双侧颞叶癫(癎)的立体定向外科治疗

目的 探讨双侧海马-杏仁核复合体毁损术治疗双侧颞叶癫(癎)的疗效.方法 回顾性分析5例双侧颞叶癫(癎)病人的临床资料,复杂部分性发作中的自动症1例,部分性继发全身性癫(癎)4例.均行深部电极引导下机器人辅助定位双侧海马-杏仁核复合体毁损术.结果 随访1～2.5年,按Engel分级:Ⅰ级2例;Ⅱ、Ⅲ、Ⅳ级各1例.术后MRI显示:双侧海马-杏仁核复合体区无严重的结构性破坏.智商、心算速度、符号数字配对、划消、数字记忆广度、指扣试验等神经心理学检查指标手术前后差异均无统计学意义(P＞0.05).结论 双侧颞叶癫(癎)无法实施切除性手术,而立体定向外科治疗可减少癫(癎)发作,且并未造成严重认知功能障碍,是一种值得尝试的外科治疗手段.     

Bilateral stereotactic radiofrequency amygdalohippocampectomy for a patient with bilateral temporal lobe epilepsy

To search for a method for treatment of bilateral temporal lobe epilepsy (BTLE), we report one patient with BTLE experienced bilateral stereotactic radiofrequency amygdalohippocampectomy (SAHE). Neuropsychological examinations were performed before and 5 days, and 6, 18, and 48 months after operation. No seizure occurred in the follow‐up time, and no long‐term memory and intelligence deficits were found except for a transient decline of the scores immediately after operation. Because severe damage of memory could be caused by bilateral resection surgery, bilateral SAHE should be considered as a possible approach for the treatment of BTLE. However, further studies with accumulation of cases are needed, especially in the detailed assessment of neuropsychological function.     

Late-onset temporal lobe epilepsy in a patient with juvenile myoclonic epilepsy

We report a patient with longstanding, severe juvenile myoclonic epilepsy who subsequently developed features of temporal lobe epilepsy, which gradually became clinically dominant. Over the years, there was an electrographic evolution from the typical generalised epileptiform patterns, characteristic of juvenile myoclonic epilepsy, to the novel appearance of interictal temporal spikes immediately preceding bisynchronous discharges, and subsequently to temporal intermittent rhythmic delta activity and temporal lobe-onset seizures. In this rare case of coexistent primary generalised epilepsy and focal epilepsy, the epileptic networks of the two forms of epilepsy appear to overlap.     

Outcome after temporal lobectomy in bilateral temporal lobe epilepsy

We determined how noninvasive presurgical data relate to prognosis after temporal lobectomy in patients with independent bilateral temporal lobe (IBTL) complex partial seizures on the intracranial electroencephalogram (EEG). Between 1986 and 1994, 28 patients had IBTL seizures on intracranial EEG. Fifteen of these 28 patients underwent temporal lobectomy and 13 were not offered surgery. Of the 15 patients who had surgery, 10 patients became seizure-free. Magnetic resonance imaging (MRI) and the Wada test were the only variables associated with a seizure-free outcome. Seven of 10 seizure-free patients had a lateralized Wada result or the presence of unilateral hippocampal sclerosis, whereas none of the patients with persistent seizures had either of these findings. Variables not found to be predictive of a seizure-free outcome included location of scalp interictal spikes, degree of seizure-onset laterality, presence of early epilepsy risk factor, duration of epilepsy, and full-scale intelligence quotient. We conclude that MRI and the Wada test provide information of prognostic value in patients with bilateral temporal seizures independent of intracranial EEG data.     

Significance of interictal bilateral temporal hypometabolism in temporal lobe epilepsy

OBJECTIVE: To assess the clinical implications and the pathophysiologic determinants of interictal bitemporal hypometabolism (BTH) in temporal lobe epilepsy (TLE) not associated with bilateral MRI abnormalities or intracranial space-occupying lesions. METHODS: The authors compared the clinical, interictal, and ictal EEG, Wada test, and neuropsychology data of 15 patients with intractable complex partial seizures of temporal lobe origin and BTH with those of 13 consecutive patients with unilateral TLE associated with unilateral temporal hypometabolism (UTH) who remained seizure free for more than 3 years after anterior temporal lobectomy. 18F-fluorodeoxyglucose PET scans were analyzed visually and semiquantitatively, and ratios of counts in individual temporal areas to the rest of the cerebrum were compared with the corresponding values from 11 normal control subjects and with the nonepileptogenic hemisphere of the 13 patients with UTH. BTH was defined as more than 2.5 SDs below control values for two or more temporal areas on each side irrespective of any asymmetry. RESULTS: BTH reflected bilateral independent seizure onset in eight patients (53%). The topography of the metabolic depression was not a reliable predictor of epileptogenicity, but involvement of the inferior temporal gyrus was related specifically to ipsilateral seizure onset (70% sensitivity, 100% specificity). In patients with unilateral TLE, contralateral hypometabolism was associated with longer disease duration and worst memory performance during the Wada test, which amounted to global amnesia after ipsilateral injection in three patients, precluding surgical treatment. Contralateral seizure spread in the ictal EEG was significantly faster in patients with BTH. CONCLUSIONS: In TLE, symmetric or asymmetric BTH may signal bilateral independent seizure onset in approximately half the patients, especially when involving the inferior temporal gyrus. Alternatively, it may reflect an advanced stage of the disease process, characterized by a breakdown of the inhibitory mechanisms in the contralateral hemisphere, and secondary memory deficit associated with higher risk of postoperative memory decline. Patients with TLE and BTH but without bilateral MRI changes may still be operated on successfully, but surgical suitability should be proved by comprehensive intracranial EEG studies and Wada test.     

