Longitudinal changes of focal cortical glucose hypometabolism in adults with chronic drug resistant temporal lobe epilepsy

Brain Imaging and Behavior - A high proportion of patients with drug-resistant temporal lobe epilepsy (TLE) show focal relative hypometabolism in the region of the epileptogenic zone on...     

Long-term outcome in children with intractable epilepsy showing bilateral diffuse cortical glucose hypometabolism pattern on positron emission tomography

The objective of this study is to determine the long-term outcome of children with intractable epilepsy who have diffuse cortical hypometabolism on 2-deoxy-2-((18)F)fluoro-D-glucose positron emission tomography (FDG-PET) scans. Seventeen children with intractable epilepsy showing bilateral, diffuse cortical hypometabolism on FDG-PET were followed up through telephone interview from 1 year 4 months to 11 years 4 months (mean: 5 years 7 months ± 2 years 1 month) after their PET scans. One child succumbed to Sanfilippo disease at age 20 years. Only 2 children were seizure free. Fifty percent had walking difficulties, 56.25% were not toilet trained, all had speech difficulties, 43.75% had behavioral problems, 37.5% had poor eye contact, 75% had socialization difficulties, and 87.5% attended special schools. Three children were found to have genetic causes, including a 4-MB deletion of the mitochondrial genome, MECP2 duplication, and Lafora disease. In conclusion, the long-term outcome in this patient population is poor, and they tend to suffer from genetic/neurodegenerative diseases.     

Correlation between interictal cerebral glucose hypometabolism and IQ in children with epilepsy

The aim of this study was to understand the relationship between IQ and glucose metabolism in brain cells in a wide variety of subjects with epilepsy. The study participants were 78 children with epilepsy and 15 healthy children for comparison. All participants were administered the Chinese Wechsler Intelligence Scale for Children (C-WISC). The verbal intelligence quotient (VIQ), performance intelligence quotient (PIQ), and full-scale intelligence quotient (FIQ) were compared between children with epilepsy and typically developing children. Seventy-eight patients underwent interictal positron emission computed tomography (PET) using 2-deoxy-2[¹⁸F]fluoro-d-glucose (FDG) as the tracer for evaluating brain glucose metabolism. Verbal intelligence quotient, PIQ, and FIQ based on the C-WISC were significantly lower in children with epilepsy than those in the healthy comparison group (P < 0.001, P = 0.001, and P < 0.001, respectively). The IQ of patients with normal metabolism, unifocal abnormal hypometabolism, and multifocal abnormal hypometabolism determined by PET differed significantly. The extent of the abnormal hypometabolism was negatively correlated with the FIQ (rs = − 0.549, P < 0.001). In patients with lateralized hypometabolism based on PET, the VIQ/PIQ discrepancy scores (|VIQ − PIQ| ≥ 15 points) differed significantly between the left hemisphere abnormal hypometabolism and right hemisphere abnormal hypometabolism subgroups, with negative values in the left and positive values in the right subgroups (P = 0.004). In conclusion, brain metabolic abnormalities are correlated with IQ, and performing interictal PET along with C-WISC can better assess the extent of severity of cognitive impairment and VIQ/PIQ discrepancy.     

Increased striatal serotonin synthesis following cortical resection in children with intractable epilepsy

BACKGROUND AND PURPOSE: Serotonin is a major regulator of structural brain plasticity, which may occur following cortical resection in humans. In this study we used positron emission tomography (PET) with alpha[11C]methyl-l-tryptophan (AMT) to evaluate serotonergic alterations in subcortical structures following cortical resection in children with intractable epilepsy. METHODS: AMT uptake in the thalamus and lentiform nucleus was evaluated postoperatively (1-89 months following resection) in 19 children (mean age: 8.7 years) with a previous cortical resection due to intractable epilepsy. Ten children with partial epilepsy but without resection and seven normal children served as controls. RESULTS: There was an increased AMT uptake in the lentiform nucleus ipsilateral to the resection as compared to the contralateral side (mean asymmetry: 4.2+/-3.0%), and the asymmetries were significantly higher than those measured in the control groups (p相似文献   

