肌萎缩侧索硬化症的磁共振弥散张量成像研究

目的初步探讨磁共振弥散张量成像(DTI)在肌萎缩侧索硬化(ALS)诊断中的应用价值。方法选取54例具有不同诊断级别的ALS患者,包括确诊(21例)、很可能(22例)、可能(11例)的患者,同时选取23名健康体检的志愿者作为健康对照。分别行DTI扫描,测量各感兴趣区(ROI)的平均扩散系数(ADC)和各向异性系数(FA),分析两组间ADC和FA的差异,并对ALS组患者ADC值及FA值与临床资料的相关性进行分析。结果与健康对照组比较,ALS患者大脑脚、内囊后肢区的ADC值升高(P0.01),大脑脚、内囊后肢及中央前回区的FA值降低(P0.01);相关分析显示ALS患者内囊后肢的ADC值与诊断级别呈负相关(r=-0.289,P0.05),与上运动神经元损伤评分呈正相关(r=0.304,P0.05);大脑脚及内囊后肢的FA值与诊断级别呈正相关(r=0.394,P0.01;r=0.547,P0.01),与上运动神经元损伤评分呈负相关(r=-0.301,P0.05;r=-0.653,P0.01).结论 DTI可客观定量评估ALS患者锥体束的损伤情况,在ALS的诊断中具有重要的辅助价值。     

运动神经元疾病患者颈髓弥散张量成像研究

目的 研究运动神经元疾病颈髓弥散张量成像(DTI)特点,探讨DTI在运动神经元疾病中的诊断价值.方法 选取临床诊断为肌萎缩侧索硬化(ALS)患者16例、肯尼迪病(KD)患者12例及健康志愿者15名.分别行颈髓常规MRI及横断面DTI检查,测定各组C3、C5椎体水平层面颈髓前索区、后索区及舣侧皮质脊髓侧束区4个区域内感兴趣区(ROI)的表观弥散系数(apparent diffusion coefficient,ADC)和部分各向异性比值(fractional anisotropy,FA),并分析影像学参数与其病程、ALSFRS-R评分、肺功能等之间的关系.结果 ALS 组患者在C3与C5水平,前索、双侧皮质脊髓侧束的FA值较KD组、健康对照组显著降低(P<0.05),而KD组与健康对照组之间差异无统计学意义(P>0.05).3组在各不同位置的ADC值之间差异无统计学意义(P>0.05).ALS组在C3水平右侧皮质脊髓束FA值与患者ALSFRS-R评分呈正相关(r=0.52,P=0.041);各水平前索、双侧皮质脊髓束的FA值、ADC值与患者病程、肺功能之间无相关性.结论 FA值可反映颈髓皮质脊髓束的功能异常,可用于评估ALS患者上运动神经元的损伤程度,有利于运动神经元疾病的鉴别诊断.     

肌萎缩侧索硬化症患者非锥体束的磁共振扩散张量成像研究

目的 研究肌萎缩侧索硬化症(ALS)患者非锥体束区域磁共振扩散张量成像(DTI)的特点.方法 选择ALS患者(病例组)21例及健康志愿者(健康对照组)20名,行轴位MRDTI扫描,选取感兴趣区(ROI),测量部分各向异性(FA)和平均扩散系数(MD).结果 病例组胼胝体压部(0.73±0.087)(u=-2.004,P<0.05)、丘脑(0.29±0.020)(u=-3.580,P<0.01)的FA值与健康对照组(0.79±0.034,0.32±0.010)比较,明显减低;两组间各ROI水平MD的差异无统计学意义.结论 除中枢运动传导系统外,ALS患者的胼胝体压部、丘脑亦被累及,ALS更有可能是一种以运动系统损害为主的多系统变性疾病.     

肌萎缩侧索硬化38例弥散张量成像及磁共振波谱表现

目的 研究肌萎缩侧索硬化(ALS)的弥散张量成像(DTI)和磁共振波谱(MRS)特点,并对ALs的病理生理机制进行初步探讨.方法 ALS患者38例,单纯下运动神经元受累疾病患者8例,混合型颈椎病5例和健康对照34名,行常规头颅MRI、DTI和1H-MRS测定.结果 21%(8/38)的ALS患者可见T2 FLAIR序列双侧锥体束走行异常高信号,强度高于皮质灰质.ALS组和健康对照组相比,中央前回(ALS组0.492±0.059,健康对照组0.552 4-0.045,F=17.150,P<0.01)、内囊后肢(分别为0.679 ±0.048、0.727±0.031,F=19.481,P<0.01)、大脑脚(左侧0.740±0.038、0.761±0.024,F=4.290;右侧0.720±0.044、0.746±0.034,F=3.264,均P<0.01)的部分各向异性(FA值)显著降低;ALS组上述部位的N-乙酰大冬氨酸/肌酸(NAA/Cr)值和健康对照组相比亦存在显著降低.AKS组各部位FA值降低百分率分别为10.9%、6.6%、2.8%～3.5%,以中央前回处最为显著;内囊前肢、枕叶处的FA值亦有不同程度降低.结论 DTI和MRS枪测不仪有助于ALS患者:运动神经元高位受累的确认和ALS的诊断和鉴别诊断,而且还可促进对ALS病理机制和病损分布的了解.     

