脑立体定向手术治疗Meige综合征二例报告并文献复习

目的 探讨立体定向深部脑刺激术( DBS)和脑内核团毁损术在Meige综合征(MS)中的临床应用.方法 2例患者术前均采用Burke - Fahn - Marsden肌张力障碍运动评分(BFMDRS),1例MS患者行双侧苍白球内侧核(GPi) DBS手术治疗,1例患者行单侧GPi射频毁损术治疗.结果 行双侧GPi - DBS患者术后随访1年改善达83.7％(BFMDRS评分由术前的22.5分减少为4分),随访2年改善率达69.1％左右(BFMDRS评分由22.5分减少为6分),疗效较为稳定.1例行立体定向右侧GPi毁损术,术后1周疗效明显,BFMDRS从28分减少到6分,改善达78.6％;术后3个月BFMDRS评分为15分,改善率为46.2％,术后6个月随访,所有症状恢复到术前水平.结论 双侧GPi - DBS对于传统治疗无效的Meige综合征患者是一种安全有效的治疗方法;对于不能接受DBS手术的患者,行苍白球或丘脑毁损手术也是值得推荐的一种方法.     

国产电刺激器左右异频治疗Meige综合征

目的观察脑深部电刺激术(DBS)使用国产刺激器异频程控治疗Meige综合征的疗效。方法回顾性分析1例Meige综合征的病例资料,在双侧苍白球内侧核(Gpi)植入国产电极刺激器,术中采用微电极准确定位靶点,术后1个月开机进行常规程控,3个月后实施左右异频程控。采用肌张力障碍评分量表(BFMDRS)评价疗效。结果术后1个月开机,症状明显改善,BFMDRS从术前22分降至6分。但由于右侧电极触点邻近内囊后肢,刺激电压较低,病人出现左侧肢体发麻现象,通过增加左侧刺激电压达到治疗效果。术后3个月时症状反复,BFMDRS评分升至10分;改用左右异频刺激,通过降低右侧刺激频率,提升右侧电压改善症状,并降低左侧电压以减少耗电量,病人左侧肢体麻感消失,症状进一步改善,BFMDRS降至4分。结论国产脑深部电刺激器实施双侧Gpi DBS治疗Meige综合征效果理想,左右异频刺激可降低不良反应,进一步提高疗效。     

脑深部电刺激双侧苍白球治疗Meige综合征3例并文献回顾

目的： Meige综合征是一种特发性累及头面部的肌张力障碍,有时也会影响到颈部。脑深部电刺激（DBS）双侧苍白球内侧部（GPi）治疗肌张力障碍的报道比较多,但治疗Meige综合征的很少。方法对3例双侧GPi-DBS术后的Meige综合征患者进行随访4、6和60个月。所有患者术前术后均采用国际通用的Burke-Fahn-Marsden Dystonia Rating 量表（BFMDRS）评分,包括BFMDRS-Ⅰ（Movement 量表）和BFMDRS-Ⅱ（Disability量表）两个部分。结果3例Meige综合征患者手术后症状均有明显改善,BFMDRS第一部分和第二部分评分的改善率分别为（78±3．1）％（范围75％～83％）和100％。结论双侧GPi-DBS是治疗原发性Meige综合征的一种有效安全的手术方式。     

脑深部电刺激术治疗外伤性肌张力障碍的长期随访研究并文献复习

目的 观察双侧丘脑底核-脑深部电刺激术治疗1例继发于脑外伤后节段性肌张力障碍患者的术后长期疗效.方法 患者17岁时遭受严重且卤外伤昏迷20余天,3年后逐渐出现肌张力障碍,呈进行性发展.21岁接受双侧丘脑底核-脑深部电刺激术,术前BFMDRS评分83分,其中运动评分56,功能障碍评分27.自术后1个月给予刺激治疗开始对其进行术后随访,评价治疗效果.结果 开肩刺激器后1周时该患的症状开始有所缓解.给予刺激3个月后该患的症状得到明显改善,BFMDRS总评分改善49%,其中运动症状评分改善50%,功能障碍评分改善48%.在随后随访过程中,该患的肌张力障碍症状进一步得到改善,3.5年时该患情况稳定,肌张力障碍症状未再出现,BFMDRS评分为8分,运动症状评分8分,功能障碍评分0分.持续刺激未引起任何不良反应,患者不再服用相关药物.结论 双侧丘脑底核脑深部电刺激术能够有效的改善外伤性节段性肌张力障碍症状,是一种可供选择的安全有效的治疗方法.     

