Journal of Pediatric Epilepsy - Volume 1, issue 2

Authors: Holmes, Mark D.

Article Type: Research Article

DOI: 10.3233/PEP-2012-013

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 2, pp. 75-76, 2012

Authors: Jovic, Nebojsa J.

Article Type: Review Article

Abstract: Juvenile myoclonic epilepsy (JME) is a common form of idiopathic generalized epilepsy. Behavioral and cognitive problems are frequently observed in JME. Although the underlying mechanisms are still unknown, recent neuropsychological and neuroimaging studies provide strong evidence for frontal lobe dysfunction. Neuropsychological studies have shown JME patients to be impaired in a number of executive functions such as concept formation, abstract reasoning, mental flexibility, and cognitive speed and planning. They scored below average on measures of attention, immediate verbal memory, working memory, and verbal fluency. The patients with JME performed worse than the group with temporal lobe epilepsy on some frontal …tasks. Some specific personality features, such as unreliability and emotional instability, poor social adjustment, high rates of anxiety, mood disorders, phobias and somatization disorders seen in patients with JME have also been described in patients with frontal lobe-lesions. Quantitative magnetic resonance imaging studies have shown regional, frontal cortical and thalamic grey matter abnormalities. These findings support the pathophysiological concept of functional abnormalities in the thalamo-cortical circuit in JME. The additional impact of interictal epileptiform electroencephalography activity on cognitive functions was suggested. Poor working memory and impaired executive and motor performance in JME patients may also be attributed to altered dopamine signaling in the striatum and frontal lobes. JME may be a non-uniform condition. Different genetic and pathophysiologic mechanisms could underlie the clinically observed differences in prognosis, cognitive functioning, personality traits, and frontal lobe deficits. Show more

Keywords: Juvenile myoclonic epilepsy, frontal dysfunction

DOI: 10.3233/PEP-2012-014

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 2, pp. 77-85, 2012

Authors: Yerdelen, Deniz | Koç, Filiz

Article Type: Research Article

Abstract: Reflex seizures refer to epileptic seizures evoked by a specific external stimulus or internal mental process or by activity of the patient. Approximately 5% of all seizures are reflex epilepsy. The most common precipitants are visual. A variety of somatosensory phenomena including thinking, reading, listening to music, and eating may also induce reflex seizures. In this review, the most common reflex epilepsies are discussed.

Keywords: Reflex seizure, reflex epilepsy

DOI: 10.3233/PEP-2012-015

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 2, pp. 87-96, 2012

Authors: Lee, Jennifer Y. | Bruno, Patricia | Rabe, Olivia T. | Thiele, Elizabeth A. | Thibert, Ronald L.

Article Type: Research Article

Abstract: To determine the safety, tolerability, and efficacy of rufinamide in treating refractory epilepsy in a largely pediatric population. A retrospective chart review was conducted of all patients prescribed rufinamide from December 2008 to July 2010 in the Pediatric Epilepsy Program at Massachusetts General Hospital for Children. Eighty-eight patients on rufinamide with sufficient data for analysis were included in the study. Eight (9.1%) patients had >90% seizure reduction, 40 (45.5%) patients had 50–90% seizure reduction, 19 (21.6%) patients had <50% seizure reduction, and 21 (23.9%) patients had no change in seizure frequency. Generally mild side effects were reported by 28 (31.8%) …patients, with the two most common side effects being decreased appetite in seven (8.0%) patients, and somnolence in six (6.8%) patients. Rufinamide appears to be an effective, safe, and well-tolerated antiepileptic drug in the treatment of broad-spectrum intractable epilepsy, expanding outside its specific Food and Drug Administration approved usage in treatment of Lennox-Gastaut syndrome. Show more

Keywords: Rufinamide, epilepsy, pediatric

DOI: 10.3233/PEP-2012-016

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 2, pp. 97-101, 2012

Authors: Hamed, Sherifa Ahmad | Darwish, Essam Saad | Youssef, Ahmed Hamdy | Abo-Fadan, Naglaa H. | Abdellah, Mostafa M. | Bathalath, Ali Mabrok Salem

Article Type: Research Article

Abstract: Studies using multimodal sensory evoked potentials (SEPs) in children with epilepsy are lacking or few and controversial. We aimed to assess the SEPs, which included: (visual, brainstem and somatosensory evoked potentials) in children with epilepsy treated with carbamazepine (CBZ), valproate (VPA) or lamotrigine (LTG) monotherapy. Forty epileptic children and 25 healthy children were included. Compared to healthy children, children on VPA had prolonged P100 and waves III and IV latencies and reduced P100 amplitude. Children on CBZ had prolonged P100, waves IV and V and N20 latencies and III-V and N9–N20 interpeak latencies. Children on LTG had prolonged N145, waves …I, II, III and IV latencies. Significant correlations were identified between the dose of VPA and P100 amplitude (P = 0.001), the dose of CBZ and P100 (P = 0.016); wave V (P = 0.049) latencies and I–III (P = 0.047) and III–V (P = 0.031) interpeak latencies and between the duration of treatment with CBZ and wave IV and V (P = 0.004; P = 0.002) latencies, between the dose of LTG and N9 (P = 0.050), N11 (P = 0.035) and N20 (P = 0.030) latencies and N9–N11 (P = 0.017) and N9–N20 (P = 0.003) interpeak latencies. No significant correlations were identified between SEPs variables and age at onset or duration of illness. This study suggested that antiepileptic drugs (AEDs) might induce changes in central SEPs indicating central nervous system impairment secondary to AEDs. Although none of the children had manifest sensory changes, however, AEDs can induce clinical manifestations with chronic or long-term use. Show more

