小儿顽固性颞叶癫痫的病理特点研究

目的 研究小儿顽固性颞叶癫痫的临床病理学表现.方法 80例小儿顽固性颞叶癫痫患者行标准前颞叶切除术,切除组织送病理学检查.术后随访1-5年,根据Engel分级评估手术效果.结果 切除组织病理学表现以单纯海马硬化(26例)、良性肿瘤(20例)、局灶性皮层发育不良(10例)和海马硬化合并颞叶其他病变(9例)四大类型为主.癫痫发作控制结果提示EngelⅠ级58例,Ⅱ级11例,Ⅲ级6例,Ⅳ级5例.结论 小儿顽固性颞叶癫痫病理表现多样,以海马硬化、良性肿瘤和局灶性皮层发育不良最常见;颞叶切除疗效肯定;病理类型与手术效果之间的关系仍待更多研究.     

Executive functioning in children with intractable frontal lobe or temporal lobe epilepsy

The aim of the present study was to shed light on the executive functioning deficits that might differentiate children with frontal lobe epilepsy (FLE) from children with temporal lobe epilepsy (TLE). Participants included 19 youth with intractable FLE and 47 youth with intractable TLE. Participants completed the Wisconsin Card Sorting Test (WCST), verbal fluency, Trail Making Test (Trails A and B), Digit Span Forward (DSF), and Digit Span Backward (DSB). When compared to the normative sample, the FLE group performed significantly worse on DSF, DSB, Trails B, and the WCST. Similarly, the TLE group performed significantly worse on DSF and DSB compared to the normative sample. Youth with FLE had significantly greater difficulty on the WCST compared to the TLE group. Overall, the results indicated that youth with FLE had significantly greater difficulty with concept formation compared to children with TLE. No differences between groups emerged on tasks assessing attention, working memory, mental flexibility, or rapid word retrieval. Both groups performed significantly below the normative sample levels on attention and working memory tasks. As a whole, it appears that some, although not all, executive dysfunction is specific to FLE.     

Comparison of temporal lobectomies of children and adults with intractable temporal lobe epilepsy

Introduction

The aim of the study was to assess the difference in clinical characteristics and postsurgical outcomes between children and adults who have undergone temporal lobectomy (TL).

Materials and methods

We retrospectively reviewed the medical records of 52 patients who had undergone TL between 2006 and 2008. Nineteen patients were classified as children (≤18 years old), and 33 patients were classified as adults (>18 years old) according to the age when TL had been performed.

Results

Twelve of 19 (63.2%) children and 24 of 33 (72.7%) adults became seizure free. Rapid secondary generalization such as generalized tonic or tonic–clonic seizures showed a tendency to be more prominent in children (four of 19, 21.1%) than in adults (three of 33, 9.1%). Patients in childhood had significantly more multifocal discharges on interictal electroencephalography (EEG) (42.1%) compared to adults (15.2%, p?=?0.014). The mean extent of surgical excision was 5.0 cm in children and 4.1 cm in adults (p?=?0.001). The incidence of hippocampal sclerosis, the most common pathologic finding in the two groups, was 57.9% (11 of 19) in children and 78.8% (26 of 33) in adults. Malformations of cortical development were significantly more frequent in children (nine of 19, 47.4%) than in adults (seven of 33, 21.2%). Dual pathology was found in 31.6% of children and in 12.1% of adults. The intelligence quotient and memory quotient values in children with temporal lobe resection remained nearly steady during follow-up period without significant decline.

Conclusion

Patients undergoing TL during childhood compared to during adulthood had distinctively different interictal EEG, resectional extents, and pathologic findings.     

