Displaying publications 41 - 60 of 72 in total

Abstract:
Sort:
  1. Ong TL, Goh KJ, Shahrizaila N, Wong KT, Tan CY
    Neurol India, 2019 12 21;67(6):1532-1535.
    PMID: 31857554 DOI: 10.4103/0028-3886.273621
    Distal acquired demyelinating symmetric neuropathy (DADS) is a variant of chronic inflammatory demyelinating polyneuropathy (CIDP) characterized by symmetrical, distal, sensory or sensorimotor involvement. DADS with M-protein (DADS-M) is less responsive to immunotherapy compared to those without M-protein (DADS-I). We report a case of DADS-I with severe clinical presentation viz. early hand involvement with marked wasting, inexcitable peripheral nerves on neurophysiology and poor response to immunotherapy. Despite the unusual presentation, ancillary tests including cerebrospinal fluid analysis, nerve biopsy and nerve ultrasound were supportive of an inflammatory demyelinating polyneuropathy. This case demonstrated the heterogeneity of the disorder and expands the clinical spectrum of DADS neuropathy.
  2. 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.
  3. Toh TH, Abdul-Aziz NA, Yahya MA, Goh KJ, Loh EC, Capelle DP, et al.
    Clin Neurophysiol, 2021 10;132(10):2722-2728.
    PMID: 34312065 DOI: 10.1016/j.clinph.2021.05.034
    OBJECTIVE: We aimed to develop a model to predict amyotrophic lateral sclerosis (ALS) disease progression based on clinical and neuromuscular ultrasound (NMUS) parameters.

    METHODS: ALS patients were prospectively recruited. Muscle fasciculation (≥2 over 30-seconds, examined in biceps brachii-brachialis (BB), brachioradialis, tibialis anterior and vastus medialis) and nerve cross-sectional area (CSA) (median, ulnar, tibial, fibular nerve) were evaluated through NMUS. Ultrasound parameters were correlated with clinical data, including revised ALS Functional Rating Scale (ALSFRS-R) progression at one year. A predictive model was constructed to differentiate fast progressors (ALSFRS-R decline ≥ 1/month) from non-fast progressors.

    RESULTS: 40 ALS patients were recruited. Three parameters emerged as strong predictors of fast progressors: (i) ALSFRS-R slope at time of NMUS (p = 0.041), (ii) BB fasciculation count (p = 0.027) and (iii) proximal to distal median nerve CSA ratio 

  4. 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.
  5. Lee LY, Tan CY, Wong KT, Goh KJ, Shahrizaila N
    J Clin Neurosci, 2023 Jan;107:40-47.
    PMID: 36502780 DOI: 10.1016/j.jocn.2022.11.017
    BACKGROUND: With progress made in neurogenetics and neuroinflammation, the indications and value of nerve biopsies in the diagnostic evaluation of peripheral neuropathies are less clear. In this study, we aimed to evaluate the diagnostic yield of nerve biopsies in patients with peripheral neuropathies.

    METHODS: We performed a retrospective review of nerve biopsy reports from April 1998 to June 2021 of patients with peripheral neuropathies presenting to the Department of Pathology, University of Malaya Medical Centre, Kuala Lumpur, Malaysia. The diagnostic value of the biopsies was determined based on the criteria by Midroni and Bilbao as follows: contributive (essential and helpful), non-contributive and inadequate.

    RESULTS: A total of 107 nerve biopsies were analysed. Sixty-four (60 %) were males and the mean age was 52 years, ranging from 13 to 86 years. Ninety-four (88 %) were sural nerve biopsies; and only one patient (1 %) each had superficial peroneal and superficial radial nerve biopsy. The indications for the procedure were vasculitis (34 %), peripheral neuropathy of unknown aetiology (34 %), amyloidosis (14 %) and chronic inflammatory demyelinating polyneuropathy (10 %). In 68 (63 %) biopsies, the diagnostic value was contributive. Of these, 28 (26 %) were essential and 40 (37 %) were helpful. In contrast, 35 (33 %) biopsies were non-contributive and 4 (4 %) were inadequate. In 66 % (71/107) of cases, the nerve biopsy did not reveal a definite pathological diagnosis. However, in the remainder, a diagnosis of vasculitis (18 %, 19/107), followed by amyloidosis (10 %, 11/107) could be determined. For 32/71 biopsies with undetermined pathological diagnosis, neuropathy remained cryptogenic in 22 % (7/32) upon follow up.

