Effect of corticosteroids in a twin child with idiopathic localization-related epilepsy

Corticosteroids have been used only in the treatment of special epileptic syndromes or epileptic encephalopathies, such as infantile spasms. We report an antiepileptic effect of corticosteroids that were used for treatment of nephropathy in a monozygotic twin child with idiopathic localization-related epilepsy (I-LRE). The patient and her monozygotic twin sister exhibited repeated partial seizures at two years of age and electroencephalogram (EEG) showed focal spikes in the occipital area and, on other occasions, the centro-parietal areas. After oral antiepileptic drugs were started, the twins still exhibited occasional seizures. The patient had IgA nephropathy at four years of age and intravenous methylprednisolone and oral prednisolone were administered. Her seizures and epileptiform discharges on EEG disappeared, while her sister continued to have seizures and EEG abnormalities. When the dose of oral predonisone was reduced, the seizures relapsed and EEG again revealed focal spikes. We conclude that corticosteroids exhibit efficacy towards seizures and epileptiform discharges on EEG in patients with I-LRE without epileptic encephalopathies.     

PLED pattern and its clinical significance in stroke patients

The pathophysiological connection between periodic lateralized epileptiform discharges (PLED) and epileptic seizures is still not clear. In the study clinical data and EEG findings were analysed in 22 patients aged 43-90 years with a history of stroke in whom EEG disclosed PLED. Eleven patients were studied in the acute phase of stroke and 11 were studied years after stroke when the diagnosis was established of poststroke epilepsy. In 2 patients in acute stroke group single epileptic seizures occurred and 5 had partial status epilepticus. In the group with poststroke epilepsy 4 had single seizures and 4 had epileptic status with partial epilepsy seizures. Thus, in 15 out of 22 patients PLEDs were noted after epileptic seizures. In all cases PLED appearance was connected with consciousness disturbances, lasting 1 to 17 days. In 6 cases PLED pattern was interrupted by seizure activity over one hemisphere, in 3 of them partial epileptic seizures were associated with it. In acute phase of stroke neuroimaging demonstrated the presence of fresh ischaemic foci, but in cases of poststroke epilepsy no such fresh foci were observed. These results suggest that PLED frequently can be associated with epilepsy, and in some patients it can be a bioelectrical manifestation of partial status epileptic.     

儿童结节性硬化症合并癫痫临床特点分析

目的 探讨儿童结节性硬化症合并癫痫的临床特征。方法 对36例结节性硬化症合并癫痫患儿的临床资料进行分析。结果 所有患儿均以癫痫发作为首发症状,发病年龄〈1岁23例（63.89%）、1-3岁8例（22.22%）、〉3岁5例（13.89%）;发作类型以部分性发作[12例（33.33%）]、全面性发作[5例（13.89%）]、痉挛发作[7例（19.44%）]和混合性发作[12例（33.33%）]为主,其中伴智力低下或发育落后者19例（67.86%）。MRI可见室管膜下结节（30例）,部分病例[26例（86.67%）]伴皮质和皮质下结节、室管膜下巨细胞型星形细胞瘤2例（6.67%）或巨脑回畸形1例（3.33%）。脑电图显示发作间期样放电[34例（94.44%）],表现为多灶性放电[12例（35.29%）]、广泛性放电[8例（23.53%）]、局限性放电[7例（20.59%）]和高度失律[7例（20.59%）];其中8例监测到临床发作,包括痉挛发作（4例）、局灶性发作（3例）、肌阵挛发作（1例）。17例患儿（47.22%）服用1种抗癫痫药物、12例（33.33%）服用2种药物、7例（19.44%）服用3种药物,其中手术治疗1例;随访0.50~10年,无发作7例（19.44%）,有效16例（44.44%）,无效13例（36.11%）。结论 癫痫发作是儿童结节性硬化症最常见的神经系统表现,多在婴儿期发病,主要发作类型为部分性发作和痉挛发作,多数患儿抗癫痫药物治疗有效。     

Amplitude-integrated electroencephalography in patients with acute encephalopathy with refractory, repetitive partial seizures

