烟雾病合并颅内血流相关性动脉瘤的血管内治疗

目的探讨烟雾病患者颅内血流相关性动脉瘤的临床特点及血管内治疗效果。方法回顾性分析2012年9月至2013年9月采用血管内栓塞治疗的7例烟雾病合并颅内血流相关性动脉瘤患者的临床资料,其中采用单纯弹簧圈栓塞3例,支架辅助弹簧圈栓塞3例,ONYX胶栓塞1例。结果7例患者中3例表现为蛛网膜下腔出血,3例表现为脑室出血,1例表现为顶枕部脑内出血;3例动脉瘤位于基底动脉顶端,1例位于脉络膜前动脉,1例位于大脑后动脉顶枕支,2例位于胼周动脉。7例均成功行血管内栓塞治疗,6例随访均获良好疗效。结论烟雾病合并血流相关性动脉瘤可导致不同类型的颅内出血,血管内栓塞治疗烟雾病合并颅内动脉瘤安全、有效。     

成人出血型烟雾病的临床特点分析

目的探讨成人出血型烟雾病的临床特点,为提高其诊治水平提供帮助。方法回顾性分析58例成人出血型烟雾病患者的临床资料。结果 58例患者均以中风发病,头颅CT示单纯脑室出血14例、脑室+脑实质出血34例、蛛网膜下腔出血6例、脑实质出血2例、脑实质出血+蛛网膜下腔出血1例。MRI见"常春藤"征者5例、基底节区血管留空影4例。DSA示合并动脉瘤或疑似动脉瘤8例。按GOS评定预后,恢复良好34例,中残15例,重残2例,植物生存1例,死亡6例,31例随访6月至10年,病史大于3年者11例,4例发生再次脑出血;病史小于3年者20例,2例发生再次脑出血。结论脑室出血合并脑实质出血是成年出血型烟雾病的主要出血方式;临床上对于不明原因的无血管病变危险因素的脑出血患者均应常规行脑血管造影检查。     

出血性烟雾病合并颅内动脉瘤的治疗方式探讨(附13例病例报道)

目的 探讨出血性烟雾病合并颅内动脉瘤患者的治疗方式。方法 回顾性分析华中科技大学同济医学院附属同济医院神经外科自2016年4月-2018年4月共收治的13例出血性烟雾病合并颅内动脉瘤患者的临床资料。结果 13例患者中共发现13个动脉瘤,其中8个动脉瘤为主干动脉型,5个为外周动脉型; 所有动脉瘤均为责任动脉瘤; 表现为蛛网膜下腔出血8例,脑室内出血3例,基底节出血破入脑室2例。8例合并主干动脉型动脉瘤患者均行弹簧圈栓塞治疗,其中有2例患者后期行血管重建术; 5例合并外周动脉型动脉瘤患者,其中1例早期行予Onyx胶栓塞治疗,后期行血管重建术,2例行开颅动脉瘤夹闭及血管重建术,另外2例早期行保守治疗,后期行血管重建术。8例烟雾病合并主干动脉型动脉瘤患者经弹簧圈栓塞术后,5例达到致密栓塞,3例瘤颈部分残余; 5例烟雾病合并外周动脉型动脉瘤患者中2例行开颅动脉瘤夹闭及血管重建术治疗,动脉瘤夹闭完全,1例行Onyx胶栓塞术后未见动脉瘤残留,未出现新鲜梗死灶,2例早期保守治疗,后期行血管重建术患者复查,其中1例动脉瘤稳定,另外1例动脉瘤消失。13例患者术后临床症状较术前均有不同程度的改善或恢复。结论 临床上出血性烟雾病合并颅内动脉瘤患者并不少见,根据动脉瘤的类型采取不同的治疗方式和积极的治疗措施能达到更好的治疗效果。     

脑血管造影诊断成人型烟雾病26例

目的对26例成人烟雾病患者的数字减影血管造影(DSA)的影像资料进行总结。方法 26例成人烟雾病患者经股动脉穿刺插管行全脑血管造影。结果 26例患者出血型18例,缺血型5例,混合型3例;大脑前动脉15例,大脑中动脉20例,同时累计大脑前动脉、中动脉者13例,大脑后动脉5例,合并动脉瘤5例。结论成人型烟雾病以出血型为主,烟雾病病变血管可以累及多支血管,可出现于单侧烟雾病,并可合并动脉瘤,DSA是确诊烟雾病的金标准。     

