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Review article
Benign pediatric localization-related epilepsies
Lama M Chahine, Mohamad A Mikati
and neuroimaging work-up, respond well to therapy, and remit without sequeale. The benign localization-related epilepsy syndromes of childhood include benign childhood epilepsy with centrotemporal spikes, Panayiotopoulos syndrome and Gastaut-type idiopathic childhood epilepsy with occipital paroxysms. Some patients initially presumed to have these or, for that matter, other benign syndromes in other age groups, follow a less typical course and continue to experience seizures or to exhibit neuropsychological deficits. Thus the diagnosis of a “possible” or “probable” benign epilepsy syndrome may need to be applied to patients initially suspected of having such syndromes until follow-up shows that they clearly follow a benign course. In Part I (Chahine and Mikati 2006) of our two-part review article, we discussed benign localization-related syndromes encountered in infancy. In this second part, we review the epidemiology, clinical manifestations, neuropsychological features, EEG findings, work-up and diagnostic criteria, differential diagnosis, genetics, management and prognosis of the three childhood-onset syndromes. In addition, we discuss their occasional overlap with or progression into other syndromes.
Original article
Anticonvulsant effects of N 6-cyclohexyladenosine microinjected into the CA1 region of the hippocampus on ento
Ali Heidarianpour, Efat Sadeghian, Javad Mirnajafi-Zadeh, Yaghoub Fathollahi, Mohammad Mohammad-Zadeh
stimulation of the entorhinal cortex. In fully kindled rats, N
<sup>6</sup>-cyclohexyladenosine (CHA; a selective A1 receptor agonist) and 1, 3-dimethyl-8-cyclopenthylxanthine (CPT; a selective A1 receptor antagonist) were microinfused bilaterally into the hippocampal CA1 region. Rats were stimulated and seizure parameters were measured. Results obtained showed that CHA (10 and 50 μ moles) decreased the afterdischarge duration (ADD) in the hippocampal CA1 region and entorhinal cortex, stage 5 seizure duration (S5D) and seizure duration (SD) only at the dose of 50 μ moles, and significantly increased the latency to stage 4 (S4L). Intrahippocampal CPT increased ADD and S5D, and significantly reduced the latency to stage 4 (S4L) at the dose of 10 μmoles. Pretreatment of rats with CPT (5 μ moles) before CHA (50 μ moles), significantly reduced the effect of CHA on seizure parameters. The results suggest that the CA1 region of the hippocampus plays an important role in spreading seizure spikes from the entorhinal cortex to other brain regions and activation of adenosine A1 receptors in this region participates in the anticonvulsant effects of adenosine agonists.
Video-EEG analysis of ictal repetitive grasping in “frontal-hyperkinetic” seizures
Elena Gardella, Guido Rubboli, Carlo Alberto Tassinari
semiology. We analysed retrospectively the presurgical, video-EEG recordings of 35 “frontal hyperkinetic” seizures (FHS) in 14 patients (age range: 9-48 years) evaluating the features of ictal grasping by means of off-line, frame-by-frame video-analysis. Ictal grasping was observed in 97.1% of the frontal hyperkinetic seizures in 100% of the patients, with a mean latency of 3.2 seconds with respect to seizure-onset; a mean number of 7.7 IG per seizure were detected. During the same FHS, both arms could perform IG in an alternating fashion. Grasping was usually preceded by a reaching movement and followed by holding or pulling. The sites of prehension were restricted to relatively few sectors, either on the patient’s body (45.5%) or the peri-personal space (54.5%). In some cases, the grasping was elicited by hand touching. We did not find a consistent relationship between side of hand grasping and side of ictal EEG discharge or MRI lesion. In conclusion, ictal grasping is an extremely frequent clinical manifestation during FHS. It was an early, forced and repetitive motor behavior, without a clear lateralizing value. Ictal grasping appeared with consistent semiological features, similar to voluntary prehension, suggesting a probable ictal release of physiological grasping behavior. [Published with video sequences]
Frontal lobe epilepsy manifesting with seizures consisting of isolated vocalization
Ricardo Rego, Stephan Arnold, Soheyl Noachtar
tonic posturing, or head version. We report on a patient whose seizures were documented by video-EEG monitoring, but in whom the observable ictal semiology consisted solely of a brief, monotonous vocalization. Ictal EEGs showed left frontal seizure patterns. Isolated vocalizations can constitute an ictal epileptic event and may be the only observable clinical manifestation of a left frontal lobe epilepsy. [Published with video sequences]
Epileptic seizures in the pediatric intensive care unit setting
Ignacio Valencia, Germán Lozano, Sanjeev V Kothare, Joseph J Melvin, Divya S Khurana, H Huntley Hardison, Sabrina S Yum, Agustín Legido
ranging from 0.8% to 3.3%, with vascular, metabolic abnormalities, and drug withdrawal being the most common etiologies. The objective of this study is to investigate the clinical characteristics of seizures in children admitted to the PICU at our institution. Methods. We performed a retrospective review of all patients with diagnoses of seizures or epilepsy, admitted to our PICU from 2002 to 2004. Of 6,820 admissions, 32 patients, aged one month to 19 years had seizures in the PICU. Results. The incidence of seizures was 0.5%. Developmental delay or mental retardation was present in 37% of patients. Seizures were generalized in 26 (81%), and focal in 6 (19%); 34% had status epilepticus. The etiology of seizures was epilepsy in 11 (34%). Seizures that do not meet the diagnosis of epilepsy were diagnosed in 21 (66%) including post-craniotomy in five (23%), febrile seizures in three (14%), encephalitis in three (14%), and hydrocephalus in three (14%). Thirty-one patients (97%) were initially treated with either lorazepam or fosphenytoin. Conclusions. Seizures in PICU have different clinical characteristics from those in adults. Recognizing the common seizure etiologies in PICU is likely to lead to a more prompt and effective treatment. Antiepileptic drug prophylaxis may be useful in post-craniotomy patients. A neurological consultation and EEG evaluation are of the utmost importance to help rule in or out epileptic disorders in the PICU.
