Displaying publications 21 - 40 of 72 in total

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  1. Shahrizaila N, Samulong S, Tey S, Suan LC, Meng LK, Goh KJ, et al.
    Muscle Nerve, 2014 Feb;49(2):198-201.
    PMID: 23649551 DOI: 10.1002/mus.23892
    Data regarding Charcot-Marie-Tooth disease is lacking in Southeast Asian populations. We investigated the frequency of the common genetic mutations in a multiethnic Malaysian cohort.
  2. Goh KJ, Tian S, Shahrizaila N, Ng CW, Tan CT
    Amyotroph Lateral Scler, 2011 Mar;12(2):124-9.
    PMID: 21039118 DOI: 10.3109/17482968.2010.527986
    Our objective was to determine the survival and prognostic factors of motor neuron disease (MND) in a multi-ethnic cohort of Malaysian patients. All patients seen at a university medical centre between January 2000 and December 2009 had their case records reviewed for demographic, clinical and follow-up data. Mortality data, if unavailable from records, were obtained by telephone interview of relatives or from the national mortality registry. Of the 73 patients, 64.4% were Chinese, 19.2% Malays and 16.4% Indians. Male: female ratio was 1.43: 1. Mean age at onset was 51.5 + 11.3 years. Onset was spinal in 75.3% and bulbar in 24.7% of the patients; 94.5% were ALS and 5.5% were progressive muscular atrophy (PMA). Overall median survival was 44.9 + 5.8 months. Ethnic Indians had shorter interval from symptom onset to diagnosis and shorter median survival compared to non-Indians. On Cox proportional hazards analysis, poor prognostic factors were bulbar onset, shorter interval from symptom onset to diagnosis and worse functional score at presentation. In conclusion, age of onset and median survival duration are similar to previous reports in Asians. Clinical features and prognostic factors are similar to other populations. In our cohort, ethnic Indians had more rapid disease course accounting for their shorter survival.
  3. Mazlan M, Rajasegaran S, Engkasan JP, Nawawi O, Goh KJ, Freddy SJ
    Toxins (Basel), 2015 Sep;7(9):3758-70.
    PMID: 26402703 DOI: 10.3390/toxins7093758
    This study aims to determine the most efficacious dose of Botulinum neurotoxin type A (BoNT-A) in reducing sialorrhea in Asian adults with neurological diseases. A prospective, double-blind randomized controlled trial was conducted over 24 weeks. Thirty patients with significant sialorrhea were randomly assigned to receive a BoNT-A (Dysport(®)) injection into the submandibular and the parotid glands bilaterally via an ultrasound guidance. The total dose given per patient was either BoNT-A injection of (i) 50 U; (ii) 100 U; or (iii) 200 U. The primary outcome was the amount of saliva reduction, measured by the differential weight (wet versus dry) of intraoral dental gauze at baseline and at 2, 6, 12, and 24 weeks after injection. The secondary outcome was the subjective report of drooling using the Drooling Frequency and Severity Scale (DFS). Saliva reduction was observed in response to all BoNT-A doses in 17 patients who completed the assessments. Although no statistically significant difference among the doses was found, the measured reduction was greater in groups that received higher doses (100 U and 200 U). The group receiving 200 U of Dysport(®) showed the greatest reduction of saliva until 24 weeks and reported the most significant improvement in the DFS score.
  4. Fong SY, Goh KJ, Shahrizaila N, Wong KT, Tan CT
    Muscle Nerve, 2016 08;54(2):244-8.
    PMID: 26790132 DOI: 10.1002/mus.25029
    INTRODUCTION: We investigated the effects of demographic and physical factors on nerve conduction studies (NCS) in a multi-ethnic Asian population.

    METHODS: One hundred sixty-six healthy Malaysians of different ethnicities (51.2% women, aged 21-77 years) underwent NCS using a standard protocol. Correlations of various factors to NCS were determined, and multiple linear regression analysis was used to develop predictive equations for each parameter.

    RESULTS: Age and ethnicity were the commonest independent factors influencing NCS followed by gender, height, weight, and body mass index. Increasing age predicted a reduction in lower limb motor and all sensory nerve action potential amplitudes and decrease in motor and sensory (except sural) conduction velocities. Ethnic Indians had slower motor and sensory conduction velocities in several nerves and also had differences in action potential amplitudes.

    CONCLUSIONS: NCS parameters in multi-ethnic Malaysians were influenced independently by various demographic and physical factors, including ethnicity. Muscle Nerve 54: 244-248, 2016.

