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Background Continuous spike wave in sleep (CSWS) is an electroencephalogram (EEG) pattern associated with developmental and epileptic encephalopathy with spike-and-wave activation in sleep (DEE-SWAS). This etiologically heterogeneous syndrome may occur because of genetic factors and congenital or acquired brain lesions. We studied the pattern of clinical presentation and underlying etiologies in patients with DEE-SWAS that respond to resective surgery. Methods We reviewed our clinical and research databases for patients who had resolution of CSWS following surgical resection of a focal lesion. Results We identified 5 patients meeting inclusion criteria. In 3 of 5, an epileptogenic structural abnormality was not apparent on brain magnetic resonance imaging (MRI). In all 3 patients, focal cortical dysplasia was identified through intracranial EEG monitoring. Significance DEE-SWAS may be a secondary bilateral network epilepsy syndrome, which can be treated with resection of the inciting focal lesion. In patients with drug-resistant CSWS, clinicians should consider a complete epilepsy presurgical workup, including intracranial EEG monitoring.

Studies have shown increased immunogenicity from heterologous boosting. This study specifically assessed boosting with Pfizer-BioNTech in Sputnik V vaccination regimens. Reactogenicity was assessed through adverse events. Immunogenicity was assessed by comparing serum anti-Spike (Anti-S) protein antibody and neutralizing antibody titers before booster administration and after 30 days. A total of 428 participants were recruited in the heterologous arm and 351 in the homologous arm. Adverse events were more frequent in the heterologous arm (pARTICLE SUMMARY LINEBoth homologous and heterologous boosting are effective in eliciting an immunological response, however heterologous boosting with Pfizer-BioNTech elicited a higher immunological response, with more adverse effects.

Heterozygous pathogenic WAC variants cause Desanto-Shinawi syndrome; affected patients have dysmorphic features, developmental impairment and behavioral abnormalities. Seizures are reported in one quarter, including tonic-clonic, absence, and febrile seizures. This study aimed to better understand the phenotypic spectrum of epilepsy and development in Desanto-Shinawi syndrome. We identified four children with seizures and pathogenic WAC variants, including two siblings. All had global developmental impairment with language affected most severely; two had diagnoses of childhood apraxia of speech and two had autism spectrum disorder. Seizure onset age ranged from six months to 14 years. Seizures always occurred from sleep and were focal impaired awareness with motor features in three patients, with one having bilateral tonic-clonic seizures of suspected focal onset. Two patients had spontaneous seizure resolution without treatment, and the remaining two were well-controlled on monotherapy. EEG was normal in two patients; one had focal right frontal spikes in drowsiness and sleep while the last had independent centrotemporal spikes from both hemispheres, activated in sleep. All patients had heterozygous truncating pathogenic WAC variants, with negative parental testing. The findings in this cohort of patients suggest that epilepsy in Desanto-Shinawi syndrome is usually focal and self-limited, and may fall within the epilepsy-aphasia spectrum.

The quarantine period imposed to travelers in many countries due to COVID19 is a major obstacle for any traveler. Lifting the quarantine period could lead to significant improvement in people’s quality of life and any country’s economy. Bahrain have used two quarantine models from arriving passengers. We report data about the incidence of COVID19 on arriving passengers at Bahrain International airport. Infection rates were reported on arrival, during quarantine and after leaving quarantine. Results showed that travelers had low incidence of COVID19 on arriving and during the quarantine period, while becoming at higher risk after leaving quarantine. We concluded that quarantine requirement maybe lifted for arriving travelers. Testing upon arrival with implementation of the public health preventative measures can minimize the risk of transmission.

The COVID-19 pandemic has affected more than 100 million cases and caused immense burdens on governments and healthcare systems worldwide. Since its emergence in December 2019, research has been focused on treating the infected, identifying those at risk and preventing spread. There is currently no known biological biomarker that predicts the risk of infection. Several studies emerged suggesting an association between ABO blood group and the risk of COVID-19 infection. In this study, we used retrospective observational data in Bahrain to investigate the association between ABO blood group and risk of infection, as well as susceptibility to severe ICU-requiring infection. We found a higher risk associated with blood group B, and a lower risk with blood group AB. No association was observed between blood group and the risk of a severe ICU-requiring infection. We extended the analysis to study the association by antibodies; anti-a (blood groups B and O) and anti-b (blood groups A and O). No association between antibodies and both risk of infection or susceptibility to severe infection was found. The current study, along with the variation in blood group association results, indicates that blood group may not be an ideal biomarker to predict risk of COVID-19 infection.

