Advances in sleep-related hypermotor epilepsy / 浙江大学学报·医学版

Sleep-related hypermotor epilepsy (SHE), formerly known as nocturnal frontal epilepsy, is characterized by asymmetrical tonic or complex hypermotor seizures during sleep, with transient, frequent and clustering attack. The accurate incidence is not known but somehow low, which is estimated about 1.8/100 000. The differential diagnosis between SHE and parasomnias may be challenging due to possible similarities between the two sleep-related manifestations. In a majority of patients, the etiology is unknown. Identified etiologies are heterogeneous and structural abnormalities,which are involved in the severity and prognosis of SHE. In terms of treatment, it mainly includes pharmacological therapy and surgery. Carbamazepine seems to be the drug of choice in SHE patients, and epilepsy surgery provides excellent results in selected drug-resistant SHE cases. This review will focus on diagnosis, pathogenesis, treatment and prognosis of SHE, aiming to promote its early diagnosis and appropriate treatment.

Electroencephalograph network connectivity of working memory disorder in frontal lobe epilepsy pa-tients / 中华行为医学与脑科学杂志

Objective To investigate the possible electroencephalograph network connectivity mech-anism of working memory disorder in patients with frontal lobe epilepsy ( FLE). Methods Seventeen adult FLE patients were enrolled as the case group and 22 normal adults were selected as the control group. The behavioral data and 34-channel electroencephalographs were recorded in the working memory behavioral par-adigm. The directed transfer function was used to construct the causal connectivity brain network of theta band of working memory delay period. Two-sample t-test or tˊ-test was used to analyze the differences in be-havioral and frontal causal connectivity between the two groups. Results (1) Compared with control group, working memory behavioral data of FLE group showed significantly lower correct rate ((92. 778±4. 399)%, (96. 258±2. 470)%,tˊ=-2. 925,P<0. 01),and significantly longer reaction time ((978. 586± 65. 161) ms,(798. 671±196. 207)ms,tˊ=4. 023,P<0. 001). (2) Compared with control group,causal connectivity of FLE group showed significantly reduced whole brain ( tˊ=-6. 008, P<0. 001), Fz channel ( tˊ=-7. 703,P<0. 001),frontal region (tˊ=-14. 667,P<0. 001),frontal-temporal interval ( t=-14. 467,P<0. 001),and frontal-central interval (t=-3. 501,P<0. 001). The causal connectivity of frontal-occipital in-terval had no significant difference( t=1. 056,P=0. 281). Conclusions Working memory disorders were found in FLE patients. The abnormal causal connectivity of frontal brain network in theta band may be one of its potential neurophysiological mechanisms.

The genes research progress in frontal lobe epilepsy / 国际儿科学杂志

Frontal lobe epilepsy,of which the morbidity is second to temporal lobe epilepsy,is one of the most common types of focal epilepsy.Its clinical manifestations are diversiform.Ictal electroencephalogram (EEG) and cranial imaging examinations are helpful in diagnosis making,but the misdiagnosis rate is still high.With the development of gene diagnosis and precision medicine in recent years,the use of genetic tests is increasing for frontal lobe epilepsy.Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) as a common type of frontal lobe epilepsy is most closely associated with gene mutations.So far,6 kinds of genetic mutations have been found associating with frontal lobe epilepsy.Research progress in pathogenic mutafons will enhance the advancement of diagnosis and treatment of frontal lobe epilepsy.

