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Rationale: Atypical benign partial epilepsy (ABPE) is characterized by centro-temporal electroencephalography (EEG) spikes, continuous spike and waves during sleep (CSWS), and multiple seizure types including epileptic negative myoclonus (ENM), but not tonic seizures. This study evaluated the localization of magnetoencephalography (MEG) spike sources (MEGSSs) to investigate the clinical features and mechanism underlying ABPE., Methods: We retrospectively analyzed seizure profiles, scalp video EEG (VEEG) and MEG in ABPE patients., Results: Eighteen ABPE patients were identified (nine girls and nine boys). Seizure onset ranged from 1.3 to 8.8years (median, 2.9years). Initial seizures consisted of focal motor seizures (15 patients) and absences/atypical absences (3). Seventeen patients had multiple seizure types including drop attacks (16), focal motor seizures (16), ENM (14), absences/atypical absences (11) and focal myoclonic seizures (10). VEEG showed centro-temporal spikes and CSWS in all patients. Magnetic resonance imaging (MRI) was reported as normal in all patients. MEGSSs were localized over the following regions: both Rolandic and sylvian (8), peri-sylvian (5), peri-Rolandic (4), parieto-occipital (1), bilateral (10) and unilateral (8). All patients were on more than two antiepileptic medications. ENM and absences/atypical absences were controlled in 14 patients treated with adjunctive ethosuximide., Conclusion: MEG localized the source of centro-temporal spikes and CSWS in the Rolandic-sylvian regions. Centro-temporal spikes, Rolandic-sylvian spike sources and focal motor seizures are evidence that ABPE presents with Rolandic-sylvian onset seizures. ABPE is therefore a unique, age-related and localization-related epilepsy with a Rolandic-sylvian epileptic focus plus possible thalamo-cortical epileptic networks in the developing brain of children., (Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Benign myoclonic epilepsy in infancy (BMEI) is the youngest form of idiopathic generalized epilepsy, characterized by myoclonic seizures (MS) in the first three years of life in otherwise normal infants, and the lack of other seizure types except for rare simple febrile seizures. Although afebrile generalized tonic-clonic seizures (GTCS) have been described to develop later in the clinical course of BMEI, mostly during adolescence, an association with GTCS in the early stage of BMEI has never been recognized. We herein report seven children who satisfied the criteria of BMEI except for the recurrence of GTCS before the onset of MS. The age of onset and ictal video-polygraphic features of MS, as well as the long-term seizure and developmental outcome in these children were similar to those of children with typical BMEI. Furthermore, these GTCS mostly disappeared within several months and were replaced by MS. Our study indicates that these children may constitute a BMEI subgroup, expanding the spectrum of BMEI., (Copyright © 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Purpose: We performed this study to evaluate the long-term efficacy and tolerability of ethosuximide (ESX), valproic acid (VPA), and lamotrigine (LTG) as initial monotherapies for patients with newly diagnosed childhood absence epilepsy., Methods: We retrospectively reviewed the medical records of 128 patients (45 boys and 83 girls) diagnosed with childhood absence epilepsy at the Seoul National University Hospital. The diagnosis was based on the criteria proposed by Panayiotopoulos in 2005. We measured the seizure-free rate and the retention rate observed during 2 years of treatment. Follow-up electroencephalography (EEG), any reported adverse events, and reasons for antiepileptic drug (AED) discontinuation were reviewed., Results: The seizure-free rate of ESX (84%) was significantly higher than that of VPA (62%) and LTG (53%) at 3 months. The seizure-free rate of ESX (90%) was significantly higher than that of LTG (63%) at 6 months. After 9 months, there was no significant difference in seizure-free rate among the three groups. There were no significant differences among the three groups in terms of normalization of EEG at 12 months (ESX, 77%; VPA, 83%; and LTG, 64%), retention rate throughout the whole treatment period, and adverse-event rates (ESX, 25%; VPA, 29%; and LTG, 14%)., Conclusion: This study suggests that ESX, VPA, and LTG are equally effective in the long-term treatment of newly diagnosed CAE patients. However, the onset of efficacy was faster for ESX compared with VPA or LTG. Efficacy, tolerability, and adverse event profiles should be carefully considered when selecting AEDs to treat individual patients with CAE., (Copyright © 2011 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Agranulocytosis caused by ethosuximide is extremely rare in children. Drug-induced agranulocytosis is an unexpected side effect of a drug, and delay in diagnosis of agranulocytosis can result in a fatal outcome. We experienced a case of a 16-month-old male infant with Down syndrome in whom fever appeared 16 days after the start of administration of ethosuximide and then severe pneumonia developed. Results of a blood test on admission showed a decreased leukocyte count of 1700/microl, and a hemogram showed that there were no granulocytes. The erythrocyte and thrombocyte counts were within normal ranges. The results of a bone marrow aspiration test showed that there was no production of any types of granulocytes. The patient required mechanical ventilation due to deterioration in his pneumonia and complication with disseminated intravascular coagulation, but the neutrophilic leukocytes began to increase from the 8th day after discontinuation of ethosuximide administration and start of treatment with granulocyte colony-stimulating factor, and the patient survived. The mechanism of onset in this case is thought to have been immunologic. Careful attention should be given to this type of agranulocytosis because of its sudden onset at 1-2 weeks after the start of administration of the causal drug. A drug-induced lymphocyte stimulation test was useful for diagnosis in this case, showing a positive reaction only for ethosuximide.

This study presents a patient with epileptic negative myoclonus who showed interictal focal epileptic discharges in the centrotemporal region. The patient's seizures were exacerbated by carbamazepine, zonisamide, and valproate, but completely controlled by ethosuximide, and were suggested to have some relation with thalamocortical oscillation mechanisms. Ethosuximide is supposed to be a drug of worth to try to use in epileptic negative myoclonus patients with centrotemporal spike foci.

We used ethosuximide as an adjunctive anticonvulsant drug in 19 children with multiple seizure types in whom the primary and most troubling seizure was falling to the ground. All patients were having multiple, daily falls, intractable to phenobarbital, valproic acid, and clonazepam. Seizures were characterized and quantitated by video-electroencephalographic telemetry. Twelve children became completely free of epileptic falls on ethosuximide. Four children showed a 50-75% decrease in the number of falls while 3 showed no change in frequency of falls. All children had an abnormal electroencephalogram (EEG) prior to starting ethosuximide with generalized bursts of spike and wave in a frequency range of 1-4 cycles per second (cps). The only EEG abnormality common to those patients who became free of epileptic falls with ethosuximide treatment was a generalized, synchronous 4 cps discharge. Our experience suggests that ethosuximide may be a valuable adjunctive treatment for epileptic falling spells that occur in the context of multiple seizure types.