OBJECTIVES:The objectives of the study were to compare the free serum concentrations after different fosphenytoin loading dose strategies in patients younger than 6 months old and to investigate the frequency of seizure cessation following a loading dose of fosphenytoin.METHODS:This retrospective cohort study included neonates and infants admitted to a 150-bed children's hospital between August 1, 2014, and February 1, 2018. Patients were included if they were younger than 6 months old and had a postload free phenytoin serum concentration collected during the specified time frame. Patients were identified through a database query screening for the inclusion criteria. Patients were separated into 2 groups with the 15 mg/kg group as per protocol and the 20 mg/kg group as noted in common practice. Data collection included demographic information, fosphenytoin dose, time of administration of the fosphenytoin loading dose, time of sampling, free phenytoin serum concentration results, concomitant antiepileptic agents, albumin serum concentration, and total bilirubin serum concentration.RESULTS:Forty-one patients were included for analysis, 12 in the 15 mg/kg group and 29 in the 20 mg/kg group. The average free phenytoin concentration after the loading dose was 2.45 ± 0.54 mg/L in the 15 mg/kg group and 2.52 ± 0.66 mg/L in the 20 mg/kg group. Seizure cessation after the fosphenytoin loading dose was achieved in 3 of 12 (25%) patients in the 15 mg/kg group and in 13 of 29 (45%) patients in the 20 mg/kg group (p = 0.305).CONCLUSIONS:The study demonstrates that a traditional range of fosphenytoin loading dose (15-20 mg/kg) led to elevated postloading dose free phenytoin serum concentrations in the majority of patients with a seizure cessation rate of approximately 39%. The question remains as to what the optimal dose and target concentration should be in this patient population to achieve the best efficacy without risking associated toxicities.

Objective:This study describes the use of continuous intravenous valproate as an abortive therapy for pediatric status migrainosus.Background:Intravenous valproate as a bolus dose has been shown to be an effective abortive therapy for status migrainosus in children; however, Valproate's pharmacokinetic profile suggests that it would be safer and more therapeutic as a continuous infusion. This dosing strategy results in less serum concentration fluctuations, more consistent therapeutic effects, and less adverse effects.Methods:A retrospective chart review between August 2009 and August 2012 identified 83 patients who had presented with status migrainosus and had received continuous intravenous valproate after failing to respond to initial abortive treatment. These patients had received a 20mg/kg loading dose, followed by continuous infusion at 1mg/kg/h. Serum valproate levels had been drawn 4 hours and 24 hours after the loading dose. Infusion rate had been adjusted to maintain serum levels of 80 to 100mcg/mL. Age-appropriate pain assessments had been recorded at regular intervals. Excellent response was defined as a 100% reduction in pain scores, moderate response as 50% to 99% reduction, and poor response as <50% reduction.Results:Of the 83 patients, 55 (66.2%) had reported an excellent response, 4 (4.8%) a moderate response, and 24 (28.9%) a poor response. Of those reporting an excellent response, 76% had responded within 24 hours. Nausea (8.4%) and vomiting (2.4%) had been the only reported side effects. Twenty-four hour serum levels had been within the goal range 91.9% of the time.Conclusions:Continuous intravenous valproate is safe, easy to monitor, and therapeutic in the abortive treatment of status migrainosus in pediatric patients.

Introduction: Surfer’s myelopathy (SM) is a rare disorder described in subjects presenting with acute paraparesis while learning how to surf. It is thought to be secondary to spinal ischemia triggered by hyperextension. Spinal magnetic resonance imaging (MRI) shows changes consistent with spinal cord ischemia on T2-weighted and diffusion-weighted imaging (DWI). Case Presentation: We report two patients who presented with acute onset paraplegia shortly after spinal hyperextension. They had no physical or radiological evidence of soft tissue injury. Their clinical and imaging findings closely resemble those described in SM. Discussion: We propose the use of the term ‘acute hyperextension myelopathy’ to categorize patients with spinal cord infarction secondary to hyperextension. DWI sequencing on MRI should be considered to evaluate for early signs of spinal cord ischemia in these patients. Use of a broader term for diagnostic classification can help include patients with spinal cord infarction due to a common mechanism.

