Journal of Pediatric Epilepsy - Volume 1, issue 4

Authors: Kanemura, Hideaki | Aihara, Masao

Article Type: Review Article

Abstract: Epilepsy with continuous spike-waves during slow sleep (CSWS) is characterized by impairment of neuropsychological abilities, frequently associated with behavioral disorders. These manifestations strongly correlate with frontal lobe dysfunctions. CSWS in childhood may affect the prefrontal cortex and leave residual mental and behavioral abnormalities as profound and permanent as the loss of speech in Landau-Kleffner syndrome. In our previous volumetric studies, frontal and prefrontal lobe volumes revealed growth disturbance in all CSWS patients compared with those of normal subjects. These studies also revealed that in the patients with shorter seizure durations and CSWS periods, developmental abnormalities of the prefrontal lobes were …soon restored to a more normal growth ratio, whereas growth disturbances of the prefrontal lobes were persistent in the patients with longer seizure durations and CSWS periods. These findings suggest that seizure and the duration of paroxysmal anomalies may be associated with prefrontal lobe growth abnormalities, which are associated with neuropsychological problems. Thus, the duration of CSWS period seems to be a significant prognostic factor. The urgent suppression of this electroencephalography abnormality may be necessary to prevent the progression of neuropsychological impairments. The best treatment options may be required to remit seizures and electroencephalography abnormalities as soon as possible to achieve optimal prognosis in CSWS. Show more

Keywords: CSWS, cognition, behavior, prefrontal lobe, brain volumetry, MRI

DOI: 10.3233/PEP-12030

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 4, pp. 187-193, 2012

Authors: Prayson, Richard A.

Article Type: Review Article

Abstract: Surgery is a well established option in treating patients with medically intractable epilepsy. Better outcomes are achieved in surgical candidates who have magnetic resonance imaging (MRI) lesions present prior to surgery. In a minority of cases, no discernible MRI lesion is evident prior to surgery (nonlesional). The most commonly documented histopathologic findings in this clinical setting are focal cortical dysplasias, nonspecific gliosis, hippocampal sclerosis and no identifiable light microscopic alteration. There are a number of potential explanations for the absence of light microscopic findings in a subset of MRI-negative or nonlesional epilepsy patients related to the surgical resection being off-target, …pathologic tissue sampling, interobserver and intraobserver variability in the diagnosis of certain pathologies, evolving pathology and lesions that are beyond light microscopic detection. This paper reviews the definition of nonlesional epilepsy, the pathologies encountered in this setting, possible explanations for the absence of apparent light microscopic abnormalities in some cases, and future directions. Show more

Keywords: Epilepsy, chronic epilepsy, nonlesional epilepsy, focal cortical dysplasia, hippocampal sclerosis, gliosis

DOI: 10.3233/PEP-12031

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 4, pp. 195-201, 2012

Authors: Anyanwu, Chinekwu | Motamedi, Gholam K.

Article Type: Review Article

Abstract: About half of patients with epilepsy will become seizure free upon treatment with the first antiepileptic drug (AED). After trying further medications, overall, two thirds of patients will become seizure free on AED(s), while the remaining one third will continue having seizures. Patients who have failed treatment with adequate trials of two appropriate and tolerated AEDs, whether as monotherapy or in combination, are considered refractory (drug resistant). Appropriate diagnosis and optimal control of seizures are essential for a successful treatment and improved quality of life of patients with epilepsy. Since the introduction of a number of newer AEDs in the …last decade, there have been improvements in tolerability and side effect profile of AED therapy. Failure to respond to the initial pharmacologic treatment should prompt trials of alternative options including reevaluation for accurate diagnosis and optimizing AED therapy, epilepsy (resective) surgery, vagus nerve stimulation, ketogenic diet, and a series of potentially effective pharmacological and surgical treatments currently under investigation. Show more

Keywords: Pediatric refractory epilepsy, drug resistant epilepsy, pharmacotherapy

DOI: 10.3233/PEP-12032

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 4, pp. 203-209, 2012

Authors: Huang, Cyrus | Marsh, Eric D. | Ziskind, Daniela M. | Celix, Juanita M. | Peltzer, Bradley | Brown, Merritt W. | Storm, Phillip B. | Litt, Brian | Porter, Brenda E.