Introduction Hydroxychloroquine (HCQ) is an antimalarial drug that received worldwide news and media attention in the treatment of patients with coronavirus disease 2019 (COVID-19). This drug was used on the basis of its antimicrobial and antiviral properties despite lack of definite evidence of clinical efficacy. In this study, we aim to assess the efficacy and safety of using HCQ in treatment of patients with COVID-19 who were admitted in acute care hospitals in Bahrain. Methods We conducted a retrospective cohort study on a random sample of patients admitted with COVID-19 between 24 February and 31 July 2020. The study was conducted in four acute care COVID-19 hospitals in Bahrain. Data was extracted from the medical records. The primary endpoint was the requirement of non-invasive ventilation, intubation, or death. Secondary endpoint was length of hospitalization for survivors. Three methods of analysis were used to control for confounding factors: logistic multivariate regression, propensity score adjusted regression, and matched propensity score analysis. Results A random sample of 1571 patients were included, 440 of whom received HCQ (treatment group) and 1131 did not receive it (control group). Our results showed that HCQ did not have a significant effect on primary outcomes due to COVID-19 infection when compared to controls after adjusting for confounders (OR 1.43, 95% CI 0.85–2.37, P = 0.17). Co-administration of azithromycin had no effect on primary outcomes (OR 2.7, 95% CI 0.82–8.85, P = 0.10). HCQ was associated with increased risk of hypoglycemia (OR 10.9, 95% CI 1.72—69.49, P = 0.011) and diarrhea (OR 2.8, 95% CI 1.4–5.5, P = 0.003), but not QT prolongation (OR 1.92, 95% CI 0.95–3.9, P = 0.06) or cardiac arrhythmia (OR 1.06, 95% CI 0.55–2.05, P = 0.85). Conclusion Our results showed no significant beneficial effect of using hydroxychloroquine on the outcome of patients with COVID-19. Moreover, the risk of hypoglycemia due to hydroxychloroquine would possess a significant risk for out-of-hospital use. Supplementary Information The online version contains supplementary material available at 10.1007/s40121-021-00397-8.

IntroductionNasopharyngeal (NP) swab is an invasive procedure that is difficult to perform in pediatric cases and those with special needs. On the other hand, saliva has been a proposed sample given the ease of collection, comfort and the ability to self-collect. The research project aims to study the presence of SARS-CoV-2 in the saliva of suspected COVID-19 patients in comparison to its presence in NP swabs.MethodologyA cross-sectional study was conducted in October 2020 in COVID19 clinic in the Bahrain Defense Force Hospital. The study compared the presence of SARS-CoV2 by PCR in saliva samples to nasopharyngeal samples. COVID-19 Clinic tests symptomatic, staff, close contacts and pre-operation patients.ResultsThe saliva PCR has shown a sensitivity of 72.9% (95% CI: 58.2% - 84.7%) and a specificity of 98.8% (95% CI: 97.8% - 99.4%). The PPV was 74.5% (95% CI 59.7% to 86.1%) and the NPV was 98.6% (95% CI 97.7% to 99.3%). Kappa coefficient of agreement between saliva and NP was 0.723 (95% CI 0.62 to 0.82, pConclusionThe findings of the study suggest that saliva samples have the potential to be used as a screening tool for SARS-CoV-2, especially in symptomatic individuals. This is especially important when it is difficult to collect NP samples. Saliva samples are however at risk of producing more false negative tests.

