肥大性橄榄核变性的研究进展

肥大性橄榄核变性(hypertrophic olivary degeneration,HOD)又称下橄榄核肥大(inferior olivary hypertrophy,IOH),是Guillian-Mollaret解剖三角病变所致的跨突触神经元变性,头部磁共振呈特征性下橄榄核T2WI高信号、T1WI等或低信号改变,下橄榄核体积常增大,临床上以腭肌震颤(symptomatic patal tremor,SPT)、眼震为特征性表现.HOD较为少见,多被临床医生所忽视,现将其进展综述如下.     

磁共振扩散张量技术在肥大性下橄榄核变性诊断中的应用(1例报道及文献复习)

目的报道1例继发于脑桥出血的肥大性下橄榄核变性病例并复习相关文献,论证头颅磁共振扩散张量成像(MR DTI)和弥散张量纤维束成像(DTT)技术用于肥大性下橄榄核变性诊断的临床价值和意义。方法收集1例患有眼肌阵挛和步态异常的男性39岁患者的临床资料。该患者分别接受MRI和DTI检查。结果 MRI显示其延髓右前外侧肥大结节灶,呈T1WI等信号、T2WI高信号、DWI高信号。MR DTI和DTT显示:(1)ADC值右侧大于左侧;(2)FA值右侧小于左侧;(3)右侧神经纤维束相对左侧减少变薄并部分呈现中断现象。结论 MR DTI和DTT在诊断肥大性下橄榄核变性时一定程度上优于常规MRI,影像学的发展提高了肥大性下橄榄核变性的诊断和鉴别诊断水平。     

脑桥出血后继发肥大性下橄榄核变性的临床及影像学特点

目的探讨脑桥出血后继发肥大性下橄榄核变性症(hypertrophic inferior olivary degenerationsyndrome,HOD)的临床及影像学特点。方法对2009年1月至2011年6月在我院确诊的脑桥出血的65例,其中63例患者进行为期1年的随访,并对随访过程中发现有继发性下橄榄核变性症的9例患者的临床资料进行分析。结果HOD发生影像学改变时仅有3例患者有临床症状,主要表现为眼震、软腭阵挛、躯干粗大震颤。脑桥出血均发生在被盖区。头颅MRI下橄榄核信号改变距离出血时间平均为4.05个月。出血病灶9例中有8例存在含铁血红素沉着。肥大的橄榄核直径多数在0.7～0.8 cm左右,下橄榄核信号改变6例为等T1WI,长T2WI改变,3例为长T1WI长T2WI改变,其中2例长T1WI患者出现典型临床症状。7例Flair显像为高信号,1例低信号,1例为等信号。治疗上,1例患者先后予森福罗、氯硝西泮、心得安、氟哌啶醇、美多巴等治疗,其中氯硝西泮及心得安有效。结论HOD多发生在脑桥被盖部出血1个月后,头颅MRI特征为下橄榄核区的圆形的T2WI长信号改变;予心得安及氯硝西泮可能有效。     

中脑梗死继发双侧肥大性橄榄核变性

肥大性橄榄核变性(HOD)是一种罕见且特殊的跨突触变性,发病率低,易被忽略。主要继发于脑干和小脑的破坏性病变。临床典型表现为腭肌震颤和共济失调,影像学特征为T2WI高信号。现总结分析1例继发于中脑梗死的典型双侧HOD病例,以期提高临床工作中对该病的认识。     

脑干小脑卒中后肥大性下橄榄核变性5例临床特点

目的探讨肥大性下橄榄核变性(hypertrophic olivary degeneration,HOD)的病因、临床表现及影像学特点。方法回顾性分析2015年至今我院诊断为HOD的5例患者,对其相关资料进行整理分析。结果 5例患者存在齿状核-红核-下橄榄核环路受累病史,其中中脑1例,脑桥2例,小脑2例,包括梗死2例,出血3例。除原发脑血管病的后遗症状外,5例患者分别于1~8个月出现新的临床表现:软腭震颤(symptomatic palatal tremor,SPT)(2例),眼震(2例),复视(2例),头部(2例)、下颌(1例)、四肢(1例)或腹部(1例)阵挛,共济失调(1例)。头MRI示同侧、对侧或双侧下橄榄核T2WI呈高信号,其中伴下橄榄核体积增大4例。结论 齿状核-红核-下橄榄核环路受累可以继发HOD,从而出现SPT等临床表现,头MRI表现为下橄榄核T2WI高信号伴或不伴体积增大,是特征性的影像学表现。     

