Focal cortical dysplasia with prolonged ictal asystole, a case report

Introduction: Cortical dysplasia carries significant morbidities such as seizures and delayed milestones. Focal cortical dysplasia (FCD) causes refractory epilepsy with various seizure types depending on the location and extent of the dysplasia. FCD in the temporal region and the insular cortex may cause ictal bradycardia (IB) and ictal asystole (IA). Video EEG (VEEG) with simultaneous EKG recording can better diagnose these cardiac abnormalities in FCD. We describe a case of refractory epilepsy. The patient\u27s clinical seizures were usually followed by syncope. VEEG revealed frequent seizures some of which were associated with prolonged ictal asystole.Results: A 15 years old female was admitted to an epilepsy monitoring unit for VEEG. There were widespread fast abnormal discharges known as FREDs with a frequency of 16-20 Hz. She developed numerous habitual seizures and syncope. Some of these were associated with an EKG change in the form of asystole. The cardiac workup was normal. MRI revealed abnormalities in bilateral insular, temporal, and right parietal lobes.Conclusion: This case highlights the significance of:●Fast rhythmic epileptiform discharges (FREDs) in cortical dysplasia.●Role of video-EEG monitoring.●Prolonged asystole and the potential role of cardiac intervention in the form of cardiac pacing and cardioneuroablation in decreasing syncope

Pattern of neurological diseases in adult outpatient neurology clinics in tertiary care hospital

Objectives: The burden of neurological diseases in developing countries is rising although little is known about the epidemiology and clinical pattern of neurological disorders. The objective of this study was to understand the burden of disease faced by neurologists a in tertiary care setting. Results: A prospective observational study was conducted of all presentations to neurology clinics at Aga Khan University Hospital Karachi over a period of 2 years. A total of 16,371 out-patients with neurological diseases were seen during the study period. The mean age of the study participants were 46.2 ± 18.3 years and 8508 (52%) were male. Headache disorders were present in 3058 (18.6%) of patients followed by vascular diseases 2842 (17.4%), nerve and root lesions 2311 (14.1%) and epilepsies 2055 (12.5%). Parkinson\u27s disease was more prevalent in male participants 564 (70.8%) as compared to female 257 (62.1%) (p = 0.002). Migraines and vertigo disease were more diagnosed in females as compared to males. Epilepsies were seen more in younger age groups. Parkinson\u27s disease was seen in 50.9% of participants between the ages of 45 and 65 years, and the frequency increased with age

Epilepsy in pakistan: national guidelines for clinicians

Epilepsy is one of the most common chronic neurological disorders requiring prolonged treatments and drugs. According to The World Health Organization (WHO), epilepsy is one of those serious brain disorders that affect not only the individual but has a deep impact on the family and society in general. Approximately 50 million people are affected with epilepsy around the world36, though proper epidemiological studies do not exist for Pakistan it is estimated that the prevalence of epilepsy is 9.99/1000. Highest prevalence is seen in people younger than 30 years of age, i.e. about 2 million people and 1/10th of the world burden of epilepsy is in Pakistan! The guidelines available in developed countries are gauged in a setting where epilepsy care is provided by epileptologists/neurologists. In Pakistan the scenario is different, there is only one neurologist for 1.4 million (14lac) population contrast to US where one neurologist for 26 thousand people 29. So there is a desperate need to adapt to alternate guidelines with strategies to provide epilepsy management at a primary care level and to standardize epilepsy care on a National level

Establishment of a comprehensive epilepsy center in Pakistan: initial experiences, results, and reflections

Background. Developing countries, home to 80% of epilepsy patients, do not have comprehensive epilepsy surgery programs. Considering these needs we set up first epilepsy surgery center in Pakistan. Methods. Seventeen teleconferences focused on setting up an epilepsy center at the Aga Khan University (AKU), Karachi, Pakistan were arranged with experts from the University of Alberta Hospital, Alberta, Canada and the University of West Virginia, USA over a two-year period. Subsequently, the experts visited the proposed center to provide hands on training. During this period several interactive teaching sessions, a nationwide workshop, and various public awareness events were organized. Results. Sixteen patients underwent surgery, functional hemispherectomy (HS) was done in six, anterior temporal lobectomy (ATL) in six, and neuronavigation-guided selective amygdalohippocampectomy (SAH) using keyhole technique in four patients. Minimal morbidity was observed in ATL and, SAH groups. All patients in SAH group (100%) had Grade 1 control, while only 5 patients (83%) in ATL group, and 4 patients (66%) in HS group had Grade 1 control according to Engel\u27s classification, in average followups of 12 months, 24 months and 48 months for SAH, ATL, and HS, respectively. Conclusion. As we share our experience we hope to set a practical example for economically constrained countries that successful epilepsy surgery centers can be managed with limited resources

Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Approach to exaggerated startle reflex: A case of hyperekplexia minor

A broad set of conditions may present with an exaggerated startle reflex in clinics. This, combined with the overall rarity of these disorders, may pose diagnostic uncertainty in the mind of the treating physician. Herein, we report a case of a patient who presented to us with the complaint of exaggerated startle reflex and outline a simple approach towards characterisation of these disorders

Dysfunctional schema modes as determinants of psychiatric comorbidities: A study in a cohort of people with epilepsy

