Cases Of Intractable Epilepsy Research Articles

Disconnection of the pathological connectome for multifocal epilepsy surgery.

OBJECTIVERecent neuroimaging studies suggest that intractable epilepsy involves pathological functional networks as well as strong epileptogenic foci. Combining cortico-cortical evoked potential (CCEP) recording and tractography is a useful strategy for mapping functional connectivity in normal and pathological networks. In this study, the authors sought to demonstrate the efficacy of preoperative combined CCEP recording, high gamma activity (HGA) mapping, and tractography for surgical planning, and of intraoperative CCEP measures for confirmation of selective pathological network disconnection.METHODSThe authors treated 4 cases of intractable epilepsy. Diffusion tensor imaging-based tractography data were acquired before the first surgery for subdural grid implantation. HGA and CCEP investigations were done after the first surgery, before the second surgery was performed to resect epileptogenic foci, with continuous CCEP monitoring during resection.RESULTSAll 4 patients in this report had measurable pathological CCEPs. The mean negative peak-1 latency of normal CCEPs related to language functions was 22.2 ± 3.5 msec, whereas pathological CCEP latencies varied between 18.1 and 22.4 msec. Pathological CCEPs diminished after complete disconnection in all cases. At last follow-up, all of the patients were in long-term postoperative seizure-free status, although 1 patient still suffered from visual aura every other month.CONCLUSIONSCombined CCEP measurement, HGA mapping, and tractography greatly facilitated targeted disconnection of pathological networks in this study. Although CCEP recording requires technical expertise, it allows for assessment of pathological network involvement in intractable epilepsy and may improve seizure outcome.

Optogenetic inhibition of chemically induced hypersynchronized bursting in mice

Synchronized activity is common during various physiological operations but can culminate in seizures and consequently in epilepsy in pathological hyperexcitable conditions in the brain. Many types of seizures are not possible to control and impose significant disability for patients with epilepsy. Such intractable epilepsy cases are often associated with degeneration of inhibitory interneurons in the cortical areas resulting in impaired inhibitory drive onto the principal neurons. Recently emerging optogenetic technique has been proposed as an alternative approach to control such seizures but whether it may be effective in situations where inhibitory processes in the brain are compromised has not been addressed. Here we used pharmacological and optogenetic techniques to block inhibitory neurotransmission and induce epileptiform activity in vitro and in vivo. We demonstrate that NpHR-based optogenetic hyperpolarization and thereby inactivation of a principal neuronal population in the hippocampus is effectively attenuating seizure activity caused by disconnected network inhibition both in vitro and in vivo. Our data suggest that epileptiform activity in the hippocampus caused by impaired inhibition may be controlled by optogenetic silencing of principal neurons and potentially can be developed as an alternative treatment for epilepsy.

Influence of full -course health education on treatment compliance among intractable epilepsy children with ketogenic diet therapy

Objective To explore the influence of full -course health education on parents' awareness of diseases and treatment compliance among intractable epilepsy children with ketogenic diet therapy. Methods Sixty nine intractable epilepsy cases with ketogenic diet therapy were randomly divided into control group (34 cases) and experimental group (35 cases) according to the admission order. The routine nursing was given to 34 cases in control group. On basis of routine nursing , full -course health education was given to 35 cases in experimental group. The analysis was carried out before and after intervention in two groups by self-designed questionnaire. x2 test was used to evaluate the parents'awareness of diseases, treatment compliance and therapeutic effects. Results The parents'awareness of diseases, treatment compliance and therapeutic effect were higher in experimental group than those in control group ( P ＜ 0. 01 ) . Conclusions Full -course health education can improve the parental awareness of the disease and treatment compliance, thereby enhance the life quality of children with intractable epilepsy. Key words: Full -course health education; Intractable epilepsy; Ketogenic diet; Compliance

Efficacy of clobazam monotherapy in three cases of symptomatic localization-related epilepsy in children

Clobazam is approved in many countries as an additional antiepileptic drug for the management of intractable epilepsy. However, a number of reports have also described its use as first-line therapy in this indication. In this article, we describe three cases involving children (aged 2–12 years) with symptomatic localization-related and intractable epilepsy who responded to low dosage (0.2–0.4 mg/kg/day) clobazam monotherapy. For periods of 3.1–4.4 years, the treatment was well-tolerated and improved electroencephalogram findings with complete disappearance of clinical convulsions. Thus, low-dose clobazam appears to be a useful treatment option in these difficult-to-manage cases of intractable epilepsy.

