1Department of Neurology, Ewha Womans University Mokdong Hospital, Ewha Womans University College of Medicine, Seoul, Korea
2Department of Neurology, Chungnam National University Sejong Hospital, Chungnam National University College of Medicine, Sejong, Korea
3Department of Neurology, Jeonbuk National University Hospital, Jeonbuk National University Medical School, Jeonju, Korea
4Research Institute of Clinical Medicine and Biomedical Research Institute, Jeonbuk National University Hospital, Jeonbuk National University Medical School, Jeonju, Korea
5Department of Neurology, Chungnam National University Hospital, Chungnam National University College of Medicine, Daejeon, Korea
6Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
7Department of Neurology, Bundang Medical Center, CHA University School of Medicine, Seongnam, Korea
8Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
9Department of Neurology, Gil Medical Center, Gachon University College of Medicine, Incheon, Korea
10Department of Neurology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea
11Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
12Department of Neurology, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea
13Department of Neurology, Korea University Guro Hospital, Korea University College of Medicine, Seoul, Korea
14Department of Neurology, Soonchunhyang University Seoul Hospital, Seoul, Korea
15Department of Genomic Medicine, Seoul National University Hospital, Seoul, Korea
16Department of Neurology, Hallym University Sacred Heart Hospital, Anyang, Korea
17Department of Neurology, Seoul Metropolitan Government-Seoul National University Boramae Medical Center, Seoul National University College of Medicine, Seoul, Korea
Copyright © 2024 The Korean Movement Disorder Society
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Conflicts of Interest
The authors have no financial conflicts of interest.
Funding Statement
This work was supported by the Seoul Metropolitan Government-Seoul National University Boramae Medical Center (SMG-SNU BMC) focused clinical research grant-in-aid (04-2022-0014).
Author contributions
Conceptualization: Jee-Young Lee, Eungseok Oh, Won Tae Yoon. Data curation: Chan Young Lee, Jee-Young Lee, Eungseok Oh, Chaewon Shin, Yun Su Hwang. Formal analysis: Chan Young Lee, Chaewon Shin. Funding acuiqition: Jee-Young Lee. Investigation: Jee-Young Lee, Chan Young Lee, Chaewon Shin, Eungseok Oh, Yun Su Hwang, Won Tae Yoon, Manho Kim, Hyun Sook Kim, Sun Ju Chung, Young Hee Sung, Jin Whan Cho, Jae-Hyeok Lee, Han-Joon Kim, Hee Jin Chang, Beomseok Jeon, Kyung Ah Woo, Kyum- Yil Kwon, Jangsup Moon, Young Eun Kim, Seong-Beom Koh. Methodology: Jee-Young Lee, Chan Young Lee, Chaewon Shin, Eungseok Oh. Project administration: Jee-Young Lee, Eungseok Oh. Resources: Jee-Young Lee, Chan Young Lee, Chaewon Shin, Eungseok Oh, Yun Su Hwang, Won Tae Yoon, Manho Kim, Hyun Sook Kim, Sun Ju Chung, Young Hee Sung, Jin Whan Cho, Jae-Hyeok Lee, Han-Joon Kim, Hee Jin Chang, Beomseok Jeon, Kyung Ah Woo, Kyum-Yil Kwon, Jangsup Moon, Young Eun Kim, Seong-Beom Koh. Supervision: Jee-Young Lee, Manho Kim, Eungseok Oh. Validation: Chan Young Lee, Chaewon Shin, Jee-Young Lee, Eungseok Oh. Visualization: Chan Young Lee, Chaewon Shin. Writing—original draft: Chan Young Lee, Chaewon Shin. Writing—review & editing: Jee-Young Lee, Chan Young Lee, Chaewon Shin, Eungseok Oh, Yun Su Hwang, Won Tae Yoon, Manho Kim, Hyun Sook Kim, Sun Ju Chung, Young Hee Sung, Jin Whan Cho, Jae-Hyeok Lee, Han-Joon Kim, Hee Jin Chang, Beomseok Jeon, Kyung Ah Woo, Kyum-Yil Kwon, Jangsup Moon, Young Eun Kim, Seong-Beom Koh.
Data are shown as the mean ± standard deviation or frequency (percentage).
