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Seminars in Pediatric Neurology

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https://www.readbyqxmd.com/read/27989333/inherited-pain-syndromes-and-ion-channels
#1
REVIEW
Francis J DiMario
Individuals rely on the perception of pain to avoid injury, to signal disease, and to warn about tissue inflammation and damage. However, the inheritance of inappropriate, extreme, or inadequate pain production is a source of significant human suffering. Substantial progress has been made in our understanding of the genetics and pathophysiology of pain through the study of individuals and families with several specific inherited pain syndromes. These studies have led to the discovery of a number of gene mutations associated with specific ion channel disturbances that produce familial inherited pain sensitivity and insensitivity syndromes...
August 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27989332/neuropathic-and-myopathic-pain
#2
REVIEW
Anthony C Rodrigues, Peter B Kang
The evaluation and management of childhood pain syndromes of neuromuscular origin have distinct challenges, as the patterns of disease presentation and the ability of a child to describe symptoms may differ from that of an adult. Advances in scientific and clinical knowledge are leading to significant progress in the care of affected children. The genetic origins of Fabry disease and the inherited form of erythromelalgia are better understood. The increasing interest in neuroimmunology among pediatric neurologists has led to more sophisticated diagnostic and therapeutic approaches...
August 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27989331/juvenile-fibromyalgia-a-primary-pain-or-pain-processing-disorder
#3
REVIEW
Lawrence Zemel, Peter R Blier
Juvenile fibromyalgia (JFM), a chronic disorder of widespread musculoskeletal pain in combination with autonomic, sensory, and cognitive dysfunction, is responsible for considerable morbidity and impaired quality of life in affected patients and their families. Historically, fibromyalgia has been incorrectly characterized as a psychosomatic or psychogenic disorder, but new understanding of the science of pain has demonstrated unambiguously that it is an organic disorder of the pain processing system itself...
August 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27989330/pain-amplification-syndrome-a-biopsychosocial-approach
#4
REVIEW
Lisa B Namerow, Emily C Kutner, Emily C Wakefield, Barbara R Rzepski, Robert A Sahl
Pediatric neurologists frequently encounter patients who present with significant musculoskeletal pain that cannot be attributed to a specific injury or illness, which can often be defined as pain amplification syndrome (PAS). PAS in children and adolescents is the result of a heightened pain sensitivity pathway, which is intensified by significant biological, psychological, and social contributors. Appropriate assessment and multimodal intervention of PAS are crucial to treatment success, including neurology and behavioral health collaborative treatment plans to restore patient function and reduce pain perception...
August 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27989329/nonpharmacologic-treatment-of-pain
#5
REVIEW
Anna Monica Agoston, Christine B Sieberg
Pain is a complex biopsychosocial experience that is influenced by neurological processes and psychosocial factors. Systematic reviews and meta-analyses of randomized controlled trials of psychological interventions have demonstrated evidence for psychological approaches in treating procedural pain and multiple types of chronic pain, including headaches, abdominal pain, and musculoskeletal pain. This article is directed toward clinicians and would provide an overview of cognitive-behavioral therapy, including specific cognitive-behavioral techniques for pediatric pain...
August 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27989328/pharmacologic-treatment-of-pain
#6
REVIEW
Eapen Mathew, Eugene Kim, William Zempsky
Adolescents and children are frequently affected by chronic pain conditions that can lead to disability and distress. The best approach to evaluation and treatment of these conditions involves use of the biopsychosocial model, which includes use of medication management. Chronic pain conditions are treated pharmacologically with a number of different medication classes via several routes of administration as drug delivery systems have progressed. These include anti-inflammatory drugs, muscle relaxants, antiepileptic medicines, antidepressants, opioids, and local anesthetics...
August 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27989327/pathogenesis-of-pain
#7
REVIEW
Pradeep Dinakar, Alexandra Marion Stillman
The pathogenesis of pain sensation includes mechanisms that result in acute or chronic pain. Pain itself is described as an unpleasant sensory and emotional experience beginning with a peripheral stimulus that undergoes a physiological process ultimately resulting in the sensation of pain. Biologists recognize pain to be a common sign of potential tissue damage. Hence, pain sensation is protective in function. However, pathologic states of pain exist secondary to disruption of the nociceptive process both peripherally and centrally or secondary to psychological conditions...
August 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27989326/pediatric-pain-measurement-assessment-and-evaluation
#8
REVIEW
Renee C B Manworren, Jennifer Stinson
Assessment provides the foundation for diagnosis, selection of treatments, and evaluation of treatment effectiveness for pediatric patients with acute, recurrent, and chronic pain. Extensive research has resulted in the availability of a number of valid, reliable, and recommended tools for assessing children's pain. Yet, evidence suggests children's pain is still not optimally measured or treated. In this article, we provide an overview of pain evaluation for premature neonates to adolescents. The difference between pain assessment and measurement is highlighted; and the key steps to follow are identified...
August 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27989325/introduction
#9
EDITORIAL
Francis J DiMario
No abstract text is available yet for this article.
August 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544475/current-and-emerging-therapies-of-severe-epileptic-encephalopathies
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
EDITORIAL
Mohamad A Mikati
No abstract text is available yet for this article.
May 2016: Seminars in Pediatric Neurology
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