Marchiafava-Bignami病的临床及影像学研究

目的 探讨Marchiafava-Bignami病(MBD)的临床及影像学改变.方法 回顾性分析了7例MBD患者的临床和CT、MRI资料,包括病灶形态、分布、信号或密度改变等影像学特征:4例同时行CT和MRI检查,2例仅行CT检查,1例仅行MRJ检查.结果 本组患者急性型5例,均表现为胼胝体肿胀及长T1、长T2信号改变,均有双侧脑室周围白质、额叶皮层下白质对称性累及:慢性型2例,胼胝体明显萎缩变薄,并呈长T1、长T2信号及FLAIR像点片状或线样低信号灶.5例患者DWI显示病灶区信号明显增高并有2例出现弥散受限改变.结论 MBD具有特征性MRJ表现,其影像学改变可能反映其临床及预后.     

颅内静脉窦血栓形成2例临床及影像学分析

目的 探讨颅内静脉窦血栓形成(CVST)的临床表现、影像学特征及治疗.方法 分析2例颅内静脉窦血栓形成患者的临床资料,并复习文献.结果 2例患者临床表现差异很大,头部MRI检查示短T1、长T2信号,其中1例伴癫(癎)发作,经治疗后患者的临床表现及影像学均很快好转.结论 分析颅内静脉窦血栓形成的临床及影像学表现有助于该病的诊断和治疗.     

垂体后叶素致迟发性脑病的临床与影像学特征

目的探讨垂体后叶素致迟发性脑病的临床及影像学特征。方法对8例由垂体后叶素治疗引起的迟发性脑病患者的临床及影像学资料进行回顾性分析。结果8例患者在停用垂体后叶素后4～12d出现不同程度的神经精神症状,以肌张力增高(8例)、运动迟缓(6例)、肢体抖动(3例)等锥体外系症状和情感言行不协调(6例)为主要表现;8例脑电图检查,4例广泛轻-中度异常,1例广泛重度异常,均以各导联慢波为主;8例血钠检查5例轻度降低;8例头颅MRI检查,均表现为豆状核、尾状核头部对称性长T1、长T2异常信号,其中合并丘脑、中脑异常信号各1例。结论应用垂体后叶素引发的迟发性脑病临床表现以锥体外系症状和大脑功能障碍为主,影像学以豆状核、尾状核头部对称性长T1、长T2异常信号为特征。推测其发病机制可能为豆纹动脉痉挛和低钠血症所致的迟发性神经元坏死。     

表现为后循环缺血性病变的小脑幕硬脑膜动静脉瘘的临床和影像学特点(附3例报告)

目的 探讨表现为后循环缺血性病变的小脑幕硬脑膜动静脉瘘(DAVF)的临床和影像学特点.方法 回顾性分析3例表现为后循环缺血性病变的小脑幕DAVF患者的临床和影像学资料.结果 本组患者起病形式多样,均表现头晕、恶心、呕吐,病变侧共济失调、水平眼震;1例患者出现交叉性感觉障碍、轻度延髓麻痹和不完全Horner征;头颅MRI均示小脑病变,其中1例合并延髓病变,病灶处均出现长T1、长T2及弥散加权成像高信号;2例MRI增强见小脑病灶强化及多条弯曲血管影;3例头部数字减影血管造影(DSA)检查均发现小脑幕DAVF瘘口.结论 小脑幕DAVF可引起小脑及脑干缺血表现,MRI增强扫描可见病灶强化及异常血管影,DSA检查可明确诊断.     

MELAS综合征临床、影像、脑电图及基因特点分析

目的探讨MELAS综合征的临床症状、影像学和脑电图表现及基因突变特点,提高临床对疾病的认识。方法本研究共纳入MELAS综合征患者12例,收集其一般临床资料,分析其临床症状、颅脑影像学结果、脑电图表现及基因突变特点。结果 12例患者平均起病年龄28岁,临床主要表现为癫痫发作(66.7%)、卒中样发作(50.0%)、认知功能下降以及精神行为异常(41.7%)、糖尿病或糖耐量异常(41.7%)、听力损害(41.7%)等;影像学检查病灶呈长T1、长T2、DWI高信号改变,主要分布在枕、颞、顶叶,6例MRS检查中5例出现异常乳酸峰,8例脑电图检查主要表现为全脑弥漫性慢波或癫痫样放电,9例基因检测均为A3243G点杂合突变。结论MELAS综合征临床表现多样,颅脑影像学、脑电图检查特异性有限,MRS成像中异常乳酸峰可提示诊断,A3243G为最常见突变类型。     