Article Type: Research Article

Abstract: Identify seizure onset electrodes that need to be resected for seizure freedom in children undergoing intracranial electroencephalography recording for treatment of medically refractory epilepsy. All children undergoing intracranial electroencephalography subdural grid electrode placement at the Children’s Hospital of Philadelphia from 2002–2008 were asked to enroll. We utilized intraoperative pictures to determine the location of the electrodes and define the resection cavity. A total of 15 patients had surgical fields that allowed for complete identification of the electrodes over the area of resection. Eight of 15 patients were seizure free after a follow up of 1.7 to 8 yr. Only one …seizure-free patient had complete resection of all seizure onset associated tissue. Seizure free patients had resection of 64.1% of the seizure onset electrode associated tissue, compared to 35.2% in the not seizure free patients (P = 0.05). Resection of tissue associated with infrequent seizure onsets did not appear to be important for seizure freedom. Resecting ≥ 90% of the electrodes from the predominant seizure contacts predicted post-operative seizure freedom (P = 0.007). The best predictor of seizure freedom was resecting ≥ 90% of tissue involved in majority of a patient’s seizures. Resection of tissue under infrequent seizure onset electrodes was not necessary for seizure freedom. Show more

Keywords: Epilepsy, epilepsy surgery, cortical dysplasia, neocortical epilepsy, intracranial electroencephalography

DOI: 10.3233/PEP-12033

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 4, pp. 211-219, 2012

Authors: Metwellay, Kotb A. | Farghaly, Hekma S. | Darweesh, Alaa E.M. | Hamed, Sherifa A.

Article Type: Research Article

Abstract: Cognitive delay is a frequent neurobehavioral manifestation in children with tuberous sclerosis complex (TSC). This study aimed to determine the relationships between cognitive delay with respect to seizure characteristics, interictal electroencephalography (EEG) changes, behavioral abnormalities and brain tubers’ count and location. This study included 24 children with TSC with a mean age of 6.2 ± 2.55 yr. Children underwent clinical, psychometric, interictal EEG and magnetic resonance imaging or magnetic resonance imaging of the brain. Nearly 58.33% of children had cognitive delay and 45.83% had autism. Compared to children without cognitive delay, children with cognitive delay were younger (P < …0.01), had higher frequencies of seizures (particularly infantile spasms with onset predominantly below 6 mo of age) (P < 0.01), severe epileptogenic EEG changes, high tuber burden (particularly in the left hemisphere with frontal and temporal predominance) (P < 0.01) and frequent behavioral abnormalities as autism (P < 0.001) and attention deficit hyperactivity disorders (P < 0.05). Cognitive scores were correlated with age of seizures’ onset (r = 0.432, P = 0.01), presence of infantile spasms (r = −0.675, P = 0.001), autism (r = −0.350, P = 0.05), and number of brain tubers (r = −0.865, P = 0.0001) but non correlated to age nor gender. In multivariate analysis, only the age of seizure onset (odds ratio 1.90; 95% confidence interval 1.02 to 3.55, P = 0.044) and the number of brain tubers (odds ratio 2.36; 95% confidence interval 0.95 to 5.81; P = 0.06) increased the odds for cognitive delay among TSC patients. The knowledge of the clinical features of TSC can help in early diagnosis and management of patients with a multidisciplinary consultation. Show more

Keywords: Tuberous sclerosis, brain tubers, cognitive delay, infantile spasms, autism, attention deficit hyperactivity disorder

