Focal Spikes Research Articles

Focal cortical dysplasia: pathophysiological approach

Clinical and experimental studies on focal cortical dysplasia (FCD) were carried out. For the experimental study, an experimental FCD model of rats was developed. Twenty Wistar rats at 0-2 days after birth were used for the study. Kainic acid (KA) solution was injected stereotaxically into medial and lateral sites of the sensori-motor cortex. Bipolar electrodes were inserted in five rats. Their behavior and electroencephalogram (EEG) were recorded using a digital-video-EEG monitoring system. After observation periods of 1, 2, and 6 months, rats were perfused for pathological study. FCD was observed adjacent to the site of KA injection in all rats more than 1 month after the injection. EEG recording demonstrated focal spike discharges in and around the site of injection. However, clinical seizure was not observed. Pathological studies showed decrease in GABA-A receptors and increase in GABA-B receptors not only in the lesion but also in perilesional areas. Fifteen surgical cases of FCD with intractable epilepsy were subjected to the clinical study. Neuro-imaging studies including high-resolution magnetic resonance imaging and single-photon emission computed tomography were performed. Conventional EEG studies demonstrated focal EEG abnormalities with epileptic phenomena. At surgery, intraoperative electrocorticography (ECoG) was performed to localize epileptic foci under neuroleptoanalgesia. Thirteen patients showed epileptiform discharges on preresection ECoG. All foci in non-eloquent areas were resected. Pathological studies including immunohistochemical staining were performed, and the characteristics of the FCD in relation to EEG findings were analyzed. Patients in whom total lesionectomy with complete focus resection was performed had favorable postoperative courses. Nine patients (64.3%) have been seizure-free with reduced medication, and significant improvement was achieved in two patients (14.3%). Electrophysiological examination revealed epileptogenecity not only in the lesions but also in perilesional areas. The immunohistochemical studies showed a decrease in GABA-A receptors and an increase in GABA-B receptors in both the lesions and perilesional areas, but N-methyl-D: -aspartate receptors were almost negative in both areas. Glutamate R1 was decreased in both areas, but glutamate R2 was increased in both areas. These findings support the results of a electrophysiological study. In conclusion, not only the epileptic property of experimental focal cortical dysplasia but also perilesional epileptogenesis was demonstrated. These findings supported the results of surgery for patients with focal cortical dysplasia. In cases of FCD, total removal of the lesion and resection of the perilesional epileptic focus are needed for a good outcome.

Magnetoencephalography in Patients with Tuberous Sclerosis and Localization‐related Epilepsy

To clarify the usefulness of magnetoencephalography (MEG) for diagnosis of the spatial relations between spike foci and suspicious epileptogenic tubers on MRI in patients with tuberous sclerosis (TS) and to compare MEG spike foci with single-photon emission computed tomography (SPECT) findings. We analyzed magnetic fields of epileptic spike discharges in 15 patients with TS and localization-related epilepsy (LRE) by using MEG (a whole-head 204-channel magnetometer system). We investigated the spatial relation between the equivalent current dipoles (ECDs) of interictal spike discharges and visible cortical tubers on MRI. We also compared results of MEG and MRI with SPECT findings. MEG detected a cluster of ECDs around one cortical tuber in six of 15 patients and clusters of ECDs around two cortical tubers in five patients. Interictal SPECT was disappointing in detection of epileptic foci in TS. However, MEG spike foci showed spatial consistency with ictal hyperperfusion areas in two patients. Three patients with single ECD clusters underwent surgical treatment: two have been seizure free, and one has obtained seizure reduction of >90%. ECDs were located around visible tuber nodules. MEG enabled precise localization of the epileptic foci and provided crucial information for surgical treatment in patients with TS and partial epilepsy. TS patients showing a single ECD cluster on MEG may be appropriate candidates for surgical treatment.

Cryptogenic Late‐onset Epileptic Spasms: An Overlooked Syndrome of Early Childhood?