Increased expression of aquaporin-1 in the anterior temporal neocortex of patients with intractable epilepsy

Objective: Aquaporin-1 (AQP1), the osmotic water channel, is located in choroidal plexus, which facilitates the cerebrospinal fluid formation in central nervous system (CNS). AQP1 has been speculated to maintain the homeostasis of intracellular and extracellular water in the brain, while the intractable epilepsy (IE) may be related to the imbalance in water and ion homeostasis.Methods: To investigate the role of AQP1 in pathophysiology of IE, we studied the expression of AQP1 in surgical samples of the anterior temporal neocortex of patients with IE and the age-matched controls samples.Results: Using immunohistochemistry, it was shown that AQP1 expression increased in astrocytes, but not in neurons or oligodendrocytes. Double-label immunofluorescence and confocal microscopy disclosed AQP1 immunoreactivity at the astrocyte membranes, where the most abundant expression was in the perivascular glial processes, which were recognized with antiglial fibrillary acidic protein antibodies (anti-GFAP).Conclusion: For the first time, we showed high expression of AQP1 water channels in IE cases and suggest two mechanisms to explain this finding. Increased AQP1 expression of astrocytes may be a cause or a consequence of IE. Thus, additional works are needed to elucidate the true mechanism underlying their relationship.

Novel variant of cortical dysplasia in two cases of infantile intractable epilepsy

Cortical dysplasia (CD) is a frequent cause of intractable epilepsy (IE), especially infantile spasms. CD is defined by neuropathologic features of neuronal disarray, dysmorphism and heterotopia, frequently together with neuronal cytologic abnormalities and “balloon cells”. We report two cases of infantile IE, a 5‐year‐old male and a 17‐year‐old female in whom neuroimaging studies showed findings consistent with CD and histopathologic examination of the corticectomies revealed severe CD with abundant Rosenthal fibers (RF), widely distributed throughout subpial regions and within subcortical white matter. The abundance and location of RF was reminiscent of neuropathologic changes seen in Alexander's disease. Sequencing of the GFAP gene was performed on DNA extracted from resected frozen tissue in one case and although no mutation was found three previously known SNPs were identified. Rare similar cases have been reported (Kupsky & Frosch 1993). The abundant RF seen in association with CD may represent a dysregulation of synthesis/metabolism of alpha B‐crystallin, the main component of the RF, aggregating in association with GFAP, or be secondary to the severity of the CD which produces a dys/upregulation of GFAP to levels sufficient to induce RF and localized leukodystrophy due to astrocytic dysfunction.

A Case of Intractable Epilepsy in a Double Cortex Syndrome

This article describes a very rare case of a double cortex syndrome in a man aged 32 years old who started from the age of 14 years having seizures and many other epileptic manifestations that continue to the present age, being always intractable to various therapeutic regimes. The neuroimaging revealed cortical ectopias in the cingulum, the visual cortex, in the middle part of the superior temporal gyrus, in the frontal pole as well as in the middle area of precentral gyrus. This article attempts to underline the behavioral disturbances, the learning difficulties, the psychological fluctuations, and the multitude of the seizures that have been released during the clinical course of the patient. The article attempts to correlate the clinical phenomena of the patient and the resistance to therapeutical interventions with the morphological changes as they have been visualized by the neuroimaging techniques, reviewing in addition relevant cases from the literature.

The family experience of "sudden health": the case of intractable epilepsy.