Keywords: Childhood, epilepsy, antiepileptic drugs, evoked potentials, valproate, carbamazepine, lamotrigine

DOI: 10.3233/PEP-2012-017

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 2, pp. 103-112, 2012

Authors: Di Rosa, Gabriella | Spanò, Maria | Lenzo, Patrizia | Parisi, Eleonora | Tripodi, Emanuela | Germanò, Eva | Siracusano, Rosamaria | Tortorella, Gaetano

Article Type: Research Article

Abstract: The relationship between migraine and epilepsy has been previously investigated in the literature. Several pathogenetical hypotheses have related the two conditions including the suggestion of a common genetic origin. The purpose of our study was to analyze the rate of migraine in our epileptic children population from Southern Italy followed-up at our unit between 2002 to 2005. Clinical data were retrospectively obtained from the patients' medical records. Migraine was far more frequent in subjects with idiopathic or cryptogenic epilepsy vs. non-epileptic controls with a 16.5 times higher risk ratio in the subjects with idiopathic or cryptogenic epilepsy vs. controls. The …group of patients with partial seizures had five-fold increased risk to have migraine compared to the group with generalized seizures. The plausible pathogenetic relationship between migraine and epilepsy needs to be further investigated in wider populations. The detection of a common background could provide evidence for a common and more effective pharmacological treatment of these two disorders. Show more

Keywords: Migraine, idiopathic, cryptogenic, epilepsy, seizures

DOI: 10.3233/PEP-2012-018

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 2, pp. 113-116, 2012

Authors: Caraballo, Roberto Horacio | García, Roberto | Betancourt, Bolivar Quito | Flesler, Santiago | Bartuluchi, Marcelo | Cersósimo, Ricardo

Article Type: Research Article

Abstract: We present 67 patients with early-onset shunted hydrocephalus and epilepsy, focusing on the prevalence of epileptic syndromes. Thirty-nine boys and 28 girls were studied. Mean follow-up was 12 yr (range, 9–17 yr). Forty-two patients had epilepsy. Epilepsy with focal seizures only was seen in 13 patients and West syndrome in 12 patients, preceded by focal symptomatic epilepsies in six. Eleven patients had epileptic encephalopathy with continuous spikes and waves during slow sleep, preceded by focal electroencephalography abnormalities in all and by West syndrome in one. Four patients presented with Lennox-Gastaut syndrome, of whom three had had West syndrome in the …first year of life. One patient had epilepsy with myoclonic-astatic seizures and another multifocal spikes associated with epileptic spasms in clusters. West syndrome evolved into focal seizures in seven. Seven patients had acute symptomatic seizures, secondary to shunt dysfunction in five and shunt infection in two. Focal epilepsy and epileptic encephalopathies were the most frequent syndromes. Hemorrhage, infections, and cortical dysplasia were associated with a higher prevalence of epilepsy. Show more

Keywords: Epilepsy, shunted hydrocephalus, syndrome, seizures

DOI: 10.3233/PEP-2012-019

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 2, pp. 117-123, 2012

Authors: Seinfeld, Syndi A. | Tye, Gary W. | Morton, Lawrence D.

Article Type: Research Article

Abstract: Epilepsy surgery is an option for many pediatric patients diagnosed with intractable seizures. Identifying pediatric patients that are candidates for surgery can be complicated. This case presented a 19-year-old female with intractable epilepsy who had chronic immune thrombocytopenic purpura and Dandy-Walker variant. The patient had previously been prescribed three appropriate anti-epileptic drugs and continued to experience seizures weekly. The patient had thrombocytopenia, which represented a potential for increased complications. The pre-operative work-up demonstrated heterotopias and dysplasias, which were felt to be the source of the focal onset seizures. She underwent focal resection and at the time of the last follow-up …visit, the patient had been seizure free for 2 yr. The outcome of the patient exemplified the importance of evaluating patients with posterior fossa abnormalities for epilepsy surgery. The data in patients with Dandy-Walker variant is limited. Show more

Keywords: Dandy-Walker malformation, seizures, ITP, resistant epilepsy, temporal lobe, children

DOI: 10.3233/PEP-2012-020

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 2, pp. 125-128, 2012

Authors: Monese, Eduardo | Cersósimo, Ricardo | Escobal, Nidia | Sassone, Adriana | Caraballo, Roberto Horacio

Article Type: Research Article

Abstract: We describe a 4.5-year-old girl with an unremarkable personal and family history. At 16 mo of age, she had a single generalized tonic-clonic seizure while awake. At 3 yr of age, she started with daily absence seizures while awake and hyperkinetic and aggressive behavior. The interictal electroencephalography showed bilateral temporo-occipital spike and wave complexes. The ictal video-electroencephalography recordings showed symmetric or asymmetric and synchronized spike and wave paroxysms associated with absences. Neurological examination revealed motor clumsiness, ataxia, and receptive and expressive language deficits. Routine laboratory and imaging studies as well as blood and urine neuro-metabolic investigations were normal. The absences …were refractory to antiepileptic drugs. Cerebrospinal fluid glucose concentration was 36 mg/dL, lactate concentration was 1 mmol/L, and cerebrospinal fluid/plasma glucose ratio was 0.49. Based on the electro-clinical manifestations, neurological examination, and the metabolic study results, glucose transporter-1 deficiency was diagnosed and the girl was started on the ketogenic diet with good response. Show more

Keywords: GLUT 1 deficiency, absences, early-onset epilepsy, hypoglycorrhachia, ketogenic diet

DOI: 10.3233/PEP-2012-021

Citation: Journal of Pediatric Epilepsy, vol. 1, no. 2, pp. 129-132, 2012