Materials and methods: We analysed prospectively collected TPE data for patients treated with centrifugation TPE at our non-acute neurology TPE unit in Kuala Lumpur Hospital between May 2015 and June 2018.
Results: A total of 245 TPE procedures were performed in 55 patients for nine neurological indications, predominantly the central nervous system (79%). Twenty four per cent (n=13) had category I and 73% (n=40) had category II indication (American Society for Apheresis (ASFA) 2019). Others (4%) were not in ASFA indications. Neuromyelitis optica spectrum disorders accounted for half (51%) of the total patients. Twenty-three (41.8%) patients experienced adverse events, with hypotensive episodes being the the most common (n=12/55, 21.8%). Five (9.1%) patients had catheter-related blood stream infection, correlating with higher exchange plasma volume (p=0.023). Symptomatic hypocalcaemia was less common (n=5/55, 9.1%) and allergic reaction to human albumin was rare (n=1/55, 1.8%). Four technical errors detected. Three involved centrifugation sets manufacturing defects and one involved error in centrifugation set installation. Seven (2.9%) procedures were terminated: 5 for adverse effects and 2 for technical errors.
Conclusion: Performing TPE among semi-critical patients with neurology manifestations in basic non-acute set-up proved safe, with predictable complications. This set-up reduced the reliance on critical care services for TPE procedures.
OBJECTIVES: We aim to determine the prevalence, demographic and clinical characteristics of MOG antibody disease (MOGAD) specifically identifying any ethnic variations unique to our local population, with global perspectives.
METHODS: This is a cross-sectional study conducted at the Neurology Department, Kuala Lumpur Hospital from January 2018 to January 2021. Out of 750 CNS IIDDs, seventy-eight consecutive anti-AQP4 antibody negative NMOSD/high risk undifferentiated relapsing or monophasic CNSIIDD subjects were tested for anti-MOG.
RESULTS: Anti-MOG was positive in thirty six out of seventy-eight (%)(46.1%) seronegative patients. The prevalence of MOGAD in our Malaysian population is 0.12 per 100,000 persons with less marked female preponderance of 2:1 and younger age at onset of 23.8 ± 14.4 years. Despite a predominantly ethnic Malay population, a high proportion of our MOGAD patients were Indian (Proportion of Malay:Chinese:Indian:others; 16:9:10:1, prevalence 0.5 per 100,000 population for Indians) with favourable disease course in the most with minor exceptions. Monophasic and relapsing disease course was seen in 11.2% and 88.8% of patients respectively. However, fulminant aggressive disease can occur especially amongst the Chinese and paediatric cohorts. Optic neuritis, NMOSD and ADEM were the commonest presentations at onset and first relapse. EDSS at diagnosis, first relapse, and last follow-up were 4.5±2.5, 3±2.0, and 1.75(range 1-3). Neuroimaging showed large, fluffy, PRES- like supratentorial cortical, periventricular deep white matter ,diencephalon lesions,enhancing anterior optic nerve with or without chiasmal sparring lesions and cervical/cervicothoracic involvement. Area post rema lesions were rare. Threshold steroid levels exist relapsing on withdrawal some fulminantly requiring Immunosuppressants(rituximab) and intravenous immunoglobulins to maintain remission.
CONCLUSION: Malaysian MOGAD profile was similar to its international descriptions of the disease with ethnic selectivity for Indians. Prolonged steroid maintenance is essential to prevent relapses. Fulminant aggressive cases of MOGAD especially amongst Paediatric patients and the Chinese cohort have been reported.
METHODS: A systematic review was conducted to include all relevant published MS/ NMOSD studies in the SEA indexed in MEDLINE, Embase, Scopus and CENTRAL from the inception of these databases to August 1, 2019. Quantity of research productivity was measured in terms of the total published documents. Quality of research impact was evaluated by assessing the study designs of the published reports, publications in journals with impact factor (IF) and PlumX Metrics (citations, usage, captures, mentions and social medias). Population size, gross domestic product (GDP) per capita, percentage (%) of GDP allocated to research and development (R&D), and the total number of neurologists reported in each country were obtained from reliable published data.
RESULTS: Out of 3,547 articles identified, only 142 articles fulfilled the eligibility criteria; therefore, the total number of publications in the SEA region related to MS/ NMOSD was deemed low in quantity. Most studies were cross-sectional and case reports/ series; hence, most studies offered low level of evidence. Since the aggregate scores in citations, usage, captures, mentions, and social medias in PlumX Metrics and publications in journals with IF were low, the overall quality of the published articles was considered low. Thailand (57 articles), Malaysia (40) and Singapore (29) contributed to the majority of publications on the topic-. GDP per capita was statistically correlated with usage. Percent GDP for R&D was positively correlated with total publications, usage, captures and social mediaindices.
