Displaying publications 1 - 20 of 90 in total

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  1. Tan JSH, Lee S, Hiew FL
    eNeurologicalSci, 2021 Mar;22:100321.
    PMID: 33553704 DOI: 10.1016/j.ensci.2021.100321
    Amyotrophic lateral sclerosis (ALS) is characterized by progressive onset motor deficits with heterogenous presentations ranging from dysarthria to foot drop. Approximately 20% of the patients present with focal bulbar symptoms, in which some may remain restricted to bulbar region (isolated bulbar palsy), and the remaining eventually spreads to involve other body regions (classical ALS). Without accompanying upper and lower motor neurons signs elsewhere, differential diagnoses for isolated bulbar symptoms are extensive, include ALS variants as well as potentially treatable mimics. Therefore, it is important to take heed on every possible aetiology that may disrupt the hypoglossal nucleus, nerve, or lingual muscle itself. Herein, we illustrated a rare presentation of Group A basilar invagination, which mimicked bulbar-onset ALS.
    Matched MeSH terms: Amyotrophic Lateral Sclerosis
  2. Ghareghani M, Ghanbari A, Eid A, Shaito A, Mohamed W, Mondello S, et al.
    Transl Neurosci, 2021 Jan 01;12(1):164-189.
    PMID: 34046214 DOI: 10.1515/tnsci-2020-0169
    Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system (CNS) in which activated immune cells attack the CNS and cause inflammation and demyelination. While the etiology of MS is still largely unknown, the interaction between hormones and the immune system plays a role in disease progression, but the mechanisms by which this occurs are incompletely understood. Several in vitro and in vivo experimental, but also clinical studies, have addressed the possible role of the endocrine system in susceptibility and severity of autoimmune diseases. Although there are several demyelinating models, experimental autoimmune encephalomyelitis (EAE) is the oldest and most commonly used model for MS in laboratory animals which enables researchers to translate their findings from EAE into human. Evidences imply that there is great heterogeneity in the susceptibility to the induction, the method of induction, and the response to various immunological or pharmacological interventions, which led to conflicting results on the role of specific hormones in the EAE model. In this review, we address the role of endocrine system in EAE model to provide a comprehensive view and a better understanding of the interactions between the endocrine and the immune systems in various models of EAE, to open up a ground for further detailed studies in this field by considering and comparing the results and models used in previous studies.
    Matched MeSH terms: Multiple Sclerosis
  3. Young Chuah Y, Yeh Lee Y
    Turk J Gastroenterol, 2023 Aug;34(8):890-891.
    PMID: 37434401 DOI: 10.5152/tjg.2023.23208
    Matched MeSH terms: Sclerosis/chemically induced
  4. Chong HT, Tan CT
    Med J Malaysia, 2008 Dec;63(5):356-61.
    PMID: 19803290 MyJurnal
    Multiple sclerosis, although a rare disease in Asia, often presents significant diagnostic challenges to clinicians. There has been rapid advancement in the understanding of the underlying genetic influence, pathophysiology, investigation and treatment recently. This paper reviewed the latest development of various aspects of the disease and examined the differences between the manifestations of Asian and Western patients. The implications of these differences to investigation and treatment were also touched upon.
    Matched MeSH terms: Multiple Sclerosis/diagnosis; Multiple Sclerosis/ethnology*; Multiple Sclerosis/therapy
  5. Ram SP, Malik AS
    Med J Malaysia, 1994 Mar;49(1):96-9.
    PMID: 8058000
