艾滋病所致的进行性多灶性白质脑病(附1例报告)

目的　提高对艾滋病所致的进行性多灶性白质脑病的认识以及引起临床重视。方法　报告 1例证实的艾滋病所致进行性多灶性白质脑病患者的临床表现、实验室及影像学特点。结果　患者神经系统表现为高级神经活动障碍 ,双侧锥体束损害 ,左侧偏身感觉减退。血清抗HIV抗体阳性。脑脊液检查正常。MR双侧额叶、右侧颞叶深部白质内均见斑片状、指状信号影 ,T1W低信号 ,T2 W高信号 ,病灶无强化。结论　艾滋病可引致进行性多灶性白质脑病 ,临床易误诊漏诊 ,应及时行有关检查及时确诊     

可逆性后部白质脑病综合征临床及影像学特点15例分析

目的探讨可逆性后部白质脑病综合征的临床和影像学特征。方法分析15例可逆性后部白质脑病综合征患者的临床和影像学资料。结果 15例可逆性后部白质脑病综合征患者继发于高血压脑病、系统性红斑狼疮、尿毒症、子痫、甲状旁腺腺瘤伴发高钙血症、非霍奇金淋巴瘤、肾移植术后、急性淋巴细胞白血病和神经白塞病。其中血压升高者10例,临床主要表现为头痛、癫痫发作、视物模糊和意识障碍。头颅CT或MRI检查主要表现为对称的枕叶白质脑水肿,部分伴有顶额颞叶等病变。经治疗后患者症状消失,头颅CT或MRI复查病灶完全或大部分消失。结论可逆性后部白质脑病综合征可由多种病因产生,影像学主要表现为大脑后部白质血管源性脑水肿改变,及时治疗后症状和影像学特征迅速改善。     

抗磷脂综合征7例患者临床、影像及病理特点分析

目的探讨抗磷脂综合征(antiphospholipid syndrome,APS)的临床表现、生化检查、神经影像和皮肤病理特点。方法回顾性分析7例诊断为APS患者的临床资料、神经影像和皮肤病理资料。结果慢性起病3例,临床表现为反复头痛、记忆力下降为主;神经影像学检查结果示病灶分布于大脑皮质下白质及侧脑室旁白质,呈脑梗死或脱髓鞘改变,颅内中等血管狭窄、小血管闭塞或继发扩张;皮肤活检病理检查:光镜示真皮内小血管周围少量淋巴细胞浸润,电镜示上皮组织下小血管管壁增厚、管腔狭窄或闭塞。结论 APS以反复头痛或脑梗死为主要临床表现,影像学以多灶性、不对称性、脑白质缺血或脱髓鞘信号改变为特点,皮肤病理检查以小血管狭窄或闭塞为特点。熟悉APS的临床、影像学与病理学特点有利于对其做出早期诊断。     

急性播散性脑脊髓炎的研究进展

急性播散性脑脊髓炎是一种特发性中枢神经系统炎症性脱髓鞘疾病,可发生于病毒感染或疫苗接种后,以儿童多见,成人亦可发生.临床表现为发热、头痛、脑膜刺激征,以及精神异常、癫癎发作、局灶性神经系统症状与体征;临床过程可轻可重,可以为单相型、复发型和多相型.神经影像学检查可见累及灰质、白质和脊髓的脱髓鞘病灶,双侧均可受累,部分病灶伴强化.诊断标准要求多灶性中枢神经系统白质脱髓鞘和存在脑病症状.鉴别诊断包括病毒性脑炎、多发性硬化、视神经脊髓炎谱系疾病、原发性中枢神经系统血管炎等.治疗方法有糖皮质激素、静脉注射丙种球蛋白、免疫抑制剂和血浆置换疗法等.     

