Displaying publications 41 - 60 of 86 in total

Abstract:
Sort:
  1. Goh LY, Shahrom EE, Ganesan CC, Vethakkan SR, Goh KJ
    Neurology Asia, 2017;22(4):325-331.
    MyJurnal
    Objective: To determine prevalence and factors associated with neuropathic pain symptoms in a multiethnic cohort of Malaysian adult diabetic patients.
    Methods: This was aprospective cross-sectional observational study of hospital-based diabetic outpatients in Malaysia. Subjects were interviewed for their demographic data and medical history. The painDETECT questionnaire was used to screen for neuropathic pain symptoms and pain intensity was assessed using the numeric pain rating scale (NPRS). Neuropathy symptoms and signs were assessed using the Neuropathy Symptom Score (NSS) and Neuropathy Disability Score (NDS).
    Results:Of 242 patients,140 (58%) were women, with a mean age of 61 + 11.4 years (range 21 to 81). Ninety nine(40.9%) were Malay, 64 (26.4%) Chinese, 76 (31.4%) Indian and three (1.2%) were Eurasian. Mean duration of diabetes was 15.9+ 9.8 years (range 1 to 53) and 232 (95.9%) patients had Type II diabetes. Peripheral neuropathy,based on NSS and NDS criteria, was found in 83 (34.3%). Thirteen (5.4%) patients were found to likely have neuropathic pain symptoms and this was independently associated with peripheral neuropathy ((OR) = 3.40, 95% confidence interval (CI): 1.04, 11.14) and Indian ethnicity (OR = 5.44, 95% CI: 1.50,
    19.57)). Patients with neuropathic pain had higher average pain intensity scores.
    Conclusions: The prevalence of neuropathic pain symptoms in a Malaysian DM patient cohort was low and was associated with the severity of neuropathy symptoms and Indian ethnicity. The causes for ethnic differences are unknown and could be due socio-cultural or physiological differences in neuropathic pain perception.
    Study site: Diabetic clinic, University of Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia
  2. Kong KH, Balcaitiene J, Berard H, Maisonobe P, Goh KJ, Kumthornthip W, et al.
    Contemp Clin Trials Commun, 2017 Jun;6:9-16.
    PMID: 29740633 DOI: 10.1016/j.conctc.2017.02.004
    Introduction: Approximately 15 million people suffer a stroke annually, up to 40% of which may develop spasticity, which can result in impaired limb function, pain and associated involuntary movements affecting motor control.Robust clinical data on spasticity progression, associated symptoms development and functional impairment is scarce. Additionally, maximal duration of muscle tone reduction following botulinum toxin type A (BoNT-A) injections remains undetermined. The ONTIME pilot study aims to explore these issues and evaluate whether abobotulinumtoxinA 500 U (Dysport®; Ipsen) administered intramuscularly within 12 weeks following stroke delays the appearance or progression of symptomatic (disabling) upper limb spasticity (ULS).

    Methods: ONTIME is a 28-week, phase 4, randomised, double-blind, placebo-controlled, exploratory pilot study initiated at four centres across Malaysia, the Philippines, Singapore and Thailand. Subjects (n = 42) with moderate to severe ULS (modified Ashworth scale [MAS] score ≥2) in elbow flexors or pronators, wrist flexors, or finger flexors will be recruited. Subjects will be randomised 2:1 to abobotulinumtoxinA 500 U or placebo (single dose 2-12 weeks after first-ever stroke).Primary efficacy will be measured by time between initial injection and visit at which reinjection criteria (MAS score ≥2 in the primary targeted muscle group and appearance or reappearance of symptomatic ULS) are met. Follow-up visits will be 4-weekly to a maximum of 28 weeks.

    Discussion: This pilot study will facilitate the design and sample size calculation of further confirmatory studies, and is expected to provide insights into the optimal management of post-stroke patients, including timing of BoNT-A therapy and follow-up duration.

  3. 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.

  4. Hasan MS, Tan JK, Chan CYW, Kwan MK, Karim FSA, Goh KJ
    J Orthop Surg (Hong Kong), 2018 7 31;26(3):2309499018789529.
    PMID: 30058437 DOI: 10.1177/2309499018789529
    BACKGROUND: Drugs used in anesthesia can affect somatosensory evoked potential (SSEP) monitoring, which is used routinely for intraoperative monitoring of spinal cord integrity during spinal surgery.

    OBJECTIVE: The objective of this study was to determine whether combined total intravenous anesthesia (TIVA) technique with propofol/remifentanil is associated with less SSEP suppression when compared to combined volatile agent desflurane/remifentanil anesthesia during corrective scoliosis surgery at a comparable depth of anesthesia.

    DESIGN: It is a randomized controlled trial.

    SETTING: The study was conducted at the Single tertiary University Hospital during October 2014 to June 2015.

    PATIENTS: Patients who required SSEP and had no neurological deficits, and were of American Society of Anesthesiologist I and II physical status, were included. Patients who had sensory or motor deficits preoperatively and significant cardiovascular and respiratory disease were excluded. A total of 72 patients were screened, and 67 patients were randomized and allocated to two groups: 34 in desflurane/remifentanil group and 33 in TIVA group. Four patients from desflurane/remifentanil group and three from TIVA group were withdrawn due to decrease in SSEP amplitude to <0.3 µV after induction of anesthesia. Thirty patients from each group were analyzed.

    INTERVENTIONS: Sixty-seven patients were randomized to receive TIVA or desflurane/remifentanil anesthesia.

    MAIN OUTCOME MEASURES: The measurements taken were the amplitude and latency of SSEP monitoring at five different time points during surgery: before and after the induction of anesthesia, at skin incision, at pedicle screw insertion, and at rod insertion.