成人烟雾病合并颅内动脉瘤13例临床分析

目的探讨成人烟雾病合并颅内动脉瘤的临床特点。方法回顾分析2015年5月至2018年3月在广东省人民医院神经外科住院确诊的141例成年烟雾病患者(其中合并颅内动脉瘤13例)的临床、影像和介入治疗资料,根据Raymond分级评估术后动脉瘤栓塞效果,依据改良Rankin评分(mRS)评价患者术后随访结果。结果合并动脉瘤的MMD患者的颅内出血发生率(8/13)显著高于单纯MMD患者(24/128)(χ~2=12.315, P﹤0.05)。13例烟雾病合并动脉瘤13个,其中8例为主干型动脉瘤(3例出血,5例表现为缺血症状),5例为周围型动脉瘤(均破裂出血,脑室内出血或脑内出血破入脑室)。烟雾病单侧型4例、双侧型9例,铃木分期2期5例、3期7例、4期1例。主干型动脉瘤行单纯弹簧圈栓塞或支架辅助弹簧圈栓塞;周围型动脉瘤中的4例超选成功予以液态栓塞剂栓塞,1例因超选困难行外科手术血流重建。合并动脉瘤患者获平均随访(11±3)个月,动脉瘤介入栓塞后均未见复发,除1例瘤颈残余外其余均获Raymond I级愈合,所有患者随访时mRS评分预后良好。结论合并动脉瘤的烟雾病患者有更高的颅内出血风险,动脉瘤的位置、形态与烟雾血管特征呈血流相关,介入栓塞是治疗此类动脉瘤安全有效的方法。     

成人烟雾病17例临床表现和影像学特点分析

目的探讨成人烟雾病的临床和影像学改变特点。方法回顾性分析17例经常规数字减影血管造影(DSA)、磁共振血管造影(MRA)和/或MRI检查确诊的成人烟雾病患者的临床和影像学资料。结果 17例患者中表现为缺血性脑卒中12例(70.6%),主要表现为偏瘫、失语、头痛及智能减退,还可表现为短暂性脑缺血发作;出血性脑卒中5例(29.4%),主要表现为头痛、意识障碍、偏瘫及抽搐。MRI检查发现缺血性脑卒中累及额叶8例,底节区3例,深部脑白质3例,枕叶3例,颞叶1例;累及单侧半球4例,双侧半球8例;脑萎缩6例。环池内烟雾血管影1例,双侧大脑中动脉累及10例。出血性脑卒中患者中单纯脑室出血2例,蛛网膜下腔出血1例,脑室出血并脑干出血1例,脑室出血并丘脑出血1例。MRA均发现双侧颈内动脉或其分支狭窄或闭塞。全脑DSA检查7例,其中双侧颈内动脉狭窄、闭塞5例,双侧大脑前动脉狭窄或闭塞4例,双侧大脑中动脉狭窄或闭塞3例。结论成人烟雾病临床表现多样,临床特点与责任病灶及支配血管相关;MRI和/或MRA有助于烟雾病的早期诊断,确诊需行全脑DSA检查。     

20例出血型烟雾病的临床及影像学分析

目的分析出血型烟雾病（MMD）的临床及影像学特征。方法回顾性分析20例出血型烟雾病的临床资料,观察其临床及影像学特点。结果本组患者主要集中在35～45岁年龄组,其中80为女性。头颅CT扫描示脑室系统出血、脑实质出血及蛛网膜下腔出血;DSA表现为颈内动脉末端狭窄或闭塞,脑底烟雾状血管网、侧枝循环血管网以及动脉瘤形成。结论DSA检查是确诊烟雾病的主要方法,临床上对于无血管病变危险因素的脑出血患者均应常规行DSA检查。     

