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New insights from a multi-ethnic Asian progressive supranuclear palsy cohort

Background: Progressive supranuclear palsy (PSP) is a rare, disabling, neurodegenerative disease, with few studies done in Asian populations.Methods: We prospectively characterized the clinical features and disease burden in a consecutively-recruited multi-ethnic Asian PSP cohort. Patients were exte...

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Main Authors: Lim, Shen -Yang, Closas, Alfand Marl F. Dy, Tan, Ai Huey, Lim, Jia Lun, Tan, Yi Jayne, Vijayanathan, Yuganthini, Tay, Yi Wen, Khalid, Raihanah Binti Abdul, Ng, Wai Keong, Kanesalingam, Ruban, -Martin, Pablo Martinez, Annuar, Azlina Ahmad, Lit, Lei Cheng, Foo, Jia Nee, Lim, Weng Khong, Ng, Adeline Su Lyn, Tan, Eng-King
Format: Article
Published: Elsevier 2023
Subjects:
R Medicine (General)
RA0421 Public health. Hygiene. Preventive Medicine
Online Access:http://eprints.um.edu.my/38783/
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spelling my.um.eprints.387832024-11-18T03:56:20Z http://eprints.um.edu.my/38783/ New insights from a multi-ethnic Asian progressive supranuclear palsy cohort Lim, Shen -Yang Closas, Alfand Marl F. Dy Tan, Ai Huey Lim, Jia Lun Tan, Yi Jayne Vijayanathan, Yuganthini Tay, Yi Wen Khalid, Raihanah Binti Abdul Ng, Wai Keong Kanesalingam, Ruban -Martin, Pablo Martinez Annuar, Azlina Ahmad Lit, Lei Cheng Foo, Jia Nee Lim, Weng Khong Ng, Adeline Su Lyn Tan, Eng-King R Medicine (General) RA0421 Public health. Hygiene. Preventive Medicine Background: Progressive supranuclear palsy (PSP) is a rare, disabling, neurodegenerative disease, with few studies done in Asian populations.Methods: We prospectively characterized the clinical features and disease burden in a consecutively-recruited multi-ethnic Asian PSP cohort. Patients were extensively phenotyped using the Movement Disorder Society (MDS-PSP) clinical diagnostic criteria and the PSP-Clinical Deficits Scale (PSP-CDS). Caregiver burden was measured using the modified Zarit Burden Interview (ZBI). Investigations (neuroimaging and genetic tests) were reviewed.Results: There were 104 patients (64.4% male; 67.3% Chinese, 21.2% Indians, 9.6% Malays), consisting of 48.1% Richardson syndrome (PSP-RS), 37.5% parkinsonian phenotype (PSP-P), and 10.6% progressive gait freezing phenotype (PSP-PGF). Mean age at motor onset was 66.3 +/- 7.7 years, with no significant differences between the PSP phenotypes. Interestingly, REM-sleep behaviour disorder (RBD) symptoms and visual hallucinations (considered rare in PSP) were reported in 23.5% and 22.8% of patients, respectively, and a family history of possible neurodegenerative or movement disorder in 20.4%. PSP-CDS scores were highest (worst) in PSP-RS; and correlated moderately with disease duration (rs = 0.45, P < 0.001) and weakly with caregiver burden (rs = 0.22, P = 0.029) in the overall cohort. Three of 48 (6.3%) patients who had whole-exome sequencing harboured pathogenic/likely pathogenic GBA variants. Conclusions: Significant heterogeneity in clinical features and disease burden, and high rates of RBD symptoms, visual hallucinations, and familial involvement were observed in this relatively large cohort. Our findings highlight important considerations when assessing Asian patients, and provide further support for the notion of overlapping neurobiology between PSP and Lewy body disorders. Elsevier 2023-03 Article PeerReviewed Lim, Shen -Yang and Closas, Alfand Marl F. Dy and Tan, Ai Huey and Lim, Jia Lun and Tan, Yi Jayne and Vijayanathan, Yuganthini and Tay, Yi Wen and Khalid, Raihanah Binti Abdul and Ng, Wai Keong and Kanesalingam, Ruban and -Martin, Pablo Martinez and Annuar, Azlina Ahmad and Lit, Lei Cheng and Foo, Jia Nee and Lim, Weng Khong and Ng, Adeline Su Lyn and Tan, Eng-King (2023) New insights from a multi-ethnic Asian progressive supranuclear palsy cohort. Parkinsonism & Related Disorders, 108. ISSN 1353-8020, DOI https://doi.org/10.1016/j.parkreldis.2023.105296 <https://doi.org/10.1016/j.parkreldis.2023.105296>. 10.1016/j.parkreldis.2023.105296