脑深部电刺激术治疗原发性全身性肌张力障碍(附2例报告)

目的 探讨关于脑深部电刺激术治疗原发性全身性肌张力障碍疗效的影响因素.方法 评估病程、病情相似的2例原发性全身性肌张力障碍患者接受脑深部电刺激术治疗的术后疗效.病例1:8岁发病,BFMDRS评分100,运动评分80,功能障碍评分20,DYT1基因突变阳性.病例2:18岁发病,BFMDRS评分98,运动评分71,功能障碍评分27,DYT1基因突变阴性.结果 2例患者术后症状均得到了改善,给予刺激治疗5个月时病例1的BFMDRS/运动评分/功能评分分别为38/32/6,病例2分别为66/44/22.病例1术后肌张力障碍症状改善的速度和程度均优于病例2,且病例2术后功能障碍改善较差.结论 脑深部电刺激术治疗原发性全身性肌张力障碍的术后疗效主要与手术时机有很大关系,应早期手术.     

苍白球内侧核脑深部电刺激联合内囊前肢毁损术治疗Meige综合征的长期疗效评估

目的探讨苍白球内侧核脑深部电刺激(GPi-DBS)联合双侧内囊前肢毁损术(BAIC)治疗Meige综合征的长期疗效。方法回顾性分析19例Meige综合征病人的临床资料,行GPi-DBS联合BAIC治疗,术前及术后不同时间段(3、6、12、18、24、36个月)采用肌张力障碍运动评分量表(BFMDRS)、Yale-Brown强迫症状量表(Y-BOCS)、Hamilton抑郁症状量表(HAMD)、Hamilton焦虑症状量表(HAMA)评分,评价手术疗效。结果术后18个月,共随访19例病人,术后3、6、12、18个月BFMDRS评分较术前明显下降(P0.05),术后12、18个月Y-BOCS、HAMA、HAMD较术前明显下降(P0.05);术后3、6、12、18个月BFMDRS评分平均改善率分别为69.8%、71.6%、72.1%、73.2%。术后24个月,共随访11例病人,BFMDRS、Y-BOCS、HAMA、HAMD术后评分较术前明显下降(P0.05);BFMDRS评分平均改善率为76%。术后36个月,随访3例病人,BFMDRS评分平均改善率为97.5%。1例随访48个月,肌张力障碍症状完全消失,同时强迫、焦虑、抑郁症状明显改善。病人均未出现颅内出血、感染及严重刺激相关并发症。结论 GPi-DBS联合BAIC治疗Meige综合征的长期疗效显著,并发症少,同时明显改善病人的焦虑、抑郁症状,是一种安全有效的治疗方法。     

脑深部电刺激术治疗肌阵挛肌张力障碍综合征三例并文献复习

目的观察脑深部电刺激术(DBS)治疗肌阵挛肌张力障碍综合征(myoclonus dystonia syndrome,MDS)的长期疗效。方法对3例MDS患者行DBS治疗,并进行术后随访。采用UMRS(unified myoclonus rating scale)肌阵挛量表和Burke-Fahn-Marsden肌张力障碍量表(Burke-Fahn-Marsden dystonia rating scale,BFMDRS)评价治疗效果并复习文献。结果本组患者采用丘脑腹外侧中间核DBS,开启刺激后,患者肌阵挛和肌张力障碍的症状均得到明显改善。1例患者因在术后第30个月开始出现新发部位的严重肌张力障碍,后加行双侧苍白球内侧部电刺激术。平均随访64.7个月,肌阵挛平均改善99.1%,肌张力障碍量表运动评分平均改善85.3%,功能障碍评分平均改善78.8%。经长期随访患者疗效稳定。结论 DBS对MDS有良好的治疗效果。     

丘脑底核电刺激术治疗原发性肌张力障碍

目的观察双侧丘脑底核(STN)脑深部电刺激(DBS)治疗原发性肌张力障碍的疗效。方法回顾性分析8例接受双侧STN-DBS的原发性肌张力障碍病人的临床资料。分别在术前和术后1、3、6个月进行Burke-Fahn-Marsden肌张力障碍评分(BFMDRS)和帕金森病综合评分量表(UPDRS),计算两项评分改善率,评价治疗效果。结果 BFMDRS和UPDRS评分的改善率:术后1个月其均值分别为47%和48%,术后3个月为69%和73%,术后6个月为75%和79%。结论 STN-DBS可有效改善原发性肌张力障碍病人的症状,术后疗效稳定。STN是DBS治疗原发性肌张力障碍的理想靶点。     