影像学引导的立体定向活检在脑疾病诊断中的价值 (附50例临床分析)

目的探讨影像学引导的立体定向活检手术在神经科临床表现及影像学检查不典型病例诊断中的应用价值。方法安装CRW立体定向框架,采用螺旋CT薄层扫描与多层图像三维重建技术,对50例临床表现及影像学检查不典型的患者施行立体定向脑组织活检手术,其中:额叶深部病灶7例,颞叶7例,顶枕叶8例,鞍区4例,丘脑和基底节区9例,第三脑室后部3例,小脑半球4例,颅内多发性病灶(病灶2~7个不等)8例。结果50例患者均得到明确的病理诊断及相应的治疗,病理诊断结果:星形细胞瘤12例(其中I级4例,I~Ⅱ级2例,Ⅱ级2例,Ⅱ~Ⅲ级2例,Ⅲ级2例),胶质母细胞瘤3例,脑胶质瘤病1例,室管膜瘤1例,生殖细胞瘤l例,松果体母细胞瘤2例,恶性淋巴瘤2例,脑白质营养不良1例,线粒体脑病1例,脑囊虫病2例,脱髓鞘改变(白质多发性硬化)5例,炎性肉芽肿10例,脑变性疾病4例,转移瘤5例。诊断成功率达100%,未出现因活检手术而造成的出血、偏瘫等严重并发症。结论影像学引导的立体定向活检手术对于神经科临床表现及影像学检查不典型病例,快速获得明确的病理学诊断,制定相应的治疗方案,是一种安全、可靠的手段,对脑疾病的诊断治疗具有重要的应用价值。     

原发性中枢神经系统淋巴瘤3例报告

目的：分析原发性中枢神经系统淋巴瘤（PCNSL）3例,探讨此病的诊断规律。方法：对3例患者的临床症状及影像学改变进行观察,对脑手术标本进行组织学和免疫组化染色。结果：临床主要表现为颅内压增高和局灶占位病变,CT、MRI、病灶多位于大脑深部,注射造影剂后可见增强,组织学特点：瘤细胞围绕血管呈套袖样排列或呈弥漫性分布。免疫组化提示1例肿瘤（T细胞型）,2例B细胞非霍奇金淋巴瘤。结论：此病临床诊断困难,主要依靠病理学检查确诊。     

进行性多灶性白质脑病(附1例临床病理报告)

报告我国首例进行性多灶性白质脑病,临床上表现为精神症状后出现以锥体外系及锥体束损害为主的进行性弥漫性脑部病变,两大脑半球白质有散在众多与血管分布无关的脱髓鞘小病灶,并具有正常少突胶质细胞消失和星形胶质细胞增生肥大的病理特征,未发现核内包涵体的存在。     

多发性硬化和脑小血管病的临床易误诊点解析(附1例病例分析)

目的探讨临床不典型多发性硬化与脑小血管病在临床和影像学表现上的差异。方法举例分析1例被误诊为多发性硬化的遗传性脑小血管病-常染色体显性遗传病合并皮质下梗死和白质脑病(cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy,CADASIL),并结合临床经验、典型图例和文献回顾讨论总结两种疾病的鉴别点。结果多发性硬化以青壮年起病,IgG指数大于0. 7,寡克隆带阳性,磁共振可有中心静脉征,很少有微出血,侧脑室旁白质病灶多不对称等特征;而脑小血管则以中老年起病,有脑血管病危险因素,磁共振表现为腔隙性脑梗死伴有微出血、脑萎缩、侧脑室旁白质病灶常常对称分布等特点与多发性硬化相鉴别。结论加强对两种疾病临床和影像学的认识有助于避免疾病误诊和尽早启动合理的治疗。     

海洛因海绵状白质脑病的临床及MRI(附3例报告)

目的 :研究海洛因中毒所致的海绵状白质脑病临床与磁共振成像特点。方法 :本文报告 3例并结合文献分析海洛因海绵状白质脑病临床和 MRI资料。结果 :本病的主要临床表现 ,起病前有明确的吸服海洛因病史 ,急性或亚急性起病 ,早期以构音障碍、步态不稳、小脑性共济失调等小脑损害症状和体征为突出表现。 MRI检查显示对称性双侧小脑半球、大脑半球后部、内囊后肢、胼胝体压部、脑干等皮质为主的多发性大片状长 T1、长 T2信号 ,加强后病灶无强化。脑病理特点是脑白质呈对称性海绵样脱髓鞘改变。结论 :本病的诊断主要依靠其临床特点、影像学及脑病理学检查 ,MRI对本病的诊断具有重要价值。     

