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The utility of ALS staging systems in a multi-ethnic patient cohort

Abdul Aziz NA, Toh TH, Loh EC, Capelle DP, Goh KJ, Abdul Latif L, et al.

Amyotroph Lateral Scler Frontotemporal Degener, 2021 08;22(5-6):341-349.
PMID: 33726578 DOI: 10.1080/21678421.2021.1893336

Objective: To compare two ALS staging systems, King's clinical staging and Milano-Torino (MiToS) functional staging, using prospective data from a multi-ethnic cohort of ALS patients. Methods: The stages of disease were determined prospectively based on existing definitions. The two systems were compared for timing of stages using box plots, correspondence using chi-square tests and association using Spearman's rank correlation. Results: The distribution of stages differed between the two systems. The proportions of disease stages of the King's staging system were more evenly distributed whereas in MiToS, there was greater weight seen at the later stages of disease. At the early stages, patients moved consecutively in the MiToS staging system but not in the King's staging system where patients tended to skip stages to reach later stages. Both systems had good correlation (Spearman's rho = 0.869) and the King's stage 4 most frequently corresponded to MiToS stage 2. Conclusion: We found the King's staging was helpful in determining the stages of disease burden, whereas both were helpful in determining the time to functional dependence with MiToS further refining the levels of dependence.
Natural history and clinical features of ALS in Malaysia

Abdul Aziz NA, Toh TH, Goh KJ, Loh EC, Capelle DP, Abdul Latif L, et al.

Amyotroph Lateral Scler Frontotemporal Degener, 2021 02;22(1-2):108-116.
PMID: 33084408 DOI: 10.1080/21678421.2020.1832121

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.
Fulltext Metabolic Profile of Scytalidium parasiticum-Ganoderma boninense Co-Cultures Revealed the Alkaloids, Flavonoids and Fatty Acids that Contribute to Anti-Ganoderma Activity

Ahmad R, Lim CK, Marzuki NF, Goh YK, Azizan KA, Goh YK, et al.

Molecules, 2020 Dec 16;25(24).
PMID: 33339375 DOI: 10.3390/molecules25245965

In solving the issue of basal stem rot diseases caused by Ganoderma, an investigation of Scytalidium parasiticum as a biological control agent that suppresses Ganoderma infection has gained our interest, as it is more environmentally friendly. Recently, the fungal co-cultivation has emerged as a promising method to discover novel antimicrobial metabolites. In this study, an established technique of co-culturing Scytalidium parasiticum and Ganoderma boninense was applied to produce and induce metabolites that have antifungal activity against G. boninense. The crude extract from the co-culture media was applied to a High Performance Liquid Chromatography (HPLC) preparative column to isolate the bioactive compounds, which were tested against G. boninense. The fractions that showed inhibition against G. boninense were sent for a Liquid Chromatography-Time of Flight-Mass Spectrometry (LC-TOF-MS) analysis to further identify the compounds that were responsible for the microbicidal activity. Interestingly, we found that eudistomin I, naringenin 7-O-beta-D-glucoside and penipanoid A, which were present in different abundances in all the active fractions, except in the control, could be the antimicrobial metabolites. In addition, the abundance of fatty acids, such as oleic acid and stearamide in the active fraction, also enhanced the antimicrobial activity. This comprehensive metabolomics study could be used as the basis for isolating biocontrol compounds to be applied in oil palm fields to combat a Ganoderma infection.
Fulltext Clinicopathological Features of Telbivudine-Associated Myopathy

