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Seminars in Pediatric Neurology

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https://www.readbyqxmd.com/read/27544475/current-and-emerging-therapies-of-severe-epileptic-encephalopathies
#1
Abeer J Hani, Mohamad A Mikati
In this article, we review the treatment options for the pediatric epileptic encephalopathies and provide an update on the new and emerging therapies targeted at the underlying pathophysiology of many of these syndromes. We illustrate how the identification of the specific genetic and autoimmune causes has made possible the evaluation and development of novel, better targeted therapies, as and at times, avoidance of potentially offending agents.
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544474/emerging-antiepileptic-drugs-for-severe-pediatric-epilepsies
#2
Basanagoud Mudigoudar, Sarah Weatherspoon, James W Wheless
The medical management of the epilepsy syndromes of early childhood (eg, infantile spasms, Dravet syndrome, and Lennox-Gastaut syndrome) is challenging; and requires careful evaluation, classification, and treatment. Pharmacologic therapy continues to be the mainstay of management for these children, and as such it is important for the clinician to be familiar with the role of new antiepileptic drugs. This article reports the clinical trial data and personal experience in treating the severe epilepsies of childhood with the recently Food and Drug Administration-approved new antiepileptic drugs (vigabatrin, rufinamide, perampanel, and clobazam) and those in clinical trials (cannabidiol, stiripentol, and fenfluramine)...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544473/amenable-treatable-severe-pediatric-epilepsies
#3
Phillip L Pearl
Vitamin-dependent epilepsies and multiple metabolic epilepsies are amenable to treatment that markedly improves the disease course. Knowledge of these amenably treatable severe pediatric epilepsies allows for early identification, testing, and treatment. These disorders present with various phenotypes, including early onset epileptic encephalopathy (refractory neonatal seizures, early myoclonic encephalopathy, and early infantile epileptic encephalopathy), infantile spasms, or mixed generalized seizure types in infancy, childhood, or even adolescence and adulthood...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544472/should-we-treat-electroencephalographic-discharges-in-the-clinic-or-in-the-intensive-care-unit-and-if-so-when-and-how
#4
REVIEW
James J Riviello
The important question that often emerges in the clinic is how aggressive the therapy for nonconvulsive status epilepticus and electrical status epilepticus in sleep ought to be and how continuous the discharges in each of these 2 entities should be before therapy is aimed at them. Additionally, as the use of electroencephalographic monitoring continues to expand to include the clinic and intensive care unit populations, it is important to identify epileptiform patterns that warrant identification and treatment...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544471/current-and-emerging-surgical-therapies-for-severe-pediatric-epilepsies
#5
Carrie R Muh
The use of epilepsy surgery in various medically resistant epilepsies is well established. For patients with intractable pediatric epilepsy, the role of intracranial electrodes, resective surgery, hemispherectomy, corpus callosotomy, neurostimulation, and multiple subpial transections continues to be very effective in select cases. Newer treatment and diagnostic methods include laser thermal ablation, minimally invasive surgeries, stereo electroencephalography, electrocorticography, and other emerging techniques...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544470/the-expanding-clinical-spectrum-of-genetic-pediatric-epileptic-encephalopathies
#6
Rolla Shbarou, Mohamad A Mikati
Pediatric epileptic encephalopathies represent a clinically challenging and often devastating group of disorders that affect children at different stages of infancy and childhood. With the advances in genetic testing and neuroimaging, the etiologies of these epileptic syndromes are now better defined. The various encephalopathies that are reviewed in this article include the following: early infantile epileptic encephalopathy or Ohtahara syndrome, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, West syndrome, severe myoclonic epilepsy in infancy (Dravet syndrome), Landau-Kleffner syndrome, Lennox-Gastaut syndrome, and epileptic encephalopathy with continuous spike-and-wave during sleep...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544469/pediatric-sudden-unexpected-death-in-epilepsy-what-have-we-learned-from-animal-and-human-studies-and-can-we-prevent-it
#7
REVIEW
Rebecca L Holt, Eric Arehart, Arsen Hunanyan, Nina A Fainberg, Mohamad A Mikati
Several factors, such as epilepsy syndrome, poor compliance, and increased seizure frequency increase the risks of sudden unexpected death in epilepsy (SUDEP). Animal models have revealed that the mechanisms of SUDEP involve initially a primary event, often a seizure of sufficient type and severity, that occurs in a brain, which is vulnerable to SUDEP due to either genetic or antecedent factors. This primary event initiates a cascade of secondary events starting, as some models indicate, with cortical spreading depolarization that propagates to the brainstem where it results in autonomic dysfunction...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544468/effect-of-seizures-on-the-developing-brain-and-cognition
#8
REVIEW
Gregory L Holmes
Epilepsy is a complex disorder, which involves much more than seizures, encompassing a range of associated comorbid health conditions that can have significant health and quality-of-life implications. Of these comorbidities, cognitive impairment is one of the most common and distressing aspects of epilepsy. Clinical studies have demonstrated that refractory seizures, resistant to antiepileptic drugs, and occurring early in life have significant adverse effects on cognitive function. Much of what has been learned about the neurobiological underpinnings of cognitive impairment following early-life seizures has come from animal models...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544467/focal-cortical-dysplasia-in-childhood-epilepsy
#9
Tarek Shaker, Anne Bernier, Lionel Carmant
Focal cortical dysplasia is a common cause of medication resistant epilepsy. A better understanding of its presentation, pathophysiology and consequences have helped us improved its treatment and outcome. This paper reviews the most recent classification, pathophysiology and imaging findings in clinical research as well as the knowledge gained from studying genetic and lesional animal models of focal cortical dysplasia. This review of this recently gained knowledge will most likely help develop new research models and new therapeutic targets for patients with epilepsy associated with focal cortical dysplasia...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544466/pediatric-epileptic-encephalopathies-pathophysiology-and-animal-models
#10
REVIEW
Li-Rong Shao, Carl E Stafstrom
Epileptic encephalopathies are syndromes in which seizures or interictal epileptiform activity contribute to or exacerbate brain function, beyond that caused by the underlying pathology. These severe epilepsies begin early in life, are associated with poor lifelong outcome, and are resistant to most treatments. Therefore, they represent an immense challenge for families and the medical care system. Furthermore, the pathogenic mechanisms underlying the epileptic encephalopathies are poorly understood, hampering attempts to devise novel treatments...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544465/introduction
#11
EDITORIAL
Mohamad A Mikati
No abstract text is available yet for this article.
