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Natural history and clinical features of ALS in Malaysia

Objective: Studies from multiethnic populations are rarely reported but do indicate differences in phenotypic presentation and survival in amyotrophic lateral sclerosis (ALS). In this study, we aimed to investigate the natural history of a cohort of ALS patients from a multiethnic population. Method...

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Main Authors:	Abdul Aziz, Nur Adilah, Toh, Tsun Haw, Goh, Khean Jin, Loh, Ee Chin, Capelle, David Paul, Abdul Latif, Lydia, Leow, Alex Hwong Ruey, Yim, Carolyn Chue Wai, Zainal Abidin, Mohd Fitry, Ruslan, Shairil Rahayu, Shahrizaila, Nortina
Format:	Article
Published:	Taylor & Francis 2021
Subjects:	R Medicine
Online Access:	http://eprints.um.edu.my/25977/ https://doi.org/10.1080/21678421.2020.1832121
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spelling	my.um.eprints.259772021-05-25T03:13:03Z http://eprints.um.edu.my/25977/ Natural history and clinical features of ALS in Malaysia Abdul Aziz, Nur Adilah Toh, Tsun Haw Goh, Khean Jin Loh, Ee Chin Capelle, David Paul Abdul Latif, Lydia Leow, Alex Hwong Ruey Yim, Carolyn Chue Wai Zainal Abidin, Mohd Fitry Ruslan, Shairil Rahayu Shahrizaila, Nortina R Medicine Objective: Studies from multiethnic populations are rarely reported but do indicate differences in phenotypic presentation and survival in amyotrophic lateral sclerosis (ALS). In this study, we aimed to investigate the natural history of a cohort of ALS patients from a multiethnic population. Methods: Data from ALS patients presenting to our multidisciplinary ALS clinic were prospectively collected from January 2015 to June 2020 as part of an ongoing hospital-based patient registry. Kaplan–Meier and Cox regression model were performed to identify potential prognostic factors. Results: A total of 144 ALS patients were recruited. We estimated the crude ALS incidence as 0.53 per 100,000 for 2019 but rises to 2 per 100,000 in patients aged 60–74 years. The majority of patients were of Chinese ethnicity (59.7%), followed by Malay (24.3%), Indian (11.1%), and others (4.9%). Malaysian Indians had a significantly steeper ALSFRS-R slope at diagnosis (p = 0.040). We found a worse prognosis in patients with bulbar-onset (HR = 1.915, p = 0.019), older age (HR = 1.052, p = 0.000), and who were fast-progressors (HR = 1.274, p = 0.000). In contrast, a higher body mass index (HR = 0.921, p = 0.007) and a longer time to diagnosis (HR = 0.967, p = 0.006), noninvasive ventilation (HR = 0.820, p = 0.000) and percutaneous endoscopic gastrostomy insertion (HR = 0.823, p = 0.000) were associated with better survival. On multivariate analysis, diagnostic delay and slow disease progression were associated with better survival. Conclusions: In our cohort, diagnostic delay and a slow disease progression were significantly associated with better survival in ALS. We also found ethnic variation with Chinese preponderance and more rapid disease progression in patients of Indian descent. © 2020 World Federation of Neurology on behalf of the Research Group on Motor Neuron Diseases. Taylor & Francis 2021 Article PeerReviewed Abdul Aziz, Nur Adilah and Toh, Tsun Haw and Goh, Khean Jin and Loh, Ee Chin and Capelle, David Paul and Abdul Latif, Lydia and Leow, Alex Hwong Ruey and Yim, Carolyn Chue Wai and Zainal Abidin, Mohd Fitry and Ruslan, Shairil Rahayu and Shahrizaila, Nortina (2021) Natural history and clinical features of ALS in Malaysia. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 22 (1-2). pp. 108-116. ISSN 2167-8421 https://doi.org/10.1080/21678421.2020.1832121 doi:10.1080/21678421.2020.1832121
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
spellingShingle	R Medicine Abdul Aziz, Nur Adilah Toh, Tsun Haw Goh, Khean Jin Loh, Ee Chin Capelle, David Paul Abdul Latif, Lydia Leow, Alex Hwong Ruey Yim, Carolyn Chue Wai Zainal Abidin, Mohd Fitry Ruslan, Shairil Rahayu Shahrizaila, Nortina Natural history and clinical features of ALS in Malaysia
description	Objective: Studies from multiethnic populations are rarely reported but do indicate differences in phenotypic presentation and survival in amyotrophic lateral sclerosis (ALS). In this study, we aimed to investigate the natural history of a cohort of ALS patients from a multiethnic population. Methods: Data from ALS patients presenting to our multidisciplinary ALS clinic were prospectively collected from January 2015 to June 2020 as part of an ongoing hospital-based patient registry. Kaplan–Meier and Cox regression model were performed to identify potential prognostic factors. Results: A total of 144 ALS patients were recruited. We estimated the crude ALS incidence as 0.53 per 100,000 for 2019 but rises to 2 per 100,000 in patients aged 60–74 years. The majority of patients were of Chinese ethnicity (59.7%), followed by Malay (24.3%), Indian (11.1%), and others (4.9%). Malaysian Indians had a significantly steeper ALSFRS-R slope at diagnosis (p = 0.040). We found a worse prognosis in patients with bulbar-onset (HR = 1.915, p = 0.019), older age (HR = 1.052, p = 0.000), and who were fast-progressors (HR = 1.274, p = 0.000). In contrast, a higher body mass index (HR = 0.921, p = 0.007) and a longer time to diagnosis (HR = 0.967, p = 0.006), noninvasive ventilation (HR = 0.820, p = 0.000) and percutaneous endoscopic gastrostomy insertion (HR = 0.823, p = 0.000) were associated with better survival. On multivariate analysis, diagnostic delay and slow disease progression were associated with better survival. Conclusions: In our cohort, diagnostic delay and a slow disease progression were significantly associated with better survival in ALS. We also found ethnic variation with Chinese preponderance and more rapid disease progression in patients of Indian descent. © 2020 World Federation of Neurology on behalf of the Research Group on Motor Neuron Diseases.
format	Article
author	Abdul Aziz, Nur Adilah Toh, Tsun Haw Goh, Khean Jin Loh, Ee Chin Capelle, David Paul Abdul Latif, Lydia Leow, Alex Hwong Ruey Yim, Carolyn Chue Wai Zainal Abidin, Mohd Fitry Ruslan, Shairil Rahayu Shahrizaila, Nortina
author_facet	Abdul Aziz, Nur Adilah Toh, Tsun Haw Goh, Khean Jin Loh, Ee Chin Capelle, David Paul Abdul Latif, Lydia Leow, Alex Hwong Ruey Yim, Carolyn Chue Wai Zainal Abidin, Mohd Fitry Ruslan, Shairil Rahayu Shahrizaila, Nortina
author_sort	Abdul Aziz, Nur Adilah
title	Natural history and clinical features of ALS in Malaysia
title_short	Natural history and clinical features of ALS in Malaysia
title_full	Natural history and clinical features of ALS in Malaysia
title_fullStr	Natural history and clinical features of ALS in Malaysia
title_full_unstemmed	Natural history and clinical features of ALS in Malaysia
title_sort	natural history and clinical features of als in malaysia
publisher	Taylor & Francis
publishDate	2021
url	http://eprints.um.edu.my/25977/ https://doi.org/10.1080/21678421.2020.1832121
_version_	1702170273281736704
score	13.211869

Natural history and clinical features of ALS in Malaysia

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