    RESULTS: Both anesthesia techniques, TIVA and desflurane/remifentanil, resulted in decreased amplitude and increased latencies of both cervical and cortical peaks. The desflurane/remifentanil group had a significantly greater reduction in the amplitude ( p = 0.004) and an increase in latency ( p = 0.002) of P40 compared with the TIVA group. However, there were no differences in both amplitude ( p = 0.214) and latency ( p = 0.16) in cervical SSEP between the two groups.

    CONCLUSIONS: Compared with TIVA technique, desflurane/remifentanil anesthesia caused more suppression in cortical SSEP, but not in cervical SSEP, at a comparable depth of anesthesia.

  5. 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.
  6. Goh YK, Marzuki NF, Tan SY, Tan SS, Tung HJ, Goh YK, et al.
    Mycology, 2016;7(1):36-44.
    PMID: 30123614 DOI: 10.1080/21501203.2015.1137985
    The influence of different medium components (glucose, sucrose, and fructose) on the growth of different Ganoderma isolates and species was investigated using mixture design. Ten sugar combinations based on three simple sugars were generated with two different concentrations, namely 3.3% and 16.7%, which represented low and high sugar levels, respectively. The media were adjusted to either pH 5 or 8. Ganoderma isolates (two G. boninense from oil palm, one Ganoderma species from coconut palm, G. lingzhi, and G. australe from tower tree) grew faster at pH 8. Ganoderma lingzhi proliferated at the slowest rate compared to all other tested Ganoderma species in all the media studied. However, G. boninense isolates grew the fastest. Different Ganoderma species were found to have different sugar preferences. This study illustrated that the mixture design can be used to determine the optimal combinations of sugar or other nutrient/chemical components of media for fungal growth.
  7. Tan CY, Arumugam T, Razali SNO, Yahya MA, Goh KJ, Shahrizaila N
    J Clin Neurosci, 2018 Nov;57:198-201.
    PMID: 30145079 DOI: 10.1016/j.jocn.2018.08.031
    Diabetic patients with poor glycaemic control can demonstrate demyelinating distal sensorimotor polyneuropathy (D-DSP) on electrophysiology. Distinguishing D-DSP from chronic inflammatory demyelinating polyneuropathy (CIDP) can be challenging. In this study, we investigated the role of nerve ultrasound in differentiating the two neuropathies. Nerve ultrasound findings of D-DSP patients (fulfilling the electrophysiological but not clinical criteria for CIDP) were compared with non-diabetic CIDP patients (fulfilling both criteria). We studied 108 and 95 nerves from 9 D-DSP and 10 CIDP patients respectively. CIDP patients had significantly larger cross-sectional areas of the median nerve at the mid-arm (17.0 ± 12.5 vs 8.7 ± 2.6; p = 0.005), ulnar nerve at the wrist (7.3 ± 3.1 vs 4.1 ± 1.0; p = 0.001), mid forearm (8.8 ± 5.3 vs 5.5 ± 1.5; p = 0.002) and mid-arm (14.5 ± 14.1 vs 7.5 ± 1.9; p = 0.013), and radial nerve at mid forearm (4.1 ± 2.4 vs 1.2 ± 0.4; p 
  8. 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.
  9. 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.
  10. Sarji SA, Abdullah BJ, Goh KJ, Tan CT, Wong KT
    AJR Am J Roentgenol, 2000 Aug;175(2):437-42.
    PMID: 10915690
    The newly discovered Nipah virus causes an acute febrile encephalitic illness in humans that is associated with a high mortality. The purpose of this study is to describe the MR imaging findings of Nipah encephalitis.
  11. 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 

  12. 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.
  13. 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.

  14. 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.

  15. Ong CE, Ahmad R, Goh YK, Azizan KA, Baharum SN, Goh KJ
    PLoS One, 2021;16(12):e0262029.
    PMID: 34972183 DOI: 10.1371/journal.pone.0262029
    Various phenolic compounds have been screened against Ganoderma boninense, the fungal pathogen causing basal stem rot in oil palms. In this study, we focused on the effects of salicylic acid (SA) on the growth of three G. boninense isolates with different levels of aggressiveness. In addition, study on untargeted metabolite profiling was conducted to investigate the metabolomic responses of G. boninense towards salicylic acid. The inhibitory effects of salicylic acid were both concentration- (P < 0.001) and isolate-dependent (P < 0.001). Also, growth-promoting effect was observed in one of the isolates at low concentrations of salicylic acid where it could have been utilized by G. boninense as a source of carbon and energy. Besides, adaptation towards salicylic acid treatment was evident in this study for all isolates, particularly at high concentrations. In other words, inhibitory effect of salicylic acid treatment on the fungal growth declined over time. In terms of metabolomics response to salicylic acid treatment, G. boninense produced several metabolites such as coumarin and azatyrosine, which suggests that salicylic acid modulates the developmental switch in G. boninense towards the defense mode for its survival. Furthermore, the liquid chromatography time-of-flight mass spectrometry (LC-TOF-MS) analysis showed that the growth of G. boninense on potato dextrose agar involved at least four metabolic pathways: amino acid metabolism, lipid pathway, tryptophan pathway and phenylalanine pathway. Overall, there were 17 metabolites that contributed to treatment separation, each with P<0.005. The release of several antimicrobial metabolites such as eudistomin I may enhance G. boninense's competitiveness against other microorganisms during colonisation. Our findings demonstrated the metabolic versatility of G. boninense towards changes in carbon sources and stress factors. G. boninense was shown to be capable of responding to salicylic acid treatment by switching its developmental stage.
  16. 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.
  17. 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 

Filters
Contact Us

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

External Links