We report amplitude-integrated EEG findings in two children with acute encephalopathy with refractory, repetitive partial seizures. Both patients had a febrile illness one week before the onset of seizure. They had reduction of consciousness and repetitive seizures refractory to first-line antiepileptic drugs. Seizure frequency rapidly increased and evolved into status epilepticus. Continuous seizure monitoring with amplitude-integrated EEG revealed frequent subclinical seizures which were missed by direct observation. In addition, the site of origin of seizures was multifocal, and seizure foci shifted from one hemisphere to the other. Their seizures were controlled after an administration of high-dose phenobarbital. Continuous seizure monitoring with amplitude-integrated EEG will contribute to correct estimation of seizure burden and efficacy of antiepileptic drugs in children with acute encephalopathy with refractory, repetitive partial seizures.     

Surgery for epilepsy in children with dysembryoplastic neuroepithelial tumor: clinical spectrum,seizure outcome,neuroradiology, and pathology

Introduction  Dysembryoplastic neuroepithelial tumors (DNTs) were first described by Daumas-Duport et al. in 1988 as a typically cortical tumor affecting young patients with long-standing, drug-resistant epilepsy. Methods  We reviewed the medical records of 29 patients with DNT between 1994 and 2007 at Hacettepe University Children’s Hospital retrospectively; age at the time of surgery, age at seizure onset, electroencephalography (EEG), MRI, medical treatment, surgical procedure, seizure outcome, and pathological findings were documented. Results  Male to female ratio was 15/14. Age at the time of evaluation ranged 4–24 years. Twenty-seven patients (93.1%) had complex partial seizures, one (3.44%) had simple partial seizures, and one patient had generalized seizures. Preop interictal EEG showed epileptiform discharges in 24 patients, while in five patients interictal EEG before surgery showed no epileptiform discharges. Pathologically, 24 of our patients were classified as complex type and five as simple type. MRI showed temporal lesion in 20 (68.9%) patients and nine patients had extratemporal DNT. We choose the type of surgery according to lesion and the epileptojenic zone. Finally, 27 patients had Engel Class IA and two patients had Engel Class IB outcome. Conclusion  Complete resection of the lesion with epileptojenic zone is important for seizure-free outcome. Timing of surgery, extent of surgery, and stopping antiepileptic drugs are still important factors.     

Periictal diffusion abnormalities of the thalamus in partial status epilepticus

Purpose:   To identify and describe thalamic dysfunction in patients with temporal as well as extratemporal status epilepticus (SE) and to also analyze the specific clinical, radiological, and electroencephalography (EEG) characteristics of patients with acute thalamic involvement.
Methods:   We retrospectively identified patients who presented with clinical and electrographic evidence of partial SE and had thalamic abnormalities on diffusion-weighted imaging (DWI) within 5  days of documentation of lateralized epileptiform discharges (group 1). The spatial and temporal characteristics of the periodic lateralized epileptiform discharges (PLEDs) and the recorded electrographic seizures were analyzed and correlated with magnetic resonance imaging (MRI)-DWI hyperintense lesions. The findings of group 1 patients were compared with those of patients with partial SE without thalamic abnormalities on DWI (group 2).
Results:   The two groups were similar with regard to clinical presentation and morphology of epileptiform discharges. Group 1 patients had thalamic hyperintense lesions on DWI that appeared in the region of the pulvinar nucleus, ipsilateral to the epileptiform activity. Statistically significant relationship was noted between the presence of thalamic lesions and ipsilateral cortical laminar involvement (p = 0.039) as well as seizure origin in the posterior quadrants (p = 0.038). A trend towards PLEDs originating in the posterior quadrants was also noted (p = 0.077).
Discussion:   Thalamic DWI hyperintense lesions may be observed after prolonged partial SE and are likely the result of excessive activity in thalamic nuclei having reciprocal connections with the involved cortex. The thalamus likely participates in the evolution and propagation of partial seizures in SE.     