Valproic acid is a versatile antiepileptic drug that is often used in the acute care setting. Intravenous valproic acid lends itself well to a continuous infusion as it exhibits a relatively short half-life. We evaluated the pharmacokinetics and clinical efficacy of continuous infusion valproic acid in hospitalized patients with migraine and seizures.

OBJECTIVES: To study the efficacy of continuous intravenous valproic acid as an abortive therapy for pediatric migraine . BACKGROUND: Valproic acid (VPA) has been reported to be effective in status epilepticus and prophylaxis of migraine headache. IV VPA as bolus dose has also shown to be effective as an outpatient abortive therapy in children but its pharmacokinetic profile suggests that it would be more effect given as a continuous infusion. DESIGN/METHODS: This is a retrospective chart review of all children admitted to Kentucky Children Hospital from August 2009 to August 2012 and treated with IV VPA. Children in migraine status were treated by standard protocol: IV VPA load 20mg/kg followed by continuous infusion of 1 mg/kg/hour. Serum VPA levels were checked at 4, 24 and 48 hours. The target serum concentration was 100(+/-10),Standard pain scoring (FACES for ages 4-8 and VAS for >8 year) was used. Response was graded according to reduction in pain scoring as Excellent (> than 50 %) Moderate (> than 30 % but < than 50 %) and Poor (< than 30 %). RESULTS: 78 subjects met criteria. They were 24 boys (32%), 54 girls (69 %), ages 4 to 18 year (mean 12.9 +/-3.34 %). . 59 (75%)reported an excellent response, 9 (11.5 %) a moderate response and 10 (12.8%) a poor response. 68%with excellent response responded within first 12 hours. Age and gender did not affect the likelihood of response. The only reported side effect, Nausea, was noticed in 5 children. CONCLUSIONS: Continuous infusion IV VPA is a safe, rapidly effective, abortive agent in pediatrics Migraine. Randomized, double blind, controlled studies are warranted

The American Academy of Neurology (AAN) represents more than 24,500 physicians who manage and coordinate care for some of the highest cost Medicare beneficiaries with complex, often progressive neurologic conditions such as Alzheimer‘s disease, stroke and epilepsy. The AAN has serious concerns about the draft recommendation to Congress under consideration by MedPAC to repeal the SGR by instituting unilateral cuts for three years and then a seven year payment freeze to non-―primary care‖ physicians as was discussed during the Commission‘s September 14-15 meeting.

When parents ask the doctor why their child is not neurologically normal, or why the youngster's development is delayed, the arms and legs are weak, or behavior is unusual, the search for an explanation begins with pregnancy. Drs. Gilles and Nelson split this critical period, separating early pregnancy (before about 20 weeks of gestation) from late pregnancy (at 20 weeks of gestation through the perinatal period). This book focuses on normal and perturbed brain development during this late period.

The editors’ declared intention is to provide a comprehensive review and update of the newest diagnostic and therapeutic tools in pediatric neurology. This is a major task for a slim volume, and the reader is left to guess about the editors’ inclusion and exclusion criteria. Because the authors are all European (and most of the chapters are affiliated with Italian centers), this volume appears to offer us a geography-based update.

If you want to learn what is happening in the broad and rapidly developing field of behavior characterized as “the autism spectrum,” this paperback volume is just for you. It is broadly inclusive, and is likely to cover any areas in which you have an interest. Simon Baron-Cohen has it right when he states in the Foreword, “This book tackles the challenge of early detection; the complex biology of autism, including genetics (from linkage, to association, to copy number variations), the autistic brain (from the perspectives of magnetic resonance imaging, diffusion tensor imaging, magnetoencephalography, neuroanatomy and epilepsy) and molecular aspects of autism (from serotonin to oxytocin); the complex psychology of autism (from cognitive models, language, memory and executive function); and the challenge of education in autism.”