Neuropsychological outcome following focal cortical removal for intractable epilepsy in children

In children, surgery for epilepsy has been recognized as a viable treatment option since publication of S. Davidson and M.A. Falconer's outcome study in 1975 [Lancet North Am Ed 5:1260-3], which demonstrated that medical outcome of children who underwent anterior temporal lobectomy paralleled that of adults. Pediatric surgical programs and the literature on medical outcome have grown considerably since that time, with surgery being offered to children with temporal but also extratemporal epilepsy foci. Comparatively little work has been conducted in the area of neuropsychological outcome. This article outlines differences in adult and pediatric outcome studies, reviews the literature on the intellectual and memory outcome in children, and discusses shortcomings of the pediatric outcome research conducted to date.     

Temporal lobe epilepsy with sensory aura: interictal glucose hypometabolism

Patients with mesial temporal lobe epilepsy (mTLE) exhibit marked depressions of the regional cerebral glucose metabolism (rCMRGlu) in the mesiotemporal region. We hypothesised that patients with temporal lobe epilepsy (TLE) who have a bilateral somatosensory or acoustic ( = temporolateral/SII-) aura can be differentiated from mTLE by rCMRGlu depressions primarily involving temporo-perisylvian locations. We therefore used this ictal semiology as a clinical criterion to define a subgroup of such patients and measured the rCMRGlu in 16 patients with TLE as evident from interictal and ictal EEG-video monitoring. Clinically, they presented with medically refractory complex partial seizures and were subjected to presurgical evaluation. The pattern of the interictal rCMRGlu in the TLE patients was different from that observed in patients with mTLE and showed significant depressions ipsilateral to the epileptic focus in mesial temporal and lateral temporal regions but spared the thalamus. The neocortical metabolic depressions were spatially more extended in right than in left TLE patients. Magnetic resonance images (MRI) were either normal (n = 5) or revealed unilateral or bilateral hippocampal atrophy/sclerosis (n = 7), or temporal or extratemporal focal cortical dysplasia (n = 4). The selected TLE patients presented here comprise a heterogeneous group showing most pronounced metabolic depressions in the lateral temporal cortex. Thus, our data suggest that non-invasive metabolic imaging can assist in identifying the neocortical symptomatogenic zone in putative temporo-perisylvian lobe epilepsy.     

Suppression-burst patterns in intractable epilepsy with focal cortical dysplasia

We report on a patient with early-onset spasms in series and partial seizures associated with focal cortical dysplasia whose EEGs showed suppression-burst patterns during early infancy. These electroclinical characteristics suggested a diagnosis of Ohtahara syndrome, but the EEG findings were atypical because of the lack of suppression-burst patterns during wakefulness. In addition, the patient did not have severe psychomotor retardation. With high-dose pyridoxal phosphate therapy, seizures were suppressed and suppression-burst patterns disappeared at 2 months of age. Focal motor seizures recurred later and they often evolved into epilepsia partialis continua. Patients with early-onset intractable seizures associated with suppression-burst patterns on EEGs have several different etiologies, and these patients should be categorized according to their etiology in addition to their syndromic diagnosis.     

Seizure precipitants in children with intractable epilepsy

PURPOSE: To investigate the seizure precipitants in children with intractable epilepsy, and to determine any distinctive clinical features contributing to seizures in these patients. METHODS: A questionnaire and seizure diary prepared by the parents of the patients. Demographic and seizure data were reviewed. RESULTS: Of 120 patients with intractable epilepsy, 74 (62%) had one (n=43), two (n=23), or three seizure precipitants (n=8). The three most common precipitants were illness or fever (32%), sleep deprivation (13%), and menstruation (10%). Of these precipitants, inducing factors (endogenous origin) were more common than triggering factors (exogenous origin): 73% versus 27%, respectively. Three distinctive clinical features - neurological abnormalities (P=0.01), status epilepticus (P=0.017), and abnormal neuroimaging (P=0.007) - were significantly more common in patients with than in patients without precipitants. CONCLUSIONS: Prompt recognition and management of seizure precipitants has practical implications for treating patients with refractory epilepsy. Such patients can be counseled to avoid specific precipitants.     