institution Universiti Malaya
building UM Library
collection Institutional Repository
continent Asia
country Malaysia
content_provider Universiti Malaya
content_source UM Research Repository
url_provider http://eprints.um.edu.my/
topic R Medicine (General)
RA0421 Public health. Hygiene. Preventive Medicine
spellingShingle R Medicine (General)
RA0421 Public health. Hygiene. Preventive Medicine
Lim, Shen -Yang
Closas, Alfand Marl F. Dy
Tan, Ai Huey
Lim, Jia Lun
Tan, Yi Jayne
Vijayanathan, Yuganthini
Tay, Yi Wen
Khalid, Raihanah Binti Abdul
Ng, Wai Keong
Kanesalingam, Ruban
-Martin, Pablo Martinez
Annuar, Azlina Ahmad
Lit, Lei Cheng
Foo, Jia Nee
Lim, Weng Khong
Ng, Adeline Su Lyn
Tan, Eng-King
New insights from a multi-ethnic Asian progressive supranuclear palsy cohort
description Background: Progressive supranuclear palsy (PSP) is a rare, disabling, neurodegenerative disease, with few studies done in Asian populations.Methods: We prospectively characterized the clinical features and disease burden in a consecutively-recruited multi-ethnic Asian PSP cohort. Patients were extensively phenotyped using the Movement Disorder Society (MDS-PSP) clinical diagnostic criteria and the PSP-Clinical Deficits Scale (PSP-CDS). Caregiver burden was measured using the modified Zarit Burden Interview (ZBI). Investigations (neuroimaging and genetic tests) were reviewed.Results: There were 104 patients (64.4% male; 67.3% Chinese, 21.2% Indians, 9.6% Malays), consisting of 48.1% Richardson syndrome (PSP-RS), 37.5% parkinsonian phenotype (PSP-P), and 10.6% progressive gait freezing phenotype (PSP-PGF). Mean age at motor onset was 66.3 +/- 7.7 years, with no significant differences between the PSP phenotypes. Interestingly, REM-sleep behaviour disorder (RBD) symptoms and visual hallucinations (considered rare in PSP) were reported in 23.5% and 22.8% of patients, respectively, and a family history of possible neurodegenerative or movement disorder in 20.4%. PSP-CDS scores were highest (worst) in PSP-RS; and correlated moderately with disease duration (rs = 0.45, P < 0.001) and weakly with caregiver burden (rs = 0.22, P = 0.029) in the overall cohort. Three of 48 (6.3%) patients who had whole-exome sequencing harboured pathogenic/likely pathogenic GBA variants. Conclusions: Significant heterogeneity in clinical features and disease burden, and high rates of RBD symptoms, visual hallucinations, and familial involvement were observed in this relatively large cohort. Our findings highlight important considerations when assessing Asian patients, and provide further support for the notion of overlapping neurobiology between PSP and Lewy body disorders.
format Article
author Lim, Shen -Yang
Closas, Alfand Marl F. Dy
Tan, Ai Huey
Lim, Jia Lun
Tan, Yi Jayne
Vijayanathan, Yuganthini
Tay, Yi Wen
Khalid, Raihanah Binti Abdul
Ng, Wai Keong
Kanesalingam, Ruban
-Martin, Pablo Martinez
Annuar, Azlina Ahmad
Lit, Lei Cheng
Foo, Jia Nee
Lim, Weng Khong
Ng, Adeline Su Lyn
Tan, Eng-King
author_facet Lim, Shen -Yang
Closas, Alfand Marl F. Dy
Tan, Ai Huey
Lim, Jia Lun
Tan, Yi Jayne
Vijayanathan, Yuganthini
Tay, Yi Wen
Khalid, Raihanah Binti Abdul
Ng, Wai Keong
Kanesalingam, Ruban
-Martin, Pablo Martinez
Annuar, Azlina Ahmad
Lit, Lei Cheng
Foo, Jia Nee
Lim, Weng Khong
Ng, Adeline Su Lyn
Tan, Eng-King
author_sort Lim, Shen -Yang
title New insights from a multi-ethnic Asian progressive supranuclear palsy cohort
title_short New insights from a multi-ethnic Asian progressive supranuclear palsy cohort
title_full New insights from a multi-ethnic Asian progressive supranuclear palsy cohort
title_fullStr New insights from a multi-ethnic Asian progressive supranuclear palsy cohort
title_full_unstemmed New insights from a multi-ethnic Asian progressive supranuclear palsy cohort
title_sort new insights from a multi-ethnic asian progressive supranuclear palsy cohort
publisher Elsevier
publishDate 2023
url http://eprints.um.edu.my/38783/
_version_ 1817841938122932224
score 13.222552

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