayden et al 1980) |
| South Africa (Cape Coloured population, mixed race) | 1976 | 3.5 | (Hayden & Beighton 1977) |
| South Africa (Mixed race) | 1979 | 0.9 | (Hayden & Beighton 1977) |
| South Africa (White and Coloured population) | 1979 | 2.2 | (Hayden et al 1980) |
| South Africa (Whites Afrikaans) | 1979 | 0.4 | (Hayden & Beighton 1977) |
| Spain (Salamanca) | 8.4 | (Ruiz et al 1985) | |
| Spain (Cadiz province) | 1968 | 1.3 | (Calcedo Ordóez 1970) |
| Spain (Valencia) | 1987-1992 | 5.4 | (Burguera et al 1997) |
| Sweden | 1965 | 4.7 | (Mattsson 1974) |
| Sweden | 1985 | 5.6 | (Al-Jader et al 2001) |
| Switzerland | 1993-1997 | 10.0 | (Laccone et al 1999) |
| Tanzania (Mount Kilimanjaro, Bantu community) | 1980 | 7.0 | (Scrimgeour 1981) |
| UK (England, Carlisle) | 1961 | 2.8 | (Brewis et al 1966) |
| UK (England, Cornwall) | 1950 | 5.6 | (Bickford & Ellison 1953) |
| UK (England, Cornwall) | 1987 | 4.9 | (Harper 1996) |
| UK (England, Devon) | 1987 | 4.6 | (Harper 1996) |
| UK (England, East Anglia) | 1971 | 9.19 | (Caro 1977) |
| UK (England, Essex) | 1965 | 2.5 | (Heathfield 1967) |
| UK (England, Leeds) | 1966 | 4.2 | (Al-Jader et al 2001) |
| UK (England, Northamptonshire) | 1967-1968 | 6.3 | (Oliver 1970) |
| UK (England, Northamptonshire) | 1960 | 7.2 | (Reid 1960) |
| UK (England, Northamptonshire) | 1954-1955 | 6.5 | (Pleydell 1955) |
| UK (England, Oxford region) | 1985 | 5.7 | (Shiwach 1994) |
| UK (England, Somerset) | 1965 | 5.5 | (Al-Jader et al 2001) |
| UK (England, Wessex) | 1987 | 3.7 | (Harper 1996) |
| UK (Indian subcontinent immigrants) | 1990 | 1.7 | (Shiwach & Lindenbaum 1990) |
| UK (Ireland, Northern) | 1991 | 6.4 | (Morrison et al 1995) |
| UK (Ireland, Northern, County Donegal) | 1991 | 1.6 | (Morrison & Nevin 1993) |
| UK (Scotland, Grampian, north-east ) | 1984 | 10.0 | (Simpson & Johnston 1989) |
| UK (Scotland, South-East) | 1967 | 7.2 | (Cameron & Venters 1967) |
| UK (Scotland, West) | 1960 | 5.2 | (Bolt 1970) |
| UK (Wales, North) | 1981 | 5.5 | (Quarrell et al 1988) |
| UK (Wales, South & Glamorgan) | 1988 | 8.4 | (MacMillan & Harper 1991) |
| UK (Wales, South Glamorgan, Glamorgan & Gwent) | 1994 | 6.2 | (James et al 1994) |
| UK (Wales, South) | 1981 | 8.9 | (Quarrell et al 1988) |
| UK (Wales, South) | 1971 | 7.6 | (Walker et al 1981) |
| UK (Wales, South) | 1971 | 7.6 | (Harper et al 1979) |
| USA & Australia (Whites) | 5.0 | (Al-Jader et al 2001) | |
| USA (Blacks) | 1940 | 1.5 | (Reed & Chandler 1958) |
| USA (Maryland) | 1980 | 5.2 | (Folstein et al 1987) |
| USA (Maryland, Blacks) | 1980 | 6.4 | (Folstein et al 1987) |
| USA (Michigan) | 1940 | 4.1 | (Reed & Chandler 1958) |
| USA (Minnesota) | 1955 | 5.4 | (Pearson et al 1955) |
| USA (Minnesota, Olmsted county) | 1990 | 2.0 | (Kokmen et al 1994) |
| USA (Minnesota, Olmsted county) | 1960 | 6.0 | (Kokmen et al 1994) |
| USA (Minnesota, Rochester) | 1955 | 6.7 | (Kurland 1958) |
| USA (New York, Jews) | 1973 | 3.5 | (Al-Jader et al 2001) |
| USA (South Carolina, Blacks) | 1980 | 1.0 | (Wright et al 1981) |
| USA (South Carolina, White) | 1980 | 4.8 | (Wright et al 1981) |
| Venezuela | 0.5 | (Paradisi et al 2008) | |
| Venezuela (Lake Maracaibo) | 1973 | 699.2 | (Avila-Giron 1973) |
| Yugoslavia (Rijeka district) | 1981 | 4.5 | (Sepci et al 1989) |
| Zimbabwe (Manicaland region, Shona population) | 1988-1989 | 0.8 | (Scrimgeour & Pfumojena 1992) |
See Harper, Conneally and Al-Jader et al for earlier reviews on HD worldwide prevalence.