Alteraciones en las ejecuciones viso-perceptuales en pacientes con síndromes electro-clínicos de epilepsias del lóbulo temporal y frontal / Visuoperceptual performances impairments in patients with electro-clinical syndromes of temporal lobe and frontal lobe epilepsies

RESUMEN INTRODUCCIÓN: Según el modelo de actividad modular encapsulada del foco epiléptico (FE), las epilepsias del lóbulo temporal (ELI) y del frontal (ELF) se asocian con alteraciones cognitivas de memoria, atención, lenguaje y función ejecutiva. Por eso la mayoría de los protocolos neuropsicológicos se centran en la evaluación de estas funciones. Las habilidades viso-perceptuales se evaluarían en los pacientes con epilepsias del cuadrante posterior (ECP). OBJETIVO: Establecer las alteraciones viso-perceptuales en pacientes adultos con síndrome electro-clínico compatible con ELT y ELF de Medellín, Colombia, al compararse con un grupo de personas sin epilepsia. Pacientes y métodos: la muestra estuvo conformada por 19 pacientes, 10 (52,6 %) mujeres, edad de 43,0±12,3 (IC 95 %:37,1-48,9) años, con síndromes electro-clínicos por video-telemetría compatibles con ELT o ELF, con capacidad intelectual total estimada 107,2±14,1 (IC95 %:100,3-114,0). Se compararon con 16 personas sin epilepsia, 9(56,2 %) mujeres, edad 41,8±12,7 (IC95 %:35,1-48,6) y capacidad intelectual de 117±17,5 (IC95 %:108,6-127,2). Se les aplicó un protocolo para evaluación de procesos viso-perceptuales. RESULTADOS: Los pacientes presentaron un rendimiento significativamente inferior (p<0,05) en discriminación de figura-fondo, orientación viso-espacial de líneas y en el reconocimiento diferido de la memoria visual de puntos. Estas dificultades fueron mayores en los pacientes con ELF. CONCLUSIONES: Se observan alteraciones en ejecuciones viso-perceptuales y de almacenamiento de memoria visual más severas en pacientes con ELF. Esto apoya el modelo de redes complejas (no modulares y no encapsuladas), tanto para la actividad epiléptica como para la cognitiva. Por esto, los procesos viso-perceptuales deben ser evaluados en los pacientes con síndromes de ELT o ELF.

SUMMARY INTRODUCTION: According to modular encapsulated activity model of the epileptic focus (EF), Temporal lobe epilepsies (TLE) and Frontal lobe epilepsies (FLE) are associated to memory, attention, language and executive function impairments. For this reason most of the neuropsychological protocols are focused in the assessment of these functions. Visuoperceptual skills only would be assessed in patients with posterior quadrant epilepsies (PQE). OBJECTIVE: To establish visuoperceptual impairment in a sample of electro-clinical syndromes compatibles with TLE and FLE from Medellín-Colombia, compared with a group of healthy people without epilepsy. Patients and methods: sample was constituted by 19 patients, 10 women (52,6%), aged 43,0±12,3 (CI95%:37,1-48,9), with electro-clinical syndromes compatible with TLE or FLE, median estimated IQ 107,2±14,1 (CI95%:100,3-114,0). They were compared with 16 healthy people without epilepsy, 9(56,2%) women, median age 41,8±12,7 (CI95%:35,1-48,6) and 117±17,5 (IC95%:108,6-127,2). A visuoperceptual protocol was administered to both groups. RESULTS: Patients presented significant lower (p<0,05) on figure-background discrimination, visuospatial line orientations, and delayed recall by recognition of dots visual memory. These impairments were significant worst in FLE patients. CONCLUSIONS: TLE and FLE patients have visuoperceptual and delayed visual memory store impairments, which were significant worst in FLE patients. These findings support the complex network (non-modular and non-encapsulated) model to explain EF and cognitive functioning in patient with TLE and FLE. Visuoper-ceptual processes should be always assessed in these patients.