    CONCLUSIONS: With the exception of vasculitis and amyloidosis, there is limited value in performing nerve biopsies in the evaluation of patients with peripheral neuropathy. However, this should be interpreted with caution as the number of patients with a clinical diagnosis of vasculitis and amyloidosis were relatively larger than patients with other diagnosis. Refinement and careful selection of cases are required to increase the diagnostic yield of nerve biopsy.

  6. Tai MLS, Goh KJ, Kadir KAA, Zakaria MI, Yap JF, Tan KS
    Singapore Med J, 2019 May;60(5):236-240.
    PMID: 30488077 DOI: 10.11622/smedj.2018150
    INTRODUCTION: Intravenous (IV) thrombolysis with alteplase (rt-PA) is effective in ischaemic stroke. The primary objective was to evaluate predictors of functional outcome in acute ischaemic stroke (AIS) patients treated with IV rt-PA. The secondary objective was to assess the outcome with the modified Rankin scale (mRS). We also examined the predictive value of the Totaled Health Risks in Vascular Events (THRIVE) score.

    METHODS: AIS patients treated with IV rt-PA from February 2012 to August 2016 were recruited. Demographic data, National Institutes of Health Stroke Scale (NIHSS) scores, timing and neuroradiological findings were recorded. Patients received a dose of 0.9 mg/kg IV rt-PA within 4.5 hours of symptom onset. mRS score was evaluated at discharge and three months, and good and poor clinical outcomes were defined as scores of 0-2 and 3-6, respectively. Baseline THRIVE scores were assessed.

    RESULTS: 36 patients received IV rt-PA. 20 (55.6%) patients had an mRS score of 0-2 at three months. Based on THRIVE score, 86.1% had a good or moderately good prognosis. On univariate analysis, poor outcome was associated with NIHSS score before rt-PA (p = 0.03), THRIVE score (p = 0.02), stroke subtype (p = 0.049) and diabetes mellitus (DM; p = 0.06). Multiple logistic regression showed that outcome was significantly associated with NIHSS score before rt-PA (p = 0.032) and DM (p = 0.010).

    CONCLUSION: Our newly developed Malaysian IV rt-PA service is safe, with similar outcomes to the published literature. Functional outcome after thrombolysis was associated with baseline NIHSS score and DM.

  7. Tan JY, Tan CY, Yahya MA, Shahrizaila N, Goh KJ
    Muscle Nerve, 2024 May;69(5):597-603.
    PMID: 38488306 DOI: 10.1002/mus.28081
    INTRODUCTION/AIMS: Muscle strength, functional status, and muscle enzymes are conventionally used to evaluate disease status in idiopathic inflammatory myopathies (IIM). This study aims to investigate the role of quantitative muscle ultrasound in evaluating disease status in IIM patients.

    METHODS: Patients with IIM, excluding inclusion body myositis, were recruited along with age- and sex-matched healthy controls (HC). All participants underwent muscle ultrasound and clinical assessments. Six limb muscles were unilaterally scanned using a standardized protocol, measuring muscle thickness (MT) and echo intensity (EI). Results were compared with HC, and correlations were made with outcome measures.

    RESULTS: Twenty IIM patients and 24 HC were recruited. The subtypes of IIM were dermatomyositis (6), necrotizing myositis (6), polymyositis (3), antisynthetase syndrome (3), and nonspecific myositis (2). Mean disease duration was 8.7 ± 6.9 years. There were no significant differences in demographics and anthropometrics between patients and controls. MT of rectus femoris in IIM patients was significantly lower than HC. Muscle EI of biceps brachii and vastus medialis in IIM patients were higher than HC. There were moderate correlations between MT of rectus femoris and modified Rankin Scale, Physician Global Activity Assessment, and Health Assessment Questionnaire, as well as between EI of biceps brachii and Manual Muscle Testing-8.

    DISCUSSION: Muscle ultrasound can detect proximal muscle atrophy and hyperechogenicity in patients with IIM. The findings correlate with clinical outcome measures, making it a potential tool for evaluating disease activity of patients with IIM in the late phase of the disease.

  8. Schee JP, Tan JS, Tan CY, Shahrizaila N, Wong KT, Goh KJ
    J Clin Neurol, 2024 Jul;20(4):422-430.
    PMID: 38951975 DOI: 10.3988/jcn.2023.0265
    BACKGROUND AND PURPOSE: Multiple acyl-CoA dehydrogenase deficiency (MADD) is an inherited disorder of fatty acid oxidation that causes lipid storage myopathy (LSM). This is the first report on MADD that describes the phenotypic and genetic features of a Malaysian cohort.