CADASIL的MRI特征及NOTCH3基因的研究

目的探讨伴皮层下梗死和白质脑病的常染色体显性遗传性脑动脉病(CADASIL)的MRI特点和基因突变类型。方法收集5例经临床确诊的CADASIL患者,对其颅脑MRI的特点和基因检测结果进行回顾性分析。结果 MRI显示为双侧大致对称的脑白质病灶,双侧颞极白质病灶(O`sullivan征),外囊T2高信号("人"字征),皮层下腔隙灶(SLLS征),基底节区、丘脑腔隙性梗死,桥脑T2高信号,脑萎缩,微出血灶。4例患者进行了基因检测,其中3例(75%)为Notch3基因外显子4位点突变,1例(25%)为Notch3基因外显子3位点突变。1例患者病理检查证实为血管平滑肌细胞表面出现嗜锇颗粒沉积。结论 MRI结合Notch3基因突变可以诊断CADASIL。     

X线摄片在脑部疾病诊断中的应用

目的:探讨X线摄片技术在脑部疾病诊断和鉴别诊断中的应用价值。方法:报道1年内我科住院患者中见到的4例较典型病例,依赖CT和MRI误诊神经系统疾病。结果:4例病人分别诊断为面肌痉挛脑梗塞的paget′s病例,初诊意识障碍原因待诊-代谢性脑病?脑干梗死?的多发性骨髓瘤病例;诊断帕金森病的股骨颈骨折病例;诊断脑梗死的肺癌病例。结论:CT和MRI决不能替代以症状和体征为基础的临床诊断学,X线摄片技术在脑部疾病诊断和鉴别诊断仍具有重要价值。     

Subacute sclerosing panencephalitis with atypical onset: clinical, computed tomographic, and magnetic resonance imaging correlations

Clinical, computed tomographic (CT), and magnetic resonance imaging (MRI) correlations of subacute sclerosing panencephalitis with an atypical onset are presented in three children. In all three patients, the disease began similarly, with unilateral neurologic deficit followed by gnosis, praxis, and memory dysfunction corresponding to massive one-sided lesions. The first patient demonstrated right frontal-lobe syndrome and polymorphic extrapyramidal hyperkinesias; MRI showed a large high-signal lesion in the right frontal lobe, while CT was normal. The second patient displayed a disease onset with left-sided hemiparkinsonism and involuntary movements correlating to the MRI finding, ie, a massive rightsided occipitotemporoparietal subcortical lesion. An acute, stroke-like episode represented the first symptom in the third child. CT visualized cerebral, mainly left-sided cortical atrophy. In all three children, CT and MRI revealed significantly progressing brain atrophy at the disease's latest stages. We discuss the role of MRI in detecting early pathologic changes in children with subacute sclerosing panencephalitis.     

Early ischemic lesion demonstrated by ictal diffusion-weighted MRI during prior TIA in a patient with cerebral infarction]

We reported a patient with transient ischemic attack (TIA), subsequently evolving to a cerebral infarction, in whom ictal diffusion-weighted magnetic resonance imaging (MRI) detected early ischemic lesion in the left hemisphere. The patient was a 30-year-old right-handed male medical doctor, who had an in-hospital episode of TIA with obtundation and right hemiparesis, which lasted for 150 minutes. Ictal diffusion-weighted MRI obtained 110 minutes after symptom onset demonstrated an area of high signal intensity in the left striatum and corona radiata, whereas T 2-weighted and FLAIR images were entirely normal. Ictal magnetic resonance angiography (MRA) showed occlusive lesions in the M 2 branches of the left middle cerebral artery. The second MRA obtained 90 minutes after resolution of the symptoms showed nearly complete recanalization of the left middle cerebral artery, suggesting that the TIA was embolic mechanism. However the patient rapidly developed similar neurological symptoms again 58.5 hours after the TIA episode, evolving finally to a completed stroke. A brain CT obtained 1 hour after the second episode demonstrated diffuse hypodense lesions in the left basal ganglia and corona radiata, and in the left temporal lobe. MRIs 3 and 7 days later displayed completed infarcts, of which distribution was consistent with that of the hypodense lesions on the earlier CT. The left middle cerebral artery remained patent on the follow-up MRAs. The patient fairly recovered and returned to his premorbid position as medical doctor with a mild residual right hand clumsiness. In this patient, ictal and post-ictal MRAs documented an occlusion and a reopening of the middle cerebral artery. The embolic mechanism remains unknown despite detailed cardiac, vascular, and hematological examinations. In addition to recurrent embolism, we would like to point out that the reperfusion injury, secondary delayed neuronal death, and other factors may be involved in the second exacerbation evolving to the completed stroke.     

