Session Information
Date: Sunday, October 7, 2018
Session Title: Choreas (Non-Huntington's Disease)
Session Time: 1:45pm-3:15pm
Location: Hall 3FG
Objective: This study is aimed to make literature review of international/national publications and conference papers on HD from Turkey, aiming to reflect at least partially the picture of HD in Turkey.
Background: There is a marked heterogeneity of the HD in Central and Eastern Europe prevalence rates changing between of 0.53-5.16/100.000 (Neuroepidemiyology). Data regarding demographicand clinical features of HD in Turkey is limited.
Methods: In this review published articles, case reports, and letters were searched in PUBMED, MEDLINE, EMBASE and SCOPUS and one national database, the ULAKBİM Turkish medical literature database Key words used for Scopus and PubMed were also‘Huntington’s Disease and Turkey’, ‘Huntington’s Chorea and Turkey’,‘Huntington’s Chorea and Turkish’ ‘Huntington’s Disease and Turkish’, ‘HD and Turkey’, ‘HD and Turkish’.
Results: The first HD case from Turkey was reported in 1945 and the fisrt English publication was written in 1961 with 35 cases. In 1993 report of a Turkish family with 7 HD patients evaluated by SPECT was published By Dutch researchers. Karabulutet all (n013), Atac et all (n027), İnce- Günal et all (n05), Ersoy et all (n=27) published genetic analysis of the patients.Akbaset all published 127 HD patients with genetic results then reported enlarged analysis of these patients (n=413) in 2016. CAG repeat was 40-50 (%76,5) in the largest series, ^50 repeat in %20,8 patients, 36-39 repeat %2,7 patients. The number of CAG repeat expansions were inversely correlated with age of onset. IN three different studies, genetic variations, modifiers and polimorfizmsof GRIK2, TBP, BDNF, HIP1, ZDHHC17, TP53 R72, GRIN were observed. Against genetic analysis, demographic and clinical reports were not reported enough. Age at onset was between 37-46 years. Juvenil HD was ^%2 patients. No gender difference was reported. Motor and neuropsychiatric symptoms were not mentioned enough in genetic reports. Chorea was reported in all of the patients, 37,2 % psychiatric problems, 28,5 % cognitive problems were reported in 1961.
Conclusions: It is likely that the paucity of Turkish reports on HD is due to under-reporting rather than to a genuinely low prevalence. Establishment of a country-wide multicentered prospective study will improve the reflection of the picture HD in Turkey and lead to further studies.
To cite this abstract in AMA style:
Y. Karadag, MA. Akbostanci, H. Hanagasi, N. Subutay Oztekin, M. Demirkiran, AN. Basak. Huntington’s Disease in Turkey: A Bird’s Eye Review of the Literature [abstract]. Mov Disord. 2018; 33 (suppl 2). https://www.mdsabstracts.org/abstract/huntingtons-disease-in-turkey-a-birds-eye-review-of-the-literature/. Accessed November 21, 2024.« Back to 2018 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/huntingtons-disease-in-turkey-a-birds-eye-review-of-the-literature/