Callosotomy and subsequent surgery for children with refractory epilepsy

Callosotomy has been considered as a palliative therapy for refractory epilepsy patients with non-focal onset seizures. Residual partial seizures and unilateral epileptiform discharges on electroencephalography (EEG) are sometimes observed after callosotomy. These findings suggest that some patients may be candidates for subsequent resective surgery. Of 149 children who received a callosotomy, resective/disconnective surgery was subsequently performed in 19 patients. Most patients had generalized seizures and epileptiform discharges on EEG before callosotomy. Two-third had normal neuroimaging studies at initial presurgical evaluation. After callosotomy, ictal symptoms evolved into asymmetric features suggesting partial onset seizures. Post-callosotomy EEG showed completely lateralized or localized epileptiform discharges responsible for residual partial seizures in 16 of 19 patients. Fifteen patients underwent resective surgery of the unilateral frontal lobe, and the remaining received hemispherotomy or posterior quadrantectomy. After subsequent surgery, favorable seizure outcomes were obtained in 11 patients (57.9%). Favorable seizure outcomes may be achieved with callosotomy and subsequent surgery in selected patients who are not candidates for a conventional resective surgery at initial presurgical evaluation.     

Focal Status Epilepticus: Clinical Features and Significance of Different EEG Patterns

PURPOSE: Focal status epilepticus is typically diagnosed by the observation of continuous jerking motor activity, but many other manifestations have been described. EEG evidence of focal status may take several forms, and their interpretation is controversial. We detailed the clinical spectrum of focal status in patients diagnosed by both clinical deficit and EEG criteria and contrasted clinical manifestations in patients with different EEG patterns. METHODS: Patients were diagnosed with a neurologic deficit and discrete recurrent focal electrographic seizures or rapid, continuous focal epileptiform discharges on EEG. Clinical findings were determined by chart review. RESULTS: Of 41 patients with focal status, acute vascular disease was the cause in 21; 10 of 41 had exacerbations of prior epilepsy. A variety of clinical seizure types occurred, both before and after the EEG diagnosis, but the diagnosis was not expected in 28 patients before the EEG. Three had no obvious clinical seizures. Focal motor seizures and an abnormal mental status were the most common manifestations at the time of the EEG. With antiepileptic drugs, almost all had control of clinical seizures, and most improved in mental status. Patients with rapid continuous focal epileptiform discharges were nearly identical in presentation, likelihood of diagnosis, subsequent seizures, response to medication, and outcome to those with discrete seizures on EEG. CONCLUSIONS: Focal status epilepticus may be seen with a wide variety of clinical seizure types or without obvious clinical seizures. The diagnosis is often delayed or missed and should be considered after strokes or clinical seizures when patients do not stabilize or improve as expected. The diagnosis should be made equally whether patients have discrete electrographic seizures or continuous rapid focal epileptiform discharges on the EEG, and the same response to medications and outcome should be anticipated for the two groups.     

Study on early-onset benign occipital seizure susceptibility syndrome.

We prospectively studied the early-onset benign occipital seizure susceptibility syndrome to confirm the benign prognosis. The patients were 37 children followed for more than 2 years after meeting the following criteria on the first examination: (1) normal development before the onset of epilepsy, (2) onset between 1 and 8 years of age, (3) normal brain MRI and cranial CT findings, (4) partial seizures manifested both initial ictal vomiting and tonic eye-deviations, and (5) normal background activity with or without epileptic EEG foci regardless of location. The incidence and clinical characteristics of seizures, response to treatment, and EEG findings were analyzed. The total number of seizures ranged from one (n = 6) to 27 times, with a median of five times. Recurrent prolonged attacks resistant to antiepileptic drugs were recognized in 15 children, who had earlier onset of epilepsy and more frequent complications than the remaining 22 children. Interictal EEG revealed occipital foci in 26 children, 17 of whom later revealed a shift in predominant foci. At the final examinations, 28 patients had been seizure-free for at least 2 years. The clinical picture of this syndrome ranges from those with a few seizures to those with recurrent prolonged seizures initially resistant to antiepileptic drugs despite ultimate remission by 12 years of age.     

Game-related seizures presenting with two types of clinical features.