    Three children with cardiac tumors are described: a 12-year-old female child who had left atrial myxoma, and two males having rhabdomyoma of the right ventricle associated with tuberous sclerosis. The child with left atrial myxoma was symptomatic and the tumour was subsequently excised. The other two children with rhabdomyoma were managed conservatively.
    Matched MeSH terms: Tuberous Sclerosis/complications*
  6. Malik AS, Hussin ZA, Shriwas SR, Kasim ZM
    Med J Malaysia, 1994 Dec;49(4):375-84.
    PMID: 7674974
    This paper reviews the subject of tuberous sclerosis complex and presents data in eight cases of this condition, admitted to Hospital Universiti Sains Malaysia over a period of 8.5 years. The average age at presentation was 53 months. Seizures were the most common presenting feature. Male to female ratio was 3:1. Family history was present in four patients. All of the patients had one or more skin lesions. Six of them had retinal in six patients. Two patients had renal angiomyolipomas. A high index of hamartomas. Cardiac tumours were found in two patients. Multiple subependymal hamartomas were detected in six patients. Two patients had renal angiomyolipomas. A high index of suspicion and a detailed physical examination is required to diagnose this rare disorder.
    Matched MeSH terms: Tuberous Sclerosis/complications*
  7. Tan CT
    Med J Malaysia, 1994 Mar;49(1):68-73.
    PMID: 8057994
    This is a study of 13 Malaysian patients with clinically definite Multiple sclerosis (MS) subjected to a hot bath test with VEPs, BAEPs, median nerve SSEPs before and after heating. Five patients (38%) developed neurological changes with the rise in body temperature. There was an average of 0.46 new sign per patient. Four patients had motor disturbances attributed mainly to aggravation of spinal cord dysfunction. Two patients had additional visual deterioration, one patient has associated VEP change. This study shows that though Uhthoff's phenomenon has not been noted in the reports of Asian MS patients, when subjected to rigorous testing, Asian MS patients also show sensitivity to body temperature change. However, the percentage of positivity of the hot bath test is much lower than that reported for Caucasians. Thus this study shows further differences between Asian and Caucasian MS patients.
    Matched MeSH terms: Multiple Sclerosis/diagnosis*; Multiple Sclerosis/genetics; Multiple Sclerosis/epidemiology; Multiple Sclerosis/physiopathology*
  8. Muthusamy E, Tan CT
    Med J Malaysia, 1988 Jun;43(2):170-2.
    PMID: 3237134
    Matched MeSH terms: Multiple Sclerosis/diagnosis*; Multiple Sclerosis/physiopathology
  9. Segasothy M, Yi AA, Mohamed S
    Med J Malaysia, 1983 Jun;38(2):94-7.
    PMID: 6621453
    Tuberous sclerosis is a disease with a well known association with renal masses, both cysts and angiomyolipomas. Reported here is a case of a 26 year old woman who had, in addition to angiomyolipomas of the kidneys, adenoma sebaceum, paraungual fibromas, shagreen patches and cerebral tubers.
    Matched MeSH terms: Tuberous Sclerosis/complications*
  10. Tay CH, Jek WT
    Med J Malaya, 1972 Jun;26(4):272-7.
    PMID: 5069417
    Matched MeSH terms: Amyotrophic Lateral Sclerosis
  11. Arumugasamy N
    Med J Malaya, 1969 Sep;24(1):45-8.
    PMID: 4243843
    Matched MeSH terms: Multiple Sclerosis/pathology*
  12. Alturkustani M, Bahakeem B, Zhang Q, Ang LC
    Malays J Pathol, 2020 Aug;42(2):187-194.
    PMID: 32860370
    INTRODUCTION: Multiple sclerosis (MS) has variable clinical presentations, prognoses, pathogeneses, and pathological patterns. We conducted a pathological review of acute MS-associated lesions that focused on the degree of axonal injury, myelin loss, and glial reaction to determine whether the observed demyelination was of the primary or secondary type.