Ambang T, Tan JS, Ong S, Wong KT, Goh KJ

PLoS One, 2016;11(9):e0162760.
PMID: 27611456 DOI: 10.1371/journal.pone.0162760

Telbivudine, a thymidine nucleoside analog, is a common therapeutic option for chronic hepatitis B infection. While raised serum creatine kinase is common, myopathy associated with telbivudine is rare. Reports on its myopathological features are few and immunohistochemical analyses of inflammatory cell infiltrates have not been previously described. We describe the clinical, myopathological and immunohistochemical features of four patients who developed myopathy after telbivudine therapy for chronic hepatitis B infection. All four patients presented with progressive proximal muscle weakness, elevation of serum creatine kinase and myopathic changes on electromyography. Muscle biopsies showed myofiber degeneration/necrosis, regeneration, and fibers with cytoplasmic bodies and cytochrome c oxidase deficiency. There was minimal inflammation associated with strong sarcolemmal overexpression of class I major histocompatibility complex (MHC class I). Upon withdrawal of telbivudine, muscle weakness improved in all patients and eventually completely resolved in three. In our series, telbivudine-associated myopathy is characterized by necrotizing myopathy which improved on drug withdrawal. Although the occasional loss of cytochrome c oxidase is consistent with mitochondrial toxicity, the overexpression of MHC class I in all patients could suggest an underlying immune-mediated mechanism which may warrant further investigation.
Fulltext Analysis of CTG repeat length variation in the DMPK gene in the general population and the molecular diagnosis of myotonic dystrophy type 1 in Malaysia

Ambrose KK, Ishak T, Lian LH, Goh KJ, Wong KT, Ahmad-Annuar A, et al.

BMJ Open, 2017 03 31;7(3):e010711.
PMID: 28363916 DOI: 10.1136/bmjopen-2015-010711

OBJECTIVE: The lack of epidemiological data and molecular diagnostic services in Malaysia has hampered the setting-up of a comprehensive management plan for patients with myotonic dystrophy type 1 (DM1), leading to delayed diagnosis, treatment and support for patients and families. The aim of this study was to estimate the prevalence of DM1 in the 3 major ethnic groups in Malaysia and evaluate the feasibility of a single tube triplet-primed PCR (TP-PCR) method for diagnosis of DM1 in Malaysia.
DESIGN, SETTING AND PARTICIPANTS: We used PCR to determine the size of CTG repeats in 377 individuals not known to be affected by DM and 11 DM1 suspected patients, recruited from a tertiary hospital in Kuala Lumpur. TP-PCR was performed on selected samples, followed by Southern blot hybridisation of PCR amplified fragments to confirm and estimate the size of CTG expansion.
OUTCOME MEASURES: The number of individuals not known to be affected by DM with (CTG)>18 was determined according to ethnic group and as a whole population. The χ2 test was performed to compare the distribution of (CTG)>18 with 12 other populations. Additionally, the accuracy of TP-PCR in detecting CTG expansion in 11 patients with DM1 was determined by comparing the results with that from Southern blot hybridisation.
RESULTS: Of the 754 chromosomes studied, (CTG)>18 frequency of 3.60%, 1.57% and 4.00% in the Malay, Chinese and Indian subpopulations, respectively, was detected, showing similarities to data from Thai, Taiwanese and Kuwaiti populations. We also successfully detected CTG expansions in 9 patients using the TP-PCR method followed by the estimation of CTG expansion size via Southern blot hybridisation.
CONCLUSIONS: The results show a low DM1 prevalence in Malaysia with the possibility of underdiagnosis and demonstrates the feasibility of using a clinical and TP-PCR-based approach for rapid and cost-effective DM1 diagnosis in developing countries.
Fulltext Deregulation of microRNAs in blood and skeletal muscles of myotonic dystrophy type 1 patients

Ambrose KK, Ishak T, Lian LH, Goh KJ, Wong KT, Ahmad-Annuar A, et al.

Neurol India, 2017 5 11;65(3):512-517.
PMID: 28488611 DOI: 10.4103/neuroindia.NI_237_16