We evaluated 22 patients with epileptic seizures in which the seizures were triggered by various games or game-related materials. Based on whether spontaneous seizure coexisted or not, these 22 patients were divided into two groups. Ten patients who experienced seizures exclusively while playing or watching specific games were referred to as Group I, while 12 patients that had both game-induced and spontaneous seizures were classified as Group II. The patients in Group I had a middle-age onset (39.1 years) with a male predominance (90%). The electroencephalogram (EEG) or brain magnetic resonance imaging revealed non-specific abnormalities in 60%, and the partial onset seizure was recognized in 30% of patients. Antiepileptic drugs had uncertain benefits in this group. In Group II, patients had a male predominance (67%), with onset during adolescence (16.3 years). Most of them had generalized tonic-clonic seizures, myoclonic seizures, and absences, and 42% showed epileptiform discharge on EEG. These 12 patients were categorized into idiopathic generalized epilepsies. Although photosensitivity was an important factor, higher mental activity seemed to be significant precipitants of seizures in Group II. Antiepileptic drugs were necessary and valproic acid alone or combined with clonazepam was effective in this group. The results showed that game-related seizures are not a unique and homogeneous syndrome and may consist of different mechanisms. Teenage onset, coexistent spontaneous seizure, and associated idiopathic generalized epilepsies were crucial factors in the determination of antiepileptic drug therapy. Moreover, avoiding the related games altogether may be a more productive preventive measure.     

A case of malignant migrating partial seizures in infancy as a continuum of infantile epileptic encephalopathy

The syndrome of malignant migrating partial seizures in infancy (MMPSI) is characterized by onset before the age of 6 months, nearly continuous electrographic seizures involving multiple independent areas of onset in both hemispheres, and poor developmental outcome. This report presents a case involving a patient with MMPSI, who later developed West syndrome. At the age of 2 months old, he showed multifocal partial seizures, which were refractory to antiepileptic drugs. His electroencephalogram (EEG) revealed characteristic migrating multifocal epileptiform activities and neuroimaging finding was normal. The focal seizures were refractory to antiepileptic drugs and ketogenic diet. When he was 9 months old, epilepic spasms were observed with hypsarrhythmia on EEG. He also showed severe developmental delay. MMPSI may be a continuum of infantile epileptic encephalpathy and could evolve to West syndrome.     

Education and Epilepsy: Assessment and Remediation

Summary: Learning difficulties in children with epilepsy may be caused by brain damage and should be investigated. In many cases, however, seizures and/or electroencephalographic (EEG) findings are the only signs of pathology. Frequency and type of seizures may be determining factors that should, if necessary, be evaluated by long-term EEG monitoring, preferably during school performance or in conjunction with neuropsychological assessment. This may prove that subclinical epileptiform discharges in the EEG can adversely affect the child's performance. Secondary psychological problems in epilepsy patients, combined with side effects of antiepileptic drugs, may cause or heighten learning problems. Prophylactic control of seizures with one appropriate drug may alleviate learning problems. Computerized neuropsychological testing with simultaneous EEG recording may reveal the influence of epileptiform discharges on cognitive function and also help to evaluate the effects of antiepileptic drugs. Objective assessment of subclinical epileptiform activity makes it easier to treat the pathology identified by the EEG with optimal dosage of the most appropriate drug. A balance is required because epileptiform discharges and even occasional seizures may be less disabling than side effects from large doses of several drugs. Information to the school and the parents concerning the patient's abilities and limitations may be as important as seizure control. Specialized teaching should be started early, when necessary, with the patient integrated into a normal school if possible. However, good functioning in a special school is preferable to marginal functioning in a normal school.     

Epileptiform EEG activities of the centromedian thalamic nuclei in patients with intractable partial motor, complex partial, and generalized seizures

Centromedian thalamic nuclei (CM) epileptiform EEG activities were recorded in patients with intractable partial motor, complex partial, and generalized seizures through implanted recording-stimulating electrodes, used for seizure control. CM epileptiform activities showed either little or no correlation to focal surface cortical and amygdaloid activities in patients with partial motor and complex partial seizures: CM paroxysmal discharges were correlated to focal epileptiform ictal activities only during the contraversive movements of partial motor and complex partial seizures. In contrast, CM epileptiform activities were consistently correlated to widespread surface cortical activities and clinical symptoms of fully developed nonconvulsive and convulsive tonic-clonic generalized seizures; i.e., unilateral CM double spike-wave complex discharges significantly preceded (p less than 0.001) the contralateral CM and bilateral surface cortical discharges and symptoms of nonconvulsive generalized seizures. Unilateral CM fast-slow-fast paroxysmal discharges significantly preceded (p less than 0.005) those of the contralateral CM and bilateral surface cortical regions and symptoms of the convulsive tonic-clonic generalized seizures. Individual spike-wave complexes from the frontal region preceded (p less than 0.001 and p less than 0.005) those at CM and other cortical regions during the nonconvulsive and clonic generalized attacks. No correlations between CM and cortical epileptiform activities were found, however, in the case of abortive, subclinical thalamocortical EEG discharges.     

