Amenably Treatable Pediatric Severe Epilepsies
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 (including 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. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - June 6, 2016 Category: Neurology Authors: Phillip L. Pearl Source Type: research
Emerging Antiepileptic Drugs for Severe Pediatric Epilepsies
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). (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - June 4, 2016 Category: Neurology Authors: Basanagoud Mudigoudar, Sarah Weatherspoon, James W. Wheless Source Type: research
Emerging Anti-Epileptic Drugs for Severe Pediatric Epilepsies
The medical management of the epilepsy syndromes of early childhood (eg., infantile spasms, Dravet syndrome, Lennox-Gastaut syndrome, etc.) is challenging; and requires careful evaluation, classification and treatment. Pharmacological treatment continues to be the mainstay of therapy for these children, and as such it is important for the clinician to be familiar with the role of new anti-epileptic drugs (AEDs). This review reports the clinical trial data and personal experience in treating the severe epilepsies of childhood with the recently FDA approved AEDs (vigabatrin, rufinamide, perampanel, clobazam) and those in cli...
Source: Seminars in Pediatric Neurology - June 4, 2016 Category: Neurology Authors: Basanagoud Mudigoudar, Sarah Weatherspoon, James W. Wheless Source Type: research
Current and Emerging Therapies of Severe Epileptic Encephalopathies
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. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - June 1, 2016 Category: Neurology Authors: Abeer J. Hani, Mohamad A. Mikati Source Type: research
The Expanding Clinical Spectrum of Genetic Pediatric Epileptic Encephalopathies
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...
Source: Seminars in Pediatric Neurology - June 1, 2016 Category: Neurology Authors: Rolla Shbarou, Mohamad A. Mikati Source Type: research
Current and Emerging Therapies of Severe Epileptic Encephalopathies
Recent advances in genetics have markedly impacted our understanding of multiple epilepsy syndromes and is expanding our therapeutic options. Epileptic encephalopathies (EEs) comprise a group of clinical entities each with a specific disease course and with electroencephalographic (EEG) abnormalities that cause progressive disturbances of cerebral functions.1 Failure to appropriately recognize an EE in question results in ongoing cognitive and behavioral impairments. EEs are often resistant to conventional antiepileptic drugs (AEDs), hence the interest in new and emerging therapies. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - June 1, 2016 Category: Neurology Authors: Abeer J. Hani, Mohamad A. Mikati Source Type: research
The Expanding Clinical Spectrum of Genetic Pediatric Epileptic Encephalopathies
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 (EIEE) or Ohtahara Syndrome, Early Myoclonic Encephalopathy (EME), Epilepsy of Infancy with Migrating Focal Seizures, West Syndrome, Severe Myoclonic Epilepsy in Infancy (Dravet Syndrome), Landau-Klef...
Source: Seminars in Pediatric Neurology - June 1, 2016 Category: Neurology Authors: Rolla Shbarou, Mohamad A. Mikati Source Type: research
Current and Emerging Surgical Therapies for Severe Pediatric Epilepsies
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 electroencephalogram (EEG), changes in electrocorticography, and other emerging techniques. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - May 30, 2016 Category: Neurology Authors: Carrie R. Muh Source Type: research
Should We Treat Electroencephalographic Discharges in the Clinic or in the Intensive Care Unit, and if So When and How?
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. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - May 23, 2016 Category: Neurology Authors: James J. Riviello Source Type: research
Effect of Seizures on the Developing Brain and Cognition
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 anim...
Source: Seminars in Pediatric Neurology - May 23, 2016 Category: Neurology Authors: Gregory L. Holmes Source Type: research
Pediatric Sudden Unexpected Death in Epilepsy: What Have we Learned from Animal and Human Studies, and Can we Prevent it?
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. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - May 23, 2016 Category: Neurology Authors: Rebecca L. Holt, Eric Arehart, Arsen Hunanyan, Nina A. Fainberg, Mohamad A. Mikati Source Type: research
Should we Treat EEG Discharges in the Clinic or in the ICU, and if so when and how?
The important question that often emerges in the clinic is how aggressive the therapy of non-convulsive status epilepticus and electrical status epilepticus in sleep ought to be and how continuous the discharges in each of these two entities should be before therapy is aimed at them. Additionally, as the use of EEG monitoring continues to expand to include the clinic and Intensive Care Unit (ICU) populations, it is important to identify epileptiform patterns that warrant identification and treatment. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - May 23, 2016 Category: Neurology Authors: James J. Riviello Source Type: research
Pediatric Epileptic Encephalopathies: Pathophysiology and Animal Models
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. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - May 23, 2016 Category: Neurology Authors: Li-Rong Shao, Carl E. Stafstrom Source Type: research
