ACHIEVING AND MAINTAINING COMPLIANCE WITH THE KETOGENIC DIET

The ketogenic diet, a treatment for intractable epilepsy, is rarely initiated because it requires strict compliance with a diet that is perceived to be unpalatable. In a case study of a 15-year-old girl with uncontrolled epilepsy, we used a stimulus-choice procedure to assess relative preferences of 33 foods from the diet and to develop two treatments based on Premack's principle. The results of a multielement analysis showed that both treatments increased dietary compliance. Compliance was maintained with generalization of treatment across settings, and was associated with a 40% reduction in seizures.     

Is Speech Arrest during Wada Testing a Valid Method for Determining Hemispheric Representation of Language?

Background and objective: The intracarotid amobarbital procedure, or Wada test, is the method of choice to determine hemispheric representation of language, and is routinely used in the presurgical evaluation for intractable epilepsy. Some investigators perform comprehensive language assessments, but others base language lateralization solely on speech arrest. This study sought to determine whether speech arrest alone during Wada testing provides valid data regarding language lateralization. Methods: The subjects (previously reported) were 21 patients evaluated for intractable epilepsy, who underwent language lateralization by Wada testing and functional MRI (FMRI). For each patient, language representation was determined by calculating: (1) a Wada laterality index based exclusively on speech arrest; (2) a Wada laterality index based on comprehensive language assessment; and (3) an FMRI laterality quotient. Correlation coefficients and categorical classifications were analyzed. Results: There was no significant correlation between the Wada laterality quotient derived from duration of speech arrest and either the comprehensive Wada language laterality score (r= .35,p= .12) or FMRI language laterality score (r= .32,p= .16). Categorical classification as left, right or bilateral language also showed marked discordance between speech arrest and the other two methods. Conclusion: Duration of speech arrest during Wada testing is not a valid measure of language dominance.     

Beyond Speech Lateralization: A Review of the Variability, Reliability, and Validity of the Intracarotid Amobarbital Procedure and Its Nonlanguage Uses in Epilepsy Surgery Candidates

While the intracarotid amobarbital procedure (IAP) was originally utilized to lateralize speech functions as an aid in the surgical treatment of epilepsy, additional uses for the IAP have emerged including: (1) the use of the IAP to predict post-surgical memory changes, including both global amnesia and smaller, yet significant, material-specific memory deficits; (2) the use of the IAP to provide confirmatory evidence of lateralization of seizure focus; and (3) the use of the IAP to predict post-surgical relief from seizures. While the literature on the IAP is extensive and growing, its utility is marred by the wide variability associated with the procedure itself from epilepsy center to center. This variability renders comparisons among IAP studies problematic and conclusions about IAP efficacy difficult. The variability associated with the amobarbital procedures, as well as the reliability and the validity of the IAP in its nonlanguage uses is reviewed here. A special emphasis is devoted to research conducted in the last decade. Also discussed is the future of the IAP including anticipated research directions.     

Dichotic listening with forced attention in patients with temporal lobe epilepsy: significance of left hemisphere cognitive dysfunction

Fifty right-handed patients with focal temporal lobe epilepsy were administered a dichotic listening test with consonant-vowel syllables under non-forced, forced right and forced left attention conditions, and a neuropsychological test battery. Dichotic listening performance was compared in subgroups with and without left hemisphere cognitive dysfunction, measured by the test battery, and in subgroups with left and right temporal epileptic focus. Left hemisphere cognitive dysfunction led to more correct responses to left ear stimuli in all three attention conditions, and fewer correct responses to right ear stimuli in the non-forced attention condition. This was probably caused by basic left hemisphere perceptual dysfunction. Dichotic listening was less affected by a left-sided epileptic focus than by left hemisphere cognitive dysfunction. General cognitive functioning influenced dichotic listening performance stronger in forced than in non-forced attention conditions. Larger cerebral networks were probably involved in the forced attention conditions due to the emphasis on conscious effort.     

Contents

Up to one third of the epilepsy population consists of children with cryptogenic localization related epilepsy (CLRE). Unfortunately, the effect of CLRE on the development is still unclear. Behavioral and academic problems have been reported, but no conclusive study concerning the impact of CLRE on neuropsychological functioning is yet published.