    MATERIALS AND METHODS: After searching the records for a 15-year period at the London Health Sciences Centre Pathology Department, we identified 8 cases of surgical acute lesion biopsies in which clinical MS diagnoses were made before or after the biopsy.

    RESULTS: The white matter pathologies in these cases could be sorted into 3 morphological patterns. The first pattern, which represents typical demyelinated plaques, was observed in 4 cases and was characterised by nearly complete demyelination accompanied by variable degrees of axon preservation and axonal swelling. The second pattern was observed in 3 cases and was characterised by demyelinating lesions containing variable numbers of myelinated axons mixed with a few demyelinated axons and variable numbers of axonal swellings. The myelinated axons ranged from scattered fibres to bands of variable thickness, and the demyelination was a mixture of primary and secondary demyelination. The third pattern was observed in 1 case and was characterised by well-demarcated areas of reduced myelin staining and numerous apoptotic nuclei. Axonal staining revealed many fragmented axons with reduced myelin staining but no definitely demyelinated axons.

    CONCLUSIONS: This report shows that the predominant pathology underlying acute MS-related lesions is not limited to demyelination but can include axonal degeneration alone or in combination with primary demyelination which reflect different pathogenesis for these acute lesions.

    Matched MeSH terms: Multiple Sclerosis/pathology*
  13. Chong PN, Sangu M, Huat TJ, Reza F, Begum T, Yusoff AAM, et al.
    Malays J Med Sci, 2018 Nov;25(6):28-45.
    PMID: 30914877 MyJurnal DOI: 10.21315/mjms2018.25.6.4
    Background: Following brain injury, development of hippocampal sclerosis often led to the temporal lobe epilepsy which is sometimes resistant to common anti-epileptic drugs. Cellular and molecular changes underlying epileptogenesis in animal models were studied, however, the underlying mechanisms of kainic acid (KA) mediated neuronal damage in rat hippocampal neuron cell culture alone has not been elucidated yet.

    Methods: Embryonic day 18 (E-18) rat hippocampus neurons were cultured with poly-L-lysine coated glass coverslips. Following optimisation, KA (0.5 μM), a chemoconvulsant agent, was administered at three different time-points (30, 60 and 90 min) to induce seizure in rat hippocampal neuronal cell culture. We examined cell viability, neurite outgrowth density and immunoreactivity of the hippocampus neuron culture by measuring brain derived neurotrophic factor (BDNF), γ-amino butyric acid A (GABAA) subunit α-1 (GABRA1), tyrosine receptor kinase B (TrkB), and inositol trisphosphate receptor (IP3R/IP3) levels.

    Results: The results revealed significantly decreased and increased immunoreactivity changes in TrkB (a BDNF receptor) and IP3R, respectively, at 60 min time point.

    Conclusion: The current findings suggest that TrkB and IP3 could have a neuroprotective role which could be a potential pharmacological target for anti-epilepsy drugs.

    Matched MeSH terms: Sclerosis
  14. Ralot TK, Singh R, Bafna C, Rajesh S, Singh S
    Malays J Med Sci, 2017 Aug;24(4):106-110.
    PMID: 28951696 DOI: 10.21315/mjms2017.24.4.13
    A female patient aged 48 years presented with sub-acute onset of weakness in right upper and lower limb over the past one month and numbness over left side of body below neck level. Multiple sclerosis (MS) presenting as Brown-Séquard syndrome is very rare. We present a case of hemicord myelitis which presented as Brown-Séquard syndrome as a first manifestation, which was later diagnosed as MS during subsequent relapses.
    Matched MeSH terms: Multiple Sclerosis
  15. Purohit B, Ganewatte E, Kollias SS
    Malays J Med Sci, 2016 Sep;23(5):91-95.
    PMID: 27904430
    Multiple sclerosis (MS) patients treated with natalizumab often face the uncommon but severe complication of developing progressive multifocal leukoencephalopathy (PML). PML may be further complicated by immune reconstitution inflammatory syndrome (IRIS) after the removal of the drug. Since both PML and IRIS are associated with high morbidity and mortality rates, early clinical and radiological diagnosis of these complications is of paramount importance. Here, we report a case of an adult male patient who was diagnosed with PML after receiving natalizumab therapy for 6 years for the treatment of MS. Upon cessation of natalizumab, he presented with a paradoxical worsening of clinical and radiological findings consistent with an inflammatory brain injury due to IRIS. He was treated with high dose corticosteroid therapy followed by a gradual improvement in clinical and imaging findings. This article illustrates the magnetic resonance imaging (MRI) features of natalizumab-associated PML-IRIS, along with a brief overview of its clinical features, complications and management strategies.
    Matched MeSH terms: Multiple Sclerosis
  16. Zahiri N, Abollahi I, Nabavi SM, Ehsani F, Arab AM, Shaw I, et al.
    Malays J Med Sci, 2017 Mar;24(1):69-80.
    PMID: 28381930 DOI: 10.21315/mjms2017.24.1.8
    BACKGROUND: Multiple sclerosis (MS) is the most widespread disabling neurological condition in young adults around the world. The purpose of this study was to investigate the impact of explicit information (EI) on motor-sequence learning in MS patients.