    METHODS: Among the >2,500 patients in a local muscle biopsy database, patients with LSM were identified and their genomic DNA were extracted from muscle samples and peripheral blood. All 13 exons of the electron-transfer flavoprotein dehydrogenase gene (ETFDH) were subsequently sequenced. Fifty controls were included to determine the prevalence of identified mutations in the normal population.

    RESULTS: Fourteen (82%) of the 17 LSM patients had MADD with ETFDH mutations. Twelve (86%) were Chinese and two were Malay sisters. Other unrelated patients reported that they had no relevant family history. Nine (64%) were females. The median age at onset was 18.5 years (interquartile range=16-37 years). All 14 demonstrated proximal limb weakness, elevated serum creatine kinase levels, and myopathic changes in electromyography. Three patients experienced a metabolic crisis at their presentation. Sanger sequencing of ETFDH revealed nine different variants/mutations, one of which was novel: c.998A>G (p.Y333C) in exon 9. Notably, 12 (86%) patients, including the 2 Malay sisters, carried a common c.250G>A (p.A84T) variant, consistent with the hotspot mutation reported in southern China. All of the patients responded well to riboflavin therapy.

    CONCLUSIONS: Most of our Malaysian cohort with LSM had late-onset, riboflavin-responsive MADD with ETFDH mutations, and they demonstrated phenotypic and genetic features similar to those of cases reported in southern China. Furthermore, we report a novel ETFDH mutation and possibly the first ever MADD patients of Malay descent.

  9. Tan JY, Tan CY, Gengadharan PN, Shahrizaila N, Goh KJ
    J Clin Neurol, 2024 Jul;20(4):412-421.
    PMID: 38951974 DOI: 10.3988/jcn.2023.0285
    BACKGROUND AND PURPOSE: Myasthenia gravis (MG) is clinically heterogeneous and can be classified into subgroups according to the clinical presentation, antibody status, age at onset, and thymic abnormalities. This study aimed to determine the clinical characteristics and outcomes of generalized MG (GMG) patients based on these subgroups.

    METHODS: Medical records of MG patients from 1976 to 2023 were reviewed retrospectively. Patients with pure ocular MG were excluded. Data on demographic, clinical characteristics, laboratory features, and outcomes were analyzed.

    RESULTS: This study included 120 GMG patients. There was a slight preponderance of female patients over male patients (male:female ratio=1:1.3), with the age at onset exhibiting a bimodal distribution. Female patients peaked at a lower age (21-30 years) whereas male patients peaked at a higher age (61-70 years). Most (92%, 105 of 114) patients had positive anti-acetylcholine receptor antibodies. Five patients were also tested for anti-muscle-specific tyrosine kinase antibodies, with two showing positivity. Thymectomy was performed in 62 (52%) patients, of which 30 had thymoma, 16 had thymic hyperplasia, 7 had an involuted thymus, and 6 had a normal thymus. There were significantly more female patients (68% vs. 45%, p=0.011) with early-onset disease (<50 years old) and thymic hyperplasia (33% vs. 0%, p<0.025). Most (71%) of the patients had a good outcome based on the Myasthenia Gravis Foundation of America postintervention status. GMG patients with early-onset disease had a significantly better outcome than patients with a late onset in univariate (58% vs. 37%, p=0.041) and multivariate (odds ratio=4.68, 95% confidence interval=1.17-18.64, p=0.029) analyses.

    CONCLUSIONS: Female patients with early-onset MG and thymic hyperplasia had significantly better outcomes, but only early-onset disease was independently associated with a good outcome. These findings are comparable with those of other studies.

  10. Tan SY, Tan CY, Yahya MA, Low SC, Shahrizaila N, Goh KJ
    Neurol Sci, 2024 Jan 25.
    PMID: 38270729 DOI: 10.1007/s10072-024-07340-y
    INTRODUCTION: There is an increasing need for a reproducible and sensitive outcome measure in patients with hereditary transthyretin amyloidosis (ATTRv) with polyneuropathy (PN) due to the emergence of disease modifying therapies. In the current study, we aimed to investigate the role of quantitative muscle ultrasound (QMUS) as a disease biomarker in ATTRv-PN.

    METHODS: Twenty genetically confirmed ATTRv amyloidosis patients (nine symptomatic, 11 pre-symptomatic) were enrolled prospectively between January to March 2023. Muscle ultrasound was performed on six muscles at standardized locations. QMUS parameters included muscle thickness (MT) and muscle echo intensity (EI). Twenty-five age- and sex-matched healthy controls were recruited for comparison. Significant QMUS parameters were correlated with clinical outcome measures.