BackgroundHydroxychloroquine is an antimalarial drug that received worldwide news and media attention in the treatment of COVID-19 patients. This drug was used based on its antimicrobial and antiviral properties despite lack of definite evidence of clinical efficacy. In this study, we aim to assess the efficacy and safety of using Hydroxychloroquine in treatment of COVID-19 patients who are admitted in acute care hospitals in Bahrain.MethodologyWe conducted retrospective cohort study on a random sample of admitted COVID19 patients between 24 February and 31 July 2020. The study was conducted in four acute care COVID19 hospitals in Bahrain. Data was extracted from the medical records. The primary endpoint was the requirement of non-invasive ventilation, intubation or death. Secondary endpoint was length of hospitalization for survivors. Three methods of analysis were used to control for confounding factors: logistic multivariate regression, propensity score adjusted regression and matched propensity score analysis.ResultsA random sample of 1571 patients were included, 440 of which received HCQ (treatment group) and 1131 did not receive it (control group). Our results showed that HCQ did not have a significant effect on primary outcomes due to COVID-19 infection when compared to controls after adjusting for confounders (OR 1.43 95% CI 0.85 to 2.37, P value=0.17). Co-administration of azithromycin had no effect on primary outcomes (OR 2.7 95% CI 0.82 to 8.85 P value =0.10). HCQ was found to be associated with increased risk of hypoglycemia (OR 10.9 95% CI 1.72 - 69.49, P value =0.011) and diarrhea(OR 2.8, 95% CI 1.4-5.5, P value =0.003), but not QT prolongation(OR=1.92, 95% CI 0.95-3.9, P value =0.06) or cardiac arrhythmia.(OR=1.06, 95% CI 0.55-2.05, P value =0.85).ConclusionOur results showed no significant beneficial effect of using hydroxychloroquine on the outcome of COVID-19 patients. Moreover, the risk of hypoglycemia due to hydroxychloroquine would possess a significant risk for out of hospital use.

IntroductionCOVID 19 has been vastly spreading since December 2019 and the medical teams worldwide are doing their best to limit its spread. In the absence of a vaccine the best way to fight it is by detecting infected cases early and isolate them to prevent its spread. Therefore, a readily available, rapid, and cost-effective test with high specificity and sensitivity for early detection of COVID 19 is required. In this study, we are testing the diagnostic performance of a rapid antigen detection test in mildly symptomatic cases. (RADT).MethodsThe study included 4183 patients who were mildly symptomatic. A nasal sample for the rapid antigen test and a nasopharyngeal sample was taken from each patient. Statistical analysis was conducted to calculate the sensitivity, specificity, positive predictive value, negative predictive value and kappa coefficient of agreement.ResultsThe prevalence of COVID 19 in the study population was 17.5% (733/4183). The calculated sensitivity and specificity were 82.1% and 99.1% respectively. Kappa’s coefficient of agreement between the rapid antigen test and RT-PCR was 0.859 (p < 0.001). A stratified analysis was performed and it showed that the sensitivity of the test improved significantly with lowering the cutoff Ct value to 24.ConclusionThe results of the diagnostic assessment of nasal swabs in the RADT used in our study are promising regarding the potential benefit of using them as a screening tool in mildly symptomatic patients. The diagnostic ability was especially high in cases with high viral load. The rapid antigen test is intended to be used alongside RT-PCR and not replace it. RADT can be of benefit in reducing the use of PCR.

IntroductionThe risk factors for transmission of SARS-CoV2 have been widely studied and it was evident that a population’s behavior has a direct effect on the risk of transmission. Public health measures and regulation are largely kept to control and direct these behaviors. In this study, we describe the change in transmission in SARS-CoV2 in relation to demographics before and after two major religious events: “Eid Alfitr” and “Ashura”MethodsThis is a national observational study conducted in Bahrain in September 2020 to compare the number and demographics of all newly diagnosed cases before and after Eid Alfitr (religious holiday) and Ashura religious event. A 10 day period before the event was compared to a 10 day period after the event by ten days. Data on the number of tests, number of new cases, their demographics (age, gender, nationality) and presence of symptoms were collected and analyzed.ResultsThere was significant increase in the number of COVID-19 cases after both Eid Alfitr (1997 more cases, with a 67% increase) and Ashura (4232 more cases with 2.19 times more cases). The majority of new cases after the religious events were found in local Bahrainis, from 472 cases to 2169 cases after Eid, and from 2201 to 6639 cases after Ashura. The rise was most notable in females (increased by 4.89 times after Eid and by 2.69 times after Ashura), children (increased by 4.69 times after Eid and by 5 times after Ashura) and elderly above the age of 60 years (increased by 5.7 times after Eid and by 3.23 times after Ashura).ConclusionIt is evident that religious events and holidays have important implications on the transmission of SARS-CoV2. This increased in transmission is related mainly to the behavior of the population in those days. Children, female, and elderly were the most affected categories due to these events. A thorough public health plan to limit the spread of the infection at these events should be planned and implemented ahead of time.

