糖尿病合并慢性炎症性脱髓鞘性多发性神经病-4例临床分析并文献回顾

目的探讨糖尿病(diabetic mellitus,DM)合并慢性炎性脱髓鞘性多发性神经病(chronic inflammatory demyelinating polyneuropathy,CIDP)的临床、电生理特点,并与糖尿病周围神经病(diabetic peripheral neuropathy,DPN)进行早期鉴别诊断。方法回顾性分析4例DM合并CIDP患者的临床表现、电生理检查及诊疗特征。结果 4例DM合并CIDP患者中,1例仅表现为对称性肢体乏力,其余3例均伴有对称性的麻木或疼痛,仅1例患者伴有颅神经损害;4例患者均存在腱反射均减弱或消失,病程均超过2个月,且均有脑脊液蛋白-细胞分离现象;4例患者肌电图检查均提示脱髓鞘病变为主,使用激素冲击治疗后症状均好转,其中2例复发患者分别采用丙种球蛋白和血浆置换术治疗后症状好转,4例患者目前均恢复良好。结论当糖尿病患者出现周围神经病变时,早期根据其临床特征及辅助检查,诊断其是否合并CIDP,并对DM合并CIDP患者合理使用免疫抑制治疗效果良好。     

不同透析方式治疗尿毒症性周围神经病变的疗效观察

目的探讨血液透析滤过(HDF)血液灌流(HP)和血液透析(HD)对尿毒症性周围神经病变的治疗作用。方法将我院及漯河市中心医院的40例合并尿毒症性周围神经病变的血液透析患者随机分为血液透析滤过组、血液灌流组和常规血液透析组。血液透析滤过组常规血液透析,2次/周,同时接受血液透析滤过治疗,1次/周;血液灌流组常规血液透析,2次/周,同时结合HD+HP治疗,1次/周;常规血液透析组行常规HD治疗,3次/周,对比每次治疗前后血清β2-MG浓度,治疗前、治疗2个月时分别观察并记录患者的临床症状,评价其疗效。结果 3组经过2个月的充分透析后,常规血液透析组患者周围神经病变的临床症状及血清β2-MG浓度无明显改善,而血液灌流组、血液透析滤过组周围神经病变的临床症状和血清β2-MG浓度较治疗前明显改善,程度显著优于血液透析组(P0.05)。结论尿毒症性周围神经病变患者在常规HD治疗同时接受HP或HDF治疗能有效地改善周围神经病变。     

多发性硬化伴发作性瘙痒20例临床分析

目的 探讨多发性硬化(MS)伴发作性瘙痒的临床特点.方法 回顾性分析20例MS伴发作性瘙痒患者的临床资料.结果 本组19例(95.0%)患者为女性,12例(60.0%)发作性瘙痒出现在首次病程,3例为MS的首发症状.瘙痒以头颈为主,突发突止,持续时间短,反复发作,瘙痒区域均伴有其他感觉障碍.MRI显示病灶位于延髓10例(头颅MRI检查15例)、颈髓11例(脊髓MRI检查14例).卡马西平等治疗后发作性瘙痒消失.结论 MS伴发作性瘙痒多为女性,发作性瘙痒有共同的临床特征,可能与延髓、颈髓病变有关,卡马西平治疗有效.     