酷似中毒性脑病的可逆性后部白质脑病综合征:6例临床分析

目的探讨可逆性后部白质脑病综合征(RPLS)的临床及影像学特点,为与中毒性脑病进行鉴别提供依据。方法回顾性分析6例RPLS患者的临床特点、影像学资料及治疗经过。结果 6例患者分别有大量饮酒史、海洛因吸食史、煤气中毒史、长期接触油漆涂料史、一氧化碳吸入史和有机溶剂接触史。临床主要表现为头痛、恶心、呕吐、癫痫发作以及血压升高。头部MRI主要表现为双侧颞叶、顶叶、额叶、侧脑室旁白质、小脑、脑干病灶,呈长T1、长T2信号,无强化。6例患者对症及去除病因治疗后临床表现及影像学表现均明显好转。结论 RPLS患者可以出现与中毒性脑病相似的临床及影像学特点,特别是当患者有毒物吸入史时,应根据该病相对特征性的临床、影像学及疗效尽早排除。     

慢性海洛因中毒性脑病

目的 :报道 3例 (男 2例 ,女 1例 )慢性海洛因中毒性脑病的临床和 MRI特征 ,并结合文献进行分析。 3例均有明确吸毒史 (吸毒时间 3～ 5年 ) ,表现为进行性智能障碍、言语障碍、肌张力障碍及共济失调等慢性脑损害的特点 ,1例 (女性 )发展为意识障碍。头颅 MRI显示双侧镜像般对称性大脑白质、内囊后肢、内侧丘系、胼胝体、中脑及小脑半球内白质广泛性长 T1 、长 T2 信号 ,无强化 ,似脱髓鞘改变。经皮质激素治疗后 ,2例 (男性 )好转 ,1例无改善。因此 ,认识慢性海洛因中毒性脑病的临床及影像学特征是十分重要的 ,及时诊治可望使病情得到缓解     

慢性一氧化碳中毒的临床与MRI表现

目的研究慢性一氧化碳(CO)中毒患者的临床和。MRI表现。方法回顾性分析5例慢性CO中毒患者的临床及MRI资料。结果5例患者早期均表现为间断性头痛、头昏、疲劳等非特异性症状，中晚期3例出现视力严重下降，2例出现认知障碍。5例患者头部MRI均可见双侧基底节区类圆形长T1、长T2改变，3例大脑白质呈弥漫性长T1、长T2改变，2例视神经呈长T1、长T2改变并可见视神经萎缩。结论慢性CO中毒患者早期症状无特异性，晚期主要表现视力减退和认知功能障碍。长期低浓度CO接触史是诊断的可靠依据。MRI检查对本病的诊断有重要意义。     

可逆性后部白质脑病二例临床分析

目的探讨可逆性后部白质脑病综合征(RPLS)的病因、临床表现、影像学特征、诊断及治疗。方法分析2例可逆性后部白质脑病综合征患者的临床资料。结果2例患者均急性起病,以头痛、频繁癫发作为首发症状;均发病于产褥期前后,均有血压升高、头晕、视物模糊等症状。1例烦躁、计算、近记忆力下降;另1例恶心、呕吐;头部CT检查2例均显示对称分布于枕、顶、额、颞叶沟回状低密度灶,其中1例广泛脑白质水肿;MRI检查2例均显示对称分布于枕、顶、额、颞叶回状或沟回弥漫片状长T2、长T1信号、Flair像示皮层下弓状纤维形高信号病灶,以脑白质为主、皮质亦见受累。经治疗2例患者的临床症状约1周消失,复查影像学恢复正常。结论头痛、视觉障碍和癫发作是RPLS主要临床表现,影像学特征主要为大脑后部白质对称性CT呈低密度灶、MRI呈长T1、长T2信号。经过正确的治疗,患者的症状、体征及神经影像学改变均可很快好转并可以完全恢复。     

