Epilepsy surgery in children with tuberous sclerosis complex: presurgical evaluation and outcome

PURPOSE: Children with tuberous sclerosis complex (TSC) benefit from excisional surgery if seizures can be localized to a single tuber. We evaluated the role of noninvasive studies to localize the epileptogenic tuber/region (ET/R) and the outcome of focal resection. METHODS: We identified 21 children with TSC, ages 3 months to 15 years (mean 4.8 years). All had video-(electroencephalogram) EEG and magnetic resonance imaging (MRI) scans, and 18 also had ictal single photon emission-computed tomography (SPECT) studies. An ET/R was localized in 17 patients. Thirteen patients underwent resection guided by intraoperative electrocorticography (n = 7) or subdural monitoring (n = 6). RESULTS: Interictal EEG revealed a principal spike focus (PSF) that corresponded to the ET/R in 14 children. In seven, PSFs occurred in rhythmic runs. PSFs were not observed remote from the ET/R. Focal polymorphic slowing and attenuation occurred in the region of the PSF in 11 patients. Sixteen patients demonstrated an ictal focus corresponding to the ET/R. Ictal SPECT revealed focal hyperperfusion correlating with the ET/R in 10 patients. Although the MRIs in all children revealed multiple tubers, the ET/R corresponded to a large discrete tuber in 8 patients and a calcified tuber in 13 patients. Patchy calcified tubers were also seen elsewhere in six patients. At a mean follow-up of 26 months, 9 of the 13 children who underwent surgery were seizure-free, one had greater than 75% reduction in seizures, two were unchanged, and one was lost to follow-up. New seizures developed in one child from a contralateral tuber. CONCLUSIONS: Surgical resection of an ET/R alleviates seizures in most children with TSC and intractable epilepsy. The scalp EEG and MRI help define the ET/R and improve case selection when ictal SPECT is nonlocalizing.     

Epilepsy surgery in tuberous sclerosis complex: early predictive elements and outcome

Aim  The aim of the study was to evaluate the surgical treatment of epilepsy and detection of possible early surgery predictive elements in patients with tuberous sclerosis complex (TSC). Materials and methods  Forty-two TSC patients with epilepsy were selected and divided into two main groups: definite and fruste forms. Definite forms were divided into different groups: patients with pharmacologically controlled epilepsy, patients with pharmacoresistant epilepsy excluded from surgery after an extensive presurgical assessment, and patients with a pharmacoresistant epilepsy who underwent surgery. We compared the definite TSC groups to identify elements that predict surgical candidacy. Second, we compared all operated patients to assess surgical outcome. Conclusion  We found several factors that could predict a surgical intervention even if identification of patients with refractory epilepsy who can benefit from surgery is an evolving process. Also, several positive factors for good surgical outcome were identified. Patients with the fruste form had excellent surgical outcome.     

Epilepsy surgery in tuberous sclerosis complex: Emphasis on surgical candidate and neuropsychology

Purpose: To discuss neuropsychological outcome and candidate of epilepsy surgery for tuberous sclerosis complex (TSC). Methods: To retrospectively analyze clinical data of 25 patients with TSC and epilepsy who underwent epilepsy surgery between 2001 and 2007. Seizure reduction was analyzed at 1‐year (1FU), 2‐year (2FU), and 5‐year (5FU) follow‐up visits after surgery, and outcomes of intelligence quotient (IQ) and quality of life (QOL) were evaluated at 2FU. Results: Resective procedures included 14 tuber resections, 9 lobectomies, and 2 tuber resections and lobectomies. Corpus callosotomies (CCTs) were performed as the adjunctive approach in eight cases with low IQ and behavioral problems. The percentages of seizure‐free cases were 72% at 1FU, 60% at 2FU, and 54.5% at 5FU, and the factors predicting seizure freedom included the course of seizures and ages of patients. Significant improvement was found in performance IQ in patients with preoperative low IQ or CCT. Significant improvement in mean QOL score was observed in all patients, especially patients with preoperative low IQ and CCT but postoperative seizure freedom. Conclusion: To be surgical candidates, patients with TSC and epilepsy should have identified epileptogenic tubers, and candidates should include patients with low IQ and multiple epileptogenic tubers. Satisfactory seizure control was often achieved with early operation, whereas improved QOL was observed frequently in postoperative seizure‐free patients. CCT could be performed as an adjunctive approach to resective operation for TSC patients with epilepsy and low IQ and render improvement of performance IQ and QOL.     

结节性硬化症癫痫的术前评估及手术方式的选择

目的 总结结节性硬化症所致癫痫患者的术前评估及手术方式,观察术后疗效.方法 回顾性分析北京三博脑科医院2004年6月至2011年6月手术治疗的20例结节性硬化症患者临床资料并系统随访1～5年.结果 经过综合评估,单纯致痫结节切除术8例;电极植入后致痫结节切除术1例;脑叶切除术4例;致痫结节切除+致痫皮层热灼术2例;脑叶切除+离断术1例;迷走神经刺激术2例;胼胝体全段切开术2例.术后随访Engel Ⅰ级11例,Ⅱ级2例,Ⅲ级5例,Ⅳ级2例,有效率达90％ (18/20).结论 经过精确术前评估,选择合适的手术方式,可以有效地控制或减轻结节性硬化引起的癫痫发作.     

