非酒精中毒性亚急性原发性胼胝体变性1例报道并文献复习

原发性胼胝体变性是一种罕见的以胼胝体受累为主的脱髓鞘和继发坏死的病变，其病因及发病机制尚不明确。该病主要见于中年男性，通常与长期大量酗酒或营养不良相关，其缺乏特异的诊断标准，容易漏诊及误诊。该文报道了1例非酒精中毒引起的亚急性原发性胼胝体变性患者。该例患者亚急性起病，有糖尿病病史，无饮酒史，主要临床表现为肢体乏力、进行性认知功能下降、行为异常及轻度意识障碍。颅脑磁共振成像示：胼胝体呈膨胀性改变，其内可见多发片状异常信号灶；液体抑制反转恢复序列呈高信号，弥散加权成像序列呈高信号，表观弥散系数序列呈低信号；以上病灶呈双侧对称性分布，累及胼胝体膝部、体部及压部。在给予补充大剂量维生素B₁、维生素B₁₂，控制血糖后，患者症状明显好转，神志清醒，言语较前流利，认知功能较前明显好转，肢体肌力基本正常。复查颅脑磁共振成像示：胼胝体对称性异常信号灶较前明显减少。该文旨在通过报道1例非酒精中毒性原发性胼胝体变性的病例，并复习该类疾病相关的国内外文献，探讨MBD的病因、发病机制、影像学表现、临床特点、诊断、鉴别诊断及治疗方法的研究进展。     

胼胝体变性六例影像学特点和临床表现

目的 探讨胼胝体变性(Marchiafava-Bignami disease,MBD)的影像学特点和临床表现.方法 对我院诊治的6例MBD患者进行回顾性分析,包括影像学检查(如头颅CT、MRI)、实验室检查、临床症状和随访情况.结果 6例患者MRI检查均表现为胼胝体肿胀及T1WI等或低信号,T2WI高信号,液体衰减反转恢复序列高信号,弥散加权成像示弥散受限.其中例1、例4和例6以急性意识障碍起病,CT和MRI检查除累及整个胼胝体外双侧半卵圆区也受累,预后较差.例2和例3以反应迟钝、记忆力下降起病,CT和MRI检查病变主要累及胼胝体膝部和压部,经积极治疗,患者基本恢复正常.例5以双下肢麻木无力、反应迟钝起病,MRI检查病变累及胼胝体压部及体部,经治疗后也恢复正常.结论 MBD临床表现复杂,但具有特征性影像学表现,其起病形式和影像学特征可作为反映其预后的重要因素之一.     

貌似椎管脓肿的白血病1例报告

1临床资料患者男,18岁,以反复发作性腰背部放射痛伴双下肢疼痛无力1月就诊。入院前2d高热,尿潴留伴便秘。体检:T40.6℃,神清,贫血貌,双耳听力差。膀胱充盈,腰4、5棘突压痛(+),双下肢深浅感觉存在,双侧直腿抬高试验(++),以右下肢为重。左下肢肌力Ⅳ级,右下肢肌力Ⅱ级。双侧腹股沟均可触及蚕豆大小淋巴结,质硬无压痛。肛门指诊骶前壁隆起欠光滑,无压痛及波动感。CT示L5～S1椎管内占位,颅脑MRI示中脑及桥脑占位性病变,脊髓MRI矢状位见L5～S1平面结节状病灶,T1WI呈等信号,T2WI呈稍高信号,边界清楚;骶骨前直肠后间隙内可见梭形肿块,T1WI…     

