A wide range of treatment options is available

Once the diagnosis of epilepsy has been made, consideration turns to appropriate treatment . Some forms of childhood epilepsy may not require any intervention other than education and reassurance For example, benign Rolandic epilepsy is a common idiopathic localization-related epilepsy of childhood that spontaneously resolves by the age of 20.8 Benign Rolandic epilepsy is also referred to as benign epilepsy with centro-temporal spikes (BECTS). Approximately 60 of patients with this condition...

Case presentation

Psg Seizure Spike And Wave

This is a nearly 3 -year-old boy who was completely well until 6 months ago when he experienced two brief (< 2 minute) generalized convulsions 1 month apart, both associated with fever Although these were diagnosed as simple febrile seizures, he was referred to a pediatric neurologist who ordered a routine 1 5T brain magnetic resonance imaging (MRI) scan . This study was interpreted as normal . However, the outpatient electroencephalogram (EEG) study revealed generalized background slowing...

What is the ketogenic diet

The ketogenic diet (KD) is a high-fat, adequate protein, very low carbohydrate diet that is both calorie and fluid restricted and carefully calculated by a ketogenic diet-trained dietitian to create and maintain ketosis 2 Typical foods eaten include butter, eggs, cheese, heavy whipping cream, canola and olive oils, mayonnaise, green vegetables, chicken, hot dogs, and ground beef Sugar-free and carbohydrate-free snacks can be incorporated to help make the KD more palatable Although the KD's...

Cost effectiveness

Previous fiscal analyses for VNS therapy indicate that the initial costs of VNS are offset over time by reductions in health care costs and hospital admissions following implantation 5 The reductions in the economic burden for both patients and society were seen even among patients with less than a 25 reduction in seizures, indicating that even those without a substantial improvement in seizure frequency receive some benefit from the device Therefore, the decision to proceed with VNS therapy...

Longterm outcome

Electroencephalography Bruxism

Although many patients respond to antiepileptic drug therapy, one-third of patients may continue to be refractory to various medical treatments Long-term outcome data on patients with ADNFLE are lacking, but it has been observed that many patients experience seizures well into adulthood reft j * __ I Z> 5t I e a I 3. A, B Electroencephalogram demonstrating a nocturnal seizure with rhythmic delta activity evolving over the frontal head regions.

Suggested reading

Bast T, Ramantani G, Seitz A, Rating D . (2006) . Focal cortical dysplasia prevalence, clinical presentation and epilepsy in children and adults . Acta Neurol. Scand. 113, 72-81 . Cepeda C, Andre VM, Levine MS et al . (2006) . Epileptogenesis in pediatric cortical dysplasia the dysmature cerebral developmental hypothesis . Epilepsy Behav. 9, 219-235 . Colombo N, Tassi L, Galli C et al. (2003) . Focal cortical dysplasia MR Imaging, histo-pathologic and clinical correlation in surgically treated...

Safety

Figure Four Epilepsy

Adverse events reported with VNS therapy are generally transient and mild, and are often related to the duration and intensity of stimulation . Serious adverse events have not been reported with standard therapy, and no patients have died or had a higher mortality risk as a result of VNS therapy.4 The most common adverse events reported during the clinical trials were mild hoarseness or voice alterations, coughing, and paresthesia (primarily at the implant site and decreasing over time) and...

Aeds Eliminated By Hepatic Metabolism And Renal ExCRETION

Many of the currently available AEDs (i e , ethosuximide, felbamate, levetiracetam, oxcarbazepine, phenobarbital, topiramate and zonisamide) are eliminated by a combination of metabolism and renal excretion Ethosuximide is eliminated primarily by CYP3A4-dependent metabolism, with approximately 20 excreted unchanged in the urine The weight-adjusted oral clearance of ethosuximide is higher in children than adults, and the ratio of ethosuximide concentration to dose was found to be 50 higher in...

Diagnostic approach

The clinical entity of CSWS, also called electrical status epilepticus of sleep (ESES), was initially described by Patry et al . in 1971 . CSWS is diagnosed on the basis of the percentage of the sleep EEG recording comprising spike-wave activity The traditional definition requires that the spike-wave index be greater than 85 during slow-wave sleep In addition to the EEG criteria, the appearance of neuropsychological regression is required during the period of CSWS activity The incidence of CSWS...

