Objective: Progressive supranuclear palsy (PSP) is a rare neurodegenerative disorder encompassing several phenotypes with various motor and cognitive deficits. We aimed to study motor and cognitive characteristics across PSP phenotypes and to assess the influence of apolipoprotein E (APOE) gene variants on PSP phenotypic expression. Methods: In this 20-year cross-sectional study, we retrospectively reviewed the charts of all patients classified as PSP patients and recategorized them according to phenotype using the Movement Disorder Society criteria (2017). Phenotypes were divided into three subgroups, Richardson’s syndrome (PSP-RS), PSP-cortical (PSP with predominant frontal presentation [PSP-F] + PSP with predominant speech/language disorder [PSP-SL] + PSP with predominant corticobasal syndrome [PSP-CBS]) and PSP-subcortical (PSP with predominant parkinsonism [PSP-P] + PSP with progressive gait freezing [PSP-PGF] + PSP with predominant postural instability [PSP-PI] + PSP with predominant ocular motor dysfunction [PSP-OM] + PSP with cerebellar ataxia [PSP-C] + PSP with primary lateral sclerosis [PSP-PLS]), based on clinical presentation during the first 3 years after symptom onset, which defines the early disease stage. Clinical and neuropsychological assessment data were collected. Genotyping of APOE was performed using restriction fragment length polymorphism polymerase chain reaction and verified by Sanger sequencing. Results: We included 112 PSP patients comprising 10 phenotypes classified into 48 PSP-RS, 34 PSP-cortical (PSP-CBS, 17.6%; PSP-F, 9.4%; PSP-SL, 8.2%) and 30 PSP-subcortical (PSP-P, 11.6%; PSP-PI, 8%; PSP-OM, 2.7%; PSP-PGF, 1.8%; PSP-C, 1.8%; PSP-PLS, 0.9%) subgroups. PSP-RS patients were older at disease onset (p = 0.009) and had more akinetic-rigid and levodopa-resistant parkinsonism (p = 0.006), while PSP-cortical patients had more tremors and asymmetric and/or levodopa-responsive parkinsonism (p = 0.025). Cognitive domains were significantly less altered in the PSP-subcortical subgroup. Overall, PSP-APOEε4 carriers developed parkinsonism earlier (p = 0.038), had earlier oculomotor dysfunction (p = 0.052) and had more altered cognitive profiles. The APOEε4 allele was also associated with a younger age of parkinsonism onset in the PSP-RS phenotype group (p = 0.026). Conclusion This study demonstrated the wide phenotypic spectrum of PSP among Tunisians. Disease onset and akinetic-rigid and levodopa-resistant parkinsonism were the hallmarks of the PSP-RS phenotype, while milder cognitive impairment was characteristic of the PSP-subcortical subgroup. The APOEε4 allele was associated with earlier parkinsonism and oculomotor dysfunction and seemed to play a role in defining a more altered cognitive profile in PSP patients.

Objective: LRRK2-G2019S is the most frequent mutation in North African Parkinson’s disease (PD) patients. Data on its impact on disease progression and treatment response remain elusive. Therefore, we investigated the clinical features, treatments, and complications of PD in Tunisian patients according to their LRRK2-G2019S profile. Methods: This longitudinal retrospective study was performed in the Department of Neurology, Razi University Hospital. We included clinically diagnosed PD patients according to the Movement Disorders Society criteria and reviewed their medical records for clinical, treatment, and neuropsychological assessments. All patients were screened for the LRRK2-G2019S mutation using Sanger sequencing. The correlation between LRRK2-G2019S and clinical PD features was evaluated. Results: We included 393 PD patients, 41.5% of whom had LRRK2-G2019S mutations. Patients with mutations were younger (p = 0.017), and female PD patients had a greater mutation frequency (p = 0.008). Mutation carriers exhibited distinct clinical features, with a greater frequency of postural instability gait difficulty forms (adjusted-p < 0.001). During disease progression, carriers showed a faster annual progression in the Unified Parkinson’s Disease Rating Scale Section III scores (adjusted-p = 0.009), and significantly higher levodopa equivalent dose values in later stages (1060.81 vs. 877.83 for 6-8 years). Motor complications, such as dyskinesia (adjusted-p < 0.001) and motor fluctuations (31.9% vs. 25.7%, adjusted-p < 0.001), were more prevalent in carriers, particularly in the later stages. LRRK2-G2019S carriers also exhibited a lower prevalence of non-motor symptoms, including episodic memory (adjusted-p < 0.001), attention (adjusted-p < 0.001), and dysexecutive disorders (adjusted-p = 0.038), as well as neuropsychiatric symptoms and dysautonomic signs. Conclusion The present study demonstrated that the variability of the clinical profile among Tunisian PD patients was explained by the incomplete penetrance of LRRK2-G2019S, which increased with age. Further studies using biomarker and disease progression data are necessary to improve PD management.