  5. Tan JS, Ambang T, Ahmad-Annuar A, Rajahram GS, Wong KT, Goh KJ
    Muscle Nerve, 2016 May;53(5):822-826.
    PMID: 26789281 DOI: 10.1002/mus.25037
    Choline acetyltransferase (CHAT) gene mutations cause a rare presynaptic congenital myasthenic syndrome due to impaired acetylcholine resynthesis.
  6. 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.
  7. Tay CG, Ong LC, Goh KJ, Rahmat K, Fong CY
    J Clin Neurosci, 2015 Dec;22(12):1994-5.
    PMID: 26254091 DOI: 10.1016/j.jocn.2015.07.001
    We report a previously well 10-month-old Somalian girl who acquired asymmetric lower limb weakness in July 2013 in Mogadishu, Banadir, before arriving in Malaysia at 12 months of age. In May 2013, there was a wild poliomyelitis outbreak in that area, as reported by the World Health Organization. Laboratory investigation, including cerebrospinal fluid, was unremarkable, and electrophysiological studies showed active axonal denervation in the left lower limb. The whole spine T2-weighted MRI revealed non-enhancing hyperintensities of the bilateral anterior horn cells, predominantly on the left side at T11-12. The viral isolations from two stool specimens at her presentation to our centre, 2 months after the onset of illness and 2 weeks apart, were negative. Despite lacking the acute virological evidence of poliomyelitis, in view of the girl's clinical, electrophysiological and classical spinal neuroradiological features, together with her temporal relationship with a World Health Organization reported wild poliomyelitis outbreak, we believe these findings are consistent with a diagnosis of imported poliomyelitis. A review at 30 months of age showed persistent left lower limb monoplegia with little recovery. Our patient reiterates the importance of maintaining awareness of wild polio importation, and keeping abreast of the latest news of global poliomyelitis outbreaks when treating patients with flaccid paralysis, even if they arrive from non-endemic poliomyelitis areas.
  8. Razali SNO, Arumugam T, Yuki N, Rozalli FI, Goh KJ, Shahrizaila N
    Clin Neurophysiol, 2016 Feb;127(2):1652-1656.
    PMID: 26228791 DOI: 10.1016/j.clinph.2015.06.030
    OBJECTIVE: To assess the longitudinal changes of nerve ultrasound in Guillain-Barré syndrome (GBS) patients.

    METHODS: We prospectively recruited 17 GBS patients and 17 age and gender-matched controls. Serial studies of their nerve conduction parameters and nerve ultrasound, documenting the cross-sectional areas (CSA), were performed at admission and repeated at several time points throughout disease course.

    RESULTS: Serial nerve ultrasound revealed significantly enlarged CSA in median, ulnar and sural nerves within the first 3 weeks of disease onset. Longitudinal evaluation revealed an improvement in the nerve CSA with time, reaching significance in the ulnar and sural nerves after 12 weeks. There was no significant difference between the demyelinating and axonal subtypes. There was also no significant correlation found between nerve CSA and neurophysiological parameters or changes in nerve CSA and muscle strength.

    CONCLUSION: In GBS, serial studies of peripheral nerve ultrasound CSA are helpful to detect a gradual improvement in the nerve size.

    SIGNIFICANCE: Serial nerve ultrasound studies could serve as a useful tool in demonstrating nerve recovery in GBS.

  9. Tan CY, Shahrizaila N, Yeoh KY, Goh KJ, Tan MP
    Clin Auton Res, 2019 06;29(3):339-348.
    PMID: 29654380 DOI: 10.1007/s10286-018-0525-z
    OBJECTIVE: The current study aimed to investigate autonomic dysfunction in Guillain-Barré syndrome (GBS) patients and describe the results of computational heart rate variability (HRV)/baroreflex sensitivity (BRS) and autonomic challenge tests.

    METHODS: GBS patients were consecutively recruited and the results were compared to age- and gender-matched healthy controls. A series of autonomic function tests including computation-dependent tests (power spectrum analysis of HRV and BRS at rest) and challenge maneuvers (deep breathing, eyeball compression, active standing, the Valsalva maneuver, sustained handgrip, and the cold pressor test) were performed.

    RESULTS: Ten GBS patients (six men; mean age = 40.1 ± 13.9 years) and ten gender- and age-matched healthy controls were recruited. The mean GBS functional grading scale at disease plateau was 3.4 ± 1.0. No patients required intensive care unit admission or mechanical ventilation. Low-frequency HRV (p = 0.027), high-frequency HRV (p = 0.008), and the total power spectral density of HRV (p = 0.015) were significantly reduced in patients compared to controls. The mean up slope (p = 0.034), down slope (p = 0.011), and total slope (p = 0.024) BRS were significantly lower in GBS patients. The diastolic rise in blood pressure in the cold pressor test was significantly lower in GBS patients compared to controls (p = 0.008).

    INTERPRETATION: Computation-dependent tests (HRV and BRS) were more useful for detecting autonomic dysfunction in GBS patients, whereas the cold pressor test was the only reliable challenge test, making it useful as a bedside measure of autonomic function in GBS patients.

  10. Tan HT, Tan CY, Teong CS, Ratnasingam J, Goh KJ
    J Clin Neurophysiol, 2020 Aug 05.
    PMID: 32773648 DOI: 10.1097/WNP.0000000000000766
    PURPOSE: Thyrotoxic periodic paralysis is characterized by recurrent episodes of reversible, severe proximal muscle weakness associated with hypokalemia and hyperthyroidism. Prolonged exercise test is an easy, noninvasive method of demonstrating abnormal muscle membrane excitability in periodic paralyses. Although abnormal in thyrotoxic periodic paralysis patients, the effects thyroid hormone levels in non-thyrotoxic periodic paralysis thyrotoxicosis patients have not been well studied. The study aims to evaluate thyrotoxicosis patients (regardless of thyrotoxic periodic paralysis history) with prolonged exercise test and correlate it with their thyroid status.

