多系统萎缩合并继发性Ondine's curse综合征的临床及影像学特点(附5例报告)

目的探讨多系统萎缩(MSA)合并继发性Ondine's curse综合征的临床特点。方法采用回顾性分析自2009年3月至2014年8月我院住院的5例MSA合并继发性Ondine's curse综合征患者的临床资料。结果 5例均为老年患者,均在病程中出现Ondine's curse综合征,2例患者以呼吸困难为首发症状;3例患者发病以头晕、共济失调、帕金森叠加综合征为首发症状,且在发病后0.5年、4年、5年时出现发作性呼吸困难症状。经呼吸机辅助呼吸后患者通气改善。结论老年MSA患者出现发作性呼吸困难时,应警惕继发性Ondine's curse综合征的发生。     

延髓病变继发Ondine’s curse综合征临床分析

目的探讨延髓病变继发Ondine’s curse综合征的临床特点、治疗及预后,以提高对本病的认识。方法收集5 y来我院临床证实的延髓病变250例,其中双侧延髓病变8例作为研究组,8例中6例继发Ondine’s curse综合征,单侧延髓病变242例作为对照组,无1例继发Ondine’s curse综合征,对两组临床特点,影像学资料及诊治预后进行回顾性分析。结果研究组与对照组在继发Ondine’s curse综合征的发生率及预后方面比较差异有统计学意义(P<0.01);研究组6例继发Ondine’s curse综合征中2例治愈,其中1例通过抢救,症状恢复,影像学病灶可逆;另1例通过逐渐延长停机间歇时间,最终脱机,恢复正常呼吸;其它4例中2例自动出院,2例死亡。结论双侧延髓病变比单侧延髓病变更易继发Ondine s curse综合征,一旦出现则病情重预后差,应提高警惕,及时诊治;单侧延髓病变继发Ondine s curse综合征相对较少,预后相对较好。     

单侧延髓梗死伴发Ondine’s curse综合征一例

<正> Ondine's curse综合征是中枢性睡眠呼吸暂停综合征(central sleep apneasyndrome)的一种特殊类型,主要表现为睡眠状态时出现的二氧化碳潴留、低氧血症及呼吸暂停"。该命名来源于德国的一个古老传说,女神Ondine的惩罚会在夜间降临到那些犯了错误的人身上。该综合征分为先天性和获得性两种类型,先天性Ondine's curse综合征又称为先天性中枢性低通气综合征(Congenital     

Wallenberg综合征的磁共振成像

目的探讨MRI和MRA对wallenberg综合征的诊断价值.方法我们复习了经临床和影像学诊断的12例wallenberg综合征患者临床资料, 其中12例作了MRI检查, 有7例同时作了MRA检查.结果 MRI检查的12例中有2例正常, 10例可见延髓背外侧梗死, 其中8例为典型的延髓背外侧梗死病灶, 另2例病变超出延髓背外侧范围; 4例梗死病变仅限于延髓, 3例延髓合并小脑梗死, 3例延髓伴桥脑梗死.在MRA检查的7例中所有患者延髓梗死同侧的椎动脉显示流空消失, 5例患者的Willis环明显动脉粥样硬化, 1例基底动脉起始部明显狭窄.结论 MRI与MRA结合使用为wallenberg综合征的诊断提供了可靠的依据.     

延髓梗死29例临床分析

延髓梗死在MRI出现前常被误诊,临床上能做出正确诊断的往往是典型的Wallenberg综合征等。随着MRI技术的发展,延髓内侧梗死和不同的Wallenberg综合征表现型逐渐被发现和总结,病因学和临床症状学研究也逐渐丰富。现对我们发现的29例延髓梗死患者进行临床分析。     

延髓梗死的临床与神经影像学分析(附73例报告)

