Olugbenga Akingbola

The First Association of a Primary Amebic Meningoencephalitis Death With Culturable Naegleria fowleri in Tap Water From a US Treated Public Drinking Water System

BACKGROUND Naegleria fowleri is a climate-sensitive, thermophilic ameba found in warm, freshwater lakes and rivers. Primary amebic meningoencephalitis (PAM), which is almost universally fatal, occurs when N. fowleri-containing water enters the nose, typically during swimming, and migrates to the brain via the olfactory nerve. In August 2013, a 4-year-old boy died of meningoencephalitis of unknown etiology in a Louisiana hospital. METHODS Clinical and environmental testing and a case investigation were initiated to determine the cause of death and to identify potential exposures. RESULTS Based on testing of cerebrospinal fluid and brain specimens, the child was diagnosed with PAM. His only reported water exposure was tap water; in particular, tap water that was used to supply water to a lawn water slide on which the child had played extensively prior to becoming ill. Water samples were collected from both the home and the water distribution system that supplied the home and tested; N. fowleri was identified in water samples from both the home and the water distribution system. CONCLUSIONS This case is the first reported PAM death associated with culturable N. fowleri in tap water from a US treated drinking water system. This case occurred in the context of an expanding geographic range for PAM beyond southern states, with recent case reports from Minnesota, Kansas, and Indiana. This case also highlights the role of adequate disinfection throughout drinking water distribution systems and the importance of maintaining vigilance when operating drinking water systems using source waters with elevated temperatures.

Pediatric noninvasive positive pressure ventilation.

Objective To review the clinical use of noninvasive positive pressure ventilation (NPPV) in both acute hypoxic and hypercarbic forms of pediatric respiratory failure, including its mechanism of action and implementation. Data Sources Studies were identified through a MEDLINE search using respiratory failure, pediatrics, noninvasive ventilation, and mechanical ventilation as key words. Study Selection All original studies, including case reports, relating to the use of noninvasive positive pressure in pediatric respiratory failure were included. Because of the paucity of published literature on pediatric NPPV, no study was excluded. Data Extraction Study design, numbers and diagnoses of patients, types of noninvasive ventilator, ventilator modes, outcome measures, and complications were extracted and compiled. Data Synthesis For acute hypoxic respiratory failure, all the studies reported improvement in oxygenation indices and avoidance of endotracheal intubation. The average duration of NPPV therapy before noticeable clinical improvement was 3 hrs in most studies, and NPPV was applied continuously for 72 hrs before resolution of acute respiratory distress. In patients with acute hypercarbic respiratory failure, application of NPPV resulted in reduction of work of breathing, reduction in CO2 tension, and increased serum bicarbonate and pH. These patients are older than patients in the acute hypoxic respiratory failure group and, in addition to improved blood gas indices, they reported improvement in subjective symptoms of dyspnea. Improvement in gas exchange abnormalities and subjective symptoms occurred within the same time span (the first 3 hrs) as in the acute hypoxic respiratory failure group. However, use of noninvasive techniques in patients with acute hypercarbic respiratory failure continued after resolution of acute symptoms. Complications related to protracted use of NPPV were common in this group. Conclusions NPPV has limited benefits in a group of carefully selected pediatric patients with acute hypoxic and hypercarbic forms of respiratory failure. The routine use of this technique in pediatric respiratory failure needs to be studied in randomized controlled trials and better-defined patient subsets.

Dexmedetomidine to Treat Lisdexamfetamine Overdose and Serotonin Toxidrome in a 6-Year-Old Girl

A 6-year-old girl displayed symptoms of serotonin syndrome after accidental ingestion of Vyvanse (lisdexamfetamine dimesylate). Dexmedetomidine was administered because of persistent neuromuscular hyperactivity and severe agitation despite initial therapy with benzodiazepines. Some children show a paradoxical reaction to benzodiazepines, and dexmedetomidine has a possible role in the treatment of serotonin syndrome.

Emergency Management of Hypertension in Children

Systemic arterial hypertension in children has traditionally been thought to be secondary in origin. Increased incidence of risk factors like obesity, sedentary life-styles, and faulty dietary habits has led to increased prevalence of the primary arterial hypertension (PAH), particularly in adolescent age children. PAH has become a global epidemic worldwide imposing huge economic constraint on health care. Sudden acute increase in systolic and diastolic blood pressure can lead to hypertensive crisis. While it generally pertains to secondary hypertension, occurrence of hypertensive crisis in PAH is however rare in children. Hypertensive crisis has been further subclassified depending on presence or absence of end-organ damage into hypertensive emergency or urgency. Both hypertensive emergencies and urgencies are known to cause significant morbidity and mortality. Increasing awareness among the physicians, targeted at investigation of the pathophysiology of hypertension and its complications, better screening methods, generation, and implementation of novel treatment modalities will impact overall outcomes. In this paper, we discuss the etiology, pathogenesis, and management of hypertensive crisis in children. An extensive database search using keywords was done to obtain the information.

Prolonged atrial fibrillation precipitated by new-onset seizures and marijuana abuse.

We report a case of prolonged atrial fibrillation (AF) precipitated by new-onset generalized tonic–clonic convulsions and marijuana abuse in a developmentally normal 18-year-old adolescent with a structurally normal heart. Our case highlights an interesting association and a unique pathophysiology between generalized tonic–clonic convulsions, marijuana abuse, and AF. We suggest that seizures and marijuana abuse should be considered in the differential diagnosis of the etiology of AF in children.