13例淋巴瘤合并周围神经病变临床分析

目的 对淋巴瘤合并周围神经病变的临床特点、辅助检查结果进行分析,并探讨其发病机制.方法 回顾分析13例淋巴瘤合并周围神经病变患者诊断、治疗经过,排除继发性带状疱疹病毒感染、相关治疗的不良反应及其他导致周围神经病变的病因.其中T细胞性非霍奇金淋巴瘤(NHL-T)3例,B细胞性非霍奇金淋巴瘤(NHL-B)9例,1例具体病理类型不详.对其周围神经受累的类型和部位、脑脊液细胞学和免疫组织化学、影像学、电生理学以及腓肠神经活体组织病理学检查结果等进行分析.结果 13例中10例周围神经系统症状出现于淋巴瘤明确诊断之前,其中8例以周围神经系统症状首发:11例脑神经受累,8例腰骶神经根受累,6例同时合并听力减退及腰骶神经根受累.脑脊液检查主要呈现蛋白定量(13例)、白细胞计数(8例)以及脑脊液压力(5例)升高,葡萄糖水平降低(7例);细胞学检查可见异型淋巴细胞(9例);免疫组织化学染色及基因重排检测主要表现为B细胞标记抗原(CD19、CD20、CD79α)阳性(3例),CD20阳性细胞数日增加(1例),以及CD4阳性细胞数目增加、T细胞(抗原识别)受体阳性和抗体IgH阴性(1例).肌电图检查显示,周围神经轴索病变或合并髓鞘损害(9例).腓肠神经活体组织病理学检查呈轴索变性和(或)脱髓鞘病变且无淋巴瘤细胞浸润(3例).头部及腰骶部MRI检查显示,双侧听神经及马尾神经根增粗、强化或脑膜强化.13例中9例系由淋巴瘤细胞直接浸润脑脊膜神经根所致,1例为淋巴瘤转移和局部肿大淋巴结压迫引起,其余3例无明确肿瘤细胞浸润证据,结合临床特点和实验室检查结果,考虑副肿瘤综合征可能.结论 淋巴瘤可通过肿瘤细胞直接浸润或副肿瘤综合征导致周围神经病变;周围神经系统症状在淋巴瘤明确诊断前即可出现,临床应对此提高认识.     

非下丘脑性发笑性癫痫发作的定位定侧分析

目的 分析发笑性癫痫发作患者的临床特点,以确定癫痫病灶位置,为手术治疗提供参考.方法 回顾性分析本院就诊10例发笑癫痫发作患者的临床特点,包括发作的特点、影像学的特征以及手术治疗结果等.结果 10例患者均接受手术治疗,其中8例完成分期颅内电极埋置.术后8例达到Engle Ⅰ级缓解,1为Ⅱ级,另外1例术后发作无明显改变.结论 非下丘脑病损所致发笑样癫痫发作者若不伴有情感症状出现,病灶多在额叶,并以辅助运动区为常见,伴有情感症状者多定位于颞叶.同时,发笑症状在发作过程的早期或者单独出现者定位很可能位于右侧大脑半球,反之,在晚期出现应考虑在左侧大脑半球的定位.     

8例家族性皮质肌阵挛震颤性癫痫临床特点分析

目的分析家族性皮质肌阵挛震颤性癫痫(FCMTE)的临床特点。方法对8例FCMTE患者的临床资料进行回顾性分析,总结家系的临床特点、遗传特征。结果 8例FCMTE患者,连续3代发病,男女均受累,均30岁以后起病,先后出现震颤、全面强直-阵挛发作。其中,5例伴头痛,6例有肢体震颤,4例有情绪焦虑,1例有共济失调症状。8例均于30岁以后癫痫发作,呈强直-阵挛发作。4例刺激左右正中神经记录的躯体感觉诱发电位(SEPs)可见巨大电位,未见C-反射。结论 FCMTE呈常染色体显性遗传,均发生于成人,表现为四肢末端细微震颤、强直-阵挛性癫痫发作,光刺激、情绪激动或惊吓时可诱发。服抗癫痫药有效,服用β受体阻滞剂或饮酒无效,为非进展性病程。神经电生理检查提示肌阵挛或震颤来源于大脑皮质。     