微电极引导立体定向核团毁损和脑深部电刺激治疗帕金森病

目的研究微电极引导立体定向颅内核团毁损和脑深部电刺激手术(deep brain stimulation,DBS)治疗帕金森病的临床疗效。方法分析我院116例应用微电极引导立体定向核团毁损术和85例应用脑深部电刺激术治疗的帕金森病患者的临床资料,获得术前、术后和DBS开启后6个月、1年、3年及5年的不同服药状态下帕金森病联合评分量表(UPDRS)的评分,比较手术前后UPDRS运动评分的差异。结果核团毁损术和DBS在术后6个月、1年和3年的随访中均能显著改善患者术前UPDRS运动评分,在第5年仅DBS组UPDRS运动评分较术前有改善,同时DBS组患者术后抗帕金森病药物用量较术前减少。结论核团毁损和脑深部电刺激手术均能显著改善帕金森病患者的UPDRS运动评分,DBS疗效更为长久。     

帕金森病丘脑底核深部脑刺激术后参数的调整

目的 探讨帕金森病丘脑底核深部脑刺激(DBS)术后脉冲发生器的参数调整.方法 回顾性分析117例帕金森病病人的临床资料,均行丘脑底核DBS,单侧电极植入45例,双侧电极植入72例;并记录术后刺激参数的调整.结果 刺激参数:双极刺激电压1-4 V,单极刺激电压1-3.6 V;脉宽60-90 μs;频率130 Hz.统一帕金森病评定量表(UPDRS)运动评分的改善率,双侧刺激较单侧刺激明显(P<0.05).治疗后1-6个月,抗帕金森药物用量减少78例(66.7%),维持术前水平39例(33.3%).结论 帕金森病丘脑底核DBS术后采用适当刺激参数可获得安全、可靠的疗效;电压调整对帕金森症状控制作用明显,脉宽及频率调整相对较少;双侧刺激效果优于单侧.     

Diagnostic Difficulties and Treatment Implications

Summary: Differentiation between types of epileptic seizures has been aided in recent years by the introduction of intensive neurodiagnostic techniques and the development of increasingly detailed classification systems. Paradoxically, these developments have not simplified the task of matching the appropriate antiepileptic drug to a particular seizure type. It is reasonable to assume that anticonvulsant drugs will have different effects on different types of seizures, but faulty, circular reasoning can enter the picture if one also assumes that responses of seizures to different drugs signify different seizure types. There are several examples of differential diagnoses that can fall prey to this problem, including the diagnosis between partial seizures with secondary generalization and generalized tonic-clonic seizures, and the diagnosis between complex partial seizures and absence seizures with automatisms, among others. Considerations of etiology in future classification systems can further complicate the problem: should one then choose an anticonvulsant drug on the basis of individual seizure type or on the basis of the type of epilepsy? Ramifications of this issue extend even to the drug approval process. Official sanction is not given for use of a drug for a seizure type not included in the original efficacy studies, even if later scientific evidence shows that seizure type to be related to a type that is included. New trials must be undertaken. These problems arise from how we choose to classify seizures.     

Cognitive Dysfunction Associated with Antiepileptic Drug Therapy

Summary: Epilepsy is frequently associated with cognitive dysfunction. However, the reasons for this correlation are unclear. Possible influential factors include patient age; duration, frequency, etiology, and type of seizures; hereditary factors; psychosocial issues; and antiepileptic drug (AED) therapy. Whereas many of these factors are beyond the physician's control, AED therapy is one element that can be addressed in treatment decisions by recognizing the potential cognitive effects of particular AEDs. For example, phenobarbital impairs memory and concentration; phenytoin affects attention, problem solving ability, and performance of visuomotor tasks. In contrast, carbamazepine may affect concentration, while valproate would appear to have minimal effects on cognition. Moreover, cognitive effects of AEDs are amplified with coadministration of multiple anticonvulsants (polytherapy). A review of studies on the cognitive effects of monotherapy with AEDs, as opposed to those of polytherapy, provides evidence that drug-related cognitive dysfunction can be reversed if patients are switched to a simpler therapeutic regimen. Future research should be directed toward developing reliable measures for assessing and monitoring cognition, and understanding the particular cognitive side effects of each AED. Physicians also need to revise their opinions about which side effects are "tolerable" for epileptic patients.     