HD, Huntington’s disease; CAG, cytosine-adenine-guanine; MoCA, Montreal Cognitive Assessment; MMSE, Mini-Mental State Examination; BDI, Beck Depression Inventory; GDepS, Geriatric Depression Scale; NPI, Neuropsychiatric Inventory; UHDRS, Unified Huntington’s Disease Rating Scale; SF, Shoulson–Fahn.
Characteristics | Total (n = 45) |
---|---|
Age, yr | 54.3 ± 13.6 |
Sex | |
Male | 24 (53.3) |
Female | 21 (46.7) |
Years of schooling | |
1–6 yr | 4 (8.9) |
7–9 yr | 6 (13.3) |
10–12 yr | 12 (26.7) |
≥ 13 yr | 23 (51.1) |
Marital status | |
Married | 36 (80.0) |
Unmarried | 8 (17.8) |
Widowed | 1 (2.20) |
Relationship between caregiver and patient | |
Blood relation | 20 (44.4) |
Sibling | 8 (17.8) |
Parent | 5 (11.1) |
Offspring | 7 (15.6) |
Spouse | 23 (51.1) |
Other | 2 (4.4) |
Caregiving duration,* yr | 4.9 ± 3.8 |
ZBI-12 | 17.6 ± 14.2 |
Characteristics | ZBI-12 score < 26 (n = 22) | ZBI-12 score ≥ 26 (n = 23) | p value |
---|---|---|---|
Characteristics of patients | |||
Age, yr | 57.0 ± 12.2 | 58.9 ± 13.1 | 0.615 |
Sex | 0.235 | ||
Male | 5 (11.1) | 9 (20.0) | |
Female | 17 (37.8) | 14 (31.1) | |
Onset age, yr | 49.1 ± 14.1 | 51.0 ± 13.5 | 0.655 |
Disease duration, yr | 9.38 ± 8.51 | 8.78 ± 5.21 | 0.487 |
CAG repeat length | 42.1 ± 2.5 | 42.8 ± 2.9 | 0.516 |
MoCA | 16.7 ± 7.2 | 12.8 ± 10.0 | 0.180 |
MMSE | 21.9 ± 6.0 | 17.4 ± 8.3 | 0.054 |
BDI | 15.7 ± 14.7 | 15.1 ± 12.0 | 0.900 |
GDepS | 11.9 ± 7.7 | 7.3 ± 5.2 | 0.061 |
NPI | 12.8 ± 15.1 | 16.3 ± 13.2 | 0.169 |
UHDRS Part I (Motor assessment) | 42.4 ± 24.4 | 49.0 ± 29.0 | 0.353 |
UHDRS Part IV-A (Functional assessment) | 13.5 ± 8.4 | 11.1 ± 7.6 | 0.220 |
UHDRS Part IV-B (Independence scale) | 74.8 ± 21.6 | 59.1 ± 29.5 | 0.062 |
UHDRS Part IV-C (Functional capacity)* | 8.0 ± 3.8 | 5.4 ± 4.3 | 0.039 |
SF staging* | 2.27 ± 1.21 | 2.96 ± 1.33 | 0.078 |
Characteristics of caregivers* | |||
Age, yr | 54.1 ± 14.6 | 54.5 ± 12.8 | 0.908 |
Sex | 0.873 | ||
Male | 12 (26.7) | 12 (26.7) | |
Female | 10 (22.2) | 11 (24.4) | |
Years of schooling | 0.964 | ||
1–6 yr | 2 (4.4) | 2 (4.4) | |
7–9 yr | 3 (6.7) | 3 (6.7) | |
10–12 yr | 5 (11.1) | 7 (15.6) | |
≥ 13 yr | 12 (26.7) | 11 (24.4) | |
Marital status | 0.846 | ||
Married | 17 (37.8) | 19 (42.2) | |
Unmarried | 4 (8.9) | 4 (8.9) | |
Widowed | 1 (2.2) | 0 | |
Blood relation | 9 (41.0) | 11 (47.8) | 0.641 |
Relationship between caregiver and patient | 0.268 | ||
Sibling | 3 (6.7) | 5 (11.1) | |
Parent | 4 (8.9) | 1 (2.2) | |
Offspring | 2 (4.4) | 5 (11.1) | |
Spouse | 11 (24.4) | 12 (26.7) | |
Other | 2 (4.4) | 0 (0) | |
Caregiving duration, yr* | 3.2 ± 6.4 | 6.4 ± 4.2 | 0.003 |
Data are shown as the mean ± standard deviation or number (percentage).
* statistically significant variables in the analysis.