糖尿病性神经病变的临床分析

糖尿病合并神经病变 (ＤＰＮ) ,包括周围神经、脑神经、植物神经病变 ,是糖尿病患者最常见的并发症。本文对 64例糖尿病性神经病变患者的年龄、性别、病程、血糖、ＨＢＡ1ｃ、晨尿微量白蛋白等进行相关性分析 ,以探讨糖尿病合并ＤＰＮ的一些临床特点。1　临床资料1 1　一般资料　本文共统计 40 5例糖尿病住院患者 ,所有病人诊断均符合 1994年标准 ,其中 64例合并ＤＰＮ ,发病率 15 8%。1 2　神经病变的诊断〔1〕　 (1)出现四肢远端 (下肢居多 )感觉、运动神经病变表现 :蚁爬感、麻木、烧灼感、刺痛、撕裂样疼痛 ,行走有踩棉花感 ,肌无力 ,…     

腓骨肌萎缩症2型的临床特点研究

目的研究腓骨肌萎缩症2型(CMT2)的临床特点。方法回顾性分析9例CMT2患者的临床资料。结果本组男5例,女4例;平均发病年龄21.7岁。平均神经病残疾评分32.6分,平均CMT评分13.3分。均为隐匿性起病,缓慢进展,主要表现为慢性对称性四肢远端肌无力和肌萎缩,感觉症状轻微。1例患者伴有限制性心肌病,临床罕见。9例患者神经电生理检查示运动、感觉神经传导动作电位波幅降低或消失,1例患者伴神经传导速度减慢(<38 m/s),周围神经病变严重。4例腓肠神经及腓短肌肌肉病理学检查符合CMT2典型神经肌肉病理表现。MFN2基因突变分析发现,1例MFN2基因第18外显子发生L710P突变。结论 CMT2的主要临床特点为对称性四肢远端肌无力和肌萎缩,周围神经损害严重但功能相对良好。限制性心肌病可能是该病的表现型之一。MFN2基因L710P突变可能为CMT2致病基因的突变型之一。     

肌电图肌源性损害合并神经源性损害71例临床分析

目的分析和探讨肌电图(EMG)肌源性损害合并神经源性损害的临床和电生理特点。方法检索作者医院EMG数据库,收集EMG表现为肌源性损害合并神经源性损害患者共71例,对其临床和电生理特点进行回顾性分析。结果在此组患者中,最常见的是结缔组织病,共63例(占88.7%)。其余依次为:肿瘤、进行性肌营养不良、AIDS、甲状腺功能亢进(甲亢)。在全部患者中,临床表现为近端无力者55例(占77.5%),有感觉症状体征者27例(占38.0%),肌肉萎缩者12例(占16.9%)。患者的三角肌52例(占73.2%)、股四头肌49例(占69.0%)EMG表现为肌源性损害,其中上下肢同时为肌源性损害者32例(占45.1%)。所合并存在的神经源性损害主要表现为多发性周围神经病(39例)、单神经病(27例)、颈或腰骶神经根病(5例)。结论EMG检查有助于检出临床下神经肌肉病变,电生理检查发现肌源性合并神经源性损害的同时分析肌病所合并周围神经病变类型将有助于揭示病变性质。     

Churge-Strauss综合征合并白细胞碎裂性血管炎的临床及病理学特点(附1例报告)

目的 探讨Churge-Strauss综合征(CSS)合并白细胞碎裂性血管炎的临床及病理学特点.方法 回顾性分析1例CSS合并白细胞碎裂性血管炎患者的临床资料.结果 本例患者以周围神经损害症状为主,病变侵犯皮肤、胃肠道、肺、中枢神经等多个系统;外周血嗜酸性粒细胞增高.病理学检查见皮肤组织和血管壁大量炎性细胞浸润、小血管壁周围多发性肉芽肿形成以及节段性纤维素样坏死性血管炎;最终因多器官功能衰竭死亡.结论 CSS合并白细胞碎裂性血管炎的临床表现以血管炎和周围神经病变为主;病理学改变为皮肤组织和血管炎性细胞浸润;后期病变侵犯多系统,预后差.     

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.     

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.     

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.     

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.     

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.     

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.