Right ventricular outflow tract obstruction in twin-to-twin transfusion syndrome undergoing laser surgery: A systematic review and meta-analysis.

INTRODUCTION: We aimed to investigate the incidence, prenatal factors and outcomes of twin-to-twin transfusion (TTTS) with right ventricular outflow tract obstruction (RVOTO). MATERIAL AND METHODS: A systematic search was conducted to identify relevant studies published until February 2023 in English using the databases PubMed, Scopus and Web of Science. Studies reporting on pregnancies with TTTS and RVOTO were included. The random-effect model pooled the mean differences or odds ratios (OR) and the corresponding 95% confidence intervals. Heterogeneity was assessed using the I2 value. RESULTS: A total of 17 studies encompassing 4332 TTTS pregnancies, of which 225 cases had RVOTO, were included. Incidence of RVOTO at time of TTTS diagnosis was 6%. In all, 134/197 (68%) had functional pulmonary stenosis and 62/197 (32%) had functional pulmonary atresia. Of these, 27% resolved following laser and 55% persisted after birth. Of those persisting, 27% required cardiac valve procedures. Prenatal associations were TTTS stage III (53% vs 39% in no-RVOTO), stage IV TTTS (28% in RVOTO vs 12% in no-RVOTO) and ductus venosus reversed a-wave (60% in RVOTO vs 19% in no-RVOTO). Gestational age at laser and gestational age at delivery were comparable between groups. Survival outcomes were also comparable between groups, including fetal demise of 26%, neonatal death of 12% and 6-month survival of 82% in RVOTO group. Findings were similar when subgroup analysis was done for studies including head-to-head analysis. CONCLUSIONS: RVOT occurs in about 6% of the recipient twins with TTTS, especially in stages III and IV and those with reversed ductus venosus a-wave. The findings from this systematic review support the need for a thorough cardiac assessment of pregnancies complicated by TTTS, both before and after laser, to maximize perinatal outcome, and the importance of early diagnosis of TTTS and timely management.

Health-related quality of life and medication adherence of people living with epilepsy in Pakistan: A cross-sectional study.

INTRODUCTION: The primary purpose of this study was to determine adherence and health-related quality of life (HRQoL) in PWE. Secondary aims were to assess association between adherence and HRQoL and determine predictors of HRQoL in PWE in Pakistan. METHODS: A descriptive cross-sectional study was conducted among PWE receiving treatment from two tertiary care hospitals of Pakistan. The HRQoL and adherence were assessed with Urdu versions of Quality of Life in Epilepsy-31 (QOLIE-31), and Medication Adherence Rating Scale (MARS). Relationship between HRQoL and adherence was assessed by Pearson's product-moment correlation coefficient. Forced entry multiple linear models were used to determine relationship of independent variables with HRQoL. RESULTS: 219 PWE with a mean (±standard deviation) age, 34.18 (± 13.710) years, participated in this study. The overall weighted mean HRQoL score was (51.60 ± 17.10), and mean score for adherence was 6.17 (± 2.31). There was significant association between adherence and HRQoL in PWE (Pearson's correlation = 0.820-0.930; p ≤ .0001). Multiple linear regression found adherence (B = 16.8; p ≤ .0001), male gender (B = 10.0; p = .001), employment status (employed: B = 7.50; p = .030), level of education (Tertiary: B = 0.910; p = .010), duration of epilepsy (>10 years: B = -0.700; p ≤ .0001), and age (≥46 years: B = -0.680; p ≤ .0001), and ASM therapy (polypharmacy: B = 0.430; p = .010) as independent predictors of HRQoL in PWE from Pakistan. CONCLUSIONS: The findings suggest PWE from our center have suboptimal adherence which affects HRQoL. Independent factors such as male gender, employment status and duration of epilepsy are predictors of HRQoL.

Injudicious use of cranio-spinal imaging in Guillain-Barré syndrome in a low resource country.