    METHODS: This is a prospective, cross-sectional study of consecutive thyrotoxicosis patients seen at the endocrine clinic of a tertiary medical center. Thyroid status was determined biochemically before prolonged exercise test. Compound muscle action potential (CMAP) amplitudes postexercise were compared against pre-exercise amplitudes and recorded as percentage of mean baseline CMAP amplitude. Comparisons of time-dependent postexercise CMAP amplitudes and mean CMAP amplitude decrement were made between hyperthyroid and nonhyperthyroid groups.

    RESULTS: Seventy-four patients were recruited, 23 (31%) men, 30 (41%) Chinese, and the mean age was 48.5 ± 16.8 years. Of 74 patients, 32 (43%) were hyperthyroid and 42 (57%) were nonhyperthyroid viz. euthyroid and hypothyroid. Time-dependent CMAP amplitudes from 10 to 45 minutes after exercise were significantly lower in hyperthyroid patients compared with nonhyperthyroid patients (P < 0.01). Mean CMAP amplitude decrement postexercise was significantly greater in hyperthyroid than nonhyperthyroid patients (23.4% ± 11.4% vs. 17.3% ± 10.5%; P = 0.02).

    CONCLUSIONS: Compound muscle action potential amplitude declines on prolonged exercise test were significantly greater in hyperthyroid patients compared with nonhyperthyroid patients. Muscle membrane excitability is highly influenced by thyroid hormone level. Thyrotoxic periodic paralysis occurs from increased levels of thyroid hormone activity in susceptible patients.

  11. 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.

  12. Tay CG, Lee VWM, Ong LC, Goh KJ, Ariffin H, Fong CY
    Pediatr Blood Cancer, 2017 Aug;64(8).
    PMID: 28139029 DOI: 10.1002/pbc.26471
    BACKGROUND: Vincristine, an essential component of childhood acute lymphoblastic leukaemia (ALL) therapeutic protocols, is associated with dose-dependent neurotoxicity, but its long-term morbidity in treated children has not been clearly elucidated. The aim of this study is to determine the prevalence of vincristine-induced peripheral neuropathy (VIPN) among Malaysian childhood ALL survivors and its impact on motor function and quality of life.

    PROCEDURE: Survivors of childhood ALL aged 4-18 years who had completed chemotherapy for 2 years or more were evaluated for VIPN using both the clinical Total Neuropathy Score (cTNS) and nerve conduction studies. Motor function and quality of life of the survivors were assessed via the Bruininks-Oseretsky Test of Motor Proficiency Brief Form, Second Edition (BOT-2 Brief Form) and the Paediatric Quality of Life version 4.0 Generic Core Scales (PedsQL4.0) questionnaire, respectively.

    RESULTS: One hundred and one survivors with a duration of follow-up ranging from 2.0 to 10.3 years were recruited. Twenty-seven (26.7%) had abnormal cTNS scores and 69 (68.3%) had electrophysiological evidence of neuropathy. Of these, 16 (15.8%) had combined clinical and electrophysiological neuropathy (VIPN). Those previously treated on the intermediate- or high-risk treatment stratification arms had a higher risk of developing VIPN (67.3 vs. 32.7%; odds ratio [OR]: 9.06, 95% confidence interval [CI]: 1.14-71.86; P = 0.014). Survivors with VIPN had significantly lower quality of life scores in the physical (P = 0.024) and social domains (P = 0.039) compared with peers without VIPN, but no association with poorer motor function was observed.

    CONCLUSIONS: Sixteen percent of ALL survivors had VIPN. VIPN should be increasingly recognised as a late effect of chemotherapy, as it significantly affects physical and social function quality of life.

  13. Goh KJ, Abdullah S, Wong WF, Yeap SS, Shahrizaila N, Tan CT
    Neurology Asia, 2014;19(4):409-412.
    MyJurnal
    We report a patient who presented with severe cold-induced allodynia and hyperhidrosis, and found to have acquired neuromyotonia (Isaacs syndrome) with high voltage-gated potassium channel (VGKC) antibody titre,positive contactin-associated protein 2 (CASPR2) and leucine-rich glioma-inactivated 1 (LGI1) antibodies. The patient also had positive anti-dsDNA and acetylcholine receptor (AChR) antibodies without clinical features of SLE or myasthenia gravis, suggesting a strong underlying autoimmune tendency. CT thorax showed no thymoma. Her symptoms improved with intravenous immunoglobulin infusion but recurred despite maintenance oral corticosteroids and carbamazepine. She has since been on regular IVIG infusions. Cold allodynia is an unusual presentation in acquired neuromyotonia.
  14. 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
  15. 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.

  16. 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.

  17. 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.

  18. 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.
  19. 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 
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