ZBI-12, 12-item Zarit Burden Interview; CAG, cytosine-adenine-guanine; MoCA, Montreal Cognitive Assessment; MMSE, Mini-Mental State Examination; BDI, Beck Depression Inventory; GDepS, Geriatric Depression Scale; NPI, Neuropsychiatric Inventory; UHDRS, Unified Huntington’s Disease Rating Scale; SF, Shoulson–Fahn.
Characteristics |
Univariable analysis |
Multivariable (Covariables: Independence scale, onset age, disease duration, CAG repeat number) |
Multivariable (Covariables: Functional capacity, onset age, disease duration, CAG repeat number) |
||||||
---|---|---|---|---|---|---|---|---|---|
B | 95% CI | p value | B | 95% CI | p value | B | 95% CI | p value | |
Age | 0.019 | -0.227, 0.264 | 0.88 | ||||||
Onset age | 0.016 | -0.220, 0.253 | 0.89 | 0.033 | -0.248, 0.314 | 0.815 | 0.038 | -0.238, 0.314 | 0.78 |
Disease duration | -0.044 | -0.502, 0.415 | 0.849 | -0.248 | -0.731, 0.235 | 0.305 | -0.158 | -0.618, 0.303 | 0.492 |
CAG repeat number | 0.421 | -0.752, 1.594 | 0.472 | 0.664 | -0.712, 2.039 | 0.334 | 0.525 | -0.834, 1.884 | 0.439 |
MoCA | -0.162 | -0.541, 0.217 | 0.39 | ||||||
MMSE | -0.367 | -0.773, 0.039 | 0.075 | ||||||
BDI | -0.077 | -0.345, 0.192 | 0.564 | ||||||
GDepS | -0.268 | -0.784, 0.247 | 0.297 | ||||||
NPI | 0.206 | -0.029, 0.440 | 0.083 | ||||||
UHDRS Part I (Motor assessment) | 0.09 | -0.024, 0.205 | 0.12 | ||||||
UHDRS Part IV-A (Functional assessment) | -0.248 | -0.634, 0.139 | 0.204 | ||||||
UHDRS Part IV-B (Independence scale) | -0.154 | -0.261, -0.047 | 0.006* | -0.174 | -0.3, -0.048 | 0.008* | |||
UHDRS Part IV-C (Functional capacity) | -1.082 | -1.751, -0.414 | 0.002* | -1.128 | -1.883, -0.373 | 0.004* |
* statistically significant variables in the analysis.
ZBI-12, 12-item Zarit Burden Interview, CAG, cytosine-adenine-guanine; CI, confidence interval; MoCA, Montreal Cognitive Assessment; MMSE, Mini-Mental State Examination; BDI, Beck Depression Inventory; GDepS, Geriatric Depression Scale; NPI, Neuropsychiatric Inventory; UHDRS, Unified Huntington’s Disease Rating Scale.
Comments on this article
Characteristics | Total (n = 65) |
---|---|
Age, yr | 56.3 ± 12.5 |
Sex | |
Male | 22 (33.8) |
Female | 43 (66.2) |
Onset age, yr | 49.3 ± 12.3 |
Young onset (< 30 yr) | 4 (6.1) |
Typical onset (30–59 yr) | 49 (75.4) |
Late onset (≥ 60 yr) | 12 (18.5) |
Disease duration, yr | 8.3 ± 6.6 |
CAG repeat length | 42.9 ± 4.0 |
Range | 38–65 |
Family history of HD | 59 (79.7) |
MoCA | 15.4 ± 8.3 |
MMSE | 20.7 ± 6.8 |
BDI | 16.3 ± 13.6 |
GDepS | 9.7 ± 7.7 |
NPI | 15.0 ± 16.1 |
UHDRS Part I (Motor assessment) (0–124) | 42.3 ± 26.0 |
UHDRS Part IV-A (Functional assessment) (0–25) | 12.4 ± 8.7 |
UHDRS Part IV-B (Independence scale) (10–100) | 69.5 ± 26.9 |
UHDRS Part IV-C (Functional capacity) (0–13) | 6.5 ± 4.7 |
SF staging | |
Stage I (11–13) | 18 (27.7) |
Stage II (7–10) | 14 (21.5) |
Stage III (3–6) | 14 (21.5) |
Stage IV, V (0, 1–2) | 19 (29.2) |