Delayed ischemic hyperintensity on T1-weighted MRI in the caudoputamen and cerebral cortex of humans after spectacular shrinking deficit

BACKGROUND AND PURPOSE: Transient internal carotid artery (ICA)-middle cerebral artery (MCA) occlusion caused by cardiogenic embolus can lead to spectacular shrinking deficit (SSD): sudden hemispheric stroke syndrome followed by rapid improvement. The aim of this study was to investigate sequential neuroradiological changes in the brains of patients after SSD compared with those after brief cardiac arrest and hypoglycemia, which we previously studied with the same methods. METHODS: We serially studied CT scans and MR images obtained at 1.5 T in 4 patients with SSD. All 4 patients suffered from transient neurological deficits due to cardiogenic embolus in ICA-MCA. The symptoms began to disappear from 25 to 50 minutes after onset. RESULTS: Repeated CT scans demonstrated no abnormal findings in the affected cerebral hemisphere in 3 of the 4 patients and a small cortical infarct in the remaining 1. In each patient, repeated MRI between day 7 and month 23 after stroke showed basal ganglionic and cortical lesions. These lesions were hyperintense on T1-weighted and relatively hypointense on T2-weighted imaging. These ischemic lesions of hyperintensity on T1-weighted MRI subsided with time. CONCLUSIONS: Transient ICA-MCA occlusion leading to SSD produces a specific ischemic change with delayed onset in the basal ganglia and cerebral cortex in humans on MRI but not CT scans. We speculate that the lesions represent incomplete ischemic injury, including selective neuronal death, proliferation of glial cells, paramagnetic substance deposition, and/or lipid accumulation. Unlike brief cardiac arrest or hypoglycemia, the localized lesions on MRI of patients after SSD seem to be incomplete and to differ from infarction or hemorrhage.     

Multiple gliomas. Illustrative cases of 4 different presentations

Multiple gliomas are uncommon and may be classified according to: a) the time of presentation in early (at diagnosis) or late (during treatment); b) the characteristics of computed tomography or magnetic resonance imaging (CT/MRI) in multifocal (with evidence of spread) and multicentric (without evidence of spread). From 212 patients with histopathologic diagnosis of glioma evaluated from March/90 to September/99, 15 (7%) had multiple lesions. We describe 4 patients: early multicentric, late multicentric, early multifocal and late multifocal, with emphasis on characteristics of CT/MRI and possible differential diagnosis. The differential diagnosis of multiple lesions in the central nervous system includes mainly infectious/inflammatory diseases and metastasis, however multiple gliomas should always be considered, even in patients with known systemic cancer, as described by others. Considering that CT/MRI features are not definite, the diagnosis should always be confirmed by histopathologic examination.     

上矢状窦血栓形成早期大脑灰质CT特殊表现(附1例报道及文献复习)

目的分析上矢状窦血栓形成的早期大脑灰质CT特点,提高对上矢状窦血栓形成早期大脑灰质CT特点的认识。方法结合文献对1例上矢状窦血栓形成患者的早期脑CT影像学特点进行分析。结果患者发病后6h行脑CT平扫显示双侧额叶皮质高密度影;第3d时脑MRIT1显示上矢状窦前部血栓形成可疑,脑FLAIR-MRI显示右侧额叶高信号及左侧额叶弧形高信号,脑MRIT1相增强扫描显示双侧额叶皮层弧线型增强,MRV显示上矢状窦前1/3处见一无信号区,周围有侧枝循环形成;治疗第6d时复查脑CT和MRI均无出血征象。结论患者早期脑CT所显示的高密度影可能是皮层静脉和毛细血管血液淤积的显示,并不全是出血,对于该征象的认识并结合病理生理过程给予的合理解释对于及时正确的认识该病具有重要意义。     