Emerging Surgical Strategies of Intractable Frontal Lobe Epilepsy with Cortical Dysplasia in Terms of Extent of Resection

OBJECTIVE: Cortical dysplasia (CD) is one of the common causes of epilepsy surgery. However, surgical outcome still remains poor, especially with frontal lobe epilepsy (FLE), despite the advancement of neuroimaging techniques and expansion of surgical indications. The aim of this study was to focus on surgical strategies in terms of extent of resection to improve surgical outcome in the cases of FLE with CD. METHODS: A total of 11 patients of FLE were selected among 67 patients who were proven pathologically as CD, out of a total of 726 epilepsy surgery series since 1992. This study categorized surgical groups into three according to the extent of resection : 1) focal corticectomy, 2) regional corticectomy, and 3) partial functional lobectomy, based on the preoperative evaluation, in particular, ictal scalp EEG onset and/or intracranial recordings, and the lesions in high-resolution MRI. Surgical outcome was assessed following Engel's classification system. RESULTS: Focal corticectomy was performed in 5 patients and regional corticectomy in another set of 5 patients. Only 1 patient underwent partial functional lobectomy. Types I and II CD were detected with the same frequency (45.45% each) and postoperative outcome was fully satisfactory (91%). CONCLUSION: The strategy of epilepsy surgery is to focus on the different characteristics of each individual, considering the extent of real resection, which is based on the focal ictal onset consistent with neuroimaging, especially in the practical point of view of neurosurgery.

Sleep frequent tongue bite / 中华实用儿科临床杂志

The 10-month baby boy,with normal development,mainly due to sleep in frequent tongue bite nearly 4 months.Bitten his tongue after faring asleep,biting bleeding,bite pain awake.Many of his tongue ulcers,serious impact on children's lives,family companionship in suffering.History found in the supplementary week before the onset of the left frontal children hurt skin bruising.Electroencephalogram showed:Sleep of epileptiform discharges in the left frontal and central anterior temporal areas,but bite the tongue during sleep electroencephalogram synchronization no relevant abnormal discharge.The final diagnosis of traumatic epilepsy,frontal lobe epilepsy syndrome automatically lead to tongue bite tongue with traumatic ulcers.Oral Clonazepam 0.25 mg before sleep,the symptoms disappeared that night,nighttime sleep peacefully.His tongue ulceration has healed after a month.Readers are advised to take advantage of these key parts of the diagnostic process and diagnostic thinking or diagnostic procedures,combined with their own clinical practice,serious thinking,learning,summarized,and benefit from it.

None mutation in the CHRNA4 gene of 6 families of South China with autosomal dominant noctumal frontal lobe epilepsy was found / 中华实用儿科临床杂志

Objective To investigate the virulence gene and mutation features in the Chinese patients with autosomal dominant noctumal frontal lobe epilepsy(ADNFLE) by using the direct sequencing(DS) PCR products with all the exons of CHRNA4 in 6 ADNFLE families,and to interpret the molecular pathogenesis in Chinese patients affected by ADNFLE.Methods Six ADNFLE families were collected,included 66 people and 24 patients with ADNFLE,and 200 healthy volunteers were selected as control group.The genomic DNA was extracted.The exons 1-6 in CHRNA4 were amplified by the PCR.The amplified products were sequenced and analyzed.All data were analyzed with SPSS 13.0 software.Results There were 4 base substitutions in exon 5,and they were c.909T ＞ G,c.1440G ＞ T,c.1458T ＞ C and c.942C ＞ T.All those base substitutions were synonymous.The first three were homozygosis substitutions,but the last one was heterozygosis substitutions.Conclusions The hot spot mutations of CHRNA4 which have been reported were not detected.Whether or not there is a correlation between ADNFLE and this substitution need to be identified by study with

Evaluación, seguimiento y rehabilitación controlada de la función motora en pacientes con cirugía de epilepsia fármaco resistente / Evaluation, monitoring and rehabilitation of motor function controlled in patients with drug resistant epilepsy surgery

La epilepsia es un trastorno neurológico crónico que afecta cerca de 45 millones de personas y responde por el 1% de la carga global de enfermedad. Entre 20 y 40% de todos los casos son epilepsia fármaco resistente, cuya opción de manejo es generalmente quirúrgica. La ocurrencia de déficit neurológico secundario a cirugía por epilepsia fármaco resistente es menor al 6%. Puede presentarse un déficit motor en cirugía de epilepsia fármaco resistente por fenómenos vasculares en el posoperatorio, a pesar de preservar la corteza motora; o secundario a resección de la zona epileptogénica, cuando está ubicada sobre el área M1-S1 o en la corteza de asociación motora. Se presentan dos pacientes con cirugía para epilepsia fármaco resistente, la evaluación, el seguimiento y la rehabilitación controlada de la función motora voluntaria, por neurorehabilitación.