Incidence, Trends, and Outcomes of Cerebral Edema Among Children With Diabetic Ketoacidosis in the United States

Introduction. There are limited data regarding the incidence, trends, and outcomes of cerebral edema among patients with diabetic ketoacidosis (DKA). Methods. NIS database was used from year 2002 to 2012. Cases with primary diagnosis of DKA were identified using International Classification of Diseases, Ninth Revision–Clinical Modification (ICD-9 CM) code 250.1 x. Cerebral edema patients were identified using ICD-9 CM code 348.5. We compared the baseline characteristics of both groups to estimate differences using the χ2 test, Student’s t test, Wilcoxon rank-sum test, and survey regression depending on the distributions of variables. For trend analysis, the χ2 test of trend for proportions was used using the Cochrane Armitage test via the “trend” command in Statistical Analysis Software (SAS). Multivariate odds ratios were calculated. P value for <0.05 was considered as significant for all analysis. Results. In all, 205 (weighted n = 974) cases of cerebral edema were identified among 52 049 (weighted n = 246 925) DKA patients, which estimates the incidence of cerebral edema at 0.39%. Trends of incidence of developing cerebral edema increased almost 2 times, from 0.34 in 2002 to 0.64 in 2012 (P < 0.001). Univariate analysis showed that both length of stay (LOS; 3 vs 2; P < 0.001) and cost of hospitalization (

De Novo Heterogeneous Mutations in SCN2A and GRIN2A Genes and Seizures With Ictal Vocalizations

10 530 vs

Asymptomatic Mycoplasma Infection Causing Acute Demyelinating Encephalomyelitis Case Report and Review of Literature

3953; P < 0.001) were statistically higher among those who developed cerebral edema. Conclusion. Our study shows that over the study period, trend in incidence of cerebral edema among DKA patients has increased. Patients with cerebral edema were found to have longer LOS and higher cost of hospitalization.

Functional Adrenal Insufficiency Complicating an Overdose of Naproxen and Ibuprofen

Seizures are the most common pediatric neurologic disorder. Approximately 30 000 among the 150 000 children, who will sustain a first-time, unprovoked seizure each year, will develop epilepsy. The incidence of epilepsy is highest among children younger than 3 years of age. Approximately 40% of the seizures in this population it is secondary to epileptic encephalopathies. Epileptic encephalopathies (EE) are a heterogeneous group of epileptic disorders that occur early in life. Epileptic Encephalopathies have been classified into various syndromes depending upon the age of onset that include early infantile epileptic encephalopathy (EIEE)/ Ohtahara syndrome (OS), Infantile spasm (West syndrome), late infantile epileptic encephalopathy, Dravet syndrome, Early myoclonic encephalopathy, Malignant migrating partial seizures of infancy and LennoxGastaut syndrome. It has been postulated that some of these syndromes may represent a spectrum of diseases along a continuum, which may evolve overtime with maturation of the nervous system. To date, 28 types of EIEE have been reported based on the de novo mutations (http://www.omim.org). These syndromes are characterized by intractable seizures resistant to anticonvulsant treatment, persistent severe electroencephalographic abnormalities, and progressive cognitive dysfunction. Epileptic encephalopathies are commonly due to sporadic, de novo dominant mutations in a single autosomal gene. However, autosomal recessive and X-linked recessive forms have also been reported. Advances in the genetic testing methods like whole exome sequencing (WES) and whole genome CNV (copy number variants) sequencing (WGS) has led to identification of newer sporadic de novo autosomal mutations causing epileptic encephalopathies. Hitherto, sporadic de novo heterogeneous mutations in voltage gated sodium channel (VGSC) [SCN2A] and N – Methyl D – Aspartate (NMDA) receptor [Glutamate receptor ionotropic NMDA2A] (GRIN2A) genes occurring individually have been reported to cause intractable pharmacoresistant seizures. However, these two mutations occurring simultaneously in a phenotypically normal neonate with structurally normal brain presenting with intermittent clusters of intractable seizures associated with ictal vocalizations have not been reported. Here, we report a case of early infantile epileptic encephalopathy/Ohtahara syndrome (EIEE/OS) secondary to sporadic de novo heterogeneous mutations in SCN2A and GRIN2A genes in a phenotypically normal neonate with structurally normal brain who presented with intermittent, clusters of intractable seizures associated with ictal vocalizations. To our knowledge this is first such case report.

High-Dose Tissue Plasminogen Activator, Topical Nitroglycerin, and Heparin for Severe Ischemic Injury in a Neonate

Acute demyelinating encephalomyelitis (ADEM) is a rare autoimmune acute demyelinating disease with an incidence of 0.64 per 100 000 persons per year with a slight male predominance. It is characterized by an intense inflammatory reaction that leads to demyelination in the brain and spinal cord. The pathological changes are localized to perivenous regions; hence, it is also known as perivenous encephalomyelitis. It has been reported to occur following various bacterial and viral infections (postinfectious encephalomyelitis) and after vaccinations (postvaccination encephalomyelitis). Mycoplasma pneumoniae is a common cause of community-acquired pneumonia (CAP). Central nervous system (CNS) complications occur in up to 0.1% of patients with M pneumoniae infection. These complications include encephalitis, aseptic meningitis, polyradiculitis, cerebellar ataxia, and myelitis. Rarely, ADEM has been seen following infection with M pneumoniae. We report and discuss a case of severe ADEM following asymptomatic mycoplasma infection.