大脑半球离断术治疗儿童难治性癫痫

目的 探讨大脑半球离断术治疗儿童难治性癫痫的手术方法及疗效.方法 2007年8月至2011年10月北京三博脑科医院进行12例大脑半球离断手术.经侧裂半球离断术1例,经纵裂半球离断并颞叶切除4例,中央区造瘘半球离断并颞叶切除5例,颞叶、岛盖切除岛周半球离断术2例.手术年龄平均7.6岁(2.1 ～11.9岁).结果 术后随访0.5 -4.5年,Engel Ⅰ级10例,EngelⅡ级1例,Engel Ⅲ级1例.术后因离断不完全再次行离断手术1例.术后患者的认知及生活能力较术前提高,无脑积水等严重神经功能损伤及死亡病例.结论 大脑半球离断术治疗儿童半球性难治性癫痫完全缓解率83％ (10/12),手术疗效确定,是治疗儿童半球病变性癫痫的安全、有效的方法.     

Cerebral blood flow in children with intractable epilepsy

A good correlation of Doppler internal carotid blood velocity determinations with hemispheric blood flow measurements by the 133-xenon inhalation method was demonstrated in 14 epileptic patients without abnormal CT findings. The highest correlation was seen between the flow of gray matter (F1) and the internal carotid end-diastolic velocity (d) (left side r = 0.841, right side r = 0.817). As end-diastolic velocity (d) well correlated with the value obtained by the 133-xenon inhalation method, the d value was compared between 77 healthy children and 13 patients with intractable epilepsy. The mean d value of both internal carotid arteries in patients was 16.7 +/- 2.9 mm (mean +/- SD), and that of healthy children 20.9 +/- 4.3 mm, the difference being statistically significant. The low cerebral blood flow in patients might be due to multiple antiepileptic drugs administered and/or mental retardation and cerebral hypofunction related to seizures.     

Flunitrazepam for sleep disturbance in children with intractable epilepsy

Add-on therapy with flunitrazepam (FNZ) was performed in 5 children with marked sleep disturbance and intractable seizures. Correction of the sleep disturbance was attained immediately after the start of FNZ administration in all patients. Furthermore, a significant decrease in the seizure frequency (3 patients) and improved quality of life (4 patients) were concomitantly observed. There was no adverse effect or interaction with conventional AEDs on long-term use.     

半球切除治疗小儿半球性病变伴顽固性癫痫

一侧大脑半球病变的小儿伴顽固性癫痫仅仅进行病灶及癫痫灶切除或者脑叶切除,很难彻底控制患儿的癫痫发作.癫痫发作得不到控制,患儿的智力、脑功能的发育将进一步受影响.这个时期如果不采取半球切除进行治疗,将丧失最好的手术时机.因为小儿的脑功能代偿能力很强,此时进行半球切除后,很大部分切除掉的半球功能将被很好的代偿,并且随着癫痫发作的控制,患儿的智力行为及脑功能均可以得到提高和发展.     

Bilateral medial prefrontal and temporal neocortical hypometabolism in children with epilepsy and aggression

