Case Series of Midazolam-Induced Seizures-Like Activity in Five Neonates

An intravenous administration of midazolam may result in seizure-like activity or movement. This report describes five neonates who developed seizure-like movements after intravenous midazolam injection. The patients presented between 2019 and 2022. The abnormal movements occurred shortly after intravenous bolus administration of midazolam. None of our patients experienced seizure-like movements after receiving midazolam infusions. The seizure-like movements were aborted either spontaneously or by antiseizure medications. Also, we did not observe any seizure recurrence in any of the infants during the later stages of their treatment. Since this adverse effect might be related to the speed of the bolus administration, intravenous midazolam must be given as a slow bolus over 2-3 minutes followed by a slow flush of normal saline. To prevent midazolam's potential adverse effect on newborns, neonatal caregivers must be aware of it. Keywords: Midazolam; Injection, intravenous, Seizures; Infant, Newborn; Hypnotics and Sedatives

Ramsay Hunt Syndrome Associated with Varicella-Zoster Virus Encephalitis in a Child

Ramsay Hunt Syndrome (RHS) is a triad of peri-auricular pain, ipsilateral facial nerve palsy and vesicular rash around the ear pinna. It is caused by reactivation of varicella-zoster virus (VZV) that lies dormant in the geniculate ganglia. It can be complicated by VZV encephalitis rarely. We report the case of an 8-year-old previously healthy boy who presented to a tertiary care hospital in Muscat, Oman in 2021 with fever, progressive left ear pain, vesicular rash around his ear pinna and left-sided facial nerve palsy. His course was complicated by VZV encephalitis where he was managed with IV acyclovir and IV corticosteroids. He improved significantly and was asymptomatic with a normal neurology examination at the 6-months follow-up. Keywords: Varicella Zoster Virus; Ramsay Hunt Syndrome; Encephalitis; Children

Case report: Cyclosporine A-induced extrapyramidal syndrome following hematopoietic stem cell transplantation

IntroductionCyclosporine A-associated neurotoxicity has been reported in up to 40% of patients and its wide range of neurological adverse effects have been reported, ranging from mild tremors to fatal leukoencephalopathy. Extrapyramidal (EP) neurotoxicity is a rare manifestation of cyclosporine. Cyclosporine-induced extrapyramidal syndrome remains a rare adverse reaction.Design/methodsA database search was performed for studies in patients from all age groups. We found a total of 10 articles reporting EP as an adverse effect of cyclosporine A. A total of 16 patients were found, and a thorough review of these patients was performed. A comparison of patients was performed to highlight common clinical presentations, investigations during the symptomatic phase, and prognosis. In addition, we describe an 8-year-old boy who developed cyclosporine-related extrapyramidal signs on day 60 post-hematopoietic stem cell transplantation for beta-thalassemia.ConclusionCyclosporine A can induce neurotoxicity resulting in diverse symptoms. Signs of EP are rare manifestations of cyclosporine neurotoxicity and should be considered when evaluating post-transplant recipients of cyclosporine when they are present with any EP symptoms. Discontinuation of cyclosporine results in good recovery in most patients

Aetiology and Outcome of Childhood Convulsive Status Epilepticus: A tertiary care experience in Oman

Objective: This study aimed to evaluate the etiology, management, and outcomes of convulsive status epilepticus (CSE) in children highlighting the factors that affect patient outcome. Methods: In a retrospective study spanning 2020 to 2023, 93 children with convulsive status epilepticus (CSE) treated at Sultan Qaboos University Hospital's emergency department (ED), High Dependency (HD), and intensive care unit (ICU) were analyzed. The Modified Rankin Scale at discharge determined CSE outcome. Results: Study of 93 children (mean age 4.84 years ± 3.64), predominantly Omani (92.47%). Acute 14 symptomatic (37.7%) and  febrile tatus (31.2%) were primary causes. Diazepam used in 67.44% 15 cases as first-line treatment, with median seizure duration of 45 minutes. Successful control achieved in 16 76.34% within 60 minutes. Return to baseline in 55.9%, 5.38% mortality, and 38.7% disability. Etiology and 17 duration significantly impacted outcomes (p < 0.05). Conclusion: Acute symptomatic is the most common etiology of CSE, and a longer duration is associated with higher mortality and neurological disability. Therefore, managing CSE promptly and appropriately is crucial. Furthermore, identifying and treating the underlying cause is essential to reduce the duration of CSE and improve the outcome. Keywords: Etiology, Outcome, Convulsive Status Epilepticus, Modified Rankin Scal