INTRODUCTION: MicroRNAs (miRNAs) are short RNA molecules of approximately 22 nucleotides that function as post-transcriptional regulators of gene expression. They are expressed in a tissue-specific manner and show different expression patterns in development and disease; hence, they can potentially act as disease-specific biomarkers. Several miRNAs have been shown to be deregulated in plasma and skeletal muscles of myotonic dystrophy type 1 (DM1) patients.
METHODS: We evaluated the expression patterns of 11 candidate miRNAs using quantitative real-time PCR in whole blood (n = 10) and muscle biopsy samples (n = 9) of DM1 patients, and compared them to those of normal control samples (whole blood, n = 10; muscle, n = 9).
RESULTS: In DM1 whole blood, miRNA-133a, -29b, and -33a were significantly upregulated, whereas miRNA-1, -133a, and -29c were significantly downregulated in the skeletal muscles compared to controls.
CONCLUSIONS: Our findings align to those reported in other studies and point towards pathways that potentially contribute toward pathogenesis in DM1. However, the currently available data is not sufficient for these miRNAs to be made DM1-specific biomarkers because they seem to be common to many muscle pathologies. Hence, they lack specificity, but reinforce the need for further exploration of DM1 biomarkers.
Absence of beta-amyloid deposition in the central nervous system of a transgenic mouse model of distal myopathy with rimmed vacuoles

Anada RP, Wong KT, Malicdan MC, Goh KJ, Hayashi Y, Nishino I, et al.

Amyloid, 2014 Jun;21(2):138-9.
PMID: 24601867 DOI: 10.3109/13506129.2014.889675
Fulltext Soil CO2 and CH4 fluxes from different forest types in tropical peat swamp forest

Busman NA, Melling L, Goh KJ, Imran Y, Sangok FE, Watanabe A

Sci Total Environ, 2023 Feb 01;858(Pt 2):159973.
PMID: 36347298 DOI: 10.1016/j.scitotenv.2022.159973

Information on temporal and spatial variations in soil greenhouse gas (GHG) fluxes from tropical peat forests is essential to predict the influence of climate change and estimate the effects of land use on global warming and the carbon (C) cycle. To obtain such basic information, soil carbon dioxide (CO2) and methane (CH4) fluxes, together with soil physicochemical properties and environmental variables, were measured at three major forest types in the Maludam National Park, Sarawak, Malaysia, for eight years, and their relationships were analyzed. Annual soil CO2 fluxes ranged from 860 to 1450 g C m⁻2 yr⁻1 without overall significant differences between the three forest sites, while soil CH4 fluxes, 1.2-10.8 g C m⁻2 yr⁻1, differed. Differences in GHG fluxes between dry and rainy seasons were not necessarily significant, corresponding to the extent of seasonal variation in groundwater level (GWL). The lack of significant differences in soil CO2 fluxes between the three sites could be attributed to set-off between the negative and positive effects of the decomposability of soil organic matter as estimated by pyrophosphate solubility index (PSI) and GWL. The impact of El-Niño on annual CO2 flux also varied between the sites. The variation in soil CH4 fluxes from the three sites was enhanced by variations in temperature, GWL, PSI, and soil iron (Fe) content. A positive correlation was observed between the annual CH4 flux and GWL at only one site, and the influence of soil properties was more pronounced at the site with the lowest GWL and the highest PSI. Variation in annual CH4 fluxes was controlled more strongly by temperature where GWL was the highest and GWL and plant growth fluctuations were the least. Inter-annual variations in soil CO2 and CH4 fluxes confirmed the importance of long-term monitoring of these at multiple sites supporting different forest types.
Fulltext Corrigendum to "Soil CO2 and CH4 fluxes from different forest types in tropical peat swamp forest" [Sci. Total Environ. 858 (2023) 159973]

Busman NA, Melling L, Goh KJ, Imran Y, Sangok FE, Watanabe A

Sci Total Environ, 2024 Feb 20;912:168867.
PMID: 38040577 DOI: 10.1016/j.scitotenv.2023.168867
Acute flaccid paralysis with chronic cough

Chai CH, Yuki N, Nor HM, Goh KJ, Shahrizaila N

Pract Neurol, 2012 Oct;12(5):328-31.
PMID: 22976064 DOI: 10.1136/practneurol-2011-000205
Idiopathic paroxysmal kinesigenic choreoathetosis: precipitation of attacks by vestibular stimulation