Facilitation of Infantile Spasms by Partial Seizures

Summary: We report 16 patients with infantile spasms in whom onset of the clusters of spasms appeared to be triggered by close temporal association with partial seizures. Common features included the presence of focal cerebral lesions in 12 infants (3 were classifiable as cryptogenic); all had partial seizures with EEG localization, clusters of bilateral spasms always preceded by partial seizures, and response to adrenocorticotropic hormone (ACTH) and traditional antiepileptic drugs (AEDs) generally was poor. Three had complete agenesis of the corpus callosum, which argues against interhemispheric callosal spread of focal discharges resulting in the generalized spasms. Surgical cortical resections were performed in 6 of the infants, with good outcomes. This group of patients supports a model in which the spasms, although probably generated at a subcortical level, are facilitated or possibly induced by focal discharges from cortical pathology.     

Does Physical Exercise Influence the Occurrence of Epileptiform EEG Discharges in Children?

Summary: Purpose: To determine if, and how, epileptiform EEG discharges in children were influenced by physical exercise.
Methods: Twenty-six children with intractable partial and generalized epilepsy exercised during video-telemetry recording, aiming at exhaustion after ∼10 min.
Results: During the exercise, epileptiform discharges decreased in 20 of 26 children (17 children had at least 25% reduction) and showed a rebound increase after the exercise (17 of 26 children) compared to baseline conditions. This exercise-induced reduction in epileptiform discharges reached statistical significance only in the 16 patients with localization-related epilepsy. Five patients showed an atypical EEG response to exercise with either unchanged or increased epileptiform activity while exercising. As compared to the other patients, these 5 children had experienced frequent clinical seizures during or immediately after exercise in their leisure time.
Conclusions: In the majority of the tested children, epileptiform EEG discharges decreased during exercise. "Exercise-EEG" may be a helpful diagnostic tool to identify patients who are disposed to have exercise-induced seizures.     

Clinical Manifestations in Children with Occipital Spike-Wave Paroxysms

The pattern of occipital-posterotemporal spike-wave paroxysms (O-PT SWPs), is a distinctive EEG abnormality observed primarily with occipital epilepsy of childhood and basilar artery migraine. We studied EEG and clinical features in 30 children and young adults with this EEG pattern. Prolonged and brief O-PT SWPs were observed. Prolonged discharges (greater than 6 s) were observed only in children with seizures (p less than 0.001), and brief discharges (1-6 s) were observed immediately after eye closure. Generalized SWPs (11 patients, 37%) and background abnormalities (17 patients, 57%) were common. Photic activation of O-PT SWPs was not observed. Twenty-four patients (80%) manifested paroxysmal phenomena-seizures (20 patients, 67%) and migraine (12 patients, 40%, 4 alone and 8 with seizures). Fifteen patients (75%) had partial seizures, and 5 (25%) had absence seizures. In 7 patients with partial seizures, an etiology was evident. Neurologic examination was more often abnormal in patients with secondary partial seizures than in those with idiopathic partial seizures (p less than 0.05) and absence seizures. Conversely, migraine was more often associated with idiopathic partial seizures than with secondary partial seizures (p less than 0.05) and absence seizures. Six children (20%) had no paroxysmal events. Generalized SWPs were uncommon in patients with idiopathic partial seizures. We conclude that O-PT SWPs is a nonspecific epileptiform abnormality that may occur in children with (a) idiopathic partial, (b) symptomatic partial, and (c) absence epilepsies, but it may also occur in patients with no evidence of seizures.     

