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Two Ethnic Clusters with Huntington Disease in Israel: The Case of Mountain Jews and Karaites
Jennifer Zitser1, Avner Thaler, Noit Inbar
1Movement Disorders Unit, Neurological Institute, Tel Aviv Medical Center, Tel Aviv, Israel.
Insights
Huntington disease (HD) shows clustering in specific Jewish groups in Israel, particularly Mountain Jews and Karaites. This study highlights over-representation in the HD patient population at a specialized center.
Area of Science:
- Neurogenetics
- Epidemiology
- Jewish Genetic Diseases
Background:
- Huntington disease (HD) prevalence estimates vary globally.
- Limited data exists on HD prevalence within Israel's diverse Jewish population.
Purpose of the Study:
- To investigate the clinical, cognitive, and demographic characteristics of Huntington disease patients in Israel.
- To identify potential population-specific patterns of HD within the Jewish community.
Main Methods:
- A cross-sectional study involving 84 Huntington disease patients at a tertiary single-center clinic in Israel.
- Assessment included clinical evaluations, cognitive tests, and demographic data collection.
Main Results:
- The cohort included Ashkenazi, Mountain (Caucasus), Sephardi Jews, and Karaites.
- Mountain Jews and Karaites were over-represented in the HD cohort compared to their general population prevalence.
- No significant differences were found in CAG repeat numbers, age at onset, disease duration, or cognitive scores among the groups.
Conclusions:
- A notable clustering of Huntington disease was observed among specific Jewish subpopulations treated at the center.
- Mountain Jews and Karaites represent disproportionately high percentages of the HD patient population in Israel.
- These findings suggest a potential genetic predisposition or specific epidemiological factors influencing HD occurrence in these groups.
Background:
Worldwide prevalence estimates of Huntington disease (HD) vary widely, with no reliable information regarding the Jewish population in Israel.
Methods:
This specialized tertiary single-center cross-sectional study assessed clinical, cognitive, and demographic characteristics of 84 HD patients who were treated at the Movement Disorder Unit of the Tel Aviv Medical Center, Israel.
Results:
Our cohort was composed of one-third Ashkenazi Jews, 27% Mountain Jews (Caucasus Jews), 18% Sephardi Jews, and 21% Karaites, with both Mountain Jews and Karaites over-represented compared to their relevant proportion in the population of the state of Israel, which is less than 1%. No between-group differences were detected regarding the number of CAG (cytosine-adenine-guanine) repeats, age at onset, disease duration, years from symptom onset to diagnosis, gender, years of education, Unified Huntington Disease Rating Scale scores, or the Montreal Cognitive Assessment scores.
Conclusion:
We detected clustering of HD among the population treated at our Medical Center, which has the only specialized HD clinic in the country, with a high percentage of HD among 2 relatively small subpopulations of Jews: Mountain Jews and Karaites.
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