磁共振弥散张量成像对阿尔茨海默病脑白质损害的评估及鉴别价值

目的：应用MR弥散张量成像（DTI）观察阿尔茨海默病（AD）患者脑白质纤维束完整性。方法：健康老年志愿者为对照组（NC）组、遗忘型轻度认知障碍（aMCI）组、AD组和皮质下缺血性血管性痴呆（SIVD）组各20例,行常规MRI和DTI扫描后,测定相同感兴趣区（ROI）的各向异性分数（FA）值和表观扩散系数（ADC）值进行比较。结果：与NC组比较, AD组前额叶、颞叶、海马等部位FA值降低,颞叶、海马等部位ADC值升高（P〈0.05）;aMCI组仅扣带束FA值降低,与AD组比差异有统计学意义（P〈0.05）;SIVD组下额枕束等部位FA值下降,ADC值升高,与AD组比较差异有统计学意义（P〈0.05）。结论：DTI可以用来评估白质纤维束完整性,AD组白质损害甚于aMCI患者;扣带束FA值可以作为aMCI患者筛查的指标;根据受累部位不同可对SIVD与AD进行鉴别。     

颈髓弥散张量成像在肌萎缩性侧索硬化诊断中的应用

目的 初步探讨颈髓弥散张量成像(DTI)在肌萎缩性侧索硬化(ALS)诊断中的应用价值. 方法 选择自2000年1月至2007年1月中山大学附属第二医院骨科收治的28例ALS患者为患者组,20例同期门诊查体健康成年人为对照组,对2组成员进行常规MRI扫描及DTI检查,获取颈髓MD值及FA值的直方图,并对ALS患者颈髓DTI弥散张量值与患者ALS残损功能评分量表(ALSFRS)评分进行相关性分析. 结果 与对照组相比,患者组颈髓FA值和颈髓横断面积明显降低,差异有统计学意义(P<0.05);MD值轻微增加,差异无统计学意义(P>0.05).患者颈髓FA值与ALSFRS评分高度相关(r=0.730,P=0.000),与MD值等指标无相关关系. 结论 ALS患者颈髓DTI显像FA值显著降低,FA值可能成为ALS诊断中的神经影像学阳性支持指标:颈髓的弥散张量值与ALSFRS结合,可以更伞面反映ALS患者的病情进展.     

高血压病合并代谢异常患者脑磁共振波谱及弥散张量成像研究

目的 应用磁共振波谱（Magnetic Resonance Spectroscopy,MRS）及磁共振弥散张量成像（Diffusion Tensor Imaging,DTI）检测病程5年以上高血压病患者的脑内代谢物、脑部各向异性（fractional anisotropy,FA）值,以例早期发现亚临床脑细胞损害.方法 本研究分为病例组（高血压病12例）及对照组（17例健康体检者）,检查一般项目（身高、体重、血压、血糖、血脂、神经功能相关量表评分）,行头颅MRI、DWI、MRS、DTI检查,测定脑部背侧丘脑、扣带回后部、侧脑室后角NAA/Cr、Cho/Cr、NAA/Cho的比值及内囊后肢、放射冠区FA值.结果 （1）病例组NAA/Cr在背侧丘脑、扣带回后部、侧脑室后角均低于对照组（分别t=-2.405、-3.710、-2.068,P＜0.05）;NAA/Cho在扣带回后部、侧脑室后角均低于对照组（分别t=-4.347、3.578,P＜0.01）;Cho/Cr在扣带回后部高于对照组（t=2.965,P＜0.01）;（2）FA值在内囊后肢、放射冠区都明显低于对照组（t=-2.543、P＜0.05;t=-3.394、P＜0.01）.结论 高血压病合并代谢异常且病程5年以上者脑细胞代谢功能及神经纤维微结构已有损害,MRS、DTI检测有助于早期发现亚临床脑细胞损害.     