Few reports detailing late-onset epileptic spasms have been published. To determine whether this condition merely represents a late variant of classic West syndrome or exhibits specific features distinct from the latter and related to a later stage of brain maturation, we analyzed the whole population with this specific seizure type, excluding symptomatic cases to avoid the effect of brain lesion. We reviewed the files of the 56 children evaluated for epileptic spasms in clusters having begun at age 12 months or later and analyzed clinical and video-EEG data of the 22 patients (4-17 years; mean, 8.5 years) without obvious cause. Interictal EEG did not show classic hypsarrhythmia. A temporal or temporofrontal slow wave and/or spike focus could be identified in all cases. Twelve children showed spasms with a tonic component. Ictal EEG revealed generalized high-voltage slow wave followed by diffuse voltage attenuation with superimposed fast activity. All children also exhibited other types of recorded seizures consisting of bursts of spike-waves with temporofrontal predominance, reminiscent of "atypical absences." In contrast with the occurrence of tonic components within a cluster of spasms, no tonic seizure stricto sensu was recorded or reported by the caregivers. In 10 children, treatment (two vigabatrin, seven hydrocortisone, one adrenocorticotropic hormone) achieved complete cessation of seizures and disappearance of focal EEG anomalies, but spasms persisted in 12 children. The cryptogenic group in our series without recognized cause and temporal or temporofrontal EEG anomalies seems to represent a type of epileptic encephalopathy intermediary between West and Lennox-Gastaut syndromes, in terms of seizure types and interictal EEG, and could correspond to dysfunction of the maturation process of the temporal lobe, possibly due to an undisclosed lesion.

Widespread and intense BOLD changes during brief focal electrographic seizures

Combined recording of EEG and fMRI has shown changes in blood oxygenation level dependent (BOLD) signal during focal interictal epileptic spikes. Due to difficult assessment of seizures inside the scanner little is known about BOLD changes during seizures. To describe BOLD changes related to brief focal electrographic seizures in a patient with right temporo-parietal gray matter nodular heterotopia. The patient underwent two EEG-fMRI sessions during which several focal seizures were recorded. EEG was acquired continuously during scanning and seizure timing was used for statistical analysis. Functional maps were thresholded to disclose positive (activation) and negative (deactivation) BOLD changes. Twenty-five focal electrographic seizures were analyzed, consisting of runs of polyspikes lasting 2 to 6 s in the right temporal region. Activation included a large volume, involving the heterotopia and the abnormal temporo-parietal cortex overlying the nodule, with a clear maximum over the angular gyrus. Deactivation was bilateral and maximum in the occipital regions. The hemodynamic response function showed a return to baseline of the BOLD signal 30 s after seizure end. The brief focal seizures resulted in high amplitude and widespread blood oxygenation level dependent (BOLD) responses taking 30 s to return to baseline. This suggests that such brief events could have important behavioral consequences despite absent overt manifestations. A clear focal BOLD peak was found at some distance from the main EEG discharge, raising the possibility that the seizure could have started in a region that did not generate a visible EEG change despite its superficial location.

Language‐induced Epilepsy, Acquired Stuttering, and Idiopathic Generalized Epilepsy: Phenotypic Study of One Family

Language-induced epilepsy involves seizure precipitation by speaking, reading, and writing. Seizures are similar to those of reading epilepsy (RE). The nosologic position of language-induced epilepsy is not clear. We performed a clinical and neurophysiological study in a multigenerational family with the association of idiopathic generalized epilepsy (IGE) with ictal stuttering as a manifestation of reflex language-induced epilepsy. Nine members on three generations were studied. All patients underwent video-polygraphic EEG recordings (awake and during sleep). A standardized protocol was applied to test the effect of language and non-language-related tasks. Six patients presented language-induced jaw jerking that mimicked stuttering and corresponded to focal myoclonus involving facial muscles. This was associated with an IGE phenotype in four of these patients. Focal EEG spikes were found in all six patients by visual analysis and/or back-averaging techniques. The focal spikes were either asymptomatic (when followed by a slow wave) or symptomatic of facial myoclonia (when isolated). Levetiracetam, used as add-on or monotherapy in four patients, suppressed ictal stuttering. One additional case only had a phenotype of IGE without focal features. This family study demonstrates the phenotypic heterogeneity of the association of IGE phenotype with ictal stuttering (language-related reflex seizure). Our data suggest that this particular form of reflex epilepsy related to language has more similarities with generalized epilepsies than with focal ones. Neurophysiological investigations should be performed more systematically in patients with acquired stuttering, especially if there is family history of IGE.