Vagus nerve stimulation for the treatment of bilateral independent temporal lobe epilepsy

PURPOSE: We studied the effect of vagus nerve stimulation (VNS) on seizure reduction in patients with intractable epilepsy with bilateral independent temporal lobe foci. METHODS: Ten patients who met the criterion of the presence of two distinctive clinical and ictal EEG seizure patterns were identified and followed up for 1 year. RESULTS: Six patients had >50% reduction in their seizure frequency that persisted up to > or =1 year of follow-up, whereas four patients reported small or no reduction in their partial seizures. CONCLUSIONS: VNS is often effective and well tolerated in this select group of intractable epilepsy patients.     

Ictal and postictal semiology in patients with bilateral temporal lobe epilepsy

Bilateral temporal lobe epilepsy is characterized by evidence of seizure onset independently in both temporal lobes. The main aim of the present study was to determine whether patients with evidence of independent bilateral temporal lobe epilepsy (biTLE) can be identified noninvasively on the basis of seizure semiology analysis. Thirteen patients with biTLE, as defined by invasive EEG, were matched with 13 patients with unilateral temporal lobe epilepsy (uniTLE). In all 26 patients, the frequency of predefined clusters of ictal and periictal signs were evaluated: ictal motor signs (IMSs), periictal motor signs (PIMSs), periictal vegetative signs (PIVSs), the frequency of early oroalimentary automatisms (EOAs), and the duration of postictal unresponsiveness (PU). Some other noninvasive and clinical data were also evaluated. A lower frequency of IMSs was noted in the group with biTLE (patients = 46.2%, seizures = 20.7%) than in the group with uniTLE (patients = 92.3%, seizures = 61.0%) (p = 0.030; p < 0.001, respectively). The individual IMS average per seizure was significantly lower in the group with biTLE (0.14; range = 0–1.0) than in the group with uniTLE (0.80; range = 0–2.6) (p = 0.003). Postictal unresponsiveness was longer than 5 min in more patients (75.0%) and seizures (42.9%) in the group with biTLE than in the group with uniTLE (patients = 30.8%, seizures = 18.6%) (p = 0.047; p = 0.002). The frequency of EOAs, PIMSs, PIVSs, and other clinical data did not differ significantly. There is a lower frequency of ictal motor signs and longer duration of postictal unresponsiveness in patients with biTLE.     

颞叶癫痫的手术治疗(附34例分析)

目的　探讨颞叶癫痫的术前评估和术式选择策略。方法　对 34例颞叶癫痫患者应用无创和有创方法进行术前综合评估 ,采用扩大额颞问号式手术切口 ,经外侧裂入路、皮层脑电监测引导下实施手术。对术前评估方法、术中脑电监测的意义和手术方式选择进行了分析。结果　 2 1例患者根据长程蝶骨电极脑电图和MRI等无创检查定位了致痫灶 ,13例根据颅内埋置电极脑电图定位了致痫灶。术后随访 15 .6± 6 .0月。 2 9例 (85 .3% )癫痫发作消失 ,3例 (8.8% )发作减少 75 % ,2例 (5 .9% )发作减少 5 0 %以上。 8例曾出现短期并发症 ,无永久性并发症发生。手术 6个月后 ,患者的日常生活能力评分与手术前相比明显改善 (P <0 .0 5 )。结论　长程视频脑电图和MRI检查是颞叶癫痫致痫灶定位可靠的无创性检查方法 ;颅内埋置电极检查是术前准确定位致痫灶必要的手段。颅内电极脑电图监测对设计切除方式有重要参考价值 ;改良的手术切口和经外侧裂入路有利于安全有效的切除前颞叶或选择性切除颞叶内侧结构     

Bilateral temporal lobe epilepsy in a patient with Turner syndrome mosaicism.

Turner's syndrome (TS), resulting from deletion of one X chromosome in women, is associated with cerebral development abnormalities, particularly in the temporal lobes. Symptomatic epilepsy is described only in cases with extensive malformations. Here, we report the first case of bilateral temporal epilepsy without macroscopic cerebral malformation in a woman with TS mosaicism. Bitemporal dysfunction was confirmed by the ictal and interictal EEG, PET, MR-spectroscopy and the neuropsychological examination, other causes than TS mosaicism were excluded. In rare cases, TS mosaicism may underlie non-lesional temporal lobe epilepsy, probably in relation to microanatomic structural and functional cerebral abnormalities. Further studies are needed to determine the frequency of this association.     