This article reports the experience of "sudden health" among six families who participated in an exploratory qualitative study of families with a member who elects to have corrective surgery for intractable epilepsy. Families were interviewed pre- and post-surgery (6-8 months) and the interviews were analyzed using a constant comparative methodology. Findings indicated that (1) families were organized in two primary ways (nesting and crisis) to deal with epilepsy and the aftermath of surgery and (2) "sudden health" had differing effects on these families depending on their organizational style, emotional communication process, and developmental dynamics.

A Case of Intractable Epilepsy: Diagnosis of Tuberous Sclerosis Based on Histopathological Findings and Immunohistochemical Expression of Hamartin and Tuberin

A Case of Intractable Epilepsy: Diagnosis of Tuberous Sclerosis Based on Histopathological Findings and Immunohistochemical Expression of Hamartin and Tuberin

Long‐Term Outcome of Functional Hemispherectomy in Two Cases of Diffuse Cortical Dysplasia of One Hemisphere

Purpse: Patients with diffuse cortical dysplasia in one cerebral cortex, such as those with cortical hemihypertrophy, are well known to have intractable epileptic seizures and severe developmental delay of mental state and motor skills. We previously reported that the region of cortical dysplasia shows the histologic characteristics of neuronal hyperactivity with impaired γ‐aminobutyric acid (GABA)‐mediated transmission. Resections of one hemisphere have historically been performed to control such epileptic seizures, although often they also cause severe complications such as massive bleeding and hydrocephalus. To decrease the severity and incidence of such complications, a procedure known as modified hemispherectomy was recently developed. We performed functional hemispherectomies on two cases of intractable epilepsy caused by unilateral cortical dysplasia. The purpose of this study was to evaluate the long‐term clinical effects of this surgery for epileptic seizure, including the effects on mental state and motor skills. Patients and Methods: Case 1 was a 4‐year‐old boy whose epilepsy first occurred 2 weeks after birth. The seizures were focal motor seizures and secondarily generalized seizures, which began from the region of the right face. The seizures occurred >l00 times every day and sometimes involved the pharyngeal region, leading to apneic episodes. The patient was severely retarded and had no head control, could not respond to his name being called, and could not track movements visually. Case 2 was a 2–year 10–month‐old boy, whose epilepsy appeared at birth and whose seizures were simple motor and secondarily generalized seizures occumng 20–50 times a day. He was a floppy infant, could not sit, could crawl only on his elbows, and could not speak. The epileptic seizures of both cases were intractable to any anticonvulsant therapy. Case 1 had unilateral cortical hypertrophy of left hemisphere on magnetic resonance imaging (MRI), and case 2 had a large cortical hyperplasia occupying almost two thirds of the left hemisphere. The epileptic seizures were confirmed to originate from the region of the cortical dysplasia by both ictal and interictal physiological examinations, including EEG and single‐photon‐emission computed tomography (SPECT). Functional hemispherectomy with resection of the parietal cortex and fiber disconnection was performed in case 1. Case 2 received a modified hemispherectomy. which involved only a fiber‐disconnection procedure, and was originally developed by Simizu. The clinical effects of the surgical treatment were evaluated in both cases with regard to epileptic seizures and mental and motor development. The follow‐up period was 3.5 years in case 1, and 1.5 years in case 2. Results: Unfortunately, case 1 had a massive intracranial hemorrhage and hydrocephalus shortly after the operation. Thereafter, however, he became stable and could eat, respond to stimuli, such as the voice of his mother, and follow movements visually. His epileptic seizures disappeared after treatment with only phenytoin (PHT). In case 2, the seizures disappeared completely with no anticonvulsant therapy from the day of the operation. His right hemiplegia recovered remarkably, and he can now stand and walk, and he can use his right fingers. He can speak, sing a song, and seems happy. Although soon after operation, he was almost completely deaf in the right ear, he can now respond even to a whisper. Conclusions: Because patients with diffuse cortical dysplasia often have severe epileptic seizures and developmental delays, surgical treatment should be considered to control the epilepsy and allow developmental “catch‐up”, and should be performed, if necessary, as soon as possible after determining its intractability to anticonvulsant medication.