Epilepsy is a chronic neurological disorder that affects about 45 million people and accounts for 1% of the global burden of disease. 20% to 40% of all cases are drug-resistant epilepsy (DRE), which management option is usually surgical. The occurrence of neurological deficit secondary to surgery DRE is less than 6%. Motor deficits may occur in the postoperative as vascular events, although preserving motor cortex or secondary to resection of epileptogenic area, when area is located on M1-S1 or in motor association cortex. This paper present two patients with surgery for DRE, its assessment, monitoring and rehabilitation of motor function by neurorehabilitation.

Executive functions in patients with frontal and temporal lobe epilepsy / 中华行为医学与脑科学杂志

ObjectiveTo investigate the executive functions of the frontal and temporal lobe epilepsy patients.MethodsFifty-five epilepsy patients (twenty-five frontal lobe epilepsy patients and thirty temporal lobe epilepsy patients) and fifty age,gender and education matched healthy controls were evaluated by means of Mini Mental State Examination ( MMSE ).Executive function was assessed using Stroop color words test and trail making test.ResultsMMSE total score of frontal and temporal lobe epilepsy patients were ( 26.02 ± 0.30) and (25.82 ± 0.67 ),respectively.There was significant abnormality between epilepsy patients and the controls (P ＜0.05).Compared with normal control group,FLP group and TLP group took longer time and their score was lower in stroop color words test (P＜0.05).There were significant difference between FLP and TLP on card B and C's reading time,correct number and strcop interference effects (P＜0.05).The epilepsy patients performed significantly worse than the controls in whole trail making test (P＜0.05).The scores indicate that the TLE group outperformed the FLE group on all analysis values (P ＜ 0.05 ).ConclusionThe frontal and temporal lobe epilepsy patients have executive function deficit.The FLE group has prominent deficits in executive functioning.

The significance of clinical feature in the source localization of epileptogenic zone in children with frontal lobe epilepsy / 中国小儿急救医学

ObjectiveTo investigate the significance of clinical feature in the source localization of epileptogenic zone in children with frontal lobe epilepsy.MethodsThirty children who were diagnosed frontal lobe epilepsy consecutively admitted to the pediatric department of Shengjing hospital from Oct 2010 to Jun 2011 were recruited for retrospective study.All the cases were subject to video-electroencephalogram monitoring.Results Seventy-six episodes with clear origins of the electroencephalogram were monitored in 30 cases,including orbital frontal attack 11 times (14.5％),dorsolateral frontal attack 33 times (43.4％),mesial frontal attack 32 times (42.1％ ).The correlation analysis showed that the head and eye deviation (47 times,61.8％ ),asymmetric tonic seizures (32 times,42.1％ ) and parakinesia seizures (29 times,38.2％ ) prompted the focus was located in the dorsolateral frontal and mesial frontal.But there was no significant difference between the two areas ( x2 =10.58,9.67,11.55,P ＞0.05).The vocalization,manual pedal automatism and emotional seizure prompted the focus was located in the dorsolateral frontal and orbitofrontal;and the emotional seizure was significant differences between the two areas(x2 =38.68,P ＜0.001 ).Emotional seizure was the characteristic of orbitofrontal attack.Conclusion The clinical features have important significance in the judgment of the source localization of epileptogenic zone in children with frontal lobe epilepsy.The emotional seizure could be considered as the characteristic attack of orbitofrontal.