表现为痉挛性斜颈的肥大性下橄榄核变性1例报告

正肥大性橄榄核变性(hypertrophic olivary degeneration,HOD)是一种继发于齿状核-红核-下橄榄核环路区(GuillianMollare三角区)病变引起的跨突触变性,该病多继发于脑桥、中脑或小脑的出血、梗死、肿瘤或创伤后的一段时期~([1])。MRI显示延髓腹外侧下橄榄核区局限性T2WI高信号,伴或不伴橄榄核体积增大~([2])。既往认为软腭阵挛是HOD最核心     

脑桥被盖部出血继发肥大性下橄榄核变性二例

肥大性下橄榄核变性又称为肥大性橄榄核变性(hypertrophic olivary degeneration,HOD),多继发于脑桥、中脑或小脑的出血、梗死、肿瘤或创伤后的一段时期,MRI显示延髓腹外侧下橄榄核区局限性T2WI高信号,伴或不伴橄榄核体积增大.     

脑干海绵状血管瘤出血继发肥大性下橄榄核变性的随访观察

目的 探讨继发性肥大性下橄榄核变性与脑干海绵状血管瘤出血的关系及其形成机制。方法 回顾性分析2013年9月到2015年10月收治的12例脑干海绵状血管瘤出血的临床资料，随访至出血后18个月。结果 12例中，4例（33%）继发肥大性下橄榄核变性（3例双侧，1例单侧），表现为腭阵挛、小脑共济失调和眼部症状，典型临床症状出现在出血后5~11个月，出血后2~11个月MRI可见下橄榄核肥大。结论 继发性肥大性下橄榄核变性是脑干海绵状血管瘤出血后容易发生的迟发性并发症，常在出血后11个月内发生，建议MRI定期随访。     

脑桥出血继发肥大性下橄榄核变性的影像分析

目的分析脑桥出血继发肥大性下橄榄核变性(hypertrophic olivary degeneration,HOD)的MRI影像学特点,以期提高对HOD的诊断水平。方法回顾性分析6例脑干出血后继发HOD的MRI表现,均行MRI常规序列和DWI、SWI序列扫描。结果 MRI表现为同侧或双侧下橄榄核体积增大或无明显变化,T1WI呈等或稍低信号,T2WI呈稍高或高信号,FLAIR呈等或稍高信号,DWI呈等信号,ADC图呈稍高信号,SWI无异常信号,对出血病灶显示最好。结论 MRI能非常清楚地显示下橄榄核的变性改变,但需结合临床病史、症状与体征才能对HOD作出明确诊断。     

表现为四肢震颤的继发性肥大性下橄榄核变性1例报告

肥大性下橄榄核变性(hypertrophic olivary degeneration,HOD)是一种特殊的跨神经元突触变性,可以在脑出血、脑梗死、血管畸形、创伤、外科手术、中毒或者肿瘤之后发生[1],为继发于齿状核-红核-橄榄核神经通路(dentate-rubro-olivary pathway,DROP)受损后的迟发性病理学改变[2]。腭肌阵挛为HOD的典型临床表现,而表现为红核震颤的HOD报告极少,红核震颤常常累及单侧上肢,罕有HOD患者表现为四肢震颤。临床上该疾病较少见,本文报告了一例初期误诊为延髓梗死,后经我院确诊为继发于脑桥出血的肥大性下橄榄核变性患者,并复习整理有关文献,以期提高临床医师对该病的认识。     

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.