    METHODS: Thirty patients with relapsing-remitting MS (RRMS), age: 29.5 (SD = 5.6) years and 30 healthy gender-, age-, and education-matched control group participants, age: 28.8 (SD = 6.0) years, were recruited for this study. The participants in the healthy group were then randomly assigned into an EI (n = 15) group and a no-EI (n = 15) group. Similarly, the participants in the control group were then randomly assigned into EI (n = 15) and no-EI (n = 15) groups. The participants performed a serial reaction time (SRT) task and reaction times. A retention test was performed after 48 hours.

    RESULTS: All participants reduced their reaction times across acquisition (MS group: 46.4 (SD = 3.3) minutes, P < 0.001, and healthy group: 39.4 (SD = 3.3) minutes, P < 0.001). The findings for the within-participants effect of repeated measures of time were significant (F(5.06, 283.7) = 71.33. P < 0.001). These results indicate that the interaction between group and time was significant (F(5.06, 283.7) = 6.44. P < 0.001), which indicated that the reaction time in both groups was significantly changed between the MS and healthy groups across times (B1 to B10). The main effect of the group (MS and healthy) (F(1, 56) = 22.78. P < 0.001) and also the main effect of no-EI vs EI (F(1, 56) = 4.71. P < 0.001) were significant.

    CONCLUSION: This study demonstrated that that RRMS patients are capable of learning new skills, but the provision of EI prior to physical practice is deleterious to implicit learning. It is sufficient to educate MS patients on the aim and general content of the training and only to provide feedback at the end of the rehabilitative session.