Chew NK, Tan CT, Goh KJ

J Clin Neurosci, 2002 Sep;9(5):604-5.
PMID: 12383430

A 24-year-old woman presented with a 3.5-year history of paroxysmal dystonia that was precipitated by sudden movement, especially when she started to walk. It was characterised by shrugging of shoulders, flexion of the neck and thoracic spine, and stiffness of the right leg followed by falls. Each attack lasted for less than 5min. Inadequate sleep and stress were exacerbating factors. There was no similar family history. Physical examination and investigations were normal. The following manoeuvres that caused vestibular stimulation precipitated attacks: turning her head from side to side while standing still, sitting still on a rotating chair and an ice-water caloric test. She had partial responses to phenytoin and levodopa, and a good response to haloperidol. Vestibular stimulation as a precipitating factor in paroxysmal kinesigenic choreoathetosis has not been reported previously.
Delayed post-irradiation bulbar palsy in nasopharyngeal carcinoma

Chew NK, Sim BF, Tan CT, Goh KJ, Ramli N, Umapathi P

Neurology, 2001 Aug 14;57(3):529-31.
PMID: 11502928

In a hospital series of 70 patients on follow-up after radiotherapy for nasopharyngeal carcinoma, 14 patients (20%) developed delayed post-irradiation bulbar palsy 1 to 18 years after radiotherapy (mean 5.5 years). Functional disability was moderate to severe. Three patients had aspiration pneumonia with one mortality. Post-irradiation bulbar palsy was a common complication and probably resulted from direct neuronal damage.
Treatment of acute Nipah encephalitis with ribavirin

Chong HT, Kamarulzaman A, Tan CT, Goh KJ, Thayaparan T, Kunjapan SR, et al.

Ann Neurol, 2001 Jun;49(6):810-3.
PMID: 11409437

Nipah virus, a newly identified paramyxovirus caused a severe outbreak of encephalitis in Malaysia with high fatalities. We report an open-label trial of ribavirin in 140 patients, with 54 patients who were managed prior to the availability of ribavirin or refused treatment as control. There were 45 deaths (32%) in the ribavirin arm; 29 deaths (54%) occurred in the control arm. This represents a 36% reduction in mortality (p = 0.011). There was no associated serious side effect. This study suggests that ribavirin is able to reduce the mortality of acute Nipah encephalitis.
The presence of Nipah virus in respiratory secretions and urine of patients during an outbreak of Nipah virus encephalitis in Malaysia

Chua KB, Lam SK, Goh KJ, Hooi PS, Ksiazek TG, Kamarulzaman A, et al.

J Infect, 2001 Jan;42(1):40-3.
PMID: 11243752

To study the excretion of Nipah virus in the upper respiratory secretions and urine of infected patients in relation to other clinical features.
Fatal encephalitis due to Nipah virus among pig-farmers in Malaysia

Chua KB, Goh KJ, Wong KT, Kamarulzaman A, Tan PS, Ksiazek TG, et al.

Lancet, 1999 Oct 9;354(9186):1257-9.
PMID: 10520635

Between February and April, 1999, an outbreak of viral encephalitis occurred among pig-farmers in Malaysia. We report findings for the first three patients who died.
High mortality in Nipah encephalitis is associated with presence of virus in cerebrospinal fluid

Chua KB, Lam SK, Tan CT, Hooi PS, Goh KJ, Chew NK, et al.

Ann Neurol, 2000 Nov;48(5):802-5.
PMID: 11079547

During the outbreak of Nipah virus encephalitis in Malaysia, stored cerebrospinal fluid (CSF) samples from 84 patients (27 fatal and 57 nonfatal cases) were cultured for the virus. The virus was isolated from 17 fatal cases and 1 nonfatal case. There were significant associations between CSF virus isolation and mortality as well as clinical features associated with poor prognosis. In addition, there was a positive linear correlation of CSF virus isolation with age. There was no significant association between CSF virus isolation and the character of the CSF, presence of Nipah-specific antibody in the serum or CSF, duration of illness before collection of samples, or sex or ethnicity of the patients. This study suggests that high viral replication in the central nervous system may be an important factor for high mortality.
Fulltext A survey on patients' disease perception and the impact of the COVID-19 pandemic on persons living with amyotrophic lateral sclerosis in Malaysia

Edgar S, Abdul-Aziz NA, Loh EC, Capelle D, Goh KJ, Latif LA, et al.