This study was a systematic cross-sectional open clinical and nonrandomized investigation, which included 68 children with CLRE. Several neuropsychological tests were analyzed and age-related normative values were used as reference. Differences between CLRE and reference values were tested with Paired-Samples t-tests. Z scores were computed to compare the different neuropsychological tests and to inspect whether a characteristic neuropsychological profile exists for CLRE. The Independent-Samples t-test was used to explore which epilepsy factors (seizure type, seizure frequency, age at onset, duration of epilepsy, and drug load) were influencing the cognitive profile of CLRE.

There seems to be a characteristic cognitive profile for children with CLRE; children with CLRE experience cognitive difficulties on a wide range of areas—in particular, alertness, mental speed, and memory. Seizure type, seizure frequency, duration of epilepsy, and drug load do not influence this neuropsychological profile. Age at onset was an important risk factor; the earlier the age at onset, the worse the cognitive performance. In spite of the influence of age at onset, the revealed profile can be seen as a stable, independent of temporary factors, neuropsychological profile for children with CLRE.     

MRI对儿童难治性、局灶性癫痫的临床诊断及预后判断

癫痫外科的临床病理研究表明,儿童局灶性皮质发育不良(FCD)及海马硬化是儿童难治性局灶性癫痫常见的致病因素。但在术前的常规的MRI检查中,这些结构上的异常经常被忽视,从而造成延误诊断。临床医生在阅片判断上的失误可能是一个原因,但癫痫的影像专业知识的不全面是重要因素。本文结合MRI技术在癫痫临床诊断领域的应用进展,阐述了儿童难治性、局灶性癫痫患者MRI影像中可能出现的结构性病变;重点阐述了临床易于忽视的局灶性皮质发育不良(FCD)及海马硬化的MRI研究、应用进展。     

脑信号导向蛋白Semaphorins3F与难治性癫痫

在神经发育过程中Semaphorins为轴突导向和神经细胞的迁移提供导向信息。这些导向分子通过抑制自发的或异常的轴突生长来维持己建立的神经连接,并参与突触可塑性以及中枢神经受损后抑制神经再生与神经细胞的死亡。目前,虽然对Semaphorins在神经发育、成熟及受损不同阶段中的功能己有初步的了解,但Semaphorins其受体多样,尚无法预测Sema-phorins与其受体的结合将产生的生物学效应以及Semaphorins在神经系统中起作用的分子机制。进一步研究Semaphorins对癫痫、精神分裂、老年痴呆症等神经系统性疾病的治疗具有深远意义。     

癫痫诊治中医患同盟建立的实践与策略

癫痫治疗目前仍以长期科学规范的药物治疗为主,为保证医生的治疗计划得到贯彻,在诊治中努力寻求医患同盟的建立尤为重要,让患者清楚地认识到医生对他的关注,认识到医生治疗的计划性、和具有应对各种情况的措施方法和能力,是寻求患方合作的基本条件。本文就医生首诊中的任务、人文关怀的重要性、如何共同进行药物选择、副反应规避、家属不良治疗心态纠正等问题进行探讨。     

Recent Innovative Studies of Memory in Temporal Lobe Epilepsy

Investigations of patients with temporal lobe epilepsy continue to result in significant advancements in the neuroscience of human memory, as they have for over 50 years. Recent reviews describing the state of the art in the clinical neuropsychology of TLE generally have emphasized (1) lateralization of cognitive deficits and the material-specific model of memory and/or (2) the relationships among pre- and post-surgery performance on standardized measures of anterograde memory, demographic and epilepsy variables and neuroimaging, neuropathology, and neurosurgery data. As information continues to accumulate about the pattern of performance on standard laboratory memory tests and the implications of these data before and after anterior temporal lobectomy for the treatment of TLE, innovative assessment techniques also are increasingly being applied. This review focuses on English language publications that addressed selected novel topics in adult TLE memory research. These topics are: (1) remote memory; (2) accelerated forgetting or long term amnesia; and (3) lateral versus mesial temporal lobe contributions to memory.