Status Epilepticus of Benign Partial Epilepsies in Children: Report of Two Cases

Benign partial epilepsies of childhood (BPEC) are one of the most frequent types of epilepsy in school-age children. Status epilepticus (SE) of these conditions have not yet been reported. Two children with clinical and EEG features consistent with the diagnosis of BPEC-SE are presented. In neither case did SE respond to current antiepileptic medications and stopped only after administration of steroids. At follow-up 1 and 2 years post SE, neurologic and intellectual development have been normal. Differential diagnoses included an atypical benign partial epilepsy, epilepsy with electrical status epilepticus during slow sleep, acquired epileptic aphasia, Lennox-Gastaut syndrome and epilepsia partialis continua.     

Epilepsia Partialis Continua in Sjögren''s Syndrome

Epilepsia partialis continua with Sj?gren's syndrome is reported. The patient had extensive involvement of the nervous system with left middle cerebral artery occlusive stroke, mononeuritis multiplex, right partial sensory motor seizures and epilepsia partialis continua involving the right big toe and foot. The EEG showed nonspecific bilateral theta slowing. The epilepsia partialis continua did not respond to antiepileptic drugs.     

Double Pathology in Rasmussen's Encephalitis: Etiologic Considerations

In a 7-year-old girl with epilepsia partialis continua (EPC) involving the left face, arm, and leg for 1 year, serial neuroimaging studies showed progressive, brain atrophy. Because medical treatment was ineffective, she underwent a large fronto-temporal surgical resection. Neuropathological examination showed loss of lamination and dysplastic neurons, gliosis, microglial nodules, and perivascular cuffing. Such “double pathology” (dysgenesia and a chronic inflammatory process) may have implications for the pathophysiology of Rasmussen's syndrome.     

Rasmussen's encephalitis: Experience from a developing country based on a group of medically and surgically treated patients

PurposeTo describe the attributes of patients with Rasmussen's encephalitis (RE) seen in a tertiary epilepsy referral center in southern India and to enquire factors helpful in predicting responsiveness to immunotherapy.MethodsWe diagnosed RE based on the European consensus criteria. To assess the factors that could potentially predict the natural course and therapeutic outcome, we subcategorized our patients according to age at onset ( 6 years), duration from onset to presentation ( 2 years), immunotherapy versus surgery, and early (≤2 years from the onset) versus late surgery.ResultsThe median age at disease onset of 19 patients was 6.0 years (range 2.3–13 years). Epilepsia partialis continua (EPC) and hemiparesis were noted in 14 (73.6%) and 16 (84.2%) patients, respectively. One patient, who presented with dysarthria due to tongue EPC, did not have hemiparesis despite having had the disease for over 15 years. The MRI findings in majority conformed to stage 3 of Bien classification. While 9/10 patients treated by surgery achieved seizure-freedom, only 1/11 patients who received immunotherapy did so. One patient expired due to subsequent development of contralateral hemispheric disease following successful hemispherectomy. None of the factors such as age at onset, age at presentation, presence/absence of antecedents, seizure burden, MRI stage predicted responsiveness to immunotherapy.ConclusionThis study from a developing country, in addition to substantiating the well known characteristics of RE, noted the following unusual findings: isolated lingual EPC abolished by focal cortical resection, bilateral RE, putaminal atrophy and absence of hemiparesis despite long standing disease.     

Long-Term Prognosis in Childhood Epilepsy: Survival and Seizure Prognosis

All children aged 0-19 years who had active epilepsy in a defined Swedish population were traced and given a clinical and psychometric investigation. Twelve years later, a follow-up study was carried out. Eleven of the 194 children had died, 8 of whom had had signs of neurodeficit, i.e., abnormal neurology and/or mental retardation. A long-standing remission of seizures occurred in 124 of the 194 children. Signs of neurodeficit, frequent seizures, and many types of seizures were negative prognostic factors. The presence of all these factors carried a bad prognosis, seizures persisting during 12 years in greater than 80%. For those who were mentally and neurologically normal and had low seizure frequency, prognosis was excellent, only 11% still having active epilepsy after 12 years. A study of the annual remission rate showed that each year approximately 13% of the children without neurodeficit had remission from epilepsy the next year. This rate appeared to be stable over the 12 years studied. Among those children with neurodeficit, the annual remission rate was high only during the first years after onset, later falling to 3% a year.     