A Kv4.2 truncation mutation in a patient with temporal lobe epilepsy

Temporal lobe epilepsy (TLE) has a multifactorial etiology involving developmental, environmental, and genetic components. Here, we report a voltage-gated potassium channel gene mutation found in a TLE patient, namely a Kv4.2 truncation mutation. Kv4.2 channels, encoded by the KCND2 gene, mediate A currents in the brain. The identified mutation corresponds to an N587fsX1 amino acid change, predicted to produce a truncated Kv4.2 protein lacking the last 44 amino acids in the carboxyl terminal. Electrophysiological analysis indicates attenuated K+ current density in cells expressing this Kv4.2-N587fsX1 mutant channel, which is consistent with a model of aberrant neuronal excitability characteristic of TLE. Our observations, together with other lines of evidence, raise the intriguing possibility of a role for KCND2 in the etiology of TLE.     

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.     

颞叶新皮质癫痫手术的疗效分析

目的分析总结颞叶新皮质癫痫患者临床特征、术前评估、手术方法和疗效。方法对36例颞叶新皮质癫痫行神经电生理、MRI,正电子发射计算断层显像计算机体层扫描（PET—CT）定位,术中皮层电极和深部电极描记,联合采用不同术式以及术后随访6个月至2年的疗效观察。结果疗效按谭启富的标准分类：Ⅰ级12例,Ⅱ级11例,Ⅲ级8例,Ⅳ级5例,Ⅴ级0例。病理报告：胶质增生23例;微小血管畸形5例;灰质异位1例,无异常发现7例。无手术致残和死亡。结论联合采取不同术式,如：行海马及杏仁核部分切除,对颞叶新皮质癫痫可达到良好的治疗效果,且无严重手术并发症。     

Diagnosis and treatment of temporal lobe epilepsy

Of the 1,200,000 Americans with partial epilepsy, temporal lobe epilepsy (TLE) occurs in more than 400,000. Temporal lobe seizures are usually stereotypic in their symptoms and duration. A typical sequence is an aura followed by arrest of motor behavior, blank stare, and automatisms. Patients with TLE often show impairments in attention, memory, mental processing speed, executive functions, mood, personality, and drive-related behaviors. Interictal depression occurs in approximately one third of TLE patients. TLE is diagnosed by a history of characteristic partial seizure symptoms. The diagnosis is confirmed by the capture of a typical episode during an electroencephalogram (EEG) or video-EEG, with epileptiform activity over one or both temporal regions. Video-EEG monitoring has revolutionized diagnosis and should be considered in patients in whom diagnosis is uncertain. TLE is treated with medications, resective surgery, and vagus nerve stimulation. Epilepsy surgery should be considered in all patients with refractory partial epilepsy.     

Ictal vomiting in a left hemisphere language-dominant patient with left-sided temporal lobe epilepsy

Ictal vomiting in patients with focal epilepsy has mostly been associated with an epileptogenic zone in the non-language-dominant hemisphere. Here we present the case of a left hemisphere language-dominant patient suffering from typical mesial temporal lobe epilepsy with histologically proven hippocampal sclerosis and ictal vomiting during complex partial seizures. He became seizure-free after selective left-sided amygdalohippocampectomy. This case implies that ictal vomiting may not necessitate invasive electrophysiological exploration of left hemisphere language-dominant patients with temporal lobe epilepsy if surface EEG and MRI indicate a left-sided epileptogenic zone. It thus corroborates that with concordant imaging and neurophysiological data, clinical signs become less valuable.     

Accuracy of ictal SPECT in mesial temporal lobe epilepsy with bilateral interictal spikes

OBJECTIVE: In mesial temporal lobe epilepsy (MTLE), the rate of correct seizure lateralization of ictal semiology and ictal EEG is better for patients with unilateral interictal spikes (UIS) than for patients with bilateral interictal spikes (BIS), possibly due to rapid seizure propagation patterns associated with bilateral epileptogenesis. In this study, the authors investigated if ictal SPECT is a reliable diagnostic test for both UIS and BIS patients. METHODS: Video-EEG recording was used as the gold standard to examine the accuracy of ictal SPECT and its relationship with interictal and ictal EEG. Ninety-three consecutive patients with MTLE associated with hippocampal sclerosis were included in the analysis. Ictal SPECT was considered accurate if two blinded observers independently lateralized the scan correctly. RESULTS: Ictal SPECT correctly lateralized 75 (80.6%) of 93 scans. The rate of correct seizure lateralization was 87.6% for the UIS group and only 55.0% for the BIS group (p = 0.0027). In the EEG epochs, 66.7% of BIS patients vs 43.4% of UIS patients had nonlateralized ictal EEG (p < 0.001). CONCLUSION: The authors conclude that the accuracy of ictal SPECT is worse for MTLE patients with BIS than for those with UIS. The role of ictal SPECT in presurgical evaluation of patients with BIS must be reviewed.