Pediatric Epilepsy Encompasses A Wide Range Of Disorders

Hypsarrhythmia West Syndrome Eeg Pattern

Imagine sitting in the waiting room of a pediatric epilepsy clinic and observing the variety of patients awaiting their turn to be evaluated. A 6-year-old child, initially referred for staring spells, is now here for a follow-up appointment with well-controlled childhood absence epilepsy. In a wheelchair you see a 13-year-old child with spastic quadriparetic cerebral palsy, moderate mental retardation, and poorly controlled symptomatic localization-related epilepsy. She is here to have the...

Pathophysiologyneurobiology of disease

JME is strongly genetically linked and several genes have been implicated that, when mutated, can alone or in combination cause the disorder Although the family history is positive in up to 50 of patients, the inheritance patterns are often not clear-cut and suggest a process influenced by multiple genes and possibly other factors . The net result of one or multiple gene defects in combination with other factors leads to increased neuronal excitability, especially a tendency for thalamocortical...

F

F-flourodeoxyglucose positron emission tomography, 185 Facial angiofibromas, 128 Facial contraction with exaggerated grimace, 119 Facial venous angioma without cerebral angiomatosis, 155 Fasting See Hypoglycemia FCD See Focal cortical dysplasia FDG-PET scan See F-flourodeoxyglucose positron emission tomography Febrile seizures, 87-91 case presentation, 87 clinical pearls, 90 defined, 87 diagnostic approach, 88 differential diagnosis, 87-88 long-term outcome, 89-90 neurobiology, 90...

V

FIGURE 16.2 A 2-Deoxy-2- 18F fluoro-D-glucose FDG positron emission tomography PET scan showed multiple areas of glucose hypometabolism black arrows in both the left and right hemispheres B a nC methyl-L-tryptophan AMT PET scan showed intense uptake in a right frontal tuber FIGURE 16.3 Histopathology of the resected right frontal cortex using hematoxylin and eosin stain H and E showing the presence of giant cells left arrow and balloon cells right arrow FIGURE 16.3 Histopathology of the...

Pathophysiology of uld

The gene responsible for ULD was identified using a positional cloning approach . The disease-causing gene, named CSTB or EPM1, is a 674 base-pair gene that contains 3 exons and localizes to chromosome 21q22 . 3 . EPM1 encodes a previously known but unmapped cysteine protease inhibitor called cystatin B CSTB . The identification of two point mutations found in EPM1 proved that this gene was responsible for ULD To date, a total of eight different point mutations have been identified in EPM1 Some...

Pathologies and etiologies

Frequent pathologies seen in infants and children who are candidates for epilepsy surgery include hypoxic-ischemic injury and stroke, malformation of cortical development, or dysembryoplastic neuroepithelial tumors such as ganglioglioma and gangliocytoma Also considered should be tuberous sclerosis complex, vascular malformations, and Sturge-Weber syndrome Mesial temporal sclerosis is rare in infants but is becoming increasingly recognized in older children and adolescents 14 Additionally,...

Delineation of the epileptogenic zone and eloquent areas of cortex

Left Insular Region

The preoperative workup aims at identification of the epileptogenic zone, which is defined as the area of cortex primarily responsible for the generation of clinical seizures . 13 The goal of epilepsy surgery is the resection or isolation through disconnection of the epileptogenic zone and the preservation of eloquent cortex Seizure freedom after resection is therefore regarded as ultimate proof of the successful localization of the epileptogenic zone The epileptogenic zone can be identified...

Aeds eliminated by mixed cyp ugt and other metabolic pathways

Several of the AEDs i . e . , clobazam, clonazepam, tiagabine, valproic acid are extensively metabolized by multiple metabolic pathways, with minimal excretion of drug unchanged in the urine Predicting age-related effects is more difficult because of the larger intersubject variability in the fraction eliminated by each pathway and lack of data on the effect of age on the non-CYP and UGT enzymes Clobazam is eliminated predominately by hepatic metabolism to multiple metabolites The primary...

Treatment strategy

The first question to consider once the correct diagnosis has been made is whether or not treatment is indicated The clinician and family must balance the side effects of medication against the impact of ongoing seizures on the developing brain Unfortunately, the first of these two is more apparent and quantifiable than the latter Given the available evidence, treatment should be offered to families with the knowledge that its ultimate effect on outcome is uncertain Based on limited case series...