Epilepsy duration, febrile seizures, and cerebral glucose metabolism

PURPOSE: Studies using magnetic resonance imaging have shown that reduced hippocampal volume is associated with a history of febrile seizures, the duration of epilepsy, and the number of generalized tonic-clonic seizures. It is uncertain whether these factors have the same influence on functional as on structural measures of the integrity of the epileptogenic zone. METHODS: We used positron emission tomography (PET) with fluorine 18 2-deoxyglucose to study 91 patients with temporal lobe seizure foci localized by ictal video-EEG. PET was performed in the awake interictal resting state with ears plugged and eyes patched. We recorded surface EEG during injection (5 mCi) and the 30-min uptake period. We used a standard template to analyze PET scans. RESULTS: A significant negative relation was found between the duration of epilepsy and hippocampal glucose metabolism ipsilateral to the epileptic focus. Patients with a history of either any febrile seizures, or complex, or prolonged febrile seizures, did not have greater hypometabolism ipsilateral to the epileptic focus than did patients without a febrile seizure history. We found no effect of generalized tonic-clonic seizure history. CONCLUSIONS: Longer epilepsy duration is associated with greater hypometabolism, suggesting that epilepsy is a progressive disease.     

国际抗癫痫联盟关于发作和癫痫分类框架术语及概念最新修订版的解读

国际抗癫痫联盟(International League Against Epilepsy,ILAE)分类和术语委员会2010年制定的发作和癫痫分类框架术语及概念修订版旨在进一步建立和规范发作和癫痫分类术语及概念的国际共识,反映术语与分类的基本关系,以指导临床实践,尤其是指导制定更合适的治疗方案。对于以往的全面性发作和局灶性发作分类法,目前强调依据网络的起源方式重新进行定义,其中双侧起源为全面性发作,局限于一侧为局灶性发作。此外,建议使用遗传性、结构性/代谢性和未知病因这3个术语以替代原来的特发性、症状性和隐源性。新的报告还首次使用了一些特定的术语,如电-临床综合征、相对明确的癫痫群体和癫痫性脑病等。本文对ILAE关于发作和癫痫分类框架术语及概念最新修订版进行了解读。     

Children with Epilepsy: The Effect of Seizures, Syndromes, and Etiological Factors on Cognitive Functioning

Summary: Overall, children with epilepsy have poorer concentration and mental processing and are less alert than age-matched controls. The relationship between cognitive functioning and epilepsy is complex, however, with widely differing degrees of intellectual impairment–ranging from minimal to severe and progressive–related to diverse types of epileptic seizures, syndromes, and etiological factors. Prolonged and frequently repeated seizures are typically associated with more severe effects on cognitive functioning, particularly if epilepsy is symptomatic, i.e., secondary to a demonstrable brain lesion. A combination of such factors may contribute to the mental deterioration seen in many children suffering from severe epilepsy.     

Seizures in Series: Similarities Between Seizures of the West and Lennox-Gastaut Syndromes

We observed seizures resembling infantile spasms in patients with Lennox-Gastaut syndrome (LGS). Infantile spasms, the type of seizures that occurs in patients who have West syndrome, have been well characterized by video-EEG studies and typically occur as a series of sudden generalized flexor or extensor jerks. The seizure types that occur in LGS have not been as clearly delineated. Some patients with West syndrome (WS) in early infancy later develop LGS. Using intensive video-EEG monitoring, we evaluated 14 LGS patients who had seizures that occurred in series. Clinically, the seizures greatly resembled infantile spasms, and the ictal EEG changes were identical to those that occur with infantile spasms. These findings expand the number of features known to be shared by these two syndromes and strengthen the hypothesis that the two syndromes represent age-related manifestations of similar epileptogenic processes.     

Photic- and pattern-induced seizures: a review for the Epilepsy Foundation of America Working Group

PURPOSE: This report summarizes background material presented to a consensus conference on visually provoked seizures, convened by the Epilepsy Foundation of America. METHODS: A comprehensive review of literature was performed. RESULTS: Photosensitivity, an abnormal EEG response to light or pattern stimulation, occurs in approximately 0.3-3% of the population. The estimated prevalence of seizures from light stimuli is approximately 1 per 10,000, or 1 per 4,000 individuals age 5-24 years. People with epilepsy have a 2-14% chance of having seizures precipitated by light or pattern. In the Pokemon cartoon incident in Japan, 685 children visited a hospital in reaction to red-blue flashes on broadcast television (TV). Only 24% who had a seizure during the cartoon had previously experienced a seizure. Photic or pattern stimulation can provoke seizures in predisposed individuals, but such stimulation is not known to increase the chance of subsequent epilepsy. Intensities of 0.2-1.5 million candlepower are in the range to trigger seizures. Frequencies of 15-25 Hz are most provocative, but the range is 1-65 Hz. Light-dark borders can induce pattern-sensitive seizures, and red color also is a factor. Seizures can be provoked by certain TV shows, movie screen images, video games, natural stimuli (e.g, sun on water), public displays, and many other sources. CONCLUSIONS: Recommendations on reducing risk of seizures have been developed by agencies in the United Kingdom, Japan, and the International Telecommunications Union, affiliated with the United Nations. The Epilepsy Foundation of America has developed a consensus of medical experts and scientists on this subject, reported in an accompanying work.     