烟雾病的临床特点及DSA分析

目的探讨烟雾病的临床特点和DSA表现。方法回顾分析我院2006年以来收治的34例烟雾病患者的临床特点和DSA表现。结果 (1)34例患者均表现为急性脑卒中,其中脑出血21例(61.8%),蛛网膜下腔出血3例(8.8%),脑梗死10例(29.4%)。(2)所有患者均经DSA确诊,DSA主要表现为单侧或双侧颈内动脉及大脑前、中动脉狭窄或闭塞,均伴有颅底不同程度烟雾状异常增生的血管网;双侧病变25例,单侧病变9例;部分病例有侧支代偿;另有4例患者检出4枚动脉瘤。结论烟雾病的临床表现以急性脑卒中为主,成人患者主要表现为脑出血,尤其是脑室出血,其次是脑梗死。DSA是诊断烟雾病的金标准,对发病年龄较轻的卒中患者应行DSA检查明确诊断。     

烟雾病合并动脉瘤栓塞治疗6例及文献复习

目的探讨烟雾病(MMD)合并颅内动脉瘤的血管内介入治疗的特点及经验。方法回顾性分析2007年5月~2015年6月期间6例烟雾病合并动脉瘤栓塞患者的临床资料及影像学表现。以脑实质内出血发病的3例患者中,2例患者考虑为合并非责任动脉瘤,行单纯栓塞;1例患者考虑为假性动脉瘤,为出血责任病灶,应用20%Glubran闭塞动脉瘤并载瘤动脉。1例未出血的眼动脉瘤患者行支架辅助弹簧圈栓塞治疗。2例表现蛛网膜下腔出血的患者,急诊行CTA检查示颅底动脉环附近动脉瘤,行单纯栓塞治疗。结果 6例患者手术均取得成功,5例弹簧圈栓塞患者中,1例取得致密栓塞,随访结果良好。结论对于烟雾病合并动脉瘤患者,因烟雾病有容易发生缺血并发症的风险,选择血管内栓塞治疗的效果是确切的,相对于开颅动脉瘤夹闭术是安全的。     

烟雾病致脑室出血的脑血管影像学特点

目的探讨烟雾病致脑室出血的脑血管影像学特点。方法回顾性分析64例烟雾病致脑室出血患者的临床资料。结果本组患者DSA检查示,双侧病变58例、单侧病变6例,合并动脉瘤4例;均见烟雾状血管。其中双侧大脑前动脉闭塞4例(6.3%),双侧大脑中动脉闭塞2例(3.1%),脉络膜前动脉远端闭塞41例(64.0%),后交通动脉远端闭塞11例(17.2%),单侧颈内动脉分叉处或大脑前动脉A1段、大脑中动脉M1段闭塞共6例(9.4%)。脉络膜前动脉异常扩张和延长108支(84.4%),后交通动脉异常扩张或延长26支(20.3%)。结论脉络膜前动脉和/或后交通动脉扩张、延长是烟雾病致脑室出血的脑血管影像学特点,并且这也可能是烟雾病致脑室出血的主要病因。     

Epilepsy and anomalies of neuronal migration: MRI and clinical aspects

Neuronal migration disorders are the result of disturbed brain development. In such disorders, neurons are abnormally located. In diagnosing these conditions, magnetic resonance imaging is superior to any other imaging technique. This enables us to improve our knowledge of the clinical correlates of neuronal migration. With reference to migrational disorder, a retrospective study of all 303 patients with epileptic seizures referred for magnetic resonance imaging during a 3-year period was performed, 13 patients (aged 12-41, mean age 27) were identified. They represent 4.3% of the entire study group. Of the patients with known epilepsy, 6.7% and of the mentally retarded, 13.7% had migrational disorders. Four patients had schizencephaly as the dominant finding, one was classified as hemimegalencephaly, 2 had isolated heterotopias, and 6 had localized pachy- and/or poly-microgyria. The clinical pictures are complex. Ectopias of grey matter are recognised foci of epilepsy, but from an epileptological and a clinical viewpoint little attention has been given to these disorders. The present study shows that malmigration is not rare in epilepsy patients, especially not in the mentally retarded.     