Although the associations between self-injurious behavior and Lesch-Nyhan syndrome, and between autism and Rett syndrome, are well-known, the relationships between other genetic disorders and behaviors often pass unrecognized. This multi-author volume explores these relationships in depth. It contains three sections. Section one, which describes the observed neurobehavioral manifestations of selected genetic syndromes in detail, will comprise the most interesting section for most readers. The opening chapter discusses the difficulties in defining behavioral phenotypes, and uses Lesch-Nyhan syndrome to demonstrate associations between behavior and brain function. The author’s review of the pathophysiology of the Lesch-Nyhan movement disorder as well as the separate orbitomedial cortical abnormality associated with injurious behaviors is particularly interesting. Subsequent chapters discuss the clinical features, neurobehavioral problems, and sleep disturbances of Smith-Magenis syndrome, and the range of abnormal behaviors in fragile X syndrome (including carriers and those with premutations), Down syndrome, Prader-Willi and autism, Williams syndrome with its relatively preserved language abilities, and fetal alcohol syndrome and related degrees of in utero exposure to alcohol.

The editors of this multiauthored volume endeavor to describe how chronic pediatric illnesses can adversely affect brain function and cause cognitive and behavioral changes. By and large, they are successful. Although the authors acknowledge that chronic illnesses per se can cause behavioral and cognitive problems, they reject the concept that chronic illness alone can explain all of the cognitive and behavioral components of those illnesses that do not primarily involve the brain. They emphasize the neurologic etiology of many of these problems and endeavor to separate the effects of the specific chronic illness from the manifestations of secondary neurological dysfunctions. This nuanced understanding of the behavioral and cognitive problems of children with chronic illness is widely accepted in the child neurology community but is not well recognized within pediatrics. A text that gathers these data in one place represents a major opportunity for child neurologists to find common ground with their pediatric associates. A unifying theme in all of the chapters—and one especially valuable to the reader—is an emphasis on explaining the biological and developmental mechanisms underlying the neurological dysfunctions that can be seen in various chronic diseases. This emphasis helps differentiate this text from other discussions of cognitive and behavioral problems in children with chronic illness and shows the unique contribution that child neurologists bring when these children are being evaluated.

This volume is the newest in the well-produced series of specialized reviews sponsored by the International Child Neurology Association, and published by the Mac Keith Press in the United Kingdom. The series emphasizes subjects of special interest to child neurologists. The senior editorship has passed from Peter Procopis in Sydney to Charles R.J.C. Newton in London, but given the length of time it takes to produce these reviews, the topic and authors for this volume were presumably Peter’s choice. The editors are American experts in this field, and they have assembled a distinguished group of authors who are, in turn, well-known for their expertise. They include, among others, the late Hugo Moser (to whom this volume is dedicated), Michael Brenner, Volkmar Gieselmann, Reuben Matalon, and Sakkubai Naidu.

At a time when there is a profusion of tests, but our knowledge of neurologic disorders is still fragmentary and incomplete, it is difficult to determine which of the available tests (if any) might benefit a patient. Practitioners are familiar with the usual handbooks, containing exhaustive lists of diagnoses and tests, that medical students and residents carry in their jackets. What is more difficult to find is a pocket text on laboratory investigations based on the idea that “the best pointer to the appropriate tests and the correct diagnosis is the clinical history” (p. 7). With their new edition of A Handbook of Neurological Investigations in Children, King and Stephenson have provided just such a book.

One of the favorite pursuits of child neurologists involves discussion of the various “spells” that look like epilepsy and are often treated as epilepsy, but that are actually nonepileptic. Perhaps it is the great number and variety of these events that please the neurologist, and cause anguish in anyone else who cares for these children. Dr. DiMario endeavors to entertain the former, and reassure the latter, with an extensive review that emphasizes the diagnostic clues leading to correct diagnoses, plus an epidemiologic overview of the extent of each problem. In considerable detail, he attempts to classify different types of events. He combines the phenomenology and circumstances of events with a child's behavior, age, and movements, to give readers a framework for differential diagnoses. This overview comprises the introductory section. In subsequent chapters, he covers specific entities, grouped as syncope and related events, sleep-related phenomena, conversion or somatoform disorders, factitious disorders, movement disorders, and headache syndromes.

This unique multiauthor book discusses the evaluation of neonates with neurologic disorders by means of three key laboratory investigations: magnetic resonance imaging and spectroscopy, electroencephalography (EEG), and cerebral ultrasound. The success of this approach, and it is very successful, says a great deal about the current state of the practice of neonatal neurology, namely, that it is heavily laboratory based.