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。     

Serial 18F-fluoro-2-deoxy-D-glucose positron emission tomography and magnetic resonance imaging of paraneoplastic limbic encephalitis

OBJECTIVES: To review and expand the existing literature of magnetic resonance imaging (MRI) and positron emission tomography (PET) of paraneoplastic limbic encephalitis (PLE). METHODS: We performed serial MRI and 18F-fluoro-2-deoxy-D-glucose (FDG)-PET in a patient with anti-Ma2-positive PLE. In addition, we reviewed the relevant literature by conducting a search in the Medline database. RESULTS: We found a total of 7 published patient studies of possible or probable PLE containing both MRI and PET data. In 1 of these reports, the diagnosis of PLE can be regarded as proven. The results of the previous studies are controversial. Epileptic activity and inflammation are assumed to be underlying mechanisms of increased FDG uptake. In our study, we found a focal tracer accumulation in the left medial temporal lobe, which increased during the first 9 months of follow-up and corresponded with an increase of serum anti-Ma2 antibody titers. The MRI findings showed a hyperintense signal change in the left medial temporal lobe without contrast enhancement, which remained unchanged over time. CONCLUSIONS: The results of functional and structural imaging in PLE may differ substantially. Results of FDG-PET can demonstrate focal hypermetabolism over a long time, which may indicate therapeutic potential. A prospective study with more patients will be needed to clarify the relevance of PET as a possible outcome measure in PLE. Future studies should include scalp or semi-invasive electroencephalographic recordings during PET acquisition.     

A case of paraneoplastic limbic encephalitis associated with malignant lymphoma presenting with a late-onset bilateral thalamic lesion

A 63-year-old man presented with cognitive impairment including disturbance of memory functions and character change. Fluid-attenuated inversion recovery (FLAIR) magnetic resonance (MR) imaging revealed signal hyperintensities in the bilateral medial temporal lobes. Cerebrospinal fluid analysis revealed high protein concentrations, positive results for the oligoclonal band, and a slightly positive result for glutamate receptor ε2 (GluRε2) antibody. Voltage-gated potassium channel (VGKC) antibody was slightly positive in serum. Computed tomography showed enlargement of the left supraclavicular, left axillary, and renal hilar lymph nodes, and 18 F-fluoro-2-deoxy-D-glucose positron emission tomography revealed increased uptake at the same sites. Lymph node biopsy findings were consistent with diffuse large B-cell lymphoma. Based on these findings, the patient was diagnosed with paraneoplastic limbic encephalitis (PLE) associated with malignant lymphoma. The patient received intravenous injection of immunoglobulin and R-CHOP chemotherapy, but his neurological condition deteriorated. MR imaging showed atrophic changes in the medial temporal lobes during immunotherapy and chemotherapy. FLAIR/T2-weighted imaging revealed signal hyperintensities in the bilateral thalami after the first course of R-CHOP chemotherapy. This is the first report of PLE associated with diffuse large B-cell lymphoma presenting with late-onset bilateral thalamic lesions.     

Breakdown in the temporal and spatial organization of spontaneous brain activity during general anesthesia

Which temporal features that can characterize different brain states (i.e., consciousness or unconsciousness) is a fundamental question in the neuroscience of consciousness. Using resting‐state functional magnetic resonance imaging (rs‐fMRI), we investigated the spatial patterns of two temporal features: the long‐range temporal correlations (LRTCs), measured by power‐law exponent (PLE), and temporal variability, measured by standard deviation (SD) during wakefulness and anesthetic‐induced unconsciousness. We found that both PLE and SD showed global reductions across the whole brain during anesthetic state comparing to wakefulness. Importantly, the relationship between PLE and SD was altered in anesthetic state, in terms of a spatial “decoupling.” This decoupling was mainly driven by a spatial pattern alteration of the PLE, rather than the SD, in the anesthetic state. Our results suggest differential physiological grounds of PLE and SD and highlight the functional importance of the topographical organization of LRTCs in maintaining an optimal spatiotemporal configuration of the neural dynamics during normal level of consciousness. The central role of the spatial distribution of LRTCs, reflecting temporo‐spatial nestedness, may support the recently introduced temporo‐spatial theory of consciousness (TTC).     