原发性脑淋巴瘤CT与MRI诊断

目的分析原发性脑淋巴瘤的CT和MRI表现,以提高对其的认识,减少误诊。方法回顾性分析17例经手术病理证实的原发性脑淋巴瘤的CT和MRI资料。结果CT和MRI主要表现为：（1）CT平扫示脑内单发或多发的等密度或较高密度瘤灶,肿瘤可沿脑脊液播散;MRI T1WI呈等或稍低信号,T2WI呈等或稍高信号;1例出现坏死及出血;②少部分病例瘤周水肿及占位效应轻至中度;（3）MRI增强扫描大多呈团块状均匀强化;1例环形强化。④除病变主体位于胼胝体膝部和压部外,瘤灶长轴多与颅脑前后径走行平行或大致相同。结论原发性脑淋巴瘤多在大脑深部,明显强化,CT和MRI表现具有一定的特异性,结合CT与MRI影像特点和临床资料综合分析,有可能在术前作出正确诊断。     

妊娠相关性脑静脉及静脉窦血栓形成和可逆性后部白质脑病的磁共振鉴别

目的探讨MRI和MRV在妊娠相关性脑静脉及静脉窦血栓形成(CVST)和可逆性后部白质脑病综合征(RPLS)鉴别诊断中的价值。方法回顾性分析3例妊娠相关性CVST和6例重度子痫前期、子痫发生RPLS患者的影像学资料。所有患者均行MRI和DSA检查,其中7例患者行MRV检查。结果 3例CVST患者中,1例孤立性大脑上静脉前组血栓形成,MRI表现为相应部位T1WI低、等信号,T2WI、FLAIR等、稍高信号,DWI为等、高信号,ADC图为低、稍高信号;2例横窦血栓形成,表现为双侧枕叶皮质、皮质下白质片状T1WI低信号,T2WI、FLAIR高信号,DWI、ADC高信号,可不对称性地累及顶叶、小脑半球,MRV与DSA检查结果相符。6例RPLS患者MRI显示双侧顶、枕叶皮质、皮质下白质多发性、斑片状、对称性病变,部分严重患者大脑半球呈弥漫性、大片状受累,表现为T1WI低信号,T2WI、FLAIR高信号,DWI、ADC高信号;1例患者MRV检查示左侧横窦未显影,DSA检查示左侧横窦通畅。结论横窦血栓形成和RPLS患者MRI均显示血管源性脑水肿,两者脑水肿主要发生于双侧枕、顶叶,但前者范围较局限,后者范围更广泛,可累及基底节、额叶、颞叶。RPLS患者MRV检查可有假阳性,DSA是鉴别两者的重要检查方法。     

中枢神经系统淋巴瘤二例

目的探讨中枢神经系统淋巴瘤的临床、脑脊液细胞学、影像学和病理学。方法对两例中枢神经系统淋巴瘤患者进行腰椎穿刺术,行脑脊液测压、常规、生化、细胞学检查及磁共振(MRI)扫描。结果例1为原发性中枢神经系统非霍奇金淋巴瘤脑病变,例2为继发性中枢神经系统非霍奇金淋巴瘤脊髓病变。例1患者临床表现主要为肢体无力、记忆力下降及脑神经损伤。脑脊液压力125mm H2O,氯化物130.0mmol·L-1,葡萄糖4.97 mmol·L-1,蛋白0.46g·L-1。头颅MRI T1WI呈低信号,T2WI呈高信号,增强后强化。病理切片可见少量小圆形肿瘤细胞,免疫组化显示为CD20阳性。例2患者临床主要表现为肢体无力,脑脊液压力180mm H2O,氯化物124.6mmol·L-1,葡萄糖3.77 mmol·L-1,蛋白1.73g·L-1。椎体MRI示T1WI低信号,T2WI呈高信号,增强后均匀强化。病理诊断为弥漫性大B细胞淋巴瘤。结论中枢神经系统淋巴瘤的临床表现和其侵犯部位有关,脑脊液中可找到肿瘤细胞,MRI表现为低T1WI信号,高T2WI信号,增强后强化。病理多显示为B细胞来源的淋巴瘤。     