INTRODUCTION: The study was performed to determine the psychological problems in children with idiopathic nephrotic syndrome (INS) while they were on steroid therapy, as compared to healthy children. METHODS: This prospective cohort study was conducted in a paediatric clinic of a tertiary hospital. Parents of the participants in the INS group and control group (comprising children without chronic illness) completed questionnaires using the Child Behavioural Checklist (CBCL). The CBCL measures a range of age-specific emotional and psychological problems, including internalising and externalising domains. Analyses of the CBCL scores between groups were done using Mann-Whitney U test. RESULTS: A total of 140 children were recruited with an equal number in the INS and control groups. There was a significant difference in the mean total CBCL scores between the INS group and the control group, specifically in the withdrawal, somatic, anxious and aggressiveness subdomains. Similar findings were demonstrated in correlation between total psychological problems and corticosteroid dosage. In the INS group, steroid dose and cushingoid features were found to have a significant positive association with internalising psychological problems. CONCLUSION Children with INS on corticosteroid treatment showed an increase in internalising and externalising scores, as compared to healthy children.
Child ; Humans ; Child Behavior Disorders/psychology* ; Nephrotic Syndrome/psychology* ; Problem Behavior/psychology* ; Prospective Studies ; Southeast Asian People ; Glucocorticoids/therapeutic use*

Introduction: Inadequate sleep among undergraduate students might have an impact on their bodies and everyday activities. This study aims to investigate the association of physical activity and dietary behaviour on sleep quality among students of Faculty of Architecture, Planning, and Surveying in UiTM Selangor. Method: This research was conducted using a cross-sectional research and convenience sampling. The data were collected through the use of self-administered questionnaires from May to June 2022. Data analysis was done using Statistical Analysis Package for Social Sciences (SPSS) version 20.0. Results: A total of 321 students responded the questionnaire. The prevalence of poor sleep quality among undergraduate students of Faculty of Architecture, Planning and Surveying was 93.5%. It was found that physical activity was not associated (p>0.05) with sleep quality using Pearson’s Chi-square test. Pearson’s Chi-square test revealed that sleep quality was associated (p<0.05) with dietary behaviour which is Western fast food, sweets, water and breakfast skipping, gender, years of study and residential. Conclusion: This study revealed a high prevalence of poor sleep quality among students of Faculty of Architecture, Planning and Surveying. This information may be helpful in creating programs to treat and promote good sleep hygiene practices that may improve students’ physical and mental health.

Introduction: Auditing of paediatric deaths has rarely been evaluated in hospitals especially in low and middle-income countries. The aim of the study is to describe the characteristics of paediatric death in hospitalised children and determine the predictors of death location in Hospital Universiti Sains Malaysia (USM). Method: Paediatric mortality cases for children aged between 29 days and 18 years old at the time of death were analyzed. These were documented cases between the year 2013 and 2020 at Hospital USM. Case notes were reviewed, and data were captured retrospectively. Multiple Logistic Regression was used to assess predictors of the location of death especially in the ward setting.Results: Out of 841 paediatric deaths identified during the period of the study, 544 cases were enrolled. The male gender has a higher proportion (1.4:1). The median age was 56 months, with infancy being the predominant age group. The median length of hospital stay was 10.5 days. More than half of the cases (58.3%) had underlying life-limiting illnesses. The majority of the patients had “Do Not Resuscitate” orders. A longer length of stay (prevalence odds ratio (POR 0.99, 95% CI 0.98, 0.99), being on ventilator (POR 3.42,95% CI 1.95, 6.01), being terminally ill (POR 0.40,95% CI 0.23, 0.70) and having underlying life limiting illness (POR 0.50, 95% CI 0.33, 0.75) were the significant predictors for the ward death. Conclusion: Understanding the characteristics of child death and the factors associated with death location is critical for improving paediatric care and treatment.