Febrile Seizures

Case Differential Diagnostic Treatment Strategy Long-Term Outcome Neurobiology Pathophysiology of Disease Clinical Pearls Suggested Reading Scenario 1 A 12-month-old child has been irritable and lethargic for the last 12 hours . He feels warm to the touch and has been placed in lukewarm water baths . Suddenly, he stiffens and then becomes limp with fine shaking of all extremities for less than 1 minute. Within approximately 15 minutes, he returns to his pre-seizure status . Scenario 2 Same...

Clinical approach

Although HH is relatively uncommon with a prevalence of about 1 in 100,000, it is almost certainly underdiagnosed by medical caregivers The gelastic seizures may initially not be typical, and the parents may simply be aware that something is wrong or that they have a baby who laughs too much . Equally common, the seizures may be more of a dycrastic, or crying, seizure, sometimes associated with strained, painful, and paroxysmal but stereotypical discomfort spells that may resemble...

Clinical pearls

Differentiation of absence and complex partial seizures often relies on EEG characteristics and may require prolonged EEG video monitoring in some cases 2 MRI should be performed in most children with partial epilepsy instead of CT to evaluate for subtle abnormalities, such as focal cortical dysplasias 3 Most patients with partial epilepsy will respond to treatment with antiepileptic medications Failure to respond to two or more medications should prompt a referral to an epilepsy...

Kevin Chapman MD

Barrow Neurological Institute, Phoenix, AZ Barrow Neurological Institute, Phoenix, AZ Taylor amp Francis Group, an informa business Cover illustration courtesy of the Barrow Neurological Institute 2007, Barrow 6000 Broken Sound Parkway NW, Suite 300 2009 by Taylor amp Francis Group, LLC CRC Press is an imprint of Taylor amp Francis Group, an Informa business No claim to original U.S. Government works Printed in the United States of America on acid-free paper International Standard Book...

Differential diagnosis

Diagnostic considerations include nonreflex epilepsies that the child's parents or caregivers have erroneously associated with certain activities, particularly if only a few seizures have occurred Proprioception or praxis-induced seizures may be confused with kinesigenic dyskinesias or with self-stimulatory behaviors in children with pervasive developmental disorders Language and or reading epilepsy may be confused with dyslexia, tics, or stuttering, and, in fact, acquired stuttering may be the...

Contributors

Professor of Pharmacy Department of Pharmacy University of Washington Seattle, Washington Danielle M. Andrade, M.D., M.Sc. Assistant Professor of Neurology Krembil Neuroscience Centre Toronto Western Hospital University of Toronto Toronto, Canada Division of Pediatric Neurology Mattel Children's Hospital at UCLA David Geffen School of Medicine at UCLA University of California at Los Angeles Los Angeles, California Clinical Neurophysiology Fellow Barrow Neurologic Institute St . Joseph's...

THE VAGuS Nerve StimuLation Therapy System

Vagus nerve stimulation VNS , which attenuates seizure frequency, severity, and duration by chronic intermittent stimulation of the vagus nerve, is intended for use as an adjunctive treatment with antiepileptic drug AED therapies . As of January 2008, more than 45,000 patients with epilepsy have been implanted with the VNS therapy system worldwide, with approximately 30 of those patients being younger than age 18 at the time of their first implant Approximately one-third of patients receiving...

Outcome

As in CSE, the outcome of NCSE is related to the underlying etiology. This is especially so with acute encephalopathies associated with NCSE or NCS . To date, no pediatric studies have shown a worse outcome in patients with NCSE or NCS, and it is thought that electrographic seizure activity may be an additive stress to the injured brain Although not as severe as in CSE, cerebral hemodynamic alterations do occur in complex partial SE but tend to be more focal 1 . NCSE may develop after the...

JJJL I f

Self Induced Seizure

FIGURE 36.1 An 8-year-old self inducing a photoconvulsive seizure by waving his fingers before his eyes. Note how the physical induction precedes the polyspike discharge and clinical absence. FIGURE 36.1 An 8-year-old self inducing a photoconvulsive seizure by waving his fingers before his eyes. Note how the physical induction precedes the polyspike discharge and clinical absence. Audiogenic Musicogenic Speech-induced Photosensitive Flash Partial Primary auditory cortex Generalized Nondominant...