Surgery removes EEG abnormalities in patients with Chiari type I malformation and poor CSF flow

Objective To study the outcome of EEG from patients with Chiari I malformations and nonspecific EEG abnormalities, after posterior fossa decompression and CSF flow normalization. Methods Three ‘apparently asymptomatic’ children who had been diagnosed with Arnold–Chiari type 1 EEG abnormalities and who exhibited (a) a wide range of abnormalities according to common anatomical Chiari MRI classifications (Elster AD, Chen MY. Chiari I malformations: clinical and radiologic reappraisal. Radiology 1992;183:347–53), (b) a lack of specific, clinical signs of increased intracranial pressure, and (c) apparently unrelated, EEG-nonspecific abnormalities (focal intermittent rhythmic delta activity (IRDA)—solely in patients 1 and 3, and with focal IRDA plus spikes and spike waves of high voltage in patient 2). Standard EEGs were recorded before surgery and within one month of surgery, which was performed in conjunction with intraoperative echo-Doppler ultrasonography to control CSF flow. Subsequent EEGs and clinical follow-ups were performed within 6–12 months of surgery. Results In all patients, intraoperative echo-Doppler ultrasonographic control demonstrated poor CSF flow, which was completely restored by posterior fossa decompression. In all patients, the EEG abnormalities disappeared within one month of surgery and the EEGs were normal at follow-up. Conclusions A new CNS symptom, identified as focal IRDA alone or focal IRDA plus spikes and spike waves of high voltage in the EEG, seems to be associated with poor CSF flow in ‘apparently asymptomatic’ patients with Chiari type I malformations. Significance The identified, paroxysmal EEG abnormalities should be interpreted as an indirect sign of subtle CNS distress.

Clinical characteristics and long-term prognosis of Landau-Kleffner syndrome

To investigate the clinical and electroencephalographic (EEG) characteristics, therapeutic response and long-term prognosis of Landau Kleffner syndrome (LKS). The clinical and EEG data of 10 children with LKS were analyzed, and therapeutic response and long-term outcome were followed up. The age of onset was from 2 to 10.5 years of age. All patients had acquired aphasia, characterized by verbal auditory agnosia. All patients had epileptic seizures. Partial motor seizures during sleep occurred in 8 patients, and other seizure type including atypical absence seizure and generalized tonic-clonic seizure were also observed. Psychological and behavioral abnormalities occurred in 9 patients. There were no abnormalities of hearing and neuro-imaging tests in all patients, and family histories were negative. All the patients had EEG abnormalities. Focal spike and waves of temporal lobe were recorded in 9 patients. Electrical status epilepticus during sleep (ESES) was observed on Video-EEG (VEEG) monitoring in 4 patients. Anti-epileptic drugs (AEDs) showed favorable effects on epileptic seizures, but no effects on aphasia. All patients responded to corticosteroid, and got language improved. Eight patients were followed up for long-term outcome. All patients were seizure free, while the level of language development was abnormal in 5 patients. The VEEG follow-up was conducted in 6 patients. Continuous epileptic discharges in slow sleep recurred in 2 patients after the discontinuation of steroid therapy. LKS is one of the childhood epileptic encephalopathy, and acquired aphasia and epileptic seizures are two main clinical characteristics. Aphasia is characterized by verbal auditory agnosia. Psychological and behavioral abnormalities are very common in children with LKS. Focal epileptic discharges were often located in temporal area, and usually generalized, and could be continuous during sleep. AEDs could control seizure but had no effects on aphasia. Early use of full dose corticosteroids could improve the language significantly. Long-term follow up showed that language impairments often remained, but the outcome in terms of EEG and epileptic seizure was good.

