Two Ethnic Clusters with Huntington Disease in Israel: The Case of Mountain Jews and Karaites

Abstract

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.

Montreal cognitive assessment for evaluating cognitive impairment in Huntington’s disease: a systematic review

“…We identified two studies that used the MoCA to assess the prevalence of cognitive impairment in an HD population 79 , 86 . Both studies were descriptive, presenting clinical and demographic characteristics of HD patients.…”

“…In addition, all the diagnostic test accuracy studies 71 , 72 , 76–78 were published before 2017. In the last 4 years, 14 studies reported the use of MoCA in HD patients: one prevalence study, 86 five neuroimaging studies, 83 , 85 , 87 , 94 , 95 six cognitive studies, 84 , 88 , 89 , 91 , 93 , 96 and two studies investigating clinical aspects of HD 90 , 92 . Nonetheless, the last diagnostic test accuracy study was done in 2013 77 …”