MRI for the management of neonatal cerebral infarctions: importance of timing

PURPOSE: Focal ischemic stroke in neonates is a rare occurrence. Diagnosis with most imaging modalities is difficult, but necessary for initiating an anticoagulatory treatment. The purpose of this study was to evaluate the sensitivity of MRI sequences within the first 14 days of birth. PATIENTS AND METHODS: Four patients with neonatal stroke presenting as seizures were examined using a standard MRI protocol including diffusion-weighted images (DWI) and magnetic resonance angiography (MRA) within 72 h of birth. The time between the onset of symptoms and MRI ranged from 6 h to 48 h. Follow-up examinations were performed on days 5 ( n=1), 7 ( n=2), and 14 ( n=1) for the control of a treatment with low-molecular heparin. RESULTS: Eight infarctions were detected in the four patients, 5 in the middle cerebral artery (MCA), three in the posterior cerebral artery (PCA) territory. All lesions were visible using DWI, four on T2-weighted images. Whereas in two patients small lesions contralateral to a large stroke were missed on T2-weighted images, the diagnosis would have been missed altogether without DWI in one patient. On follow-up, the visibility of the infarctions had declined using DWI after 5 days and the lesions were invisible after 1 week. Fourteen days after the stroke, an increased diffusion was detected in the infarcted brain tissue. By this time, all lesions were visible on T2-weighted images. CONCLUSION: In neonates, infarcted brain tissue can be detected using DWI with high sensitivity during the first 2 days after stroke and before other sequences are diagnostic. However, the diffusion restriction does not persist beyond 1 week. After 5 days, diagnosis has to rest mainly on T2-weighted images.     

Japanese encephalitis presenting with left hemiplegia and thalamic neglect--a case report]

This report concerns a 51-year-old right-handed man with Japanese encephalitis, showing left hemiplegia and left hemispatial neglect. On admission, he had a slight fever, mild consciousness disturbance, left hemiplegia, and left hemispatial neglect but no neck stiffness, headache nor nausea. He was treated on the basis of cerebral infarction, but his fever and consciousness disturbance worsened. We found pleocytosis (145/mm3) in the cerebrospinal fluid (CSF) and right thalamic edema on a brain CT scan obtained 4 days later. He was finally diagnosed as having Japanese encephalitis on the basis of an increase in anti-viral antibodies observed in paired CSF and serum samples. In the exacerbation phase, 123I-IMP single photon emission CT (SPECT) demonstrated a marked decrease in cerebral perfusion in the right hemisphere, while a brain MRI revealed irregular lesions localized the right thalamus (mainly posterior and medial parts), showing low intensity on T1-weighted and high intensity on T2-weighted images. In the recovery phase, asymmetrical perfusion was no longer observed on SPECT and the symptoms including the left hemispatial neglect had improved. These findings suggest that the left hemispatial neglect in this patient might been caused by the right thalamic lesion resulting in damage to the activating system of the right hemisphere. This case thus shows that acute onset of hemispatial neglect could be caused by cerebral encephalitis.     

Asymmetric Neuroimaging in Creutzfeldt-Jakob Disease: A Ruse

Creutzfeldt-Jakob disease (CJD) causes diffuse neurological symptoms, but asymmetric lesions have been found on conventional magnetic resonance imaging (MRI) and diffusion-weighted imaging (DWI). Less often, position emission tomography (PET) scanning can also reveal asymmetric lesions in patients with CJD. Such imaging may mislead clinicians. The authors present a case of a woman with CJD who was diagnosed as having suffered a stroke because she had asymmetric T2-weighted imaging (T2WI) MRI abnormalities that were interpreted as a stroke. It was noted that the patient had clinical features consistent with CJD, including rapidly progressive dementia, myoclonus, cerebellar dysfunction, and pyramidal and extrapyramidal signs. This diagnosis was supported by periodic epileptiform discharges on the electroencephalogram (EEG) and by elevated 14-3-3 protein in the cerebrospinal fluid. MRI T2WI and DWI showed dramatically asymmetric abnormalities involving the left cortex. A PET study found decreased metabolism in the left cerebral and right cerebellar hemispheres. The patient's clinical, EEG, and laboratory data were all consistent with CJD, not other diseases, but the MRI and PET had atypical, asymmetric findings. This case demonstrates that CJD should be considered in the differential diagnosis of patients with rapidly progressive neurological decline, even if they have asymmetric imaging findings.     

100例脑卒中患者利手与语言优势半球关系的研究

选择１９８８年５月～１９９６年８月经头颅ＣＴ证实为单侧脑卒中且病灶波及语言相关区的病人１００例，在发病２周内进行失语检查，要求患者接受检查时神志清楚，定向力及记忆力好，无智能障碍，既往无脑病疾病史，其检测结果，左脑病变８０例中，有６６例失语；右脑病变２０例中，仅４例失语。经统计学处理差别有高度显著性，说明中国人左脑和右脑均可能控制语言，语言优势侧多在左侧，同时发现，右利手中，６８例左脑病变者有５８