Parents reported improvement in quality of life and particularly in levels of alertness in a study of Danish children receiving the MA and ketogenic diets. Most children will experience some adverse effects of dietary therapies, although the MA and LGIT diets might be better tolerated than the classic ketogenic diet. Virtually all children experience gastrointestinal tract adverse effects, especially constipation, but also nausea and vomiting. Hypercholesterolemia is common but can often be addressed by modifying fat sources eg, decreasing butter in favor of coconut oil.
Many families report initial fatigue or lethargy, but these effects usually resolve spontaneously. An excellent guideline outlines the ideal evaluation, initiation, and ongoing treatment for patients receiving the ketogenic diet and its variations. Most centers initiate the classic ketogenic diet with an inpatient hospital admission. The admission allows for medical surveillance of hypoglycemia, dehydration, and acidosis, for example, and permits intensive education programs for caregivers.
Traditionally, the ketogenic diet was initiated with a period of fasting. However, a randomized trial demonstrated that gradual initiation of the diet, by increasing the ratio of fats to carbohydrates and protein for several days, is equally effective and better tolerated. Intensive follow-up, including the combined efforts of the dietician and neurologist, are required to maintain dietary therapies.
Patients must undergo assessment in person at regular intervals to measure growth variables and to evaluate adverse effects and efficacy. In addition, caregivers require frequent informal support via telephone calls and emails from nursing, dietary, and often social work staff. Follow-up care and laboratory testing should be tailored to meet individual patients' needs, but typically children must be examined in the clinic about every 3 months for the first year and then somewhat less frequently thereafter.
Micronutrient status must be assessed regularly because the ketogenic and MCT diets are known to result in nutritional deficiencies, 27 some of which can result in clinically important symptoms. For example, Bergqvist et al 28 described a patient whose selenium deficiency, induced by ketogenic diet therapy, resulted in heart failure.
Poor bone mineralization and levels of vitamin D below the laboratory reference range are known to be prevalent among patients with epilepsy, and these issues are exacerbated among those treated with ketogenic diets. Resective surgery may be indicated for children with treatment-resistant focal epilepsy if debilitating seizures continue despite appropriate antiseizure medications, and the potential benefit of seizure control outweighs the risk of resecting the cortex where the seizures originate the epileptogenic zone.
A careful presurgical evaluation is required to identify the epileptogenic zone and to determine whether resection of this area is likely to result in unacceptable new neurologic deficits.
Removal of the epileptogenic zone is necessary and sufficient to achieve seizure freedom 30 - 32 and offers the best hope for a cure among appropriately selected patients. The ideal surgical candidate will have a single, well-localized focus of seizure onset in noneloquent cortex.
The components of the presurgical evaluation are outlined in the Table. These data are evaluated by a multidisciplinary team, including neurologists, neurosurgeons, radiologists, psychologists, and social workers, to determine the best surgical strategy and the most likely risks and benefits of the proposed procedure.
Interictal epileptiform discharge patterns can assist the clinician to refine the choice of further presurgical studies and are associated with a good surgical prognosis when unifocal. A combination of neuroimaging modalities is often used to complement and corroborate the EEG findings and increase the clinicians' confidence in the epileptogenic zone localization.
The most widely used and reliable tool for identifying the ictal focus is magnetic resonance imaging MRI. The presence of a distinct lesion on MRI can help to guide the pathway for surgical candidacy and predict a favorable surgical outcome. In general, T1-weighted, T2-weighted, gadolinium contrast, fluid-attenuated inversion recovery, coronal, and axial images should be obtained.
When no lesion is identified on MRI, other noninvasive imaging modalities may delineate the epileptogenic zone. A nuclear medicine technique, peri-ictal single-photon emission computed tomography SPECT , and a subtraction image coregistered to MRI SISCOM may demonstrate a focal increase in blood flow at the time of a seizure, thereby localizing the region of seizure onset.