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27464367/in-memoriam-steven-leroy-linder-md-1945%C3%A2-2015
#12
Roy D Elterman
No abstract text is available yet for this article.
February 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27464366/in-memoriam-donaldw-lewis-md-1951%C3%A2-2012
#13
Andrew D Hershey, Jack Gladstein, Kenneth J Mack
No abstract text is available yet for this article.
February 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27017029/migraine-and-hormones
#14
REVIEW
Ann Pakalnis
This article discusses the role that hormones play in adolescent girls and young women with headaches, which are very common in adolescent girls, in particular, migraine. In many cases, migraine onset may occur shortly around the time of menarche, prevalence of recurrent migraine in this population approaches 15%, and typically the symptoms continue through adulthood. Hormonal changes associated with puberty and the menstrual cycle may significantly influence migraine in young women. This article reviews the following topics: management of menstrually related headaches, changes in ovarian hormones and their relationship to migraine, and oral contraceptives and pregnancy effects on migraine...
February 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27017028/the-comorbidity-of-migraine-and-epilepsy-in-children-and-adolescents
#15
Michael K Sowell, Paul E Youssef
Migraine and epilepsy share a number of clinical attributes, including pathophysiology and clinical expression. Both are paroxysmal in nature and thus constitute episodic disorders, yet either may be chronic and/or recurrent. Epileptic seizures and migraine headaches may be mistaken one for the other and may even overlap. In particular, occipital lobe seizures may be misdiagnosed as migraine auras. In this article, we review the relationship between migraine and epilepsy, including the known genetic contributions to both conditions, prodromal, ictal, and postictal headache and shared pathophysiology and treatment options...
February 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27017027/infant-colic
#16
Amy A Gelfand
This article reviews the evidence for an association between infant colic and migraine. Infant colic, or excessive crying in an otherwise healthy and well-fed infant, affects approximately 5%-19% of infants. Multiple case-control studies, a cross-sectional study, and a prospective cohort study have all found an association between infant colic and migraine. Although infant colic is often assumed to have a gastrointestinal cause, several treatment trials aimed at gastrointestinal etiologies have been negative...
February 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27017026/dizziness-and-orthostatic-intolerance-in-pediatric-headache-patients
#17
REVIEW
William Qubty, Sita Kedia
Children and adolescents with headaches commonly complain of dizziness that creates significant distress among the patients and their families. Dizziness is largely due to either orthostatic intolerance or vertigo; this distinction is the initial step in the evaluation of a child with co-occurrence of headaches and dizziness. Vertiginous symptoms are most commonly due to vestibular migraine or benign positional vertigo. This review would focus its attention on the diagnosis, evaluation, and management of orthostatic intolerance, specifically postural orthostatic tachycardia syndrome...
February 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27017025/comorbid-psychological-conditions-in-pediatric-headache
#18
REVIEW
Hope L O'Brien, Shalonda K Slater
Children and adolescents with chronic daily headaches (CDH) often have comorbid psychological conditions, though their prevalence is unclear. Pediatric patients with CDH may have higher rates of disorders such as anxiety and depression. However, some researchers have found that scores on depression and anxiety screening measures for pediatric patients with migraine are within reference range. Barriers to identify patients with psychiatric disorders have included limited validated screening tools and lack of available mental health resources...
February 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27017024/medical-comorbidities-in-pediatric-headache
#19
Howard Jacobs, Samata Singhi, Jack Gladstein
Comorbid conditions frequently occur in pediatric headaches and may significantly affect their management. Comorbidities that have been associated with pediatric headaches include attention-deficit or hyperactivity disorder, autism, developmental disabilities, depression, anxiety, epilepsy, obesity, infantile colic, atopic disorders, inflammatory bowel disease, and irritable bowel syndrome. The goal of this article is to review these comorbidities associated with pediatric headache, thereby empowering child neurologists to identify common triggers and tailor management strategies that address headache and its comorbidities...
February 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27017023/migraine-and-puberty-potential-susceptible-brain-sites
#20
Nasim Maleki, Carolyn Bernstein, Vitaly Napadow, Alison Field
Puberty is a sensitive and critical period for brain development. The relationship between developmental processes in the brain during puberty and the onset of migraine disease in relation to the potential sites of susceptibility in the brain remains largely unknown. There are few data on how such processes interact with each other in influencing the migraine onset during puberty or even later in adulthood. Focusing on the migraine brain during pubertal development may provide us with a "window of opportunity" both to better understand the mechanisms of the disease and, also more importantly, to effectively intervene...
February 2016: Seminars in Pediatric Neurology
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