难治性颞叶癫(癎)的相关基因表达谱

目的 筛选难治性颞叶癫(癎)相关基因表达谱,并通过对表达谱筛选所发现的差异基因进行验证,从分子水平探讨颞叶癫(癎)可能的发病机制.方法 在利用基因芯片对难治性颞叶癫(癎)患者手术切除颞叶组织与对照间行基因表达谱分析研究的基础上,扩大样本量,应用RT.PCR方法,验证SH3GL2、BTN2A2、KCNJ4基因的mRNA在颞叶癫(癎)患者脑内的表达差异.结果 芯片研究结果显示,颞叶癫(癎)脑组织标本与对照组相比,表达趋势一致的已知基因包括:免疫相关因子、离子通道相关因子、信号转导相关基因、细胞增殖周期因子、细胞凋亡相关基因等;经RT.PCR证实,颞叶癫(癎)组突触重建调控因子SH3GL2的mRNA表达水平(1.022±0.547)明显高于对照组(0.446±0.171,t=-3.181),免疫调控基因BTN2A2的mRNA表达在颞叶癫(癎)组(0.481±0.196)同样高于对照组(0.243±0.111,t=3.351),而编码内向整流钾通道亚单位基因KCNJ4 mRNA的表达水平在颞叶癫疴组(0.438±0.178)则明显低于对照组(0.795±0.112),差异均有统计学意义(P<0.05).结论 在颞叶癫(癎)发生发展各个阶段均有基因的参与,是一个复杂的调控过程;芯片试验结果对进一步探讨疾病可能的发病机制以及为探求疾病治疗的新靶点提供了依据.     

Atypical temporal lobe language representation: MEG and intraoperative stimulation mapping correlation

Functional brain imaging techniques hold many promises as the methods of choice for identifying areas involved in the execution of language functions. The success of any of these techniques in fulfilling this goal depends upon their ability to produce maps of activated areas that overlap with those obtained through standard invasive procedures such as electrocortical stimulation. This need is particularly acute in cases where active areas are found outside of traditionally defined language areas. In the present report we present two patients who underwent mapping of receptive language areas preoperatively through magnetoencephalography (MEG) and intraoperatively through electrocortical stimulation. Language areas identified by both methods were located in temporoparietal regions as well as in less traditional regions (anterior portion of the superior temporal gyrus and basal temporal cortex). Importantly there was a perfect overlap between the two sets of maps. This clearly demonstrates the validity of MEG-derived maps for identifying cortical areas critically involved in receptive language functions.     

Atypical handedness in mesial temporal lobe epilepsy

ObjectiveThe main aim of our study was to investigate the handedness of patients with mesial temporal lobe epilepsy (MTLE). We also sought to identify clinical variables that correlated with left-handedness in this population.MethodsHandedness (laterality quotient) was assessed in 73 consecutive patients with MTLE associated with unilateral hippocampal sclerosis (HS) using the Edinburgh Handedness Inventory. Associations between right- and left-handedness and clinical variables were investigated.ResultsWe found that 54 (74.0%) patients were right-handed, and 19 (26%) patients were left-handed. There were 15 (36.6%) left-handed patients with left-sided seizure onset compared to 4 (12.5%) left-handed patients with right-sided seizure onset (p = 0.030). Among patients with left-sided MTLE, age at epilepsy onset was significantly correlated with handedness (8 years of age [median; min-max 0.5–17] in left-handers versus 15 years of age [median; min-max 3–30] in right-handers (p < 0.001).ConclusionsLeft-sided MTLE is associated with atypical handedness, especially when seizure onset occurs during an active period of brain development, suggesting a bi-hemispheric neuroplastic process for establishing motor dominance in patients with early-onset left-sided MTLE.     

Patterns of verbal learning and memory in children with intractable temporal lobe or frontal lobe epilepsy

PurposeThe objective of this study was to provide a better understanding of the verbal learning and memory (VLM) patterns that might differentiate children with frontal lobe epilepsy (FLE) from children with temporal lobe epilepsy (TLE) and to examine the impact of variables thought to influence outcomes (seizure laterality, age at seizure onset, age at assessment, epilepsy duration, number of antiepileptic drugs).MethodsRetrospective analyses were carried out for children with intractable unilateral TLE (n = 100) and FLE (n = 27) who completed standardized measures of VLM entailing lists of single words or lists of word pairs.ResultsMean intelligent quotients and VLM scores on single words fell within the average range for both groups, whereas scores fell within the low average to borderline range on word pairs. No significant overall differences in VLM were found between the group with TLE and the group with FLE.Older age at assessment and older age at seizure onset were generally associated with better VLM in both groups but were related to better performance in a number of indices in the group with TLE and only fewer intrusions in the group with FLE.ConclusionsThe VLM profiles of children with TLE and FLE are generally similar. Older age at assessment and older age at seizure onset have a favorable impact on both groups but are related to better encoding, retrieval, and monitoring processes for the group with TLE and improved memory monitoring (i.e., as indicated by fewer intrusions) in the group with FLE.     