Introduction and aims: Symptomatology of epilepsy and its\u27 associated alteration in brain processes, stigma of experiencing seizures, and adverse sequelae of anti-epileptics have been demonstrated to impact behaviour and exacerbate psychopathology. This study examines the role of dysfunctional schema modes in People with Epilepsy (PWE) and their association with psychiatric symptoms.Methods: Semi-structured interviews were conducted with 108 PWE treated with anti-epileptics for at least one year and with no history or mental disorder or psycho-active substance use. Clinical symptoms were measured utilising the Symptom Checklist-90 (SCL-90) with schema modes measured utilising the Schema Mode Inventory (SMI).Results: Maladaptive coping and child schema modes were significantly higher in individuals from lower socio-economic status group (p \u3c 0.01), with several maladaptive schema modes more prevalent in males. Hostility symptoms were increased in individuals from lower socio-economic classes and were more prevalent early in disease course. Several psychological symptoms including somatisation, interpersonal, obsession, depression, paranoia, hostility, phobia, anxiety, and psychoticism, were predicted by various maladaptive schema modes (p \u3c 0.001).Conclusion: This study highlights the impact of maladaptive schemas, suggesting that PWE might benefit from the introduction of appropriate psychotherapeutic interventions such as schema-focused therapy, particularly if from lower socio-economic classes or in the early stages of their disease course

Experiences from an International Tele-Epilepsy Collaboration

Objective: Our main objective was to use videoconferencing as a primary means to: a) assist in launching an epilepsy surgery program in Pakistan, 2) participate in case conferences on complex epilepsy Patients in each country. Methods: Extensive testing using both point to point and bridged integrated service digital network (ISDN) and internet protocol (IP) connections was carried out using bandwidths of 384-768 kilobits per second (kbps). Videoconferences between sites were arranged two to three weeks in advance and connections were tested a day prior to the scheduled conference. Sharing of PowerPoint presentations, neuroimaging and video-EEG was available to all sites. Discussions centered on Patients with medically refractory epilepsy. Results: Between July 2006 and June 2008, 17 sessions were booked. Five of these conferences bridged in specialists from West Virginia University. Most successful connections occurred using IP point to point calls or a bridge connecting end points through IP at 512 kbps. We conducted three surgeries for medically refractory temporal lobe epilepsy in Pakistan. At follow-up in January 2009, two Patients have been seizure free and one had two breakthrough seizures after sudden unsupervised discontinuation of Levetiracetam. Conclusion: Our international tele-epilepsy collaboration has proven feasible and valuable to all participants. Our experience suggests considerable thought and preparation are needed before a teleconference to ensure its success. We provide a recipe to set-up similar telemedicine collaborations. Considerations include time zone differences, equipment type, interoperability between endpoints, connection capabilities, bandwidth availability, and backup plans for unsuccessful connections. Telemedicine can facilitate epilepsy care around the world, identifying with the concept of a Global Health Village

Establishment of a Comprehensive Epilepsy Center in Pakistan: Initial Experiences, Results, and Reflections

Background. Developing countries, home to 80% of epilepsy patients, do not have comprehensive epilepsy surgery programs. Considering these needs we set up first epilepsy surgery center in Pakistan. Methods. Seventeen teleconferences focused on setting up an epilepsy center at the Aga Khan University (AKU), Karachi, Pakistan were arranged with experts from the University of Alberta Hospital, Alberta, Canada and the University of West Virginia, USA over a two-year period. Subsequently, the experts visited the proposed center to provide hands on training. During this period several interactive teaching sessions, a nationwide workshop, and various public awareness events were organized. Results. Sixteen patients underwent surgery, functional hemispherectomy (HS) was done in six, anterior temporal lobectomy (ATL) in six, and neuronavigation-guided selective amygdalohippocampectomy (SAH) using keyhole technique in four patients. Minimal morbidity was observed in ATL and, SAH groups. All patients in SAH group (100%) had Grade 1 control, while only 5 patients (83%) in ATL group, and 4 patients (66%) in HS group had Grade 1 control according to Engel’s classification, in average followups of 12 months, 24 months and 48 months for SAH, ATL, and HS, respectively. Conclusion. As we share our experience we hope to set a practical example for economically constrained countries that successful epilepsy surgery centers can be managed with limited resources

A prospective, observational, multicentre study to evaluate the efficacy of brivaracetam as adjuvant therapy for epilepsy: The Bravo study

Background: Epilepsy is a persistent tendency to experience epileptic seizures and can lead to various neurobiological disorders, with an elevated risk of premature mortality. This study evaluates the efficacy of brivaracetam adjuvant therapy in patients with epilepsy. Methods: A prospective observational multicentre study that was conducted in Pakistan from March to September 2022, by using a non-probability convenience sampling technique. The population consisted of 543 individuals with a diagnosis of epilepsy for whom adjunctive brivaracetam (Brivera; manufactured by Helix Pharma Pvt Ltd., Sindh, Pakistan) was recommended by the treating physician. The research sample was drawn from various private neurology clinics of Karachi, Lahore, Rawalpindi, Islamabad and Peshawar. Data originating from routine patient visits, and assessments at three study time points, were recorded in the study case report form. Results: Across four clinical sites, 543 individuals participated, with a mean age of 32.9 years. The most prescribed dosages were 50 mg BD, followed by 100 mg BD. Notably, brivaracetam combined with divalproex sodium was the most prevalent treatment, followed by brivaracetam with levetiracetam. At both the 14th and 90th day assessments, a significant reduction in seizure frequency was observed, with 63.1% of individuals showing a favourable response by day 90. Treatment-naive individuals exhibited higher rates of seizure freedom and response compared with treatment-resistant individuals. Conclusions: The study demonstrates the effectiveness of brivaracetam combination therapy in epilepsy management, with notable reductions in seizure frequency and favourable clinical responses observed, particularly in treatment-naive individuals