Clinical and electroencephalographic characteristics of frontal lobe epilepsy / 中国综合临床

Objective To explore the clinical and electroencephalographic characteristics of frontal lobe ep- ilepsy(FLE).Methods The clinical and electroencephalographic data of 80 patients with FLE were analyzed.Re- suits The seizure types of frontal lobe epilepsy were simple focal seizure-complex focal seizure and secondary gen- eral tonic.clonic seizure.Seizures were often exhibited in relatively short duration,hyper motor,tonic or postural.Fre- quent nocturnal attacks and slight postictal mental confusion was increased.Interictal EEG manifested sharps or spikes or slow wave in the frontal area.Ictal EEG showed paroxysm rhythms.Conclusion FLE is a distinct epilepsy syndrome.

Non-epileptic paroxysmal events during sleep: Differentiation from epileptic seizures / 소아과

This review describes the wide spectrum of paroxysmal events during sleep in infancy and childhood. The differential diagnosis between sleep-related non-epileptic paroxysmal events and epileptic seizures is difficult in special occasions. The nocturnal frontal lobe seizure and of the more common non- epileptic paroxysmal events during sleep are described. The main differentiating features characterizing parasomnias are: onset in early childhood, rare episodes of long duration, relatively lower frequency per night, absence of stereotypy, gradual disappearance of older age. Video-polysomnography is the gold standard to diagnosing and differentiating parasomnias from nocturnal frontal lobe seizures.

Surgical Treatment of Intractable Frontal Lobe Epilepsy / 대한간질학회지

PURPOSE: Frontal lobe epilepsy is difficult to be managed with medical or surgical treatment. So the authors analyzed and reviewed the clinical results of the frontal lobe epilepsy patients who underwent surgical treatment. METHODS: From 1996 to 2004, Thirty-nine patients were treated surgically. The authors reviewed retrospectively the radiological, clinical and pathological data of the patients. The surgical outcome was classified using Engel's classification. RESULTS: The mean follow-up period is 70.5 (+/-29.7) months. The preoperative MR image was normal in 19 cases and abnormal in 20 cases. There were 11 encephalomalacias, 4 cortical dysplasias and 5 brain tumors. The postoperative pathological finding was normal in 5 cases and abnormal finding in 34. There were 20 cortical dysplasias, 4 encephalomalacias, 3 gliosises, 2 fibrous scars, 2 cavernous malformations, 1 dysembryoplastic neuroepithelial tumor, 1 paragonimiasis and 1 venous thrombosis. The postoperative surgical outcomes were as follows: Class I, 41%; Class II, 25.6%; Class III, 20.5%; Class IV, 12.8%. CONCLUSIONS: The surgical outcomes of frontal lobe epilepsy have improved, compared with previous series. The pathological abnormality was observed in large portion of the frontal lobe epilepsy patients.

Analysis of Positron Emission Tomography and Video Electroencephalogram in Children with Nocturnal Frontal Lobe Epilepsy / 实用儿科临床杂志

0.05).Of essay group 19 children whose PET were normal or slight abnormal,8 children's VEEG had epileptifrom abnormalities only appear in lucid interval,8 children's VEEG had epileptifrom abnormalities appear in nocturnal sleep period,3 children's VEEG had epileptifrom abnormalities appear in lucid interval and nocturnal sleep period.Of essay group,7 children whose PET were serious abnormal,6 children's VEEG had epileptifrom abnormalities appear in lucid interval and nocturnal sleep period.The PET outcome was relate with the time of VEEG epileptic discharge(r=0.461 P

Clinical and Electroencephalography Features of Childhood Frontal Lobe Epilepsy / 实用儿科临床杂志