Characteristics | Total (n = 45) |
---|---|
Age, yr | 54.3 ± 13.6 |
Sex | |
Male | 24 (53.3) |
Female | 21 (46.7) |
Years of schooling | |
1–6 yr | 4 (8.9) |
7–9 yr | 6 (13.3) |
10–12 yr | 12 (26.7) |
≥ 13 yr | 23 (51.1) |
Marital status | |
Married | 36 (80.0) |
Unmarried | 8 (17.8) |
Widowed | 1 (2.20) |
Relationship between caregiver and patient | |
Blood relation | 20 (44.4) |
Sibling | 8 (17.8) |
Parent | 5 (11.1) |
Offspring | 7 (15.6) |
Spouse | 23 (51.1) |
Other | 2 (4.4) |
Caregiving duration, |
4.9 ± 3.8 |
ZBI-12 | 17.6 ± 14.2 |
Characteristics | ZBI-12 score < 26 (n = 22) | ZBI-12 score ≥ 26 (n = 23) | p value |
---|---|---|---|
Characteristics of patients | |||
Age, yr | 57.0 ± 12.2 | 58.9 ± 13.1 | 0.615 |
Sex | 0.235 | ||
Male | 5 (11.1) | 9 (20.0) | |
Female | 17 (37.8) | 14 (31.1) | |
Onset age, yr | 49.1 ± 14.1 | 51.0 ± 13.5 | 0.655 |
Disease duration, yr | 9.38 ± 8.51 | 8.78 ± 5.21 | 0.487 |
CAG repeat length | 42.1 ± 2.5 | 42.8 ± 2.9 | 0.516 |
MoCA | 16.7 ± 7.2 | 12.8 ± 10.0 | 0.180 |
MMSE | 21.9 ± 6.0 | 17.4 ± 8.3 | 0.054 |
BDI | 15.7 ± 14.7 | 15.1 ± 12.0 | 0.900 |
GDepS | 11.9 ± 7.7 | 7.3 ± 5.2 | 0.061 |
NPI | 12.8 ± 15.1 | 16.3 ± 13.2 | 0.169 |
UHDRS Part I (Motor assessment) | 42.4 ± 24.4 | 49.0 ± 29.0 | 0.353 |
UHDRS Part IV-A (Functional assessment) | 13.5 ± 8.4 | 11.1 ± 7.6 | 0.220 |
UHDRS Part IV-B (Independence scale) | 74.8 ± 21.6 | 59.1 ± 29.5 | 0.062 |
UHDRS Part IV-C (Functional capacity) |
8.0 ± 3.8 | 5.4 ± 4.3 | 0.039 |
SF staging |
2.27 ± 1.21 | 2.96 ± 1.33 | 0.078 |
Characteristics of caregivers |
|||
Age, yr | 54.1 ± 14.6 | 54.5 ± 12.8 | 0.908 |
Sex | 0.873 | ||
Male | 12 (26.7) | 12 (26.7) | |
Female | 10 (22.2) | 11 (24.4) | |
Years of schooling | 0.964 | ||
1–6 yr | 2 (4.4) | 2 (4.4) | |
7–9 yr | 3 (6.7) | 3 (6.7) | |
10–12 yr | 5 (11.1) | 7 (15.6) | |
≥ 13 yr | 12 (26.7) | 11 (24.4) | |
Marital status | 0.846 | ||
Married | 17 (37.8) | 19 (42.2) | |
Unmarried | 4 (8.9) | 4 (8.9) | |
Widowed | 1 (2.2) | 0 | |
Blood relation | 9 (41.0) | 11 (47.8) | 0.641 |
Relationship between caregiver and patient | 0.268 | ||
Sibling | 3 (6.7) | 5 (11.1) | |
Parent | 4 (8.9) | 1 (2.2) | |
Offspring | 2 (4.4) | 5 (11.1) | |
Spouse | 11 (24.4) | 12 (26.7) | |
Other | 2 (4.4) | 0 (0) | |
Caregiving duration, yr |
3.2 ± 6.4 | 6.4 ± 4.2 | 0.003 |
Characteristics | Univariable analysis |
Multivariable (Covariables: Independence scale, onset age, disease duration, CAG repeat number) |
Multivariable (Covariables: Functional capacity, onset age, disease duration, CAG repeat number) |
||||||
---|---|---|---|---|---|---|---|---|---|
B | 95% CI | p value | B | 95% CI | p value | B | 95% CI | p value | |
Age | 0.019 | -0.227, 0.264 | 0.88 | ||||||
Onset age | 0.016 | -0.220, 0.253 | 0.89 | 0.033 | -0.248, 0.314 | 0.815 | 0.038 | -0.238, 0.314 | 0.78 |
Disease duration | -0.044 | -0.502, 0.415 | 0.849 | -0.248 | -0.731, 0.235 | 0.305 | -0.158 | -0.618, 0.303 | 0.492 |