Seizure semiology and neuroimaging findings in patients with midline spikes

PURPOSE: Midline epileptiform discharges are rare compared with discharges at other scalp locations. Neuroimaging results and semiologic seizure characteristics of patients with midline spikes are not adequately described. The aim of this study was to describe the neuroimaging findings and detailed seizure semiologies in patients with midline spikes. METHODS: We reviewed the EEG database of the University of Michigan Medical Center and identified 35 patients with midline spikes. Information about seizure types and neuroimaging results was obtained from a review of medical records. The seizures were classified according to the International League Against Epilepsy (ILAE) criteria and semiologic classification. RESULTS: Twenty-nine (83%) patients had a history of seizures. Complex partial seizures and simple partial seizures were the most common seizure types, experienced by 66% of patients. The age at seizure onset was within the first 10 years in 90% of patients. According to the semiologic seizure classification, automotor seizures and tonic seizures were the most common seizure types. Neuroimaging studies were abnormal in 45% of patients. When focal abnormalities were detected, they were lateralized to one of the frontal lobes in all cases. CONCLUSIONS: Our results indicate that in the majority of patients, midline spikes represent focal epileptiform activity rather than fragments of generalized discharges, and are most commonly associated with seizures of partial onset. Automotor seizures and tonic seizures are the most common semiologies. Focal radiologic abnormalities tend to be lateralized to one of the frontal lobes.     

Electroencephalographic studies of simple partial seizures with subdural electrode recordings

We used subdural electrodes to study the EEG features of simple partial seizures in 7 patients. We detected epileptiform discharges in 61 of 68 subdurally recorded simple partial seizures compared with 6 of 55 simple partial seizures recorded with scalp electrodes (p less than 0.0001). The onset of 36 nonmotor simple partial seizures was detected only by the medial and basal temporal subdural electrodes, and the onset of 25 simple partial seizures with motor manifestations was recorded by subdural electrodes only from the lateral cortex of the posterior frontal lobe. There was a close correspondence between the area first involved in the epileptiform discharge during simple partial seizures and the area first involved during complex partial and secondary generalized tonic-clonic seizures. Subdural electrodes may be effective in localizing the onset and spread of simple partial seizures, including those that arise from the medial temporal lobe.     

EEG of partial seizures.

EEG remains the primary technique in the diagnosis, characterization, and localization of partial seizures. This review examines the significance and character of interictal epileptiform abnormalities, periodic lateralized epileptiform discharges, and ictal patterns in patients with partial epilepsy. Interictal epileptiform discharges are common and assist in the diagnosis and localization of partial seizures. Fortunately, true "false positive" EEGs with focal epileptiform abnormalities are distinctly rare. Periodic lateralized discharges have characteristics of both interictal and ictal activity and are an area of controversy as to their clinical significance. Ictal patterns in partial seizures are variable, with the most distinctive features seen in seizures from a mesial temporal lobe origin. The unifying EEG feature of a partial seizure is in its evolution. A partial seizure begins with a clear delineation of the onset of activity that is distinct from the preceding background, followed by an evolution of this activity in both frequency and amplitude and terminating with an identifiable cessation of the rhythmic pattern that merges again into the background activity.     

Aggravation of seizures and/or EEG features in children treated with oxcarbazepine monotherapy

PURPOSE: Exacerbation of epilepsy may occur following initiation of therapy with antiepileptic drugs (AEDs). The aim of this study is to analyze the clinical and EEG characteristics of a group of pediatric patients with worsening of seizures and/or EEG deterioration while on oxcarbazepine (OXC). METHODS: A retrospective analysis of a clinical database was performed to identify patients with epilepsy treated with OXC over the past 3 years. History, neurological examination, and EEG findings were reviewed to identify any who had developed exacerbation of seizures or new abnormalities on EEG. RESULTS: Of 290 patients on OXC, we identified 12 patients with new onset seizures, all with initial normal neurological exam and normal EEG, who developed either worsening of preexisting seizures, new seizure types, and/or EEG deterioration following introduction of OXC monotherapy. EEG changes were primarily characterized by new onset of generalized epileptiform activity not reported on the initial baseline EEG. Following substitution of OXC with a broad spectrum AED, significant improvement of seizure control and improvement in the EEG was observed. CONCLUSIONS: These findings suggest that OXC can aggravate seizures and/or worsen EEG features in children. Following initiation of therapy with OXC, monitoring of patients with follow-up EEGs may be important, especially in patients who do not show adequate response to therapy.