    RESULTS: Muscle volume of first dorsal interosseus (FDI) muscle [measured as cross-sectional area (CSA)] was significantly lower in symptomatic patients compared to healthy controls and pre-symptomatic carriers (98.3 ± 58.0 vs. 184.4 ± 42.5 vs. 198.3 ± 56.8, p 

  11. Lau KF, Tan KS, Goh KJ, Ramli N, Tai SM
    Ann Acad Med Singap, 2019 Mar;48(3):109-111.
    PMID: 30997481
  12. Gengadharan PN, Ong WT, Tan JY, Shahrizaila N, Goh KJ, Tan CY
    Acta Neurol Belg, 2024 Dec 02.
    PMID: 39621280 DOI: 10.1007/s13760-024-02693-9
    INTRODUCTION: Myasthenia gravis (MG) can be classified according to clinical features into ocular MG (OMG) and generalised MG (GMG). However, OMG carries the risk of conversion to GMG. In this study, we aimed to determine the predictive factors for the secondary generalisation of OMG patients.

    METHODS: OMG patients followed-up in our hospital from January 1999 to November 2023 were retrospectively reviewed. Demographic and clinical characteristics data were collected from medical records. OMG patients with follow-up of

  13. Abdul Aziz NA, Toh TH, Loh EC, Capelle DP, Goh KJ, Abdul Latif L, et al.
    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.
  14. 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.
  15. Tan AH, Toh TH, Low SC, Fong SL, Chong KK, Lee KW, et al.
    J Mov Disord, 2018 Sep;11(3):149-151.
    PMID: 30086616 DOI: 10.14802/jmd.18017
  16. Yaiw KC, Crameri G, Wang L, Chong HT, Chua KB, Tan CT, et al.
    J Infect Dis, 2007 Sep 15;196(6):884-6.
    PMID: 17703419
    Tioman virus, a relatively new paramyxovirus, was isolated from fruit bats (Pteropus species) on Tioman Island, Malaysia, in 2001. The objective of this study was to determine the prevalence of antibodies to T. virus in island inhabitants, by use of comparative ELISA and serum neutralization assays. Of the 169 human sera analyzed, 5 (approximately 3.0%) were positive for T. virus, by comparative ELISA. Of these 5 sera, 3 (1.8% of the total) had neutralizing antibodies against T. virus, suggesting previous infection of this study population by this virus or a similar virus.
  17. Tai ML, Norhatta N, Goh KJ, Moy FM, Sujarita R, Asraff AA, et al.
    PLoS One, 2015;10(1):e0115838.
    PMID: 25629323 DOI: 10.1371/journal.pone.0115838
    BACKGROUND: Dyspepsia and headache frequently co-exist, but the clinical implication of this association is uncertain. We planned to examine the prevalence and impact of dyspepsia in adults with headache.
    METHODS: A cross-sectional study was conducted in a secondary care setting. Clinical, psychological and health-related quality of life (HRQOL) data were compared between subjects with headache and controls (non-headache subjects). The impact of dyspepsia was analysed further in subjects with headache alone.
    RESULTS: 280 subjects (93 cases with headache and 187 matched controls) were recruited. The following baseline characteristics of subjects were as follows: mean age 45.0 ± 17.3 years, 57.0% females and ethnic distribution-Malaysian = 45 (48.4%), Chinese n = 24 (25.8%) and Indians n = 24 (25.8%). Headache sub-types among cases with headache were as follows: tension-type headache (TTH) n = 53 (57.0%) and migraine n = 40 (43.0%). Dyspepsia was more prevalent in cases with headache compared to controls (25.8% vs 12.8%, p = 0.011), and headache was independently associated with dyspepsia (OR 2.75, 95% CI 1.39-5.43). Among cases with headache, there was a trend towards a higher prevalence of dyspepsia in those with migraine (27.5%) compared to TTH (24.5%). Subjects with headache and dyspepsia, compared to those with headache alone, had a greater severity of headache symptoms (63.67 ± 22.85 mm vs 51.20 ± 24.0 mm VAS, p = 0.029). Overall HRQOL scores were lower in headache subjects with dyspepsia (EQ-5D summary score 0.82 ± 0.18 vs 0.90 ± 0.16, p = 0.037 and EQ-5D VAS 62.08 ± 17.50 mm vs 72.62 ± 18.85 mm, p = 0.018), compared to those without dyspepsia.
    CONCLUSION: Dyspepsia is associated with more severe headache symptoms and results in a lower HRQOL in patients with headache.
Filters
Contact Us

Please provide feedback to Administrator (afdal@afpm.org.my)

External Links