The current COVID-19 pandemic has affected more than 22 million cases and caused immense burdens on governments and healthcare systems worldwide. Since its emergence in December 2019, research has been focused on ways to not only treat the infected but also identify those at risk and prevent spread. There is currently no known biological biomarker that can predict the risk of being infected. A growing set of studies have emerged that show an association between ABO blood group and the risk of COVID-19 infection. In this study, we used retrospective observational data in Bahrain to investigate the association between ABO blood group and risk of infection as well as susceptibility to a more severe ICU-requiring infection. We found that individuals with blood group B were at a higher risk of infection, while those with blood group AB were at a lower risk. No association was observed between blood group and the risk of a severe ICU-requiring COVID-19 infection. We extended the analysis to study the association by antibodies present; anti-a (blood groups B and O) and anti-b (blood groups A and O). Antibodies were not found to be associated with either risk of infection or susceptibility to severe infection. The current study, along with the variation in blood group association results, indicates that blood group may not be the most ideal biomarker to predict risk of COVID-19 infection.

Introduction: Sturge-Weber syndrome (SWS) is often associated with drug resistant epilepsy. The literature is unclear as to how often these patients can be weaned off of antiepileptic drugs (AEDs) to become seizure-free. Case Description: We describe two patients with SWS. After initial treatment with various AEDs, breakthrough seizures still occurred. However, after periods with no seizure activity, they were weaned off of their medications. They have been off for 4 and 3 years and seizure-free for 13 and 12 years, respectively. No surgical procedure was necessary. Conclusion: We hypothesize that spontaneous involution or pathological disconnection of the vascular malformations might underly the patients’ recovery. The initial aggressive therapy, close follow-up, choice of AEDs, or natural evolution of the disease may have played a role in their recovery. Therefore, in patients with SWS and lesional structural epilepsy, medication freedom is possible and invasive management options including surgery should be discussed carefully.

Lateral medullary syndrome is rare in pediatrics. It is characterized by neurological deficits due to an ischemic lesion in the lateral medulla. The authors describe a 17-year-old boy who developed lateral medullary syndrome in the context of a hyperflexion neck injury while diving in shallow water with traumatic vascular injury. He had “crossed” neurological deficits above and below the neck. His magnetic resonance angiography showed intra- and extracranial left vertebral artery occlusion and his magnetic resonance imaging showed signal abnormality involving the left lateral medulla and inferomedial cerebellum in keeping with an infarct secondary to left vertebral artery and left posterior inferior cerebellar artery occlusion. Good neurological recovery was observed on heparin therapy started after surgical treatment of traumatic injury. To our knowledge, this is the first reported case of lateral medullary syndrome in a pediatric population related to a flexion neck injury. The authors emphasize the importance of a high level of suspicion for accurate diagnosis.

Background Acute hemorrhagic encephalomyelitis (AHEM) is considered as a rare form of acute disseminated encephalomyelitis characterized by fulminant encephalopathy with hemorrhagic necrosis and most often fatal outcome. Objective To report the association with Ran Binding Protein (RANBP2) gene variant and the response to decompressive craniectomy and high-dose intravenous methylprednisolone (IVMP) in life-threatening AHEM. Design Single case study. Case report A 6-year-old girl known to have sickle cell disease (SCD) presented an acquired demyelinating syndrome (ADS) with diplopia due to sudden unilateral fourth nerve palsy. She received five pulses of IVMP (30 mg/kg/day). Two weeks after steroid weaning, she developed right hemiplegia and coma. Brain magnetic resonance imaging showed a left frontal necrotico-hemorrhagic lesion and new multifocal areas of demyelination. She underwent decompressive craniotomy and evacuation of an ongoing left frontoparietal hemorrhage. Comprehensive investigations ruled out vascular and infectious process. The neurological deterioration stopped concomitantly with combined neurosurgical drainage of the hematoma, decompressive craniotomy, IVMP, and intravenous immunoglobulins (IVIG). She developed during the following months Crohn disease and sclerosing cholangitis. After 2-year follow-up, there was no new neurological manifestation. The patient still suffered right hemiplegia and aphasia, but was able to walk. Cognitive/behavioral abilities significantly recovered. A heterozygous novel rare missense variant (c.4993A>G, p.Lys1665Glu) was identified in RANBP2, a gene associated with acute necrotizing encephalopathy. RANBP2 is a protein playing an important role in the energy homeostasis of neuronal cells. Conclusion In any ADS occurring in the context of SCD and/or autoimmune condition, we recommend to slowly wean steroids and to closely monitor the patient after weaning to quickly treat any recurrence of neurological symptom with IVMP. This case report, in addition to others, stresses the likely efficacy of combined craniotomy, IVIG, and IVMP treatments in AHEM. RANBP2 mutations may sensitize the brain to inflammation and predispose to AHEM.