Purpose: Macular edema, serous retinal detachment, and retinal pigment epithelial detachment have been reported in patients with nephrotic syndrome. However, there is limited data about macular thickness in children with nephrotic syndrome. The aim of this study was to compare the mean macular thickness in children with nephrotic syndrome and in a control group and to correlate it with visual acuity and level of proteinuria. Methods: The comparative cross-sectional study included 66 children aged 6 to 17 years with nephrotic syndrome and healthy control seen in two tertiary centers in Malaysia. We recorded demographic data, as well as visual acuity, level of proteinuria, and the mean macular thicknesses in both groups. The mean macular thickness was measured using Stratus optical coherence tomography according to nine areas of the Early Treatment Diabetic Retinopathy Study map. Results: The mean foveal thickness was 238.15 ± 22.98 µm for children with nephrotic syndrome and 237.01 ± 22.60 µm for the control group. There was no significant difference in the mean macular thickness between the groups (p = 0.843). A significant correlation with visual acuity was observed in the superior outer macula (r = –0.41, p = 0.019), the nasal outer macula (r = –0.41, p = 0.019), and the inferior outer macula (r = –0.40, p = 0.021). There was no significant correlation between the mean macular thickness and level of proteinuria (p = 0.338), although those with higher levels of proteinuria demonstrated a trend towards increased macular thickness. Conclusions The mean macular thickness in children with nephrotic syndrome was similar to that of healthy children. A significant correlation between the mean thickness of the outer macular layer and the presenting visual acuity was observed. There was no correlation between the mean macular thickness and the level of proteinuria.

Background: The spread of COVID-19 forced many countries including Malaysia to switch from on-campus learning to exclusively online learning. The modality of the synchronous session allows the learners to interact with the instructor and obtain instant feedback. The purpose of this study was to evaluate the effectiveness of synchronous simulation among medical students in clinical years in the absence of hospital-based clinical education. Methods: This was a prospective observational study involving 54 clinical year students, conducted in the simulation lab at International Medical University. Scenarios for each session were built around clinical skills targeting specific learning outcomes. The perception of the simulation session was evaluated using a 5-point Likert Scale. Synchronous debriefing followed each simulated session allowing active participation by all students. Focus group discussion was conducted among 6 students who volunteered representing online participants to obtain feedback on their learning experience. Results: The highest mean scores were obtained for the items referring to team communication (4.09±0.734), debriefing (4.06±0.811) and timetabling (3.92±1.007). Results from the focus group discussion revealed that: simulated sessions assisted in application of knowledge by observing their peers; the repetitive process of observing and performing was preferred over observing per se; observing the performance of peers from the same cohort was preferred, and critiques on technical logistics were reported. Conclusion The synchronous simulation sessions were well received by the students. It is believed that this will be a novel teaching modality to adopt even after the passing of the pandemic.
Students, Medical ; Education, Medical

COVID-19 virus is a causative agent of viral pandemic in human beings which specifically targets respiratory system of humans and causes viral pneumonia. This unusual viral pneumonia is rapidly spreading to all parts of the world, currently affecting about 105 million people with 2.3 million deaths. Current review described history, genomic characteristics, replication, and pathogenesis of COVID-19 with special emphasis on Nigella sativum (N. sativum) as a treatment option. N. sativum seeds are historically and religiously used over the centuries, both for prevention and treatment of different diseases. This review summarizes the potential role of N. sativum seeds against COVID-19 infection at levels of in silico, cell lines and animal models.
Animals ; COVID-19 ; Humans ; Nigella ; Pandemics ; Pathology, Molecular ; SARS-CoV-2

Introduction: Caring for children who have chronic kidney disease (CKD) is challenging. The goal of this study was to find out the prevalence of burnout and what factors contribute to it. We also want to know the correlation between caretaker’s strain and burnout. Methods: This was a cross-sectional study that involved caretakers of children with CKD. They were recruited from a university hospital in Kelantan, Malaysia. Self-administered questionnaire in Malay language comprised of Demographic Information Form, Copenhagen Burnout Inventory (CBI-M) and Caregiver Strain Index (CSI-M) was used. Results: Eighty-eight caretakers were involved in this study. The mean age of caretakers was 42 years old, majority were female (72.4%), Malay (99.0%) and Muslim (99.0%). The mean age of children with CKD was 11 years old, most of them were on medication (69.3%) and mean duration of illness was 4.6 years. The prevalence of burnout in all domains (personal, work-related, client-related) among the caretakers was 5.7%. Eight caretakers had personal burnout (9.1%) while five (5.7%) had client-related and work-related burnout respectively. Multiple linear regression showed positive association of duration since the initial diagnosis and total ward admission within six months with total burnout score. Pearson correlation revealed a positive and fair correlation between strain and burnout. Conclusion: Prevalence of burnout among caretakers was generally low, but personal burnout outnumbered other domains. Children with CKD with longer duration of illness and more frequent ward admission are the important factors leading to burnout among caretakers. A high number of caretakers having significant strain indicated that there were possibilities of developing burnout later on.