颅咽管瘤非典型MRI表现

目的探讨颅咽管瘤非典型MRI表现,以提高对此病的认识。方法回顾性分析4例经手术病理证实的颅咽管瘤的MRI资料。结果4例肿瘤均为囊性:例1肿瘤位于鞍上,瘤内可见脂液平面,脑室、脑池及脑沟内可见大小不等的脂肪小滴;例2肿瘤的大部分位于桥小脑角区,仅小部分位于鞍上,T1WI和T2WI均为高信号,脂肪抑制亦为高信号;例3肿瘤位于鞍上,突向鞍内并延伸至胼胝体压部下方,蝶鞍稍增大,垂体未显示,T1WI和T2WI均为高信号;例4肿瘤位于鞍内及鞍上,蝶鞍扩大,垂体未显示,肿瘤前部T1WI和T2WI均为高信号,后部T1WI信号稍高,T2WI与白质信号相同。结论颅咽管瘤存在方式多种多样,有特征性的MRI表现,也可有非典型MRI表现,肿瘤可向邻近部位延伸,亦可破裂播散至蛛网膜下腔内。囊性颅咽管瘤的信号特点与囊液的成分有关。     

可逆性胼胝体压部病变综合征四例分析

目的总结可逆性胼胝体压部病变综合征的临床表现和影像学特点,探讨其病因和发病机制。方法回顾分析4例可逆性胼胝体压部病变综合征患者的临床表现、影像学特点和病因。结果4例患者中2例(例1和例2)有颅脑创伤史,1例(例3)于饮酒后出现发作性双眼视物模糊,1例(例4)于感冒后出现头痛、晕厥,伴间断性视物模糊和慢性肾功能衰竭;MRI表现为胼胝体压部孤立性圆形或类圆形、边界清晰的病变,呈T_1WI等或稍低信号,T_2WI、扩散加权成像和FLAIR成像高信号,表观扩散系数低信号,无明显水肿和占位效应,1例(例2)除胼胝体压部病变外,磁敏感加权成像可见额叶出血。均经对因和对症支持治疗后症状完全缓解,病程8~15 d。复查MRI,3例胼胝体异常信号消失,1例(例2)病变信号范围和强度较前明显好转,均未遗留神经功能障碍。结论可逆性胼胝体压部病变综合征是一种临床影像学综合征,病因多样,癫及其相关疾病是最常见病因,颅脑创伤也可以引起可逆性胼胝体压部病变综合征,临床表现无特异性,经对因治疗后预后良好。     

获得性肝脑变性二例

目的探讨获得性肝脑变性(AHCD)患者的临床及MRI特点。方法报道2例AHCD患者的临床及MRI资料,结合文献进行分析。结果 2例患者均有肝硬化病史,临床上例1表现为反复发生的主观性眩晕、精神行为异常,例2表现为头晕/眩晕,2例患者均未见运动障碍、小脑性共济失调等症状。本组2例患者MRI上均显示双侧基底核区、大脑脚对称性的T_1WI高信号。其中例1双侧内囊、大脑脚T_2Wl/FLAIR、DWI/ADC也呈稍高信号;例2双侧大脑脚DWI呈稍高信号,相应区域ADC相呈等信号,同时可见额叶、颞叶、顶叶、海马萎缩。结论 AHCD是一类与慢性肝病相关的神经系统损害综合征,临床表现复杂多样,其MRI特征表现为双侧基底核区、大脑脚对称性T_1WI高信号,可见部分患者DWI上亦呈高信号。     

MRI表现酷似颅内感染的多灶性脑梗死1例报告

目的:报道1例头颅MRI表现酷似颅内感染的多灶性脑梗死。方法:患者行头颅MRI检查,脑脊液等检测,脑标本行多部位病理检查。结果:MRI:两侧额叶及胼胝体膝部、体部多发片状异常信号,T1WI稍低信号,T2WI稍高信号,FLAIR高信号,DWI稍高信号,增强后病灶环形强化。病理诊断:脑动脉重度粥样硬化伴大脑顶叶、基底节、胼胝体及脑桥多发梗死伴出血灶形成。结论:多灶性脑梗死头颅MRI增强亦可出现酷似颅内感染的多灶性环型增强。     

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.