A case of intractable epilepsy with left pachygyria. The effect of TRH-T treatment with a nasal nebulizer

A case of intractable epilepsy with left pachygyria. The effect of TRH-T treatment with a nasal nebulizer

A case of intractable epilepsy associated with congenital hypotonia and intermittent episodes of psychomotor regression and hemiparesis

A case of intractable epilepsy associated with congenital hypotonia and intermittent episodes of psychomotor regression and hemiparesis

A case of intractable epilepsy with mental deterioration: Detection of measles virus genome in cerebrospinal fluid and peripheral mononuclear cells using reverse transcriptase‐polymerase chain reaction

Psychiatry and Clinical NeurosciencesVolume 49, Issue 3 p. S294-S295 A case of intractable epilepsy with mental deterioration: Detection of measles virus genome in cerebrospinal fluid and peripheral mononuclear cells using reverse transcriptase-polymerase chain reaction TASUKU MIYAJIMA MD, Corresponding Author TASUKU MIYAJIMA MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanDepartment of Pediatrics, Tokyo Medical College, 6–7-1 Nishi-shinjuku, Shinjuku-ku, Tokyo 160, Japan.Search for more papers by this authorHISASHI KAWASHIMA MD, HISASHI KAWASHIMA MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this authorAKINORI HOSHIKA MD, AKINORI HOSHIKA MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this authorMASAAKI OGIHARA MD, MASAAKI OGIHARA MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this authorNAOTO YAMADA MD, NAOTO YAMADA MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this authorWANG CHUAN-YU MD, WANG CHUAN-YU MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this authorKEIKO KINOUE MD, KEIKO KINOUE MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this authorKOUJI TAKEKUMA MD, KOUJI TAKEKUMA MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this author TASUKU MIYAJIMA MD, Corresponding Author TASUKU MIYAJIMA MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanDepartment of Pediatrics, Tokyo Medical College, 6–7-1 Nishi-shinjuku, Shinjuku-ku, Tokyo 160, Japan.Search for more papers by this authorHISASHI KAWASHIMA MD, HISASHI KAWASHIMA MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this authorAKINORI HOSHIKA MD, AKINORI HOSHIKA MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this authorMASAAKI OGIHARA MD, MASAAKI OGIHARA MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this authorNAOTO YAMADA MD, NAOTO YAMADA MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this authorWANG CHUAN-YU MD, WANG CHUAN-YU MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this authorKEIKO KINOUE MD, KEIKO KINOUE MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this authorKOUJI TAKEKUMA MD, KOUJI TAKEKUMA MD Department of Pediatrics, Tokyo Medical College, Tokyo, JapanSearch for more papers by this author First published: June 1995 https://doi.org/10.1111/j.1440-1819.1995.tb02214.xAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume49, Issue3June 1995Pages S294-S295 RelatedInformation

Clinical application of SPECT to the localization of corticoepileptogenic focus of intractable epilepsy.

This paper presented 51 cases of intractable epilepsy, of which 36 were males, and 15 females, with a mean age of 21. They were all verified through CT/MRI, among which 17 were proved to have lesions on the brain, and 34 to have no lesion. Before operation all the patients were identified by SPECT and cerebral blood flow imaging (CBF). During the operation they were monitored under subdural strip electrode ECoG for the localization of corticoepileptogenic focus. The results showed that the sensitivity rate of localization with SPECT for corticoepileptogenic focus location was 97.87%.

A Case of Intractable Epilepsy with Pachygyria: the Effect of TRH-T Treatment

A Case of Intractable Epilepsy with Pachygyria: the Effect of TRH-T Treatment

EEG scalp electrode projection onto three-dimensional surface rendered images of the brain.