    Matched MeSH terms: Multiple Sclerosis; Multiple Sclerosis, Relapsing-Remitting
  17. Sayuthi S, Tharakan J, Pieter MS, Salmah WM, Madhavan M, Tahir A, et al.
    Malays J Med Sci, 2009 Jan;16(1):39-43.
    PMID: 22589647 MyJurnal
    We present our preliminary experience in neuropsychological testing in epilepsy surgery patients to demonstrate how these tests contributed to decide the laterality of epileptic focus, and to assess the effect of surgery on patient's cognitive function and quality of life. Preoperative neuropsychological tests consisting of Wechsler Adult Intelligence Scale-III (WAIS) for IQ, Wechsler Memory Scale-III (WMS) for memory and patients' quality of life (QOLIE 31) were administered to refractory epilepsy patients under evaluation for surgical treatment. These tests were repeated one year after surgery and we studied any changes in trends. A total of seven patients were recruited in this study between July 2004 and July 2006. The aetiologies of refractory epilepsy were pure mesial temporal sclerosis (MTS) in five patients, dysembryogenic neuroepithelial tumour (DNET) in one and dual lesion of cavernous angioma with ipsilateral MTS in one. The preoperative neuropsychological tests were all in concordance to MRI finding, and showed good contralateral function; five lateralises to the right and two to the left. The post-operative Engel seizure count (median 8.00, IQR 7.00-8.75), general IQ (88 vs. 79), performance IQ (94 vs. 79), verbal memory (89 vs. 71), non-verbal memory (88 vs. 75) and QOLIE (53.14 vs. 44.71) were better compared to preoperative values. The verbal IQ (84 vs. 84) was unchanged. Neuropsychological tests are useful as ancillary investigations to determine the laterality of seizure focus and integrity of function in the contralateral temporal lobe. Following successful surgical treatment, there is a trend towards improvement in memory, IQ and quality of life scores in this small group of patients.
    Matched MeSH terms: Sclerosis
  18. Ismail NF, Rani AQ, Nik Abdul Malik NM, Boon Hock C, Mohd Azlan SN, Abdul Razak S, et al.
    J Mol Diagn, 2017 03;19(2):265-276.
    PMID: 28087349 DOI: 10.1016/j.jmoldx.2016.10.009
    Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder characterized by tumor growth in multiple organs and caused by mutations in either TSC1 or TSC2 genes. Because of their relatively large genomic sizes, absence of hotspots, and common type of mutations, mutation detection in TSC1 and TSC2 genes has been challenging. We devised a combination of multiple ligation-dependent probe amplification (MLPA) and amplicon sequencing (AS) to simplify the detection strategy, yet we come up with reasonably high detection rate. Thirty-four Malaysian patients diagnosed with TSC were referred to Human Genome Center, Universiti Sains Malaysia. We used a combination of MLPA to detect large copy number changes and AS to detect smaller mutations. TSC1 pathogenic or likely pathogenic mutations were found in 6 patients (18%) and TSC2 in 21 patients (62%), whereas 6 patients (18%) show no mutations and 1 patient (2%) showed only TSC2 missense variant with uncertain significance. Six of the mutations are novel. Our detection strategy costs 81% less and require 1 working week less than the conventional strategy. Confirmatory sequencing using Sanger method on a few representative mutations showed agreement with results of the AS. Combination of MLPA and Illumina MiSeq AS provides a simplified strategy and reasonably high detection rate for TSC1/TSC2 mutation, which suggested application of the strategies into clinical molecular diagnostics.
    Matched MeSH terms: Tuberous Sclerosis/diagnosis*; Tuberous Sclerosis/genetics*
  19. Said H, Razi Hadi A, Akmal SN, Lokman S
    J Laryngol Otol, 1988 Nov;102(11):1064-7.
    PMID: 3209947 DOI: 10.1017/s0022215100107297
    The term tumefactive fibroinflammatory lesion is used to describe an unusual fibrosclerosing disorder that behaves in a malignant way clinically but is histologically benign. Lesions in the head and neck are believed to be part of the broader fibrosclerosing syndrome which include pseudotumour of the orbit, Reidel's thyroiditis, sclerosing cholangitis and mediastinal fibrosis. Clinical diagnosis is often difficult and relies heavily on histopathology. Steroid therapy is suggested as the first line of management, while surgery or radiation therapy is reserved for failures.
    Matched MeSH terms: Sclerosis
  20. Qi Y, Montague P, Loney C, Campbell C, Shafie INF, Anderson TJ, et al.
    Eur J Neurosci, 2019 12;50(12):3896-3905.
    PMID: 31336405 DOI: 10.1111/ejn.14526
    Canine degenerative myelopathy (DM) is a progressive neurological disorder that may be considered to be a large animal model for specific forms of the fatal human disease, familial amyotrophic lateral sclerosis (fALS). DM is associated with a c118G>A mutation of the superoxide dismutase 1 (Sod1) gene, and a significant proportion of cases are inherited in an autosomal recessive manner in contrast to the largely, but not exclusively, dominant mode of inheritance in fALS. The consensus view is that these Sod1/SOD1 mutations result in a toxic gain of function but the mechanisms remain unclear. Here we used an in vitro neuroblastoma cell line transfection system to monitor wild-type and mutant forms of SOD1 fusion proteins containing either a Cherry or an enhanced green fluorescent protein (EGFP) tag. These fusion proteins retained SOD1 enzymatic activity on a native gel assay system. We demonstrate that SOD1 aggregate density is significantly higher in DM transfectants compared to wild-type. In addition, we show by co-immunoprecipitation and confocal microscopy, evidence for a potential interaction between wild-type and mutant forms of SOD1 in co-transfected cells. While in vitro studies have shown SOD1 heterodimer formation in fALS models, this is the first report for DM SOD1. Therefore, despite for the majority of cases there is a difference in the mode of inheritance between fALS and DM, a similar interaction between wild-type and mutant SOD1 forms can occur. Clarifying the role of SOD1 in DM may also be of benefit to understanding the role of SOD1 in fALS.
    Matched MeSH terms: Amyotrophic Lateral Sclerosis/genetics*
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