难治性颞叶癫(癎)的手术治疗

目的 探讨难治性颞叶癫(癎)的手术疗效.方法 回顾性分析2003年7月至2007年2月我科手术治疗的72例难治性颞叶癫(癎)患者临床资料.结果 按我国谭氏标准,满意:59例;显著改善:2例;良好:3例;效差:2例;无改善:5例;死亡:1例.结论 手术治疗难治性颞叶癫(癎)疗效确切可靠,在有条件的医院可推广.     

Prefrontal hypofunction in patients with intractable mesial temporal lobe epilepsy

We compared the cognitive functions and interictal cerebral glucose metabolism of 11 patients with mesial temporal lobe epilepsy (MTLE) with frequent seizures to those of 10 patients with MTLE with rare seizures; the groups were matched for age, sex, education, IQ, and focus side. The frequent-seizure group had more set-shifting impairment that correlated with glucose hypometabolism in the prefrontal cortices. Our results suggest that frequent seizures in MTLE are associated with hypofunction of the prefrontal cortex.     

Effects of anomalous language representation on neuropsychological performance in temporal lobe epilepsy.

OBJECTIVE: To examine the effects of anomalous language representation (i.e., mixed- and right-cerebral dominant) on neuropsychological performance. BACKGROUND: Right cerebral language dominance resulting from early cerebral injury is associated with relatively preserved language function with decreased visuospatial ability. However, previous reports of this phenomenon have examined patients with relatively large cerebral injuries (e.g., infantile hemiplegia) or limited sample sizes. METHODS: A total of 561 patients with complex partial seizures of left temporal lobe origin were studied. Patients were classified into left (n = 455), bilateral (n = 58), and right (n = 48) language dominant groups based on Wada testing. RESULTS: Right language dominant patients performed more poorly on multiple tests of visuospatial function, including Performance IQ (PIQ), than did left language patients. No significant group differences were detected for measures of language or general verbal function. The effects of bilateral language on PIQ differed according to handedness. Lowered PIQ was present in the bilateral nondextral group but not for bilateral dextral patients, and this pattern was observed with other visuospatial measures. CONCLUSIONS: In patients with relatively small lesions restricted to the left mesial temporal lobe, a shift in language dominance to the right hemisphere is associated with decreased visuospatial functions but preserved verbal abilities. Nondextral patients with bilateral language representation also displayed decreased visuospatial performance, although dextral patients with bilateral language did not.     

Frontal lobe dysfunction in children with temporal lobe epilepsy

There is evidence that adults with temporal lobe epilepsy present executive impairments. However, there is limited information in children, especially when using a comprehensive neuropsychologic battery. We aimed to: 1) investigate the presence and severity of executive dysfunctions in children with temporal lobe epilepsy, and 2) determine the implications of clinical variables (including etiology) in the occurrence and severity of executive dysfunction, using eight paradigms. Thirty-one children with temporal lobe epilepsy were evaluated and compared with 21 age-matched controls. Patients with temporal lobe epilepsy had significantly worse performance than controls. Intragroup analysis indicated that patients with symptomatic epilepsy were more impaired than those with cryptogenic epilepsy. In the former group, patients with mesial lesions performed worse than those with lateral lesions. Regarding the severity of executive dysfunction, 83.87% manifested severe to moderate executive impairment. Early age of onset, longer duration of epilepsy, and use of polytherapy were correlated with worse executive dysfunction. These findings indicated the presence of frontal lobe dysfunction in children with temporal lobe epilepsy, with worse performance in those with mesial temporal lobe epilepsy, early onset, longer duration of disease, and use of polytherapy. Our study corroborates the hypothesis that temporal lobe epileptogenic activity affects the extratemporal regions that mediate attentional and executive functions.     