Classifications of Epileptic Syndromes: Advantages and Limitations for Evaluation of Childhood Epileptic Syndromes in Clinical Practice

The advantages and limitations of the two most recent International League Against Epilepsy classifications of the epilepsies and epileptic syndromes have been assessed after examining the clinical records of 645 consecutive outpatients aged 1 month to 15 years followed at the Children's Epilepsy Center of the University of Milan, Italy, from 1977 through 1985. The percentage of cases that could be classified according to the 1970 and 1985 proposals for classification were 94.1 and 98.1%, respectively. According to the 1985 proposal, partial epilepsies (PE) and generalized epilepsies (GE) were almost equally represented (45.0 vs. 47.2%). Among PE, symptomatic epilepsies were the commonest variety. In the group of GE, idiopathic and/or symptomatic epilepsies were most common. Childhood absence epilepsy was the largest subgroup among idiopathic GE. Newly diagnosed patients, a less biased sample of the epileptic population represented 38.9% of the entire sample, and a proper classification was possible in 96% of cases. Idiopathic epilepsies were about twice as frequent and idiopathic and/or symptomatic GE less frequent in newly diagnosed patients when compared with the remainder. Marked differences in the frequency of the epilepsies were found in comparison with other reports in the literature which used the 1970 classification. This finding probably depends on different diagnostic assessment, selection bias, and different geographic and ethnic components, but it can also reflect the variable interpretation of the clinical and EEG features of a patient with epilepsy in the light of the artifactual categories of the classification.     

Treatment Strategies for Myoclonic Seizures and Epilepsy Syndromes with Myoclonic Seizures

Summary:   Despite the availability of numerous treatment options, the diagnosis and treatment of myoclonic seizures continue to be challenging. Based on clinical experience, valproate and benzodiazepines have historically been used to treat myoclonic seizures. However, many more treatment options exist today, and the clinician must match the appropriate treatment with the patient's epilepsy syndrome and its underlying etiology. Comorbidities and other medications must also be considered when making decisions regarding treatment. Rarely, some antiepileptic drugs may exacerbate myoclonic seizures. Most epileptic myoclonus can be treated pharmacologically, but some cases respond better to surgery, the ketogenic diet, or vagus nerve stimulation. Because myoclonic seizures can be difficult to treat, clinicians should be flexible in their approach and tailor therapy to each patient.     

Photic- and pattern-induced seizures: expert consensus of the Epilepsy Foundation of America Working Group

PURPOSE: In August, 2004, the Epilepsy Foundation of America convened a workshop to begin to develop an expert consensus on photosensitive seizures. METHODS: Literature and data were reviewed, and consensus was derived from discussion. RESULTS: A flash is a potential hazard if it has luminance >or=20 cd/m2, occurs at a frequency of >or=3 Hz, and occupies a solid visual angle of >or=0.006 steradians (approximately 10% of the central visual field or 25% of screen area at typical viewing distances). A transition to or from saturated red also is considered a risk. A pattern with the potential for provoking seizures contains clearly discernible stripes, numbering more than five light-dark pairs of stripes in any orientation. When the light-dark stripes of any pattern collectively subtend at the eye from the minimal-expected viewing distance a solid angle of >0.006 steradians, the luminance of the lightest stripe is >50 cd/m2, and the pattern is presented for >or=0.5 s, then the pattern should display no more than five light-dark pairs of stripes, if the stripes change direction, oscillate, flash, or reverse in contrast; if the pattern is unchanging or smoothly drifting in one direction, no more than eight stripes. These principles are easier to apply in the case of fixed media, for example, a prerecorded TV show, which can be analyzed frame-by-frame, as compared with interactive media. CONCLUSIONS: A consensus view of stimuli likely to provoke visually evoked seizures can be developed.     