A technique is described for generating magnetic resonance-based, surface rendered images of the brain with electroencephalographic (EEG) scalp electrode positions projected onto the cortical surface. This technique (EEG electrode projection) was used in 10 patients who subsequently underwent surgery for medically intractable frontal lobe epilepsy. In most cases of intractable epilepsy, successful surgery entails the resection of electrophysiologically abnormal cortical tissue rather than an identifiable mass lesion. EEG electrode projection is a unique and useful surgical tool because it provides images that spatially correlate the surface anatomy of the brain and the electrophysiologic abnormality recorded at the scalp. Excellent correlation was found between cortical topography delineated by the surface rendered images and cortical anatomy at surgery. Agreement between EEG electrode projection and electrocorticography as to the location of the electrophysiologic abnormality increases confidence that appropriate cortical areas have been identified for resection. The technique provides new and unique insight into important anatomic-electrophysiologic relationships and aids in formulation of surgical strategy.

Clinical Course and Prognosis of Childhood Epilepsy

Remarkable progress has been accomplished in epileptology in recent years. The remission rate in childhood epilepsy has been improved by the introduction of new technologies and better diagnostic as well as therapeutic methods. Investigation of prognosis is an important field of epileptology, because prognostic improvement reflects its overall progress. A long-term follow-up study of childhood epilepsy was undertaken in the Okayama University Hospital. Ten to 15 years of follow-up was possible in 730 of 1,295 patients who were first diagnosed at ages below 15 years, from 1968 to 1971. The 3-year remission rate amounted to 82.0% and 5-year remission was obtained in 79.1%. These high rates of remission indicate the favorable prognosis of childhood epilepsy. On the other hand, cases of intractable epilepsy also amounted to a considerable number. Intractable epilepsy consisted mainly of age-dependent epileptic encephalopathy (Ohtahara's syndrome, West syndrome and Lennox-Gastaut syndrome) and severe myoclonic epilepsy in infancy. Development of effective therapy for these intractable epileptic syndromes will be an important subject of future studies.

Chronic Cerebellar Stimulation in the Treatment of Epilepsy

Six cases of intractable epilepsy who were subjected to chronic cerebellar stimulation are reported. They had all been treated with a wide range of anticonvulsant drugs without success. The ages varied between 17 and 55 years. Three were genetic-inherent, 2 were of unknown etiology, and 1 was symptomatic (infection? trauma?). Four had generalized major convulsions and 2 had a mixture of major and minor attacks, 1 with a strong temporal lobe component. Stimulation was commenced in all cases as soon as the patients had a postoperative seizure. Headache occurred in all cases and necessitated reduction in the strength of the stimulation to between 2 and 4 V in all cases except 1. Occipital nerve block temporarily relieved 1 patient, but posterior root section of C2-C3 and part of C4 did not prevent headaches in another case. Two patients were greatly improved and managed to hold employment whereas formerly they had been unemployed. One patient felt 50% improved but there was no reduction in his seizure frequency; another had a 50% reduction in seizures but felt no better. One patient was noted to be improved intellectually although her seizures were more frequent. The remaining patient was unchanged. One patient committed suicide and another drowned. One prosthesis became infected and had to be removed.

Diagnostic and Prognostic Significance of Electroencephalography with Intravenous Diazepam in Epilepsy

Thirty cases of intractable epilepsy in children were investigated to observe the correlation between the suppression of seizure discharges to intravenous diazepam and the type of seizure or effects of subsequent therapy. Suppression of seizure discharges were bilaterally poor in 75% of the cases with infantile spasms and 89% of cases with Lennox syndrome and other generalized seizures while it was unilaterally poor in 80% of the cases with focal or unilateral seizures. ACTH or steroid therapy was clinically and electroencephalographically effective in 80% of the cases with good suppression responses and in none of cases with good suppression that was resistant to the therapy. On the other hand, clinical seizures were not controlled in 73% of the cases showing poor suppression and EEG did not reveal improvement in 82% of the cases with poor suppression. EEG with intravenous diazepam is valuable for understanding the pharmacophysiological mechanism of epilepsy and it may be possible to select cases as candidates for ACTH or steroid therapy using this technique.