Guillain-Barre Syndrome Associated with SARS-CoV-2 in Two Pediatric Patients

Guillain-Barre syndrome (GBS) is a recognized complication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). We report two children with GBS associated with SARS-CoV-2 presented to a tertiary center in Muscat, Oman in 2021: The first patient was a 3-month-old female infant who presented with bradypnea, encephalopathy, and generalized weakness that required mechanical ventilation. Polymerase chain reaction (PCR) testing of the nasopharyngeal swabs (NPS) was positive for SARS-CoV-2. She had axonal variant GBS based on a nerve conduction study, cerebrospinal fluid analysis, and neuroimaging findings. The second patient was a 6-year-old girl with fever, vomiting, and diarrhea followed by ascending weakness who presented with quadriplegia and facial weakness. Subsequently, she developed respiratory muscle weakness and required mechanical ventilation. PCR testing of NPS was negative for SARS-Cov-2, however IgG serology analysis was positive. The clinical course of these two patients was rapidly progressive and both of them required mechanical ventilation. The patient with axonal variant GBS made an incomplete recovery. Keywords: Acute Inflammatory Demyelinating Polyradiculoneuropathy, SARS-CoV-2, Oma

Transient response to high‐dose niacin therapy in a patient with NAXE deficiency

Abstract Background NAXE‐encephalopathy or early‐onset progressive encephalopathy with brain edema and/or leukoencephalopathy‐1 (PEBEL‐1) and NAXD‐encephalopathy (PEBEL‐2) have been described recently as mitochondrial disorders causing psychomotor regression, hypotonia, ataxia, quadriparesis, ophthalmoparesis, respiratory insufficiency, encephalopathy, and seizures with the onset being usually within the first three years of life. It usually leads to rapid disease progression and death in early childhood. Anecdotal reports suggest that niacin, through its role in nicotinamide adenine dinucleotinde (NAD) de novo synthesis, corrects biochemical derangement, and slows down disease progression. Reports so far have supported this observation. Methods We describe a patient with a confirmed PEBEL‐1 diagnosis and report his clinical response to niacin therapy. Moreover, we systematically searched the literature for PEBEL‐1 and PEBEL‐2 patients treated with niacin and details about response to treatment and clinical data were reviewed. Furthermore, we are describing off‐label use of a COX2 inhibitor to treat niacin‐related urticaria in NAXE‐encephalopathy. Results So far, seven patients with PEBEL‐1 and PEBEL‐2 treated with niacin were reported, and all patients showed a good response for therapy or stabilization of symptoms. We report a patient exhibiting PEBEL‐1 with an unfavorable outcome despite showing initial stabilization and receiving the highest dose of niacin reported to date. Niacin therapy failed to halt disease progression or attain stabilization of the disease in this patient. Conclusion Despite previous positive results for niacin supplementation in patients with PEBEL‐1 and PEBEL‐2, this is the first report of a patient with PEBEL‐1 who deteriorated to fatal outcome despite being started on the highest dose of niacin therapy reported to date

Pancreatic surgery outcomes: multicentre prospective snapshot study in 67 countries

Background: Pancreatic surgery remains associated with high morbidity rates. Although postoperative mortality appears to have improved with specialization, the outcomes reported in the literature reflect the activity of highly specialized centres. The aim of this study was to evaluate the outcomes following pancreatic surgery worldwide.Methods: This was an international, prospective, multicentre, cross-sectional snapshot study of consecutive patients undergoing pancreatic operations worldwide in a 3-month interval in 2021. The primary outcome was postoperative mortality within 90 days of surgery. Multivariable logistic regression was used to explore relationships with Human Development Index (HDI) and other parameters.Results: A total of 4223 patients from 67 countries were analysed. A complication of any severity was detected in 68.7 percent of patients (2901 of 4223). Major complication rates (Clavien-Dindo grade at least IIIa) were 24, 18, and 27 percent, and mortality rates were 10, 5, and 5 per cent in low-to-middle-, high-, and very high-HDI countries respectively. The 90-day postoperative mortality rate was 5.4 per cent (229 of 4223) overall, but was significantly higher in the low-to-middle-HDI group (adjusted OR 2.88, 95 per cent c.i. 1.80 to 4.48). The overall failure-to-rescue rate was 21 percent; however, it was 41 per cent in low-to-middle-compared with 19 per cent in very high-HDI countries.Conclusion: Excess mortality in low-to-middle-HDI countries could be attributable to failure to rescue of patients from severe complications. The authors call for a collaborative response from international and regional associations of pancreatic surgeons to address management related to death from postoperative complications to tackle the global disparities in the outcomes of pancreatic surgery (NCT04652271; ISRCTN95140761)