Epilepsy Genes and the Genetics of Epilepsy Syndromes: The Promise of New Therapies Based on Genetic Knowledge

Summary: Treatment strategies based on the molecular biology of the epilepsies may soon become a reality. Critical steps in this process are identifying molecular genetic defects in specific epilepsies, understanding of the neurobiologic consequences of those defects, and developing methods to correct the molecular defects or their downstream consequences. Identification of molecular defects is easier in single-gene epilepsies than in those with complex inheritance, although the latter are more common. A number of epilepsies have been mapped and, in two cases, specific genes have been identified. Unverricht-Lundborg disease is caused by defects in the cystatin B gene, with absence of the gene product. Autosomal dominant nocturnal frontal lobe epilepsy in some families is caused by mutations in the a4-subunit of the nicotinic acetylcholine receptor gene. In vitro studies suggest that the mutations lead to impaired function of the acetylcholine receptor, raising the possibility of cholinergic therapy for this condition. Advances in the molecular biology of the epilepsies are likely to change our understanding radically and to allow opportunities to develop innovative new treatments for epilepsy.     

Epilepsy in Rural Kentucky: Prevalence in a Population of School Age Children

Seizure prevalence among school age children residing in a rural western Kentucky area (Hardin County) was determined utilizing a method designed to minimize false positives and to allow estimation of false negatives. The observed prevalence of epilepsy is 5.7/1,000 and of febrile seizures 17/1,000. Because of the high rate of false negative responses detected by random sample interview, the true rate of febrile seizures is believed to be closer to a projected rate of 31/1,000.     

Ontogeny of Feline Temporal Lobe Epilepsy, II: Stability of Spontaneous Sleep Epilepsy in Amygdala-Kindled Kittens

We previously described a model of spontaneous "sleep epilepsy" in kindled kittens with temporal lobe epilepsy (TLE). We now describe the postkindling course of this model from preadolescence to maturity and suggest pathophysiologic mechanisms. Spontaneous epilepsy, particularly generalized tonic-clonic convulsions (GTCs), developed 1h to 4 months after amygdala kindling and persisted to adulthood. At first, GTCs were detected only in sleep; later, convulsions also occurred during wakefulness. Two factors were consistently associated with the sequential onset of sleep and waking GTCs: seizure clusters and anatomic seizure localization. (1) Seizure clusters. Cats with infrequent or unclustered GTCs continued to exhibit "sleep epilepsy," defined by convulsions occurring exclusively during sleep. In contrast, cats with frequent seizure clusters developed recurrent or terminal convulsive status in conjunction with GTCs during waking and sleep. Severe seizure manifestations therefore appeared to contribute to the dissociation of convulsions from the sleep-wake cycle. (2) Anatomical seizure localization. Focal seizure origin appeared to differentiate sleep from waking GTCs. Onset during sleep was first recorded in the kindled amygdala, whereas onset during waking was initially detected outside the temporal lobe. Findings thus suggest secondary "kindling" of multifocal epilepsy. Secondary epileptogenesis is consistent with "transsynaptic" kindling effects. This phenomenon is defined in mature animals by rapid secondary site kindling (transfer) and subtle morphologic changes distal to the stimulating electrode. Transfer may be accentuated by youth, because kittens developed spontaneous seizure foci in previously unstimulated tissue. Moreover, multifocal interactions and diffuse cell loss were implicated as possible mechanisms. Collectively, the findings indicate complications with early onset TLE in kindled cats. Onset during youth can have an unfavorable prognosis, reflected by recurrent status epilepticus and multifocal epilepsy with convulsions distributed throughout the sleep-wake cycle.     

Prevalence, Classification, and Severity of Epilepsy and Epileptic Syndromes in Children

Summary: Purpose: To determine the point prevalence of active childhood epilepsy in a defined area and evaluate the usefulness of ILAE classification of seizures, and epilepsies/syndromes with special interest in severe epilepsies. Methods: By using the latest ILAE International Classification of Epileptic Seizures (ICES, 1981) and Epilepsies and Epileptic Syndromes (ICE, 1989), we determined the age- and sex-specific prevalence rates of epilepsy, type of seizures, epilepsies, and recognizable epileptic syndromes, as well as the proportion of severe cases in each seizure/epilepsy/syndrome category in all children 0–15 years of age from a geographically defined area in Finland. All medical records, neurophysiological recordings and available clinical data were reviewed retrospectively. Results: Point prevalence of active epilepsy on December 12, 1992 was 3.94 per 1,000. According to ICESDCE, we were able to classify 96% of seizures and 90% of epilepsies and syndromes. Generalized seizure and epilepsy/syndrome types were more prevalent in children 0–6 years of age and partial/ localization-related in children 6–15 years of age. Epilepsy was intractable in 17% of all cases and correlated significantly with symptomatic etiology and early onset of epilepsy, as well as with additional neuroimpairments. Conclusions: A considerable number of cases fell into the nonspecific categories of ICE, which limits the value of present epilepsy/syndrome classification in terms of prognosis, prediction, and indication for special investigations in individual cases. A number of intractable cases was relatively low, indicating good prognosis in many childhood epilepsies, especially when additional neuroimpairments are absent.