TY - JOUR
T1 - Two Ethnic Clusters with Huntington Disease in Israel
T2 - The Case of Mountain Jews and Karaites
AU - Zitser, Jennifer
AU - Thaler, Avner
AU - Inbar, Noit
AU - Gad, Alona
AU - Faust-Socher, Achinoam
AU - Paleacu, Diana
AU - Anca-Herschkovitch, Marieta
AU - Balash, Yakov
AU - Shabtai, Hertzel
AU - Ash, Elissa L.
AU - Merkin, Ludmila
AU - Manor, Yael
AU - Kestenbaum, Meir
AU - David, Aya Bar
AU - Peretz, Chava
AU - Naiman, Tova
AU - Bar-Shira, Anat
AU - Orr-Urtreger, Avi
AU - Dangoor, Nira
AU - Giladi, Nir
AU - Gurevich, Tanya
N1 - Publisher Copyright:
© 2017 S. Karger AG, Basel. Copyright: All rights reserved.
PY - 2017/12/1
Y1 - 2017/12/1
N2 - 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.
AB - 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.
KW - Huntington disease
KW - Karaites
KW - Mountain Jews
UR - http://www.scopus.com/inward/record.url?scp=85028362740&partnerID=8YFLogxK
U2 - 10.1159/000479375
DO - 10.1159/000479375
M3 - Article
C2 - 28848105
AN - SCOPUS:85028362740
SN - 1660-2854
VL - 17
SP - 281
EP - 285
JO - Neurodegenerative Diseases
JF - Neurodegenerative Diseases
IS - 6
ER -