CONCLUSION: In conclusion, the scientific impact of MS/ NMOSD in the SEA was considered low in quantity and quality. This study must encourage researchers in the SEA to produce greater volumes of high-quality publications in this particular field and motivate governments to increase % GDP for R&D for the benefit of patients suffering fromthese rare and disabling conditions.
METHODS: (1) A population-based study was undertaken to estimate NMOSD prevalence in the multi-ethnic Penang Island, Malaysia, comprising Chinese, Malays, and Indians. Medical records of NMOSD patients followed up at the Penang General Hospital (the neurology referral centre in Penang Island) were reviewed. The 2015 diagnostic criteria of the International Panel for NMO Diagnosis were used for case ascertainment. (2) A review of population-based prevalence studies of NMOSD worldwide was carried out. PubMed and conference proceedings were searched for such studies.
RESULTS: Of the 28 NMOSD patients, 14 were residents of Penang Island on prevalence day [13 (93%) Chinese and one (7%) Malay]. All 14 patients were females and aquaporin 4 seropositive. The prevalence of NMOSD in Penang Island was 1.99/100,000 population; according to ethnicities, the prevalence in Chinese was significantly higher than in Malays (3.31/100,000 vs 0.43/100,000, respectively, p = 0.0195).
CONCLUSION: Based on our and other population-based studies, among Asians, East Asian origin populations (Chinese and Japanese) appear to have higher NMOSD prevalence than other Asian ethnic groups. Worldwide, Blacks seem to have the highest NMOSD prevalence. More studies in different geographical regions and ethnic groups will be useful to further inform about potential factors in NMOSD pathogenesis.
Objective: To evaluate the peripapillary RNFL thickness and optic nerve functions in fellow eye of NMO with unilateral optic neuritis.
Materials and Methods: A comparative cross-sectional study was conducted in 2 tertiary hospitals from August 2017 to May 2019. RNFL thickness and optic nerve functions were evaluated. Statistical analysis was performed using Statistical Package for Social Science version 24.
Results: A total of 26 NMO patients and 26 controls were involved in this study. The median age (IQR) of NMO patients was 32.5 (12) years old. The RNFL thickness was significantly reduced in NMO patients with non-ON eyes as compared to control group. Best corrected visual acuity between the 2 groups were comparable (0.20 vs 0.00, p=0.071). Contrast sensitivity was also reduced in NMO patients (non-ON eyes) at all 5 spatial frequencies. In NMO group, 34.6% have normal colour vision. The mean deviation (MD) of Humphrey visual field (HVF) was higher in NMO group (p<0.001). There was a moderate correlation between RNFL thickness and contrast sensitivity. Weak correlation was found between the RNFL thickness with visual acuity and mean deviation of visual field test.
Conclusion: Our study showed that the fellow eye of NMO patients with unilateral ON revealed a significant reduction in RNFL thickness and all the optic nerve functions have subtle early changes that signify a subclinical retinal damage.
METHODS: In this randomized, double-blind, time-to-event trial, 143 adults were randomly assigned in a 2:1 ratio to receive either intravenous eculizumab (at a dose of 900 mg weekly for the first four doses starting on day 1, followed by 1200 mg every 2 weeks starting at week 4) or matched placebo. The continued use of stable-dose immunosuppressive therapy was permitted. The primary end point was the first adjudicated relapse. Secondary outcomes included the adjudicated annualized relapse rate, quality-of-life measures, and the score on the Expanded Disability Status Scale (EDSS), which ranges from 0 (no disability) to 10 (death).
RESULTS: The trial was stopped after 23 of the 24 prespecified adjudicated relapses, given the uncertainty in estimating when the final event would occur. The mean (±SD) annualized relapse rate in the 24 months before enrollment was 1.99±0.94; 76% of the patients continued to receive their previous immunosuppressive therapy during the trial. Adjudicated relapses occurred in 3 of 96 patients (3%) in the eculizumab group and 20 of 47 (43%) in the placebo group (hazard ratio, 0.06; 95% confidence interval [CI], 0.02 to 0.20; P<0.001). The adjudicated annualized relapse rate was 0.02 in the eculizumab group and 0.35 in the placebo group (rate ratio, 0.04; 95% CI, 0.01 to 0.15; P<0.001). The mean change in the EDSS score was -0.18 in the eculizumab group and 0.12 in the placebo group (least-squares mean difference, -0.29; 95% CI, -0.59 to 0.01). Upper respiratory tract infections and headaches were more common in the eculizumab group. There was one death from pulmonary empyema in the eculizumab group.
CONCLUSIONS: Among patients with AQP4-IgG-positive NMOSD, those who received eculizumab had a significantly lower risk of relapse than those who received placebo. There was no significant between-group difference in measures of disability progression. (Funded by Alexion Pharmaceuticals; PREVENT ClinicalTrials.gov number, NCT01892345; EudraCT number, 2013-001150-10.).