Infantile Spasms as an Adverse Outcome of Neonatal Cortical Sinovenous Thrombosis

Cerebral sinovenous thrombosis is a rare but potentially serious condition often occurring in children with nonspecific presenting features. Much remains to be learned about the long-term outcome of infants with cerebral sinovenous thrombosis. We report a series of four patients taken from a prospective database of neonates with sinovenous thrombosis who subsequently developed infantile spasms, three with hypsarrythmia on electroencephalography and one with multiple independent spike foci. The first patient presented at 2 weeks of age with hypernatremia, dehydration, and seizures. He was found to have extensive thrombosis and hemorrhagic infarction of the right basal ganglia. The second patient presented at 5 weeks of life and was found to have sagittal sinus thrombosis with bilateral intracranial hemorrhage. The third patient presented with seizures on day 1 of life and was found to have venous thrombosis involving the torcular, extending into the sagittal sinus. The fourth patient presented at 3 weeks with lethargy and seizures. He was diagnosed with bacterial meningitis and also had extensive sinus thrombosis. All patients developed infantile spasms at ages 9, 7, 11, and 10 months, respectively. This is the first report in the English literature describing infantile spasms as a possible outcome of sinovenous thrombosis in early infancy.

Independent component analysis in the distinction between focal and multifocal spikes.

Independent component analysis (ICA) is a novel method that extracts independent sources in recorded signals. One of its capabilities is to separate epileptiform activity of different origins. The goal of this study was to demonstrate that ICA is useful for differentiating focal and multifocal epilepsies. Using ICA, the authors analyzed 160 samples of patients with unifocal (temporal: 50 samples from 5 patients; frontal: 50 samples from 5 patients) or multifocal epilepsy (bitemporal: 30 samples from 3 patients; multifocal extratemporal: 30 samples from 5 patients). Each sample included at least two spikes. ICA was applied using the JADE algorithm implemented in a Matlab platform. The components were identified visually. The EEG and the isopotential map of the suspected components were reconstructed to demonstrate the nature and location of each spike. In multifocal epilepsies, the spikes were separated in distinct components in all cases. In unifocal temporal epilepsies, ICA extracted all the spikes from the same location into a single component. In the patients with unifocal frontal epilepsy, one component included all the spikes in 80% of the samples; in some of these cases, other components were responsible for small parts of the spikes, but with a close topography to the main component. The waves were separated in different components both in the unifocal and in the multifocal samples. Therefore, in epilepsies with independent foci, ICA separates the spikes of different origins into distinct components. In unifocal epilepsies, ICA tends to locate the spikes from the same area into the same component, especially in temporal epilepsies. The authors conclude that ICA might be a useful tool to distinguish between unifocal and multifocal epilepsies.

Independent component analysis in the distinction between focal and multifocal spikes.

Independent component analysis (ICA) is a novel method that extracts independent sources in recorded signals. One of its capabilities is to separate epileptiform activity of different origins. The goal of this study was to demonstrate that ICA is useful for differentiating focal and multifocal epilepsies. Using ICA, the authors analyzed 160 samples of patients with unifocal (temporal: 50 samples from 5 patients; frontal: 50 samples from 5 patients) or multifocal epilepsy (bitemporal: 30 samples from 3 patients; multifocal extratemporal: 30 samples from 5 patients). Each sample included at least two spikes. ICA was applied using the JADE algorithm implemented in a Matlab platform. The components were identified visually. The EEG and the isopotential map of the suspected components were reconstructed to demonstrate the nature and location of each spike. In multifocal epilepsies, the spikes were separated in distinct components in all cases. In unifocal temporal epilepsies, ICA extracted all the spikes from the same location into a single component. In the patients with unifocal frontal epilepsy, one component included all the spikes in 80% of the samples; in some of these cases, other components were responsible for small parts of the spikes, but with a close topography to the main component. The waves were separated in different components both in the unifocal and in the multifocal samples. Therefore, in epilepsies with independent foci, ICA separates the spikes of different origins into distinct components. In unifocal epilepsies, ICA tends to locate the spikes from the same area into the same component, especially in temporal epilepsies. The authors conclude that ICA might be a useful tool to distinguish between unifocal and multifocal epilepsies.