目的探讨延髓梗死的临床与神经影像学特点。方法回顾性分析73例延髓梗死患者的临床资料和影像学检查结果。结果本组男性53例,女性20例。首发临床表现复杂多样,其中浅感觉障碍41例,眩晕39例,饮水呛咳36例,恶心呕吐32例,吞咽困难31例,构音障碍29例。梗死病灶位于延髓上段27例,中段29例,下段12例,同时累及上段和中段3例,中段和下段2例。病灶位于背外侧61例,腹内侧10例,背内侧2例。表现为经典的延髓综合征共15例,包括Wallenberg综合征12例,Dejerine综合征1例,Avellis综合征1例,Babinski-Nageotte综合征1例。结论延髓梗死常见临床表现是浅感觉障碍和后循环缺血症状,经典延髓综合征少见;延髓梗死以中上段最常见,主要发生于背外侧;头部MRI对延髓梗死的诊断有极其重要的意义。     

延髓梗死11例患者MRI与临床特点对照研究

目的 探讨延髓梗死患者MRI病灶部位与临床表现特点的关系.方法 总结11例患者的临床症状和体征,对照MRI结果对梗死病灶进行定位,讨论病灶部位与临床表现的关系.结果 延髓外侧梗死5例,延髓内侧梗死6例(其中双侧梗死2例);延髓外侧梗死常见症状是言语不清、眩晕、饮水呛咳、吞咽困难及面部麻木,延髓内侧梗死常见症状是肢体瘫痪、言语不清;延髓外侧梗死常见体征是构音障碍、感觉障碍、Horner征及面瘫,延髓内侧梗死常见体征是肢体瘫痪、周围性舌瘫.结论 不同病灶部位的延髓梗死患者症状、体征各异,借助MRI可帮助诊断.     

延髓背外侧综合征36例临床分析

目的总结分析延髓背外侧综合征的临床表现及影像学特点。方法回顾性分析36例延髓背外侧综合征患者的临床资料及CT、MRI特点。结果本组起病较急,主要表现为感觉障碍、共济失调、眩晕、恶心呕吐、吞咽困难及饮水呛咳等,本组病例经CT及MRI检查均发现延髓背外侧梗死病灶。结论延髓背外侧综合征主要临床表现复杂,影像学检查对于明确诊断及定位定性价值较大,脑血管检查可以进一步明确病因。     

延髓背外侧综合征22例临床分析

目的总结延髓背外侧综合征的临床表现与影像学改变。方法对22例延髓背外侧综合征的临床资料及影像学进行回顾性分析。结果延髓背外侧综合征的主要表现为眩晕、恶心、呕吐、构音障碍、饮水呛咳、吞咽困难、眼球震颤、Honer征、共济失调、交叉性感觉障碍等。头部CT检查未发现延髓病变,17例头部MRI示延髓背外侧梗死。结论延髓背外侧综合征的主要病因为动脉粥样硬化,诊断主要依据临床特征及MRI。     

31例延髓梗死患者的临床分析

目的:根据头颅MRI对延髓梗死病灶的定位并结合延髓的解剖学特点,探讨延髓梗死临床表现的特征.方法:31例急性延髓梗死患者均行头颅MRI检查,对其病灶位置分布、神经系统症状体征,出院当天改良Rankin量表(mRS)评分等指标进行分析.结果:31例急性延髓梗死病例中:外侧延髓梗死(LMI)16例;内侧延髓梗死(MMI)13例;双侧延髓梗死(BMI)2例.LMI组病灶位于延髓背外侧或外侧;MMI组病灶位于延髓腹内侧,且多位于延髓上段.LMI多表现为不典型或部分性的"Wallenberg综合征",最常见的症状或体征为眩晕,Homer征、构音障碍、吞咽障碍、软腭麻痹,肢体共济失调.MMI多表现为感觉运动性卒中(SMS)或纯运动性卒中(PMS),最常见的症状或体征为对侧肢体偏瘫、构音障碍、中枢性面舌瘫.31例延髓梗死患者中,BMI 2例均为双侧MMI,其中1例出现四肢瘫痪、双侧肢体深浅感觉障碍、核间性眼肌麻痹、构音以及吞咽障碍,另1例表现为左侧肢体偏瘫、构音障碍、强哭、强笑等症状体征.结论:典型的延髓梗死综合征在临床上少见,LMI多表现为不典型,部分性的"Wallenberg综合征".而MMI往往与桥脑、基底节区腔隙性梗死的临床表现相似.双侧MMI导致四肢瘫痪等双侧锥体束受损表现,预后较差.     

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”.