Objective To explore the clinical and electroencephalography features of children's frontal lobe epilepsy(FLE)and improve the diagnosis and therapy.Methods Medical records were reviewed and neuropsychological evaluations of patients with FLE diagnosed from 2000 to 2006.Children were examined by 24 h EEG and imaging examinations.Their charts for family medical history,seizure,aura,simultaneous phenomenon and anti-epilepic drug therapeutic effects were reviewed.Results Seizures were clonus in 24 cases,tonic in 4 cases,adversive,atonic,vomit in 2 cases,respectively,paresthesia,cephalic,visul,sensory in 1 case,Imaging finding were in 9 of 40.All the 40 cases were bought into 1 to 6 years' follow-up.Long-term control was achieved in 35 of 40.Partial control was achieved in 3 of 40.The left 2 cases had no response to the anti-epileptic drugs.Conclusions The common features of FLE included high seizure frequency,short duration and nocturnal preponderance.There is high positive rate of active EEG in detecting epileptic discharges,and active EEG shoud be necessary.Carbamazepine is recommended to be the first choice to FLE.

Significance of Biological Rhythm on Selective Nocte Treatment on Autosomal Dominant Nocturnal Frontal Lobe Epilepsy / 实用儿科临床杂志

0.05).The adverse effect of treatment group was significantly less than control group(P

Forntal Lobe Epilepsy Presenting with Blinking and Leg Jerking

Eye blinking is a rare semiology in frontal lobe epilepsy. However, eye blinking with leg jerking as a manifestation of simple partial seizure has not been reported. We report a patient with frontal oligodendroglioma who showed simple partial seizure presenting with eye blinking and leg jerking.

Case Control-Study on Efficacy of Carbamazepine and Oxcarbazepine in Treating Frontal Lobe Epilepsy as Monotherapy in Children / 实用儿科临床杂志

0.05).Conclusions Both CBZ and OXC are effecive in treating typical frontal lobe seizures.

A Case of Suspected Autosomal Dominant Nocturnal Frontal Lobe Epilepsy / 대한소아신경학회지

Autosomal dominant nocturnal frontal lobe epilepsy(ADNFLE) is a recently identified partial epilepsy. This disorder is characterized by a variable age of onset(mostly in childhood), autosomal dominant inheritance, clusters of brief frontal nocturnal seizures, negative findings on neuroimaging, and fairly good prognosis. Carbamazepine is known to be the most useful drug in this type of epilepsy. We experienced a case of 5-year-old boy who had unusual arousal and irritability every night for 3 years and had sharp & wave discharges from frontal lobe region in long-term video-EEG monitoring, who was suspected as an autosomal dominant nocturnal frontal lobe epilepsy. We report a case with a brief review of literatures.

Clinical Characteristics of Complex Partial Seizures: a Temporal versus a Frontal Lobe Onset

PURPOSE: This study was performed to correlate clinical behaviours with either a temporal or frontal site of origin and then to identify behaviours that might have a significant practical value in differentiating a temporal from a frontal focus and thus reduce the need for invasive monitoring. METHODS: We analysed 129 seizures that occured during video-EEG monitoring in 13 patients with temporal lobe epilepsy (TLE) and in 9 patients with frontal lobe epilepsy (FLE) as well as neuroimaging studies. We compared first the clinical behaviours that occurred in frontal lobe seizures to those of temporal lobe seizures (second stage analysis). To eliminate clinical behaviours that could occur as the discharge propagated another lobe, we compared only clinical seizure events in which we had no evidence of spread to other regions, or that spread only to the homologous contralateral lobe (first stage analysis). The Fisher exact test was used for analysis. RESULTS: Although staring, sitting up, leg movement, and tonic-clonic movement, generalization occurred more frequently in FLE, and oral, alimentary and hand automatisms were more frequent in TLE, no statistically significant difference was found between the two groups. In second stage analysis, sitting up, tonic-clonic movement were seen only in FLE and oral, alimentary and hand automatisms only in TLE. Staring, hand posturing occurred more frequently in FLE. CONCLUSION: We therefore conclude that the reliability of clinical behaviour alone to predict the site of origin of an epileptic discharge is limited when the surface EEG is equivocal or neuroradiologic evidence of a focus is not clear.