DOI: 10.3233/PEP-12034

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 4, pp. 221-228, 2012

Authors: Abd El-Aziz, Sahar A. | El-Serogy, Hesham

Article Type: Research Article

Abstract: Status epilepticus (SE) represents a medical emergency that is associated with a high morbidity and mortality. The most accepted definition of SE is a condition during which epileptic activity persists for 30 min or longer or is repeated frequently enough that the individual does not regain consciousness between seizures. Neuron-specific enolase (NSE) is a marker of acute brain injury and blood-brain barrier dysfunction, which is elevated in SE. Serum NSE is an accepted marker of acute brain injury, and an increase in NSE has been correlated with the duration and outcome of generalized convulsive SE. Increase in serum NSE in …generalized convulsive SE would provide new information about the degree of brain injury in generalized convulsive SE. The aim of this study was to evaluate whether convulsive SE causes brain damage or not, based on the serum levels of NSE and to determine the correlation between the serum NSE and the duration of SE. Thirty children with convulsive SE, 20 males and 10 females with the age range 6–14 yr and the mean age was (9.1 ± 2.3 yr). Thirty healthy children 18 males and 12 females with a similar age distribution and sex, their age range was 8–14 yr and the mean age was (9.9 ± 1.6 yr) served as the control children. Twenty children with idiopathic epilepsy 15 males and five females, newly diagnosed suffering from various types of seizures generalized and partial types, aged 1 yr to 14 yr with the mean age was 10.1 ± 3.1 yr for comparison. Serum NSE levels were measured in children with SE 24 hr after the control of seizures and compared with the control children and children with idiopathic epilepsy, the duration of SE was determined and correlated with the peak NSE level. The mean serum NSE was significantly higher in children with convulsive SE than the control children and children with idiopathic epilepsy (P < 0.001), also there was significant correlation between the serum NSE and the duration of seizures in children with SE. Children with convulsive SE had significantly higher serum levels of NSE than the control children. The extremely higher levels of NSE in convulsive SE reflect the severity of the acute neurological insults. In addition, higher serum NSE levels in generalized convulsive SE reflect the long duration of SE and the potential for brain injury. Show more

Keywords: Neuronal injury, neuron-specific enolase, status epilepticus, children

DOI: 10.3233/PEP-12035

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 4, pp. 229-235, 2012

Authors: Keiner, Doerthe | Gaab, Michael R. | Schroeder, Henry W.S. | Baldauf, Joerg | Oertel, Joachim

Article Type: Research Article

Abstract: In the past years, neuroendoscopic procedures have been applied with a constantly broadening of indications. One major indication for neuroendoscopic surgery is the physiological restoration of cerebrospinal fluid circulation in obstructive hydrocephalus. Possible complications include contusion of fornix, hypothalamus or pituitary gland or lesion of cranial nerves. Only rare data exist on the onset of epileptic seizures after neuroendoscopic procedures. Between April 1993 and October 2007, 134 neuroendoscopic procedures were performed in 126 children with different intracranial pathologies. The postoperative clinical course and the outcome of the patients was analyzed retrospectively with special regard to pre- and postoperative epileptic seizures. …Endoscopic surgery was performed for the treatment of aqueductal stenosis in 23 patients (18.3%), intracranial tumor formation in 48 patients (38.1%), intracranial cysts in 24 patients (19%), brain malformation in 20 patients (15.9%), intracerebral hemorrhage in six patients (4%) and isolated ventricle in five patients (4.8%). Most children presented with mild to severe signs of increased intracranial pressure. Postoperatively, transient complications occurred in 17 cases (12.7%) and permanent complications occurred in two patients (1.5%). In seven children, seizures have been observed preoperatively. The seizures have been observed in different pathologic entities. Additionally, a convulsive disorder did not occur postoperatively as a complication. A new onset of seizures after neuroendoscopic surgery has not been found. The present data do not speak for an increased risk of developing a convulsive disorder in children after neuroendoscopic surgery. Show more

Keywords: Epileptic seizures, neuroendoscopy, surgical technique, occlusive hydrocephalus, endoscopic third ventriculostomy

DOI: 10.3233/PEP-12036

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 4, pp. 237-242, 2012

Authors: Rana, Kamer S. | Roy, Shuvendu | Singh, Daljit

Article Type: Research Article

Abstract: A cross-sectional study was done in a pediatric neurology referral center over 2 yr. Our aim was to study the incidence and profile of intractable epilepsies. Children of either sex; presented with seizures were screened for intractability. Children with intractable seizures were further evaluated for intractability. Two hundred and sixty one children with seizures were identified as having intractable epilepsy. Pseudo-intractability was observed in 69% (non-epileptic events 35%, poor compliance, precipitating factors and surgical causes 24%, neurological symptoms wrongly diagnosed as seizures and pseudo seizures following true seizures in 9%). True medical intractability constituted 31%.

Keywords: Epilepsy, intractable seizures

DOI: 10.3233/PEP-12037

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 4, pp. 243-248, 2012

Article Type: Other

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 4, pp. 249-251, 2012

Article Type: Other

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 4, pp. 253-254, 2012