Background: Many patients being investigated for Guillain-Barré syndrome (GBS) undergo unnecessary neuroimaging. The objective of this study was to determine the proportion of patients with GBS undergoing neuroimaging investigation, and to investigate any association with different GBS variants using the Brighton criteria. Methods: This cross-sectional observational study was conducted in the leading tertiary care hospital in Pakistan; 148 patients being investigated for, and subsequently diagnosed with GBS between January 2017 and March 2020 were enrolled. Participants were asked if they had undergone neuroimaging of the craniospinal axis before or during hospital admission, and the purpose of any computed tomography (CT) scan was investigated. We enquired whether fundoscopy had been performed before lumbar puncture (LP) and determined the level of certainty based on the Brighton criteria. Results: The majority of participants were men (n = 107, 73%), with a mean age of 42.85 ± 18.40 years. The mean waiting time to their first interaction with a neurologist was 5.20 ± 4.01 days, and the demyelinating variant of GBS was more common than the axonal variant (1.6:1). Most patients were diagnosed with level I certainty using the Brighton criteria (n = 113, 76%). Brain and spine magnetic resonance imaging (MRI) were performed ahead of admission in 48 (32%) and 59 (39%) patients, respectively. Brain CT scan was performed in 121 (82%) patients before LP, while 27 (18%) only underwent fundoscopic examination before LP. Conclusion: Clinical examination is fundamental in the diagnosis of GBS. Neuroimaging may be inappropriate and unnecessary, and may detract attention from crucial peripheral neuropathy measures while misusing limited resources.

Excessive Daytime Sleepiness and Sleep Quality and their Impact on Health-Related Quality of Life in People with Epilepsy: A study from Pakistan.

BACKGROUND: Epilepsy is one of the most common but manageable neurological disorder. The relation between epilepsy, sleep, and health-related quality of life (HRQoL) in culturally distinct environment of Pakistan remains unclear. The purpose of this study was to determine prevalence and predictors of excessive daytime sleepiness (EDS), poor sleep quality (SQ), and to analyze their association with HRQoL in people with epilepsy (PWE). METHODS: A study was conducted among PWE attending two tertiary care hospitals of Islamabad and Rawalpindi, Pakistan. The EDS, SQ, and HRQoL were evaluated by Urdu versions of Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), and Quality of Life in Epilepsy-31 (QOLIE-31). RESULTS: The study included 200 PWE and 51 healthy controls with mean (SD) ages of 33.1 ±â€¯13.9, 32.9 ±â€¯10.9, and a disease duration of 5.01 ±â€¯6.17, respectively. The majority of the respondents (n = 130, 65%) had poor seizure control, and most of these (n = 88, 68%) were on combination antiepileptic drug (AED) therapy. In comparison to controls, a higher number of PWE had EDS by ESS (score ≥ 11, 10% vs. 40%, p-value 0.00), and poor SQ by PSQI (score > 5, 9% vs. 71%, p-value 0.00). A multiple logistic regression analysis reveals that the factors significantly associated with EDS were: female gender; increasing age; seizure control; duration of epilepsy; and combination AED therapy. A second multiple binary logistic regression analysis suggests that factors significantly associated with poor SQ were: increasing age; female gender; poor seizure control; and combination therapy. The Hierarchical multivariate analysis suggests that poor seizure control, EDS, and poor SQ were significant predictors of low HRQoL. CONCLUSION: The findings suggest high prevalence of EDS and poor SQ in PWE in Pakistan. A significant negative association exists between sleep complaints and HRQoL. During routine clinical consultations, awareness about sleep hygiene practices must be provided to enhance HRQoL.

Neurological complications of SARS-CoV-2: A single-center case series authors.

The neurological manifestations of SARS-CoV-2 are wide-ranging from simple headache to severe demyelinating brain disease. This is a review of collected case reports of patients with SARS-CoV-2 with neurological manifestations presenting to the Pakistan Institute of Medical Sciences (PIMS). Neurological manifestations associated with SARS-CoV-2 such as encephalitis, acute cerebrovascular disease, encephalitis with chorea, post-COVID myositis and Guillain-Barré Syndrome (GBS) are of great concern but are often overlooked in the presence of life-threatening abnormal vital signs in severely ill SARS-CoV-2 patients. There is a need to diagnose these manifestations at the earliest opportunity to limit long-term consequences and complications. Much research is needed to explore the role of SARS-CoV-2 in causing these neurological manifestations by isolating it either from the cerebrospinal fluid (CSF) or the brain tissue of the deceased on autopsy. We also recommend exploring the risk factors that lead to the development of these neurological manifestations.

Tocilizumab and Cytokine Release Syndrome in COVID-19 Pneumonia: Experience From a Single Center in Pakistan.