Carbamazepine Efficacy in Adults With Partial and Generalized Tonic-Clonic Seizures

Summary: Carbamazepine and phenytoin are drugs of choice in initial monotherapy for adult partial and secondarily generalized tonic-clonic seizures. These designations reflect the results of the Veterans Administration Epilepsy Cooperative Study Group of 1985. An earlier comparative study of carbamazepine and phenytoin by Ramsay and associates found both drugs equally effective in controlling new-onset seizures. Among the advantages of carbamazepine is that it causes relatively few cognitive and dysmorphic side effects. Its disadvantages are its unavailability in parenteral formulation and its metabolic autoinduction. The latter must be compensated for by planned dosage increases to maintain therapeutic plasma steady-state levels during the first 2 or 3 months of treatment. Carbamazepine is judged a drug of choice in the treatment of these secondarily generalized tonic-clonic seizures, and the drug of choice in children, adolescents, and women susceptible to the dysmorphic side effects associated with other anticonvulsant agents.     

Etiologic and Preventive Aspects of Epilepsy in the Child–Bridging the Gap Between Laboratory and Clinic

Summary: Four broad categories of basic phenomena are pertinent to developing ways to prevent epilepsy. These include mechanisms of epileptogenesis, ictal initiation and temporary entrainment by the seizure discharge of normally functioning brain, seizure propagation, and control mechanisms that function both to restrain the cascade of epileptic events culminating in a seizure and to arrest the epileptic event and restore the interictal state. In newborns and children, hypoxia-ischemia is a major factor leading to epileptogenesis, and several schemes are proposed to classify, quantify, and prevent hypoxic-ischemic encephalopathy. Control mechanisms must be better understood in order to develop prophylactic recommendations for epilepsy, and an experimental model of "kindling antagonism" may increase our understanding of these. Programs of prevention of seizures in children will evolve only if basic researchers and clinicians work productively together to develop an adequate understanding of factors important in epileptogenesis and antiepileptogenic control mechanisms.     

Predisposing and Causative Factors in Childhood Epilepsy

Summary: We review information from large studies of defined populations, examining the role of known factors and especially of prenatal and perinatal factors in contributing to nonfebrile seizure disorders of early childhood. We depend especially, but not exclusively, on the recently completed analyses from the Collaborative Perinatal Project of the National Institute of Neurological and Communicative Disorders and Stroke, the NCPP. About 4% of children in the NCPP who had at least one non-febrile nonsymptomatic seizure by the age of 7 years had a previous seizure during acute neurologic illness, such as meningitis or during the acute illness after trauma. Many such seizures should potentially be preventable. Of children with seizures, 10% had had a neonatal seizure and 13% had had a febrile seizure. Among the hundreds of prenatal and perinatal factors explored as predictors of childhood seizure disorders, the principal predictors identified were congenital malformations of the fetus, cerebral and noncerebral; family history of certain neurologic disorders; and neonatal seizures. In agreement with the British National Child Development Study, labor and delivery factors in the NCPP appeared to contribute very little to childhood seizure disorders. Maldevelopment, rather than damage at birth to an initially intact nervous system, appeared to be the more common mechanism. Most seizure disorders of early childhood remained unexplained by the large set of prenatal and perinatal characteristics examined.     

Anticonvulsant Drugs and Cognitive Function: A Review of the Literature

Summary: Alterations of cognitive function are separate from disturbances of behavior seen in association with epilepsy. The nature of the cognitive disability may to a certain extent depend on the seizure type. Partial seizures, mainly derived from a temporal lobe focus, impair memory tasks, while generalized seizures seem to have more effect on attentional abilities. A number of studies, reviewed in this paper, suggest that anticonvulsant drugs further impair cognitive function. Maximal impairments are seen in patients receiving polytherapy: rationalization of polytherapy improves cognitive abilities. Studies in children and adults have allowed differentiation of the effects of various commonly used antiepileptic agents. Maximal cognitive deficits are seen with. phenytoin, while phenobarbital and sodium valproate induce moderate disturbances, and carbamazepine seems relatively free from such toxicity. Further research is needed on the interrelationship between types of seizure disorders, types of anticonvulsant medications, and cognitive function.     