The time to injection is critical, with decreasing data reliability as the time between seizure onset and tracer injection increases. In a study of children with polymicrogyria, SISCOM not only identified the location of the epileptogenic zone but also served as a primary prognostication tool for epilepsy surgery. Fludeoxyglucose F 18—labeled positron emission tomography and subsequent voxel-based analysis using statistical parametric mapping may localize areas of decreased cerebral metabolism, which are presumed to represent the epileptogenic zone.
Magnetoencephalography is a relatively new functional imaging modality that delineates the epileptogenic zone by detecting magnetic fields produced by interictal epileptiform activity. Among children and adults, magnetoencephalography accurately localized the epileptogenic zone in All potential candidates for epilepsy surgery must undergo a neuropsychological assessment to evaluate presurgical cognition, the relationship of eloquent cortex to the epileptogenic lesion, and potential postsurgical deficits.
Typically, a battery of standardized neuropsychometric tests is administered to evaluate general intelligence, attention, executive functioning, memory, behavior, and motor and sensory functions with a focused speech and language assessment. For language and memory lateralization, additional testing is often required.
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The intracarotid amobarbital procedure also known as Wada testing involves injection of amobarbital sodium via invasive angiography into 1 carotid artery to inactivate the ipsilateral cerebral hemisphere temporarily, allowing memory and language testing of the contralateral hemisphere.
Intracranial EEG monitoring is most often used to refine the localization of extratemporal epileptogenic zones. Surgically placed subdural electrodes are used regularly for intracranial monitoring in infants and children. Such EEG monitoring can allow precise localization of the seizure focus and an opportunity for cortical function mapping, minimizing the size of the resection and avoiding resection of eloquent cortex. The efficacy and outcome of pediatric surgery varies widely depending on the procedure. Because the epileptogenic zone can extend beyond a visible brain lesion, a traditional lesionectomy may not be sufficient to render the patient free of seizures.
Although a lesionectomy minimizes the resection of normal surrounding tissue, a tailored resection aims at maximizing the resection of electrically abnormal tissue and may extend beyond the borders of the visible lesion. No conclusive randomized studies compare lesionectomy with tailored resection, and the choice of procedure is individualized on a case-by-case basis, often guided by EEG data recorded from subdural electrodes.
Patients with brain tumors can present with acute symptomatic seizures or chronic epilepsy. The most common epileptogenic neoplasms are low-grade tumors, including astrocytoma, mixed glioma, oligodendroglioma, and dysembryoplastic neuroepithelial tumor. Identification of these tumors has therapeutic and prognostic implications, with surgical resection offering the best survival outcome, reducing seizure burden, and limiting long-term effects of chemotherapy and radiation therapy.
Hemispheric malformations of cortical development primarily or exclusively involve a significant or a complete portion of 1 cerebral hemisphere and are characterized by treatment-resistant epilepsy, intellectual disability, and often contralateral hemiparesis. These malformations include hemimegalencephaly, porencephaly, and other similar but less well-characterized hemispheric malformations. Such lesions are often amenable to aggressive, early surgical intervention, with preservation of the child's cognitive functioning when seizures are controlled.
Rasmussen encephalitis is an acquired, slowly progressive disorder characterized by treatment-resistant focal seizures that may evolve into epilepsia partialis continua, worsening contralateral hemiparesis, and cognitive dysfunction, with corresponding progressive hemispheric atrophy.
Causes of Epilepsy in Childhood
In general, rates of seizure freedom after hemispherectomy are lower among children with hemispheric malformations of cortical development than those with acquired disorders, such as Rasmussen encephalitis or ischemic stroke, because of commonly coexisting pathology. Postsurgical care varies widely among institutions. A suggested approach for short-term follow-up of children undergoing epilepsy surgery is outlined in the Table.