Classification of methods in transcranial Electrical Stimulation (tES) and evolving strategy from historical approaches to contemporary innovations

Transcranial Electrical Stimulation (tES) encompasses all methods of non-invasive current application to the brain used in research and clinical practice. We present the first comprehensive and technical review, explaining the evolution of tES in both terminology and dosage over the past 100 years of research to present day. Current transcranial Pulsed Current Stimulation (tPCS) approaches such as Cranial Electrotherapy Stimulation (CES) descended from Electrosleep (ES) through Cranial Electro-stimulation Therapy (CET), Transcerebral Electrotherapy (TCET), and NeuroElectric Therapy (NET) while others like Transcutaneous Cranial Electrical Stimulation (TCES) descended from Electroanesthesia (EA) through Limoge, and Interferential Stimulation. Prior to a contemporary resurgence in interest, variations of transcranial Direct Current Stimulation were explored intermittently, including Polarizing current, Galvanic Vestibular Stimulation (GVS), and Transcranial Micropolarization. The development of these approaches alongside Electroconvulsive Therapy (ECT) and pharmacological developments are considered. Both the roots and unique features of contemporary approaches such as transcranial Alternating Current Stimulation (tACS) and transcranial Random Noise Stimulation (tRNS) are discussed. Trends and incremental developments in electrode montage and waveform spanning decades are presented leading to the present day. Commercial devices, seminal conferences, and regulatory decisions are noted. We conclude with six rules on how increasing medical and technological sophistication may now be leveraged for broader success and adoption of tES.     

Hepatic Considerations in the Use of Antiepileptic Drugs

Summary: Virtually all of the major antiepileptic drugs (AEDs) can cause hepatotoxicity, although fatal hepatic reactions are rare. The mechanisms, incidences, and risk profiles for such reactions differ from drug to drug. With carbamazepine and phenytoin, hepatotoxicity may be due to drug hypersensitivity. Although the profiles of patients at risk have not been well-defined for these two antiepileptic drugs, it would appear from reports in the literature that older adolescents and adults are at higher risk than children of developing serious or fatal hepatotoxicity. Once hepatotoxicity develops, mortality rates are 10–38% with phenytoin and 25% for carbamazepine. The risk profile for valproate fatal hepatotoxicity has been more clearly defined. Those at primary risk of fatal hepatic dysfunction are children under the age of 2 years who are receiving multiple anticonvulsants and also have significant medical problems in addition to severe epilepsy. The risk is considerably lower for patients over the age of 2 years on valproate monotherapy. In contrast to the risk profile with other AEDs, adults receiving valproate as monotherapy have the lowest risk of hepatotoxicity. Fatal hepatic dysfunction coincident with valproate may be the result of aberrant drug metabolism. Concomitant use of AEDs that induce microsomal P450 enzymes (e.g., phenytoin and phenobarbital) may enhance the production of a toxic metabolite, and hence the greater risk of hepatotoxicity with polypharmacy.     

Vascular Malformations and Epilepsy: Clinical Considerations and Basic Mechanisms

Summary: Vascular malformations (VMs) are associated with epilepsy. The natural history of the various VMs, clinical presentation, and tendency to provoke epilepsy determine treatment strategies. Investigations have probed the mechanisms of epileptogenesis associated with these lesions. Electrophysiologic changes are associated with epileptogenic cortex adjacent to VMs. Putative pathophysiologic mechanisms of epileptogenesis include neuronal cell loss, glial proliferation and abnormal glial physiology, altered neurotransmitter levels, free radical formation, and aberrant second messenger physiology.     