Effects of NMD A- and AMPA-Receptor Antagonists on Different Forms of Epileptiform Activity in Rat Temporal Cortex Slices

Summary: Lowering extracellular magnesium induces different patterns of epileptiform activity in rat hippocampus and entorhinal cortex. Short recurrent epileptiform discharges in the hippocampus are stable over time, whereas seizurelike events (SLEs) in the entorhinal cortex, the subiculum, and the neighboring neocortex develop into late recurrent discharges which are not blocked by clinically employed antiepileptic drugs. We tested the sensitivity of the different epileptiform discharge patterns to. /V-methyl-D-aspartate (NMDA)- and non-NMDA-receptor antagonists. As NMDA-receptor antagonist we used dextrorphan, ket-amine, and 2-aminophosphonovalerate (2APV); as α-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid (AMPA)-receptor antagonist we employed the quinoxaline derivative glutamate 6-cyano-7-nitroquinoxaline-2,3-dione (CNQX). The findings show that the different patterns of epileptiform activity, including the late recurrent discharges, are sensitive to all NMDA-receptor antagonists. However, when dextrorphan was employed to suppress seizure-like events, later recurrent discharges did not develop during the remaining time course of the experiment. CNQX reversibly suppressed recurrent discharges in the hippocampus and SLEs in the entorhinal cortex. However, late recurrent discharges become insensitive to CNQX, even at a high concentration of 60 μM m. This finding suggests a prominent role for NMDA receptors in the generation of late recurrent discharges.     

The influence of comorbid depression on seizure severity

PURPOSE: To determine the relation between depressive symptoms and seizure severity among people with epilepsy. METHODS: A postal questionnaire was used to survey a nationwide community sample about seizures and depression. The Seizure Severity Questionnaire (SSQ) assessed the severity and bothersomeness of seizure components. The Centers for Epidemiological Studies-Depression scale categorized levels of depression. RESULTS: Respondents categorized as having current severe (SEV, n = 166), mild-moderate (MOD, n = 74), or no depression (NO, n = 443) differed significantly in SSQ scores (all p < 0.0001). People with SEV or MOD reported significantly worse problems than did those with NO depression for overall seizure recovery (mean, 5.3, 4.9, 4.5, respectively); overall severity (5.0, 4.5, 4.2); and overall seizure bother (5.3, 4.8, 4.4) (all p < 0.005). Cognitive, emotional, and physical aspects of seizure recovery also were rated worse among people with SEV than with NO depression (all p < 0.05). Symptoms of depression were significantly correlated with higher levels of all components of generalized tonic-clonic seizure severity (r = 0.33-0.48; all p < 0.0001), and partial seizures (r = 0.31-0.38; all p < 0.01). CONCLUSIONS: Clinically depressed people with epilepsy reported higher levels of perceived severity and bother from seizures, as well as greater problems with overall seizure recovery than did nondepressed people experiencing similar types of seizures. The pervasive influence of depressive symptoms on reports of seizure activity suggests that people with epilepsy should be screened for depression. These data highlight the importance of detecting and treating depression among people with epilepsy.     

Une phénoménologie de la dépendance à l'alcool. Une expérience primordiale de la « nostrité »

The phenomenological approach to alcoholism interestingly focuses on specific dynamics of interpersonal relationships displaying the founding of the Self from a primary “us” and its original basis in the human feast. Priorities for treatment intervention recommend to involve social setting and relationships of the patients, reaching their active participation to a motivational and long term group treatment, underlying the specific therapeutic effect of world exchanges. Biopsychosocial determination of alcoholism could be primarily based on components of interpersonal relationships. Regarding social background, drinking is one of the most famous supports for the achievement of the feast, a founding marker of present time. Taking an existential point of view, the feast appears as the heart of mankind because it presents a primary “us”, a plural state which indicates the beginning and founding of the Self from the others. During the feast, we regularly have to reach our Self from the “us” while avoiding two main dangers, drunkenness, an increase in the dizziness of upright verticality, and addiction, an opposite vertical surrender to alcohol and falling into in the alcoholic relapse, both situations imply a spatial domination and the disappearance of others. Treatment programs of alcohol addicts need to integrate the necessity of reaching the existential basic trust from the support of a group to the appropriation of the community which can be defined as an original “usness”.