CAG repeat number | 0.421 | -0.752, 1.594 | 0.472 | 0.664 | -0.712, 2.039 | 0.334 | 0.525 | -0.834, 1.884 | 0.439 |
MoCA | -0.162 | -0.541, 0.217 | 0.39 | ||||||
MMSE | -0.367 | -0.773, 0.039 | 0.075 | ||||||
BDI | -0.077 | -0.345, 0.192 | 0.564 | ||||||
GDepS | -0.268 | -0.784, 0.247 | 0.297 | ||||||
NPI | 0.206 | -0.029, 0.440 | 0.083 | ||||||
UHDRS Part I (Motor assessment) | 0.09 | -0.024, 0.205 | 0.12 | ||||||
UHDRS Part IV-A (Functional assessment) | -0.248 | -0.634, 0.139 | 0.204 | ||||||
UHDRS Part IV-B (Independence scale) | -0.154 | -0.261, -0.047 | 0.006 |
-0.174 | -0.3, -0.048 | 0.008 |
|||
UHDRS Part IV-C (Functional capacity) | -1.082 | -1.751, -0.414 | 0.002 |
-1.128 | -1.883, -0.373 | 0.004 |
Characteristics | Multivariable (Covariables: Independence scale, onset age, disease duration, CAG repeat number) |
Multivariable (Covariables: Functional capacity, onset age, disease duration, CAG repeat number) |
||||
---|---|---|---|---|---|---|
B | 95% CI | p value | B | 95% CI | p value | |
Caregiver age | 0.113 | -0.163, 0.389 | 0.411 | 0.085 | -0.194, 0.363 | 0.541 |
Caregiving duration | 1.18 | -0.084, 2.445 | 0.066 | 1.089 | -0.029, 2.207 | 0.056 |
Blood relation | 0.541 | -6.398, 7.479 | 0.875 | 0.635 | -6.154, 7.424 | 0.85 |
Years of schooling | -1.517 | -5.251, 2.216 | 0.415 | -1.582 | -5.223, 2.06 | 0.384 |
Marital status | 3.716 | -6.187, 13.62 | 0.451 | 3.717 | -5.96, 13.393 | 0.44 |
Data are shown as the mean ± standard deviation or frequency (percentage). HD, Huntington’s disease; CAG, cytosine-adenine-guanine; MoCA, Montreal Cognitive Assessment; MMSE, Mini-Mental State Examination; BDI, Beck Depression Inventory; GDepS, Geriatric Depression Scale; NPI, Neuropsychiatric Inventory; UHDRS, Unified Huntington’s Disease Rating Scale; SF, Shoulson–Fahn.
Data are shown as the mean ± standard deviation or number (percentage). two caregivers did not report their caregiving duration. HD, Huntington’s disease; ZBI-12, 12-item Zarit Burden Interview.
Data are shown as the mean ± standard deviation or number (percentage). statistically significant variables in the analysis. ZBI-12, 12-item Zarit Burden Interview; CAG, cytosine-adenine-guanine; MoCA, Montreal Cognitive Assessment; MMSE, Mini-Mental State Examination; BDI, Beck Depression Inventory; GDepS, Geriatric Depression Scale; NPI, Neuropsychiatric Inventory; UHDRS, Unified Huntington’s Disease Rating Scale; SF, Shoulson–Fahn.
statistically significant variables in the analysis. ZBI-12, 12-item Zarit Burden Interview, CAG, cytosine-adenine-guanine; CI, confidence interval; MoCA, Montreal Cognitive Assessment; MMSE, Mini-Mental State Examination; BDI, Beck Depression Inventory; GDepS, Geriatric Depression Scale; NPI, Neuropsychiatric Inventory; UHDRS, Unified Huntington’s Disease Rating Scale.
ZBI-12, 12-item Zarit Burden Interview; CAG, cytosine-adenine-guanine; CI, confidence interval.
Manho Kim
March 31, 2024