Neonatal Seizures: Problems in Diagnosis and Classification

Summary: The clinical identification of neonatal seizures is critical for the recognition of brain dysfunction; however, diagnosis is often difficult because of the poorly organized and varied nature of these behaviors. Current classification systems are limited in their ability to communicate motor, autonomic, and electroencephalo-graphic features of seizures precisely and to provide a basis for uniform effective diagnosis, therapy, and determination of prognosis. Recent investigations of neonates, utilizing bedside electroencephalographic/polygraphic/ video monitoring techniques, have provided the basis for improved diagnosis and classification of seizures in the newborn. These studies have demonstrated that not all clinical phenomena currently considered to be seizures require electrocortical epileptiform activity for their initiation or elaboration. In addition, the specific clinical character of the phenomena considered to be seizures, the clinical state of the infant, and the character of the EEG indicate the probable pathophysiological mechanisms involved and suggest probable etiologies, prognosis, and therapy. Similarities between animal models that demonstrate reflex physiology and neonates with motor automatisms and tonic posturing suggest that these clinical behaviors may not be epileptic in origin but, rather, primitive movements of progression and posture mediated by brainstem mechanisms. Although not all clinical behaviors currently considered to be neonatal seizures may have similar pathophysiological mechanisms, they are clinically significant because they all indicate brain dysfunction.     

Valproate Monotherapy in the Management of Generalized and Partial Seizures

Summary: For decades, therapeutic tradition has promoted the concept of polypharmacy in the management of epilepsy. In recent years, however, studies have shown that, for most patients, monotherapy can provide comparable or better seizure control than administration of multiple anticonvulsants, while diminishing the potential for adverse reactions, drug interactions, and poor compliance. Valproate is an important monotherapeutic agent that is highly effective in the control of idiopathic primary and secondarily generalized epilepsies, and partial seizures that do not generalize. Comparative studies have found that valproate is at least as effective as phenytoin and carbamazepine in the treatment of generalized and partial seizures. Given the similar efficacy, other factors such as pharmacokinetics and side effects may therefore determine anticonvulsant selection for monotherapy.     

Carbamazepine Efficacy and Utilization in Children

Summary: Carbamazepine is effective for preventing partial and generalized tonic-clonic seizures in children. Although absence epilepsies are more common in children than adults, an estimated 80% of children with epilepsy have seizure types or epilepsies that are potentially responsive to carbamazepine. The differential diagnosis of ictal staring is an especially important issue in children because absence and atypical absence seizures are more prevalent in children than adults. Age-related pharmacokinetic differences and drug interactions are major considerations in children. On average, children have higher clearance rates of carbamazepine, shorter half-lives, and higher ratios of carbamazepine-10, 11-epoxide to carbamazepine than adults. In addition, children with severe epilepsy are more likely to require multiple-drug therapy, which can lead to complex drug interactions. When carbamazepine is administered along with valproate, drug protein binding interactions can cause intermittent side effects.     

Un nouveau cadre conceptuel de travail pour la psychiatrie

In an attempt to place psychiatric thinking and the training of future psychiatrists more centrally into the context of modern biology, the author outlines the beginnings of a new intellectual framework for psychiatry that derives from current biological thinking about the relationship of mind to brain. The purpose of this framework is twofold. First, it is designed to emphasize that the professional requirements for future psychiatrists will demand a greater knowledge of the structure and functioning of the brain than is currently available in most training programs. Second, it is designed to illustrate that the unique domain which psychiatry occupies within academic medicine, the analysis of the interaction between social and biological determinants of behavior, can best be studied by also having a full understanding of the biological components of behavior.     

Special Pharmacokinetic Considerations in Children

Summary: Pediatric patients have greater degrees of pharmacokinetic variability and unpredictability than adults. This variability results from the effects of pharmacogenetics, age and growth, prior and current comedication, and disease. Newborns with seizures have the least predictable dosage requirements, and their needs change as drug-eliminating mechanisms mature in the neonatal period. Infants have the highest relative capacities to eliminate antiepileptics of any age group and require the largest relative doses. In addition to age-related trends, children demonstrate the same drug-specific, pharmacokinetic phenomena that adults do, including nonlinear phenytoin elimination, nonlinear valproate binding, and autoinduction of carbamazepine. Intercurrent illness and drug interactions further modify the age-related pharmacokinetic patterns in children and make dosage requirements even more unpredictable. Recent studies have shown that febrile illness can affect drug elimination, sometimes decreasing drug levels by 50% or more. Intermittent treatment with benzodiazepines administered either orally or rectally can be an important adjunct and help minimize this type of problem for children with marginally controlled epilepsy. Intermittent benzodiazepines are also helpful for children who have febrile seizures and who need only occasional antiepileptic protection.