Depending on the magnitude of the resection, patients may require short- or medium-term physical medicine and rehabilitation services. For those whose seizure outcome is favorable, medication therapy can often be slowly tapered after an appropriate waiting period months, depending on the clinical scenario. Patients with persistent seizures may benefit from follow-up neuroimaging to evaluate for subtotal resection of the epileptogenic lesion. Additional surgery is sometimes an option if results of repeated EEG and imaging studies suggest that an improved outcome will be achieved. For some children with challenging epilepsy syndromes, focal resection is not an option and complete seizure freedom is not the goal.
Rather, reducing the seizure-related morbidity and thereby improving quality of life is a reasonable objective. Options can include corpus callosotomy or multiple subpial transections MSTs. Corpus callosotomy is far from a new surgical technique, but it retains a role in palliation for some children with debilitating generalized seizures, particularly those with Lennox-Gastaut syndrome and frequent atonic seizures. Some patients cannot undergo epilepsy surgery because resection of primary speech, motor, sensory, or visual cortex would result in unacceptable functional deficits.
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To overcome this challenge, MST was developed. The MSTs interrupt the horizontal synchronizing neuronal networks while preserving vertical functional units. In 1 study, 12 of 26 children with varying neuropathologic findings dysplasia, tumor, etc who underwent limited cortical resection followed by MST became seizure free. Despite the decades-long interest in neurostimulation for reducing seizure frequency and severity, the development of devices and procedures for clinical use is relatively recent.
The most widely used and best known neurostimulation device is the vagus nerve stimulator VNS.
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The VNS generator is implanted under the skin in the left pectoral area, with a wire leading to the left vagus nerve. The generator is programmed to deliver a current at regular intervals, with an option for manual activation to provide a stronger signal when needed, to abort seizures. In the s, animal studies showed that VNS reduced interictal epileptiform discharges.
Studies have indicated VNS to be a well-tolerated and safe therapeutic option when resective epilepsy surgery is not feasible. Ideal candidates are those whose cognitive and motor abilities allow them to activate the device manually at the onset of a seizure. Children with persistent seizures who did not tolerate or are not candidates for dietary therapies or surgical options are also potential candidates. Adverse effects of VNS include hoarseness of voice, coughing, or throat discomfort, all of which are usually transient.
Gastroesophageal reflux is also commonly aggravated. Vagal nerve stimulation may exacerbate obstructive sleep apnea, and untreated apnea has adverse medical effects and can worsen seizure control. Thus, clinicians should screen for any history suggestive of sleep apnea when patients undergo evaluation for treatment with VNS.
Other areas of the brain have also been targets of electrical stimulation for epilepsy treatment. The basis of developing these techniques is interruption of epileptiform activity in the epileptogenic zone or the pathways along which seizures propagate. Besides neurostimulation, other novel methods are being developed to treat seizures, such as localized cerebral hypothermia, local drug perfusion, and the use of optical light stimulation to cerebral cortex.
Despite the recent introduction of several new antiseizure medications, many children with epilepsy have treatment-resistant seizures. Evaluation by a pediatric epileptologist should be considered for these children to determine whether they may be candidates for dietary therapies, epilepsy surgery, or VNS. Early and aggressive consideration of nonmedication therapies for treatment-resistant childhood epilepsy can identify some children whose seizures can be cured or significantly reduced, providing the best chance for optimal neurodevelopmental outcome and quality of life.
Correspondence: Sucheta M. Submitted for Publication: February 13, ; final revision received April 17, ; accepted April 18, Published Online: November 12, Analysis and interpretation of data : Joshi, Singh, and Shellhaas. Drafting of the manuscript : Joshi, Singh, and Shellhaas.
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The mission of the Epilepsy Foundation is to lead the fight to overcome the challenges of living with epilepsy and to accelerate therapies to stop seizures, find cures, and save lives. Skip to main content. Sign In Register find us donate. Causes of Epilepsy in Childhood. Seizures in children have many causes.
Common causes of childhood seizures or epilepsy include fever these are called febrile seizures genetic causes head injury infections of the brain and its coverings lack of oxygen to the brain hydrocephalus excess water in the brain cavities disorders of brain development Most seizures in childhood are not associated with a definite cause, however.
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