Background Tocilizumab (TCZ), an interleukin-6 (IL-6) receptor blocker, emerged as a treatment for cytokine release syndrome (CRS) in patients with severe COVID-19 pneumonia. The main objective of the study is to discuss the treatment response of TCZ in severe and critically ill patients with COVID-19 pneumonia. Patient demographics, laboratory parameters before and after TCZ therapy, and clinical outcomes in 20 patients in a single center were prospectively reviewed. Results Out of 120 patients, 96 (80%) were males and 24 (20%) were females. Only eight (10%) patients did not have any previously known comorbidity. There were 78 (65%) patients with severe disease, while 42 (35%) have critically severe disease. Of the 120 patients, only 36 required a second dose of TCZ in our study based on clinical background. Neutrophils and C-reactive protein (CRP) levels were observed to be raised in all patients, while lymphopenia was observed in 114/120, and D-dimer levels were elevated in 102 (85%) patients. After the second dose of tocilizumab, 102 (85%) patients reduced oxygen requirement within four days, and 14 patients were removed on the second dose of tocilizumab on clinical grounds. Of these 120 patients, in two weeks, 30 (25%) were discharged. Within three weeks, 60 of them were discharged, while 12 were discharged after three weeks, and 18 patients died in our study despite treatment. Conclusion TCZ appeared to be a good treatment option in patients with CRS and severe and critical pneumonia, and for patients with raised IL-6 levels despite single TCZ therapy, a repeat dose is recommended.

Coexistent Mitral Stenosis and Multiple Sclerosis: An Unprecedented Case.

Mitral stenosis is a valvular heart disease characterised by narrowing of mitral orifice. It can lead to a dilated left atrium with atrial fibrillation culminating into thrombus formation. Patients with mitral stenosis, presenting with stroke-like episodes, most likely experience cardio-embolic phenomenon; but unusual and unprecedented associations do occur. Reported association of cardiac MS with demyelinating CNS MS has never been proven; but authors have speculated theories based on case reports and series. We narrate the case of a 28-year gentleman known to have severe mitral stenosis, who presented with history of multiple stroke-like episodes. During each episode, he had CT brain done and was labelled as having recurrent embolic strokes. We investigated in detail, and the patient was found to have classical MRI features and CSF findings of multiple sclerosis with multiple lacunar infarcts, warranting different line of treatment in addition to cardio-embolic strokes.

High HbA1c is associated with higher risk of ischaemic stroke in Pakistani population without diabetes.

CONTEXT: The role of glycated haemoglobin (HbA1c) in the prediction of ischaemic stroke in individuals without diabetes is underestimated. AIMS: We performed a study to analyse the role of HbA1c in the risk prediction of ischaemic stroke in Pakistani population without diabetes. We further studied the difference between HbA1c values of individuals with diabetes and without diabetes with stroke. SETTINGS AND DESIGN: Single centre, case-control. MATERIALS AND METHODS: In phase I, a total of 233 patients without diabetes with ischaemic stroke and 245 as controls were enrolled. Association of HbA1c levels, lipid profiles and blood pressure recordings with ischaemic stroke was analysed. In phase II, comparison was done between diabetics and non-diabetics with stroke. STATISTICAL ANALYSIS: Comparison of the mean variables was performed with Student's t-tests. Logistic regression analysis with ischaemic stroke as the dependent variable was performed for phase I. RESULTS: In phase I, the ischaemic stroke group had significantly higher HbA1c levels (5.9±2.9% vs 5.5±1.6%) compared with controls (p<0.05). Triglyceride cholesterol, high-density lipoprotein cholesterol, systolic blood pressure, diastolic blood pressure and HbA1c were the significant determinants of stroke (p<0.05). In phase II, mean HbA1c values were significantly higher in the diabetes group (7.6±2.1 vs 6.1±2.3) (p<0.05) but other parameters were not statistically significantly different (p>0.05). CONCLUSIONS: Higher HbA1c indicated a significantly increased risk for ischaemic stroke. An HbA1c value above 5.6% (prediabetic range) predicted future risk of stroke and efforts to maintain glucose level within the normal range (≤5.6%) in individuals with high cardiovascular risk are important.

Subarachnoid hemorrhage secondary to forceful sneeze.

Subarachnoid hemorrhage (SAH) is a relatively less common but important neurological condition comprising 5% of all the cerebrovascular accidents. In most populations the reported incidence is 6-7 per 100,000 person-years and one-third of survivors become dependent. It is a serious but potentially treatable cause of neurological morbidity. Multiple authors have identified the most unusual novel associations and triggers of subarachnoid bleeds over the past decade. We herein report a rare case of subarachnoid hemorrhage leading to focal neurological deficit in a middle aged man secondary to forceful sneeze.