Complex partial status epilepticus in paraneoplastic limbic encephalitis.

Paraneoplastic limbic encephalitis (PLE) results from tumor-related autoimmune mediated inflammation and degeneration of the mesial temporal structures. Cognitive and behavioral changes and seizures occur in PLE. Seizures are an uncommon presenting symptom of PLE occurring in 6 of 50 patients in one series. We present a report of complex partial status epilepticus (CPSE) as the presentation of PLE with anti-neuronal antibodies and improvement in mental status following treatment of seizures.     

Cortical Gyrification,Psychotic-Like Experiences,and Cognitive Performance in Nonclinical Subjects

BackgroundPsychotic-like experiences (PLE) are present in nonclinical populations, yet their association with brain structural variation, especially markers of early neurodevelopment, is poorly understood. We tested the hypothesis that cortical surface gyrification, a putative marker of early brain development, is associated with PLE in healthy subjects. MethodsWe analyzed gyrification from 3 Tesla MRI scans (using CAT12 software) and PLE (positive, negative, and depressive symptom dimensions derived from the Community Assessment of Psychic Experiences, CAPE) in 103 healthy participants (49 females, mean age 29.13 ± 9.37 years). A subsample of 63 individuals completed tasks from the Wechsler Adult Intelligence Scale and Controlled Oral Word Association Test. Estimated IQ and a composite neuropsychological score were used to explore mediation pathways via cognition. ResultsPositive PLE distress was negatively associated with gyrification of the left precuneus. PLE depression dimension showed a negative association with gyrification in the right supramarginal and temporal region. There was no significant mediating effect of cognition on these associations. ConclusionOur results support a neurobiological psychosis spectrum, for the first time linking an early developmental imaging marker (rather than volume) to dimensional subclinical psychotic symptoms. While schizophrenia risk, neurodevelopment, and cognitive function might share genetic risk factors, additional mediation analyses did not confirm a mediating effect of cognition on the gyrification-psychopathology correlation.     

The Self and Its Prolonged Intrinsic Neural Timescale in Schizophrenia

Schizophrenia (SCZ) can be characterized as a basic self-disorder that is featured by abnormal temporal integration on phenomenological (experience) and psychological (information processing) levels. Temporal integration on the neuronal level can be measured by the brain’s intrinsic neural timescale using the autocorrelation window (ACW) and power-law exponent (PLE). Our goal was to relate intrinsic neural timescales (ACW, PLE), as a proxy of temporal integration on the neuronal level, to temporal integration related to self-disorder on psychological (Enfacement illusion task in electroencephalography) and phenomenological (Examination of Anomalous Self-Experience [EASE]) levels. SCZ participants exhibited prolonged ACW and higher PLE during the self-referential task (Enfacement illusion), but not during the non-self-referential task (auditory oddball). The degree of ACW/PLE change during task relative to rest was significantly reduced in self-referential task in SCZ. A moderation model showed that low and high ACW/PLE exerted differential impact on the relationship of self-disorder (EASE) and negative symptoms (PANSS). In sum, we demonstrate abnormal prolongation in intrinsic neural timescale during self-reference in SCZ including its relation to basic self-disorder and negative symptoms. Our results point to abnormal relation of self and temporal integration at the core of SCZ constituting a “common currency” of neuronal, psychological, and phenomenological levels.     

Complicated paraneoplastic neurological syndromes: a report of two patients with small cell or non-small cell lung cancer

Paraneoplastic neurological syndromes are frequently associated in patients with small cell lung cancer (SCLC) and antineuronal antibodies are involved in the autoimmune mechanism. Multiple syndromes are sometimes complicated in a single patient with SCLC. However, little is known about non-SCLC-associated neurological manifestations. We report two patients with complicated paraneoplastic neurological syndromes. Patient 1 showed paraneoplastic limbic encephalitis (PLE), paraneoplastic sensory neuropathy (PSN) and Lambert-Eaton myasthenic syndrome (LEMS) associated with SCLC. Patient 2 developed opsoclonus-ataxia and probable PLE associated with non-SCLC. Analysis of various antineuronal antibodies revealed that anti-Hu and P/Q-type voltage-gated calcium channel (VGCC) antibodies were positive in Patient 1 but any antibodies were not in Patient 2. Brain MRI demonstrated high intensity signals in temporal lobes particularly on fluid-attenuated inversion recovery (FLAIR) or diffusion-weighted images. These findings suggest that complicated paraneoplastic neurological syndromes occur in non-SCLC as well as SCLC and that unidentified antineuronal autoantibodies may underlie the pathophysiology.     