PURPOSE: To identify brain regions with abnormal function in children with intractable partial epilepsy and aggressive behavior by using 2-deoxy-2-[18F]fluoro-D-glucose (FDG) positron emission tomography (PET). METHODS: Six children (mean age, 9.9 years) with intractable partial epilepsy and aggressive behavior underwent detailed psychodevelopmental assessment and FDG-PET scanning. The objective technique of statistical parametric mapping (SPM) was applied to define focal abnormalities of glucose metabolism, and compared those with those of a group of normal adult subjects (n = 17) as well as age-matched children with epilepsy with similar seizure characteristics but without aggression (n = 7). The findings were analyzed further by using a region-of-interest (ROI) approach. RESULTS: The aggressive children all showed developmental delay, and four of them also manifested autistic symptoms. SPM analysis demonstrated extensive glucose hypometabolism in the aggressive group bilaterally in the temporal and prefrontal cortex compared with that in normal adult controls. A focal area of medial prefrontal glucose hypometabolism was defined in the aggressive children as compared with the nonaggressive pediatric group with SPM, whereas ROI comparison of these groups confirmed prefrontal hypometabolism and also showed glucose hypometabolism of the temporal neocortex in the aggressive children. Severity of aggression correlated inversely with glucose metabolism of the left temporal as well as bilateral medial prefrontal cortex. CONCLUSIONS: Bilateral prefrontal and temporal neocortical brain glucose hypometabolism in children with epilepsy and aggressive behavior may indicate a widespread dysfunction of cortical regions, which normally exert an inhibitory effect on subcortical aggressive impulses. PET studies may be used to elucidate the neurobiologic basis of aggressive behavior in children.     

儿童难治性癫痫的长期治疗

治疗癫痫患者的最终目标是摆脱癫痫发作,但有些癫痫患儿可能需要终身治疗,尤其是难治性癫痫患儿更加现实的目标是降低具有致残性发作类型的发作频率。长期发作会影响患儿社会活动、心理、认知等功能,因此对这些患儿的长期管理需要儿科和神经内科医师,以及社会工作者联合给予终身关爱。在治疗过程中,临床医师应定期评价患儿病情,注意预防和控制抗癫痫药物的不良反应,监测抗癫痼药物相关不良事件;为难治性癫痫患儿选择最佳治疗方案,在收益与风险之间达到平衡,提高患儿生活质量。     

Altered distribution of KCC2 in cortical dysplasia in patients with intractable epilepsy

PURPOSE: To examine the distribution of KCC2, a neuron-specific K(+)-Cl(-) cotransporter, in human cortical dysplasia (CD). METHODS: The immunohistochemical expression of KCC2 was investigated in 18 CD specimens obtained during epilepsy surgery. The histopathologic diagnoses were focal CD (FCD) type I (eight cases), FCD type II (six cases), and hemimegalencephaly (HME; four cases). Tissue sections were immunostained for KCC2 and compared with control sections. RESULTS: In the mature nondysplastic cortex, all the layers showed diffuse neuropil staining for KCC2. The somata were stained much less, although subcortical ectopic neurons displayed dense staining in the cytosol (intrasomatic staining). In FCD type I, the cortex showed neuropil staining for KCC2 with less-stained somata. Aberrant giant pyramidal neurons were also less stained at the soma, whereas immature neurons showed intrasomatic staining. Increased numbers of ectopic neurons with intrasomatic staining were noted in the subcortical white matter. In FCD type II, dysmorphic neurons displayed dense intrasomatic staining with reduced staining of the neighboring neuropils. Balloon cells did not stain for KCC2. Dysmorphic neurons in HME also showed intrasomatic staining. CONCLUSIONS: Neurons in CD tissues expressed KCC2. However, the subcellular distribution of KCC2 was altered, which might have affected the ionic homeostasis of Cl(-) and K(+) involved in epileptic activity within CD tissues.     

Sleep pattern in children with intractable epilepsy and mental retardation

Four cases with intractable epilepsy and mental retardation (Epi + MR), four cases of mental retardation (MR), one case of mental retardation without epileptic seizures for the last several years (MR + (Epi] and two normal children were studied on their sleep pattern. Besides these, two cases of epilepsy (Epi) were examined. Awake time increased in the Epi + MR group. Slow wave sleep decreased markedly in the Epi + MR group. REM sleep decreased in the MR + (Epi) and Epi + MR groups. REM density was lowered in the following order: normal----Epi----MR----Epi + MR groups. The difference of sleep pattern among the normal, Epi and MR groups was not exhibited clearly, but severe sleep disturbances were shown in the Epi + MR group, implicating the severe brain dysfunction in the cortex and the brain stem.