Objective: To evaluate the epidemiological features of Huntington’s disease (HD) according to the Centre for Movement Disorders and Botulinum Therapy of Republic of Tatarstan (Centre…) in the region.

Background: .

Methods: We have 44 patients with manifest HD (total – 37 families), of whom 26 (59%) female patients and 18 (41%) male patients according Centre… The age of patients ranged from 19 to 79 years; mean age – 47,3 ± 12,5 years (among female patients 22 – 79 years; the average age – 47,7 ± 10,5 years, male – 19-77 years; mean age – 46,6 ± 15 ,8 years).

All patients were divided into 3 groups: 1-the total group (44 people), 2 -female patients (26 people), 3 – male patients (18 people); also highlighted two other groups of patients: 1 – inherited disease on his father’s side, 2nd – on the mother.

The study design included the identification and analysis of clinical data, epidemiological and genealogical data, laboratory parameters.

Results: It was found the prevalence of the disease – 1,1 cases per 100 000 population in Tatarstan. A higher prevalence of HD occurred in the northwestern region of the Republic of Tatarstan and the north-eastern parts of the Republic (46% and 32% of the total number of cases, respectively), even with the 11,4% of patients after migration from other regions of Russia. Smoking and alcoholism are equally were detected in 6,8%, with HD and only male patients.

The weak correlation was found (0,29) between the age of onset and number of CAG-repeats in patients with male HD, i.e., the higher the expansion of trinucleotide repeats, the earlier the disease debut. Psychiatric manifestations prevailed in 75% in the debut of HD, regardless of gender and inheritance path. It revealed a moderate correlation between the severity of motor symptoms on the number of CAG-repeats in HD patients with paternal (0,35) and a weak correlation of these parameters in patients with HD in the female line. No significant difference in the severity of clinical manifestations in patients with HD on the father’s and women’s lines have been identified. The weak correlation (-0,29) with the total functional capacity of the time the disease has been established in female patients.

It was noted earlier onset of illness in patients and their ancestors on the paternal line (average 28 ± 7,6 years and 41,2 ± 6,9 years, respectively; p˂0,05).

Conclusions: .

References: .

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MDS Abstracts - https://www.mdsabstracts.org/abstract/huntingtons-disease-in-the-republic-of-tatarstan-epidemiological-features/