Cortical and thalamic fMRI responses in partial epilepsy with focal and bilateral synchronous spikes

Objective To determine the blood oxygen level-dependent (BOLD) responses to epileptic discharges in the thalamus and cerebral cortex in patients with partial epilepsy. Methods Among 64 tested patients, 40 had EEG spikes during scanning and were divided in two groups: unilateral or bilateral independent spikes (29 patients) and bilaterally synchronous spikes (11 patients). Each spike topography was analyzed separately, yielding 40 studies in the first group and 17 in the second. Results Forty-five percent of focal spike studies showed significant BOLD responses. Cortical activation (positive BOLD) represented the dominant response and had a better correlation with spike location than cortical deactivation (negative BOLD). In the second group, all patients had significant BOLD responses; they were more widespread compared to the first group, and deactivated areas were as important as activated regions. A thalamic response was seen in 12.5% of studies in the first group and 55% in the second. Conclusions The thalamus is involved in partial epilepsy during interictal discharges. This involvement and also cortical deactivation are more commonly seen with bilateral spikes than focal discharges. Significance These findings show evidence for a role for the thalamus and a more important role for inhibition in secondary bilateral synchrony.

Cognitive Deficits Related to Focal Spike Location in Benign Partial Epilepsy

The topographic relation between focal spikes and neuropsychological deficits in children with benign partial epilepsy (BPE) was investigated using magnetoencephalography (MEG), electroencephalography (EEG) and MRI in a study of 27 children at University Children’s Hospital, Tuebingen, Germany, and University of Trento, Italy.

Clinical Neurophysiology: EEG–Video Monitoring

Clinical Neurophysiology: EEG–Video Monitoring

Benign Partial Epilepsy in Childhood: Selective Cognitive Deficits Are Related to the Location of Focal Spikes Determined by Combined EEG/MEG

Benign partial epilepsy (BPE) in childhood is characterized by the occurrence of interictal stereotyped focal spikes with variable localization in the EEG. Children with BPE often exhibit neuropsychological deficits. It is unclear whether a correlation exists between these deficits and the localization of spikes, several EEG studies giving inconsistent results. Magnetoencephalography (MEG) improves the accuracy of spike localization. Therefore by using combined MEG/EEG, we investigated the topographic relation between focal spikes and neuropsychological findings in children with BPE. Twenty-seven children diagnosed consecutively with BPE were enrolled in the study. All were examined by combined MEG/EEG and magnetic resonance imaging (MRI). Location of spikes was determined by dipole source estimation. A standardized neuropsychological assessment was conducted, including Kaufman ABC battery, language tests, and motor performance series. All children with sufficient MEG data were included in the correlation analysis (N = 20). Focal spikes were located in the perisylvian region in 13 children, in the occipital region in seven, and in the frontal region in one. Five children had bilateral or multiple foci. Children with left perisylvian spikes did not differ from the others in global IQ, but performed significantly lower in language tests (p = 0.01). Children with occipital spikes performed significantly lower in simultaneous information processing (p = 0.01), especially in visual transformation tasks. Combined MEG/EEG investigation is a useful tool to examine interictal focal spikes. Our results show a correlation between the location of spikes and selective cognitive deficits in children with BPE. These findings indicate that focal interictal spikes may interfere with complex cognitive functions.

Pilot survey of Hashimoto's encephalopathy in children

Hashimoto's encephalopathy (HE) is a steroid responsive, relapsing encephalopathy associated with thyroid autoantibodies. Paediatric literature mainly consists of case reports of the disease. A questionnaire survey of 68 consultant paediatric neurologists was undertaken through the British Paediatric Neurology Association in 2002 to gather preliminary data about this condition. The response rate was 68% and a total of ten patients were identified: nine from the UK and one from Ireland. Complete clinical details were available on eight patients (seven females and one male). Age at presentation ranged from 10 to 15 years (mean 12y 7mo, SD 1y 1mo). Presenting features included seizures (n=7/8), encephalopathy (n=7/8), cognitive decline (n=4/8), behavioural problems (n=3/8), psychosis (n=2/8), myoclonus (n=1/8), and tremors (n=1/8). All had thyroid autoantibodies and four were also hypothyroid at diagnosis. One patient became hypothyroid during follow-up. Electroencephalograms (EEGs) showed high amplitude slow background rhythms in all patients, and one patient also had focal spikes. Cognitive deficits were identified in four patients and persisted in one over 2 years of follow-up. Six patients improved with steroids and two improved spontaneously. Two had relapsing courses during follow-up afer diagnosis (range 12-48 months). HE may be currently under-recognized in children and increased awareness can result in prompt diagnosis and treatment. Steroid therapy appears to be beneficial. Neuropsychological assessment is required in all cases and may guide steroid therapy. Long-term prognosis for cognition remains guarded at this time.