儿童孤独症的脑扩散张量成像研究

目的 研究孤独症儿童脑扩散张量成像的异常.方法 对24例孤独症儿童(孤独症组)和10名正常儿童(正常对照组)进行脑扩散张量成像扫描,测量并比较两组的双侧胼胝体膝部、压部和双侧内囊后肢表观扩散系数(ADC值)和各向异性分数(FA值),分析孤独症儿童各被检测部位ADC值、FA值与孤独症治疗评估量表(ATEC)得分的相关性.结果 (1)孤独症组双侧内囊后肢[左右侧分别为(6.8±0.3)×10-9 mm2/s和(6.7±0.4)×10-9 mm2/s]、左侧胼胝体膝部[(8.0±0.5)×10-9 mm2/s]ADC值高于对照组[分别为(6.6±0.2)×10-9 mm2/s,(6.6±0.4)×10-9 mm2/s,(7.4±0.5)×10-9 mm2/s;P＜0.05～0.01];孤独症组右侧胼胝体压部(0.73±0.14)FA值低于正常对照组(0.84±0.07;P＜0.05),孤独症组右侧胼胝体膝部(0.75±0.07)FA值高于正常对照组(0.68±0.17;P＜0.05).(2)孤独症组右侧胼胝体压部ADC值与ATEC健康/行为评分呈正相关(r=0.490),右侧胼胝体压部FA值与ATEC说话/语言评分呈负相关(r=-0.453),右胼胝体膝部FA值与社交评分呈正相关(r=0.427),均P＜0.05.(3)孤独症组内囊后肢、胼胝体膝部FA值左右侧的差异有统计学意义(P＜0.05).结论 孤独症儿童胼胝体神经纤维连接存在异常.     

磁共振弥散张量成像在帕金森病诊断与定量分析中的应用价值

目的探讨磁共振弥散张量成像(DTI)在帕金森病(PD)诊断与定量分析中的应用价值。方法选取2017年12月-2019年10月我院收治的PD患者45例作为观察组,同期健康体检者39例作为对照组。两组均接受DTI检查,测量各向异性指数(FA)及表观扩散系数(ADC)值,进行统计学分析。结果观察组丘脑、黑质FA值均低于对照组,差异有统计学意义(P 0. 05)。两组丘脑、黑质、脑桥、小脑上脚及中脚ADC值比较差异均无统计学意义(P 0. 05)。ROC曲线分析显示:丘脑FA值、黑质FA值诊断PD的AUC分别为0. 704、0. 798(均P 0. 05)。Hoehn-Yahr中期PD患者黑质FA值低于早期,丘脑ADC值高于早期,差异有统计学意义(P 0. 05)。Pearson相关性分析显示:黑质FA值与PD患者Hoehn-Yahr分期呈负相关(r=-0. 457,P=0. 002);丘脑ADC值与PD患者Hoehn-Yahr分期呈正相关(r=0. 405,P=0. 006)。结论 DTI对PD具有定量诊断价值,且黑质FA值、丘脑ADC值与PD病情具有相关性。     

DTI评价轻度认知障碍及轻中度阿尔茨海默病脑白质微细结构损害的研究

目的应用磁共振弥散张量成像(DTI)技术研究轻度认知障碍(MCI)及轻中度阿尔茨海默病(AD)患者脑白质微细结构的改变。方法对MCI患者、轻中度AD患者各12例及健康老年人12名(对照组)行常规MRI及DTI检查,测量其胼胝体压部、额叶、顶叶、颞叶、枕叶、内囊前肢及内囊后肢白质区部分各向异性分数(FA)和平均弥散率(MD)。将3组的FA、MD值进行比较,并与MMSE评分、单词回忆及单词再认评分进行相关性分析。结果 (1)MCI患者顶叶白质FA值为0.489±0.079,与对照组(0.558±0.079)相比下降(P0.05)。(2)AD患者额叶、顶叶及颞叶FA值分别为0.405±0.072、0.454±0.069和0.363±0.056,与对照组(分别为0.499±0.081、0.558±0.079和0.440±0.061)比较差异均有统计学意义(P0.05)。AD患者胼胝体压部、额叶及顶叶MD值分别为0.978±0.082、0.920±0.054和0.81 7±0.045,均高于对照组(分别为0.801±0.093、0.820±0.084、0.712±0.096)(P0.05)。AD、MCI两组内囊前、后肢及枕叶FA及MD值分别与健康对照组比较均无统计学差异(P0.05)。(3)3组顶叶、颞叶FA值与MMSE、单词回忆及单词再认评分均有相关性(分别r=0.869、-0.621、-0.759,均P0.01;r=0.446、-0.486、-0.361,均P0.05),胼胝体压部FA值与单词再认评分有相关性(r=-0.343,P0.05);3组胼胝体压部及顶叶MD值与MMSE、单词回忆及单词再认评分均有相关性(分别r=-0.612、0.547、0.586,均P0.01;r=-0.576、0.499、0.519,均P0.01),内囊前肢MD值与MMSE评分相关(r=-0.340,P0.05)。结论 MCI及轻中度AD患者存在脑白质选择性微细结构损害,且该损害出现在与高级皮层功能相关的脑区,而与初级功能相关的区域未见明显受损。     

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.