Disseminated necrotizing leukoencephalopathy following low-dose oral methotrexate

Leukoencephalopathy is a recognized complication with intrathecal or intravenous methotrexate (MTX). We report a 59-year-old lady who developed MTX leukoencephalopathy with long-term low-dose oral MTX. She developed posterior leukoencephalopathy (PLE) that initially was reversible on discontinuation of oral MTX. Four months later, she developed disseminated necrotizing leukoencephalopathy (DNL), and was left with devastating neurological deficits. The sequential conventional magnetic resonance imaging (MRI), diffusion weighted imaging (DWI), MR perfusion (MRP) and MR spectroscopic (MRS) changes are highlighted in this report. MRP and MRS showed more wide spread abnormalities than DWI. Stereotactic biopsy from the lesion revealed demyelination with macrophagic infiltration, pericapillary lymphomononucear aggregation, fibrinoid changes in the capillaries and neovascularization. Of the two cases of PLE with oral MTX reported in literature, one reversed clinically and radiologically with the discontinuation of MTX. To the best of our knowledge, this is the first reported case of DNL following oral MTX in the world literature.     

Magnetic resonance imaging detection of mesial temporal sclerosis in children

The objective of this study was to investigate the prevalence and clinical characteristics of mesial temporal sclerosis as diagnosed by brain magnetic resonance imaging in children. A total of 390 consecutive brain magnetic resonance imaging studies in children were reviewed for evidence of mesial temporal sclerosis. Subsequently, the magnetic resonance imaging scans and charts of patients with mesial temporal sclerosis were reviewed and their clinical details were evaluated. The magnetic resonance imaging studies had been performed for multiple indications, including seizures, headache, and developmental problems. In children, the prevalence of mesial temporal sclerosis among all brain magnetic resonance imaging studies was 3.1% (12 of 390 studies) and 12.1% (12 of 99 studies) among all brain magnetic resonance imaging studies performed for seizures. These children all presented with a history of seizure disorder, often had other medical problems, and histopathology (when available) nearly always (5 of 6 patients) confirmed their magnetic resonance imaging diagnosis of mesial temporal sclerosis. The prevalence of mesial temporal sclerosis is low among all pediatric patients who had magnetic resonance imaging brain studies. All our mesial temporal sclerosis patients had clinical seizures; i.e., it was never an "incidental finding". Children with mesial temporal sclerosis often had comorbid conditions, and the diagnosis of mesial temporal sclerosis made by magnetic resonance imaging was accurate when compared with the available histopathology.     

A case of parietal lobe epilepsy with ictal laughter

We report here about an 8-year-old boy with parietal lobe epilepsy (PLE) and ictal laughter. At the age of 6, he began to experience drop seizures, followed by sensory fits. Interictal EEG showed frequent spikes at C3, C4, P3 and Cz. Despite treatment with antiepileptic drugs, he often fell down in seizures after feeling abnormal sensations in the right shoulder. On ictal video EEG at the age of 7 years, (1) he became motionless and complained of fear and pain in the right hand, (2) he had clonic seizures of the right upper limb and fell down to his left, (3) he laughed though he did not feel funny. Ictal EEG showed spikes which originated in Pz and then were generalized. In many of the previously reported cases, ictal laughter is associated with hypothalamic hamartomas, infantile spasms,. complex partial seizures of frontal, temporal, or parietal origin. We diagnosed the present case as having PLE. However, other localization could not be roled out because the spikes were generalized quickly. To date, there are two reported cases of ictal laughter with PLE, but ictal EEG is lacking in these patients. Ictal laughter is rare in non-lesional cryptogenic PLE, but it may imply PLE's pathogenesis.