Evolution of seizures and electroencephalographical findings in 23 cases of deletion type Angelman syndrome

Angelman syndrome (AS) is a genetic disorder with characteristic clinical and EEG findings. We report here the results of long-term follow-up studies on the epileptic seizures and EEG findings of 23 cases of deletion type AS confirmed by FISH analysis, including seven cases previously reported by Matsumoto et al. in 1992. The age at last follow-up in 23 patients was from 1 to 37 years of age (average: 18.0 years), with 10 patients (43.5%) in their 20s, and five over 30. Epileptic seizures were seen in all patients, and the age at seizure onset ranged from 3 to 50 months (average: 21.7 months). Status epilepticus was seen in 11 patients (47.8%). The percentages of cases seizure-free for more than 3 years were 25% (4/16) at 10 years of age, 70% (7/10) at 20, and 80% (4/5) at 30. The EEG findings were classified into six patterns according to the previous report: N (no spike, including focal slow waves), HVS (diffuse high-voltage slow bursts with or without spikes), F (focal spikes or multifocal spikes), S (diffuse spike and waves), C (continuous diffuse spike and waves), Hy (hypsarrythmia or hypsarrhythmia like waves). Hy was noted at ages 0–2 years in two cases. C was observed from the ages 2 to 15 years, being most frequently noted at 3–6 years of age, and it was never seen after 16 years of age. S was observed from ages 1 to 21 years. F was seen from 2 to 21 years of age, and most frequently during the ages of 2–7 years. HVS was seen from 0 years, and still remained after the age of 20. After 22 years of age, all patients showed N pattern including focal slow waves. One of the two patients who had bilateral frontal dominant delta slow waves in their 30s, had a recent seizure. Even if the spikes disappear with age, when bi-frontal focal slow waves remain, seizures may occur even in patients over 30.

Symposium I

Symposium I

Lipofuscinosis neuronal ceroidea infantil tardía: aspectos clínicos y electroencefalográficos

In this paper we describe the clinical characteristics, and particularly the epileptic seizures and electroencephalographic findings, in 15 patients with a pathology diagnosis of late infantile neuronal ceroid lipofuscinosis (NCL). Nine female and six male patients were studied and their clinical records covering the period February 1990 to June 2003 were analysed. Neuroimaging, neurometabolic studies, ERG, PE and repeated EEG were carried out in all cases. The mean age on onset of the disease was 3 years (range: 1-5 years). The initial symptom was epilepsy in all cases. Massive myoclonias and myoclonic-atonic seizures were the most frequent kinds of attacks. Focal myoclonias were observed in six patients. Other types of epileptic seizures observed included generalised tonic-clonic, absence, motor focal and complex focal. The epileptic seizures were resistant to therapy. Progressive neurological and visual impairment, pyramidal and cerebellar signs, as well as mental retardation were present in all cases. Intercritical EEG recordings showed diffuse paroxysms with spike and polyspike waves, multifocal spikes and, less often, focal spikes that were predominant in posterior regions. Photostimulation showed high amplitude (300-450) occipital spikes during the application of light stimulation between 1 and 8 Hz. ERG, VEP and SSEP results were pathological. Images showed signs of brain and cerebellar atrophy. Seven of the patients died between 8.5 and 11 years of age. Late infantile NCL must be considered in the case of a child aged between 1 and 5 years who presents seizures that are predominantly generalised myoclonias and myoclonic-atonic, in association with progressive neurological deterioration including pyramidal, cerebellar and visual signs and an EEG trace showing occipital paroxysms triggered by low frequency photostimulation.