Neurodegener Dis Manag, 2021 08;11(4):307-314.
PMID: 34284643 DOI: 10.2217/nmt-2021-0004

Aim: To investigate the patients' perception of their disease, its management and the impact of the COVID-19 pandemic on persons living with amyotrophic lateral sclerosis (ALS) in Malaysia. Patients & methods: An online survey comprising 42 questions was conducted on ALS patients during the peak of the COVID-19 pandemic. Results: Responses were received from 37/60 (62%) participants with ALS directly or through their caregivers. During the COVID-19 pandemic, two-thirds of patients were negatively impacted by the sudden disruption to their hospital appointments, rehabilitation sessions and reduced social interactions. Conclusion: This study provided insight into patients' perception of their care and management of ALS in Malaysia which will facilitate in implementing changes that can improve care to persons living with this devastating illness.
Mutation analysis of SOD1, C9orf72, TARDBP and FUS genes in ethnically-diverse Malaysian patients with amyotrophic lateral sclerosis (ALS)

Edgar S, Ellis M, Abdul-Aziz NA, Goh KJ, Shahrizaila N, Kennerson ML, et al.

Neurobiol Aging, 2021 12;108:200-206.
PMID: 34404558 DOI: 10.1016/j.neurobiolaging.2021.07.008

Recent studies have identified SOD1, FUS, TARDBP and C9orf72 as major ALS-related genes in both European and Asian populations. However, significant differences exist in the mutation frequencies of these genes between various ancestral backgrounds. This study aims to identify the frequency of mutations in the common causative ALS genes in a multi-ethnic Malaysian cohort. We screened 101 Malaysian ALS patients including 3 familial and 98 sporadic cases for mutations in the coding regions of SOD1, FUS, and TARDBP by Sanger sequencing. The C9orf72 hexanucleotide repeat expansion was screened using the repeat-primed polymerase chain reaction assay. Mutations were found in 5.9% (6 of 101) of patients including 3.0% (3 of 101) of patients with the previously reported SOD1 missense mutations (p.V48A and p.N87S) and 3.0% (3 of 101) of patients with the C9orf72 repeat expansion. No mutations were found in the FUS and TARDBP genes. This study is the first to report the mutation frequency in an ethnically diverse Malaysian ALS population and warrants further investigation to reveal novel genes and disease pathways.
Longitudinal extensive transverse myelitis with cervical epidural haematoma following dengue virus infection

Fong CY, Hlaing CS, Tay CG, Abdul Kadir KA, Goh KJ, Ong LC

Eur. J. Paediatr. Neurol., 2016 May;20(3):449-53.
PMID: 26900103 DOI: 10.1016/j.ejpn.2016.01.012

Longitudinal extensive transverse myelitis associated with dengue infection is rare with no reported paediatric cases.
Bickerstaff's brainstem encephalitis with overlapping Guillain-Barré syndrome: Usefulness of sequential nerve conduction studies

Fong CY, Aung HWW, Khairani A, Gan CS, Shahrizaila N, Goh KJ

Brain Dev, 2018 Jun;40(6):507-511.
PMID: 29459060 DOI: 10.1016/j.braindev.2018.02.001

Bickerstaff's brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a rapidly ascending paralysis of all four limbs and disturbance of consciousness. The initial impression was BBE with overlapping GBS. This was supported by sequential nerve conduction study (NCS) findings compatible with an acute inflammatory demyelinating polyneuropathy (AIDP). He received intravenous pulse methylprednisolone, intravenous immunoglobulin and plasmapharesis with complete clinical recovery after 6 weeks of illness and improved NCS findings from week 16. This is the first case of paediatric BBE with overlapping GBS with an AIDP subtype of GBS. It expands the clinical spectrum of this condition in children. Our case highlights the importance of sequential NCS in paediatric BBE with overlapping GBS for accurate electrophysiological diagnosis and prognosis particularly if the first NCS findings are not informative.

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