Interictal language functions in temporal lobe epilepsy

OBJECTIVE: To evaluate interictal language functions in patients with medically intractable left and right sided mesial temporal lobe epilepsy (TLE). METHODS: Spontaneous speech, language comprehension, confrontation naming, repetition, reading, writing, and word fluency were examined in 12 patients with left sided TLE and 11 patients with right sided TLE. RESULTS: Four patients out of 23 displayed language deficits in more than one language domain. Three further patients exhibited isolated language deficits. Linguistic deficits were observed in both left TLE and right TLE. In quantitative analyses left and right TLE only differed in spontaneous speech (p = 0.02); no difference was found in other language functions, laterality quotient of Wada test, or overall IQ. Qualitative error analysis of object naming, however, showed typical errors associated only with left TLE. Patients with linguistic deficits were older at testing compared to patients without linguistic deficits (p = 0.003), whereas other factors including side of TLE, handedness, educational level, age at epilepsy onset, and duration of epilepsy did not differ between groups. CONCLUSIONS: Possible explanations for these findings include neuronal cell loss and deafferentiation in cortical areas, and disruption of the basal temporal language area pathways. Our study suggests that some patients with chronic mesial TLE exhibit linguistic deficits when specifically tested, and underlines the need to routinely investigate linguistic functions in TLE.     

Atypical intrinsic neural timescales in temporal lobe epilepsy

Objective

Temporal lobe epilepsy (TLE) is the most common pharmacoresistant epilepsy in adults. Here we profiled local neural function in TLE in vivo, building on prior evidence that has identified widespread structural alterations. Using resting-state functional magnetic resonance imaging (rs-fMRI), we mapped the whole-brain intrinsic neural timescales (INT), which reflect temporal hierarchies of neural processing. Parallel analysis of structural and diffusion MRI data examined associations with TLE-related structural compromise. Finally, we evaluated the clinical utility of INT.

Methods

We studied 46 patients with TLE and 44 healthy controls from two independent sites, and mapped INT changes in patients relative to controls across hippocampal, subcortical, and neocortical regions. We examined region-specific associations to structural alterations and explored the effects of age and epilepsy duration. Supervised machine learning assessed the utility of INT for identifying patients with TLE vs controls and left- vs right-sided seizure onset.

Results

Relative to controls, TLE showed marked INT reductions across multiple regions bilaterally, indexing faster changing resting activity, with strongest effects in the ipsilateral medial and lateral temporal regions, and bilateral sensorimotor cortices as well as thalamus and hippocampus. Findings were similar, albeit with reduced effect sizes, when correcting for structural alterations. INT reductions in TLE increased with advancing disease duration, yet findings differed from the aging effects seen in controls. INT-derived classifiers discriminated patients vs controls (balanced accuracy, 5-fold: 76% ± 2.65%; cross-site, 72%–83%) and lateralized the focus in TLE (balanced accuracy, 5-fold: 96% ± 2.10%; cross-site, 95%–97%), with high accuracy and cross-site generalizability. Findings were consistent across both acquisition sites and robust when controlling for motion and several methodological confounds.

Significance

Our findings demonstrate atypical macroscale function in TLE in a topography that extends beyond mesiotemporal epicenters. INT measurements can assist in TLE diagnosis, seizure focus lateralization, and monitoring of disease progression, which emphasizes promising clinical utility.     

Progressive hippocampal atrophy in chronic intractable temporal lobe epilepsy

We report on a 28-year-old man with long-standing intractable complex partial and secondary generalized seizures, whose magnetic resonance imaging scans 4 years apart documented progressive decrease in the left hippocampal volume. Left anterior temporal lobectomy with amygdalohippocampectomy rendered the patient seizure free at 12 months' follow-up. The findings demonstrate that patients with uncontrolled temporal lobe seizures may develop progressive atrophy of the hippocampus, in the absence of status epilepticus.     