0062 Epilepsy in cystic lesions of the pineal region in childhood

Introduction: Lesions of the pineal gland constitute 0, 4-1, 0% of all intracranial tumors. The most common clinical symptoms related are headache, vertigo, visual disturbancies and less often vomiting, opthalmic symptoms and epilepsy. Sofar details on epileptic seizures were not presented. AIM: To present clinical characteristics of epilepsy and EEG findings in five children with cystic lesions of the pineal region. Material and Methods: The patients are two boys and three girls aged 11 to 15 years with epilepsy as presenting symptom. All examinees had MRI verified cystic lesion of the pineal region. Diagnostic work up excluded all other possible etiology for symptomatic epilepsy. The size of the lesions varied from 8x8x10 mm to 15x12x10 mm in diameter. Three children had generalized epilepsy (absance, grand mal), one had secondary generalized and one partial complex seizures. All the children had abnormal epileptic EEG recordings ranging from focal spike or sharped-waves to diffuse paroxismal polyspike, spike-waves complexes and were put on valproate therapy. Stabilization of epileptic seizures in all children was obtained over 6 months to 2 years follow-up period. On subsequent MRI examination in six months intervals no changes were noticed in comparison to initial findings. Therefore neurosurgical intervention wasn't considered as yet necessary and clinical, EEG and MRI follow up was recommended. Conclusion: In our patients, children with MRI verified cystic lesions of the pineal region epileptic seizures were absance, grand mal, partial complex and partial seizures with secondary generalization. Epilepsy was stabilized by administration of antiepileptic drug.

Free Communications: Clinical Cases

Abstract *NaokoToyota, *KazuyoshiWatanabe, *TamikoNegoro, *AkihisaOkumura, *JunkoTakenaka, and *AyakoSofue *Department of Pediatrics, Nagoya University School of Medicine, Nagoya, Japan Purpose: Some patients clinically have complex partial seizures (CPSs) but their interictal EEGs demonstrate diffuse spike-and-wave discharges (D-SpW). They are often difficult to control with antiepileptic drugs (AEDs) usually considered to be the first choice for partial seizures. The aim of this study was to determine the effectiveness of drug therapy in such patients. Methods: Fourteen patients meeting the following criteria were selected for this study from among those who had been treated and followed-up for >2 years at Nagoya University Hospital: (a) The main seizure type was CPSs, defined as those characterized by a duration of ≥1 min, impaired consciousness, signs suggestive of partial seizures such as eye deviation, autonomic signs such as cyanosis, nausea, and/or emesis, and postictal sleep; (b) A short duration (<5 s) of D-SpW on interictal EEG. Patients who had absences were excluded. Results: Of the six male and eight female patients, none had any abnormal history around birth. Two had mild mental retardation, and one, moderate mental retardation and mild cerebral palsy. Eight patients had a history of febrile seizures. The age at onset of afebrile seizures ranged from 1 to 11 years. No patients exhibited abnormal findings in neuroimaging studies [computed tomography (CT) and/or magnetic resonance imaging (MRI)]. Nine patients had CPSs alone, one had simple partial seizures (SPSs) with CPSs, and four had secondarily generalized seizures (SGSs) with CPSs. Interictal EEG revealed D-SpW alone in nine patients, and D-SpW and focal spikes in five. We needed to change the drugs in nine patients because of a lack of a response to the initial therapy. The drug that was ineffective was carbamazepine (CBZ) in six patients, phenobarbital (PB) in three, phenytoin (PHT) in two, and clonazepam (CZP) in two. We controlled the seizures in 13 patients for >2 years with the abolition of D-SpW in eight. The effective drugs were valproate (VPA) in six patients, CZP in two, VPA+CZP in two, and PB, VPA+PB and PHT+PB in one each. The serum-level ranges of the effective drugs were 47.5–112.65 μg/ml for VPA, 9.2–19.0 ng/ml for CZP, 16.7–19.12 μg/ml for PB, and 13.3 μg/ml for PHT. Conclusions: In patients who exhibited CPSs and D-SpW on interictal EEG, the seizures were often refractory to antiepileptics, such as CBZ, that are usually indicated for CPSs, but were controlled with drugs, such as VPA, that are usually prescribed for generalized seizures. We should consider not only the type of seizures but also the types of paroxysmal abnormalities on interictal EEG for the treatment of epilepsy.