结节性硬化致癫痫发作的临床特点及治疗观察

目的　分析结节性硬化 (TS)所致癫痫的临床特点及治疗效果。方法　对 2 0例TS所致癫痫患者的临床发作特点 ,电生理、影像学资料及抗癫痫药物 (AEDs)的治疗效果进行分析。结果　本组癫痫发作均出现在 1 0岁之前 ,其中 2岁之前发病 1 5例 (75 .0 % ) ,主要发作类型为部分性发作 1 0例 (50 .0 % )。 5例患者在疾病发展过程中 ,从部分发作转化为全身强直 阵挛发作。 2例结节位于皮质且直径 >1 0mm ,其位置与脑电图 (EEG)主要放电的部位相符。 1 1例 (61 .1 1 % )AEDs治疗有效。 1 0例随诊 1年以上 ,其中 3例恶化。结论TS所致癫痫患者发病年龄低 ,主要发作类型为部分性发作 ,在疾病的发展过程中 ,发作形式可发生转变。皮质结节的大小与致痫灶有关。AEDs治疗有效 ,但随年龄增长 ,疗效下降     

Psychiatric outcomes of epilepsy surgery: a systematic review

Purpose: The objective of this systematic review was to identify: (1) prevalence and severity of psychiatric conditions before and after resective epilepsy surgery, (2) incidence of postsurgical psychiatric conditions, and (3) predictors of psychiatric status after surgery. Methods: A literature search was conducted using PubMed, EmBase, and the Cochrane database as part of a larger project on the development of an appropriateness and necessity rating tool to identify patients of all ages with potentially resectable focal epilepsy. The search yielded 5,061 articles related to epilepsy surgery and of the 763 articles meeting the inclusion criteria and reviewed in full text, 68 reported psychiatric outcomes. Thirteen articles met the final eligibility criteria. Key Findings: The studies demonstrated either improvements in psychiatric outcome postsurgery or no changes in psychiatric outcome. Only one study demonstrated deterioration in psychiatric status after surgery, with higher anxiety in the context of continued seizures post‐surgery. One study reported a significantly increased rate of psychosis after surgery. The two main predictors of psychiatric outcome were seizure freedom and presurgical psychiatric history. De novo psychiatric conditions occurred postsurgery at a rate of 1.1–18.2%, with milder psychiatric issues (e.g., adjustment disorder) being more common than more severe psychiatric issues (e.g., psychosis). Significance: Overall, studies demonstrated either improvement in psychiatric outcomes postsurgery or no change. However, there is a need for more prospective, well‐controlled studies to better delineate the prevalence and severity of psychiatric conditions occurring in the context of epilepsy surgery, and to identify specific predictors of psychiatric outcomes after epilepsy surgery.     

Neurological manifestations of tuberous sclerosis complex

CNS lesions of tuberous sclerosis complex (TSC) are due to a developmental disorder of neurogenesis and neuronal migration. MRI studies provide excellent in vivo demonstration of the various pathologic lesions. Symptoms of cortical tubers may include seizures, mental retardation, learning disabilities, and abnormal behavior. Seizures have a focal or multifocal origin, this clinical feature depending on the localization of the cortical tubers. Epilepsy associated with TSC is often intractable, but seizure control has benefited from the introduction of the new antiepileptic drugs. Carefully selected drug-resistant patients can be assessed with intensive monitoring as candidates for surgical removal of epileptogenic lesions. The success of epilepsy surgery is predicated on the clear identification of epileptogenic foci.     

儿童结节性硬化症合并癫痫临床特点分析

目的 探讨儿童结节性硬化症合并癫痫的临床特征。方法 对36例结节性硬化症合并癫痫患儿的临床资料进行分析。结果 所有患儿均以癫痫发作为首发症状,发病年龄〈1岁23例（63.89%）、1-3岁8例（22.22%）、〉3岁5例（13.89%）;发作类型以部分性发作[12例（33.33%）]、全面性发作[5例（13.89%）]、痉挛发作[7例（19.44%）]和混合性发作[12例（33.33%）]为主,其中伴智力低下或发育落后者19例（67.86%）。MRI可见室管膜下结节（30例）,部分病例[26例（86.67%）]伴皮质和皮质下结节、室管膜下巨细胞型星形细胞瘤2例（6.67%）或巨脑回畸形1例（3.33%）。脑电图显示发作间期样放电[34例（94.44%）],表现为多灶性放电[12例（35.29%）]、广泛性放电[8例（23.53%）]、局限性放电[7例（20.59%）]和高度失律[7例（20.59%）];其中8例监测到临床发作,包括痉挛发作（4例）、局灶性发作（3例）、肌阵挛发作（1例）。17例患儿（47.22%）服用1种抗癫痫药物、12例（33.33%）服用2种药物、7例（19.44%）服用3种药物,其中手术治疗1例;随访0.50~10年,无发作7例（19.44%）,有效16例（44.44%）,无效13例（36.11%）。结论 癫痫发作是儿童结节性硬化症最常见的神经系统表现,多在婴儿期发病,主要发作类型为部分性发作和痉挛发作,多数患儿抗癫痫药物治疗有效。     

Everyday memory measures in multiple sclerosis: a systematic review

ABSTRACT

Everyday memory is one of the most affected cognitive functions in multiple sclerosis (MS). Assessing everyday memory problems is crucial for monitoring the impact of memory deficits on individuals’ day-to-day lives and evaluating the effectiveness of interventions that aim to improve cognitive functions. The aim of this systematic review was to identify the research literature on everyday memory measures used with people with MS, describe the types of measures used, and summarise their psychometric properties. Empirical studies of cognitive function in MS using standardised everyday memory measures were included. Online databases (MEDLINE, PsycINFO, PsycARTICLES, Embase) and Google Scholar were searched. Forty-four studies met the inclusion criteria. A total of 12 measures were identified, with varied uses and administration methods. The majority of papers did not report any psychometric properties for MS populations. The few papers that did, reported that the measures have good reliability and appear to have good face, concurrent, and ecological validity, but these need to be evaluated further. This review presents researchers and clinicians with an overview of the various everyday memory measures used in studies with people with MS, to help them choose the appropriate measure for their evaluations.