Speech recognition impairments in patients with intractable right temporal lobe epilepsy

PURPOSE: To evaluate speech recognition in patients with focal intractable epilepsy and surgical resections in the nondominant (right) hemisphere. METHODS: Speech recognition was tested prospectively, under different listening conditions, in 22 patients with right temporal lobe (11 patients) or extra-temporal lobe epilepsy. All were left-hemisphere dominant for language on preoperative intracarotid sodium amobarbital testing. RESULTS: All patients demonstrated normal auditory recognition of words and environmental sounds before and after surgery. However, when real-world listening conditions were simulated by using acoustically degraded (filtered) words, patients with temporal lobe epilepsy performed significantly worse than patients with frontal or parietooccipital lobe epilepsy before and after surgery (p<0.0001). CONCLUSIONS: Patients with intractable right temporal lobe epilepsy are at risk for speech recognition impairments in real-world listening environments, independent of surgery. The impact of speech recognition difficulties on verbal communication, coupled with the prevalence of adverse listening environments, underscores the importance of testing speech recognition under different listening conditions in patients with intractable right temporal lobe epilepsy.     

Subtemporal hippocampectomy preserving the basal temporal language area for intractable mesial temporal lobe epilepsy: preliminary results

PURPOSE: Decline in verbal memory as a surgical complication remains an unresolved problem in mesial temporal lobe epilepsy. Some areas in the temporal lobe associated with the language function, often including the basal temporal language area, have been removed or transected by conventional surgical procedures. The authors defined the basal temporal language area and removed only the epileptogenic zone with a subtemporal approach. METHODS: The basal temporal language area was evaluated by using long-term subdural electrodes in five patients with language-dominant-side mesial temporal lobe epilepsy. While preserving this area, the hippocampus and the parahippocampal gyrus were removed by using a combined subtemporal, transventricular, transchoroidal fissure approach. Verbal memory performance was assessed with the Wechsler Memory Scale-Revised (WMS-R) before and after the operation. RESULTS: The basal temporal language area, defined as a part of the inferior temporal gyrus, the fusiform gyrus, and the parahippocampal gyrus, was spared by entering the temporal horn via collateral sulcus. Verbal memory was significantly improved by 3 months and 1 year after the operation. CONCLUSIONS: In language-dominant-side mesial temporal lobe epilepsy, preserving the basal temporal language area would have potential to improve verbal memory outcomes after removal of the epileptogenic zone.     

颞叶癫痫患者颞叶切除术前后的记忆评估

目的探讨颞叶切除术对颞叶癫痫患者术后记忆功能的影响。方法回顾性分析2012年7月至2013年12月收治的17例颞叶癫痫患者的临床资料,采用成人韦氏量表测量患者颞叶切除术前及术后6个月记忆商（MQ）值。结果术前MQ平均值为（89.94±4.45）,术后6个月为（104.76±4.96）,差异显著（P〈0.05）。术前长时MQ值为（37.53±1.95）,术后为（40.53±1.70）,术后长时记忆改善不明显（P〉0.05）。术前短时记忆MQ值为（46.29±2.43）,术后为（54.94±3.18）;术前瞬时MQ均值为（6.12±1.37）,术后为（9.29±1.00）;术后短时及瞬时MQ均较术前有显著差异（P〈0.05）。EngleⅠ～Ⅱ级15例病人中,术后MQ较术前改善11例,无明显变化4例;EngleⅢ～Ⅳ级2例病人中,MQ值较术前下降1例,无改变1例。结论颞叶切除术可明显改善颞叶癫痫患者术后MQ。     

Atypical hemispheric language dominance in left temporal lobe epilepsy as a result of the reorganization of language functions

Hemispheric language dominance, as determined by intracarotid short-acting barbiturate injections (Wada testing), was retrospectively evaluated in 44 right-handed patients with medically intractable left (n=26) or right (n=18) temporal lobe epilepsy (TLE). Atypical hemispheric language dominance (right or bilateral) was revealed in 13.6% of all patients investigated. A significantly higher rate of deviance from complete left hemisphere dominance was observed in left TLE (23.1%) than in right TLE (0%). Patient age at the time of seizure onset was proven a critical variable affecting atypical speech development in left TLE. The mean patient age at the time of seizure onset in left TLE patients with atypical language representation was 5.61 years, but was 13.13 years in patients with left hemisphere language dominance. Our results strongly support the influential impact of epileptiform activity spreading from the left temporal lobe structures on the reorganization of language functions in patients with early brain injury.