Benign childhood epilepsy with centro-temporal spikes (BCECTS): early onset of seizures is associated with poorer response to i
Su Jeong You, Deok-Soo Kim, Tae-Sung Ko
examine features that could suggest refractoriness at onset. Methods. We retrospectively reviewed the medical records of 144 children with BCECTS diagnosed at the Division of Pediatric Neurology, Asan Medical Center, from March 1, 1995, to April 30, 2002 and treated with AEDs. The patients were subdivided into two groups according to the number of antiepileptic drugs used for effective seizure control. Results. Of the 144 patients, 75 were male and 69 were female, with a mean age at seizure-onset of 7.2 ± 2.3 years (range, 2.1-14.3 years); 119 children were taking one antiepileptic drug (AED) (Group A), and 25 were taking more than one (Group B). There were no significant group differences in female-to-male ratio, prescribed AEDs, number of seizures before the start of treatment, interval between seizure-onset and start of treatment, presence of secondarily generalized seizures, or presence of bilateral EEG abnormalities. The groups differed however, in mean age at seizure onset (7.6 ± 2.2 years versus 5.1 ± 1.9 years, p < 0.05) and percentage of patients with seizure-onset before 3 years (p < 0.05). Conclusions. When treated with AEDs, children with BCECTS usually respond well. However, an earlier onset of seizures is associated with more frequent seizures and initial refractoriness to medical treatment.
Case report
Taylor-type focal cortical dysplasia: is the epilepsy always resistant to medical treatment?
Ennio Del Giudice, Maria Cristina Bianchi, Michela Tosetti, Andrea Varrone, Alfonso Romano, Angela Francesca Crisanti, Luigi Titomanlio, Floriana Imperati
proves to be the only treatment approach leading to control of seizures. We describe a 17-year-old girl with TTFCD who exhibited a very long period of seizure remission. Combined clinical and neuroimaging findings were compatible with a diagnosis of a balloon cell-subtype TTFCD. As for the clinical course, partial motor seizures began at one year of age and ceased at five: our patient has had no seizure recurrence over a 12-year-follow-up. Moreover, throughout the 15-year follow-up, neurological examinations and cognitive abilities always remained within normal limits. Neuropsychological assessment clearly showed no impairments in executive functions: planning abilities, working memory, attention and impulse control, or constructive aspects of motor coordination. The predominant deficits pertained to verbal abilities in the context of borderline intellectual performances. To our knowledge, this case report documents the longest duration of seizure remission in a patient with TTFCD, thus emphasizing the possible benign course of such dysplastic lesions which usually have a poor prognosis, leading to early surgical treatment.
Focal occipital seizures with cerebral polyopia
Asma Zakaria, Irfan Lalani, Laura Belorgey, P Jay Foreman
seizures. We report the case of a man with multiple cavernous angiomas and occipital lobe seizures manifesting as cerebral polyopia.
Video case report
Euphoric (hedonic) theta hypersynchrony in early childhood
Michael Koutroumanidis
child who responded to pleasurable stimulation with a robust, diffuse theta activity, and also samples some of the thoughts that such a response can generate. [Published with video sequences]
Epileptic nystagmus
Pinar Bekdik, Ufuk Sener, Irem Fatma Asan, Murat Ozcelik, Yasar Zorlu
who presented a long-lasting (>30mn) episode of left head and eyes deviation and left-beating nystagmus not crossing the midline. Interictal EEG showed right temporal abnormalities.[Published with video sequences]
Special article
Diet therapy in refractory pediatric epilepsy: increased efficacy and tolerability
Hoon-Chul Kang, Heung Dong Kim
However, the KD is not a convenient therapy, especially because the customary diets of Asian countries contain substantially less fat than traditional Western diets. In addition, there are various complications associated with the diet that should be considered. Unfortunately, no international protocols have been developed with the exceptions of the Johns Hopkins Hospital protocol adopted by a substantial number of hospitals. While the Hopkins protocol has been the basic model, several revisions of the initial protocol have been suggested. Changes to the applicable ages, seizure types, etiologies, the initiation of the diet, the ratio of constituents to reduce the fat content, the duration of the diet, and revised formulae, such as ketogenic milk or the all-liquid KD, have attempted to extend the indications of the KD and increase its tolerability and palatability. Recently, less restrictive KDs, including a modified Atkins diet and low-glycemic-index treatment, have been clinically tested. Here, we review these approaches toward a safer and more convenient therapeutic diet for refractory pediatric epilepsy.
Letter to the Editor
Lamotrigine-induced leucopenia
Serkan Kilbas
