以TIA为唯一表现的子痫前期患者合并可逆性脑血管痉挛综合征1例报告

<正>可逆性脑血管收缩综合征(reversible cerebral vasoconstriction syndrome,RCVS)是临床表现为严重头痛,伴或不伴神经功能缺失,并以脑影像学显示动脉可逆性多灶性狭窄为特征的一组临床综合征。近些年来,国内外有妊娠引发RCVS的报道,但患者均伴有严重头痛。经文献检索,以TIA为唯一临床表现的RCVS国内尚未见相关报道。现将我院     

获得性肝脑变性25例临床分析

目的提高对获得性肝脑变性认识。方法收集2016年1月至2019年2月中国医科大学附属盛京医院25例获得性肝脑变性患者的临床资料及影像学特点,结合文献进行总结分析。结果 25例获得性肝脑变性患者均患有慢性肝病,肝脏影像学均显示肝硬化伴侧支循环形成,头颅MRI均显示苍白球T1WI高信号,部分伴有邻近部位受累,中脑9例,内囊3例,丘脑3例。25例患者均有不同程度的神经系统症状,以帕金森病样症状和小脑损害症状组合为主。同时合并有急性肝性脑病发作7例,经治疗急性肝性脑病后症状可缓解,但其帕金森病样症状及小脑损害症状未见明显缓解。结论获得性肝脑变性是与慢性肝病相关的神经系统慢性疾病,目前认为主要与微量元素锰离子沉积导致的基底节区病变相关。抗帕金森病药物治疗对症状无明显改善,早期肝移植可能是有效的治疗手段。     

可逆性脑血管收缩综合征:一例报告并文献分析

目的 报告1例临床罕见的可逆性脑血管收缩综合征病例,以期提高对该病的认识.方法 对1例可逆性脑血管收缩综合征患者的临床症状、影像学特征及临床转归进行回顾性分析,并复习相关文献.结果 女性患者,25岁.以声音刺激后发作性、雷击样、剧烈头痛为首发症状.继之出现右侧肢体无力,症状持续.MRI检查显示右侧外囊、左侧枕叶及左侧额顶叶皮质多发长T1、长T2信号;TCD检查显示双侧颈内动脉末端、大脑中动脉血流速度明显增快伴涡流、杂音,提示血管狭窄;MRA检查双侧大脑前动脉、大脑中动脉、大脑后动脉呈节段性狭窄,似"串珠样"改变.经氯吡格雷、羟乙基淀粉和维生素B1治疗后,临床症状完全恢复.出院后复查TCD显示血流速度和频谱基本正常,MRI显示病灶明显缩小,MRA可见血管形态恢复正常.临床诊断为可逆性脑血管收缩综合征.结论 临床表现为雷击样头痛的患者应考虑可逆性脑血管收缩综合征的可能,其继发性脑梗死易发生于大脑后循环供血区和分水岭区.TCD和MRA检查可作为诊断、药物疗效评价及随访观察的重要方法.     

特发性低颅压综合征的临床和影像学特征

目的:探讨特发性低颅压综合征(spontaneousintracranialhypotension,SIH)的临床特点和影像学特征。方法:回顾性分析9例SIH患者的临床和CT、MRI资料。结果:本组患者均有特征性体位性头痛症状,伴有头晕4例,耳鸣3例,颈项强直2例,9例均行腰穿检查,脑脊液压力均<70mmH2O,4例患者脑脊液中红细胞数有不同程度升高。头部影像学表现硬脑膜增厚6例,硬膜下积液2例,硬膜下出血1例,伴有脑下垂3例。结论:体位性头痛是SIH的临床特征,影像学表现为硬脑膜增厚,硬膜下积液或出血,伴或不伴有脑下垂。     

线粒体脑肌病伴高乳酸血症和卒中样发作综合征的临床特点分析

目的探讨线粒体脑肌病伴高乳酸血症和卒中样发作(MELAS)综合征的临床、影像学、肌肉病理及分子生物学特点。方法回顾性分析6例MELAS患者的临床资料。结果首发症状表现为肢体抽搐4例,轻偏瘫1例,失语1例;其他症状包括发作性头痛和视力障碍。合并心脏功能异常2例,ECG提示预激综合征。6例患者行血乳酸运动试验,均为阳性。6例患者MRI检查显示大脑皮质和皮质下与血管分布不一致的长T_1、长T_2病灶。3例伴有脑萎缩,其中2例为小脑萎缩,1例为全脑萎缩。1例患者4个月后复查头颅MRI提示病灶完全消失。磁共振波谱可见病灶内多个体素的乳酸峰值升高,N-乙酰天冬氨酸值降低,乳酸/胆碱比降低。2例患者肌肉组织活检的病理均见到不同程度的萎缩肌纤维,未见到破碎红纤维。5例患者基因检测发现线粒体DNA(mtDNA)A3243G点突变,1例患者发现mtDNA T3271C点突变。结论 MELAS综合征临床表现复杂多样,以头痛、肢体抽搐及反复卒中样发作为突出特点。卒中样发作期影像学主要表现为脑内与供血区分布不一致的病灶;血乳酸运动试验阳性;典型肌肉病理检查MGT染色可见破碎红纤维;分子遗传学检查可发现基因突变。最终确诊有赖于基因检测。     

皮质基底节变性综合征四例临床及影像学分析

目的 探讨皮质基底节变性综合征的临床特征、影像学表现和治疗.方法 对4例临床诊断皮质基底节变性患者的资料进行回顾性分析.结果 4例患者的首发症状均为单侧肢体的帕金森样表现,对左旋多巴治疗无反应,4例均有肢体失用、构音障碍,3例伴肌阵挛,2例伴有痴呆,1例伴不自主运动.4例患者头部MRI扫描均显示受累肢体对侧脑皮质萎缩,以额顶叶显著;头葡萄糖代谢正电子发射断层扫描(FDG-PET)检查均显示不对称的大脑皮质和基底节区葡萄糖代谢减低.结论 皮质基底节变性综合征患者的临床特征为不对称的帕金森样表现,可伴有认知功能障碍、失用、构音障碍、肌阵挛和不自主运动等症状;头MRI、FDG-PET检查有助于诊断;目前尚无有效治疗方法.     

线粒体脑肌病伴高乳酸血症和卒中样发作三例

目的分析3例线粒体脑肌病伴高乳酸血症和卒中样发作(MELAS)患者的临床表现影像学特点并探讨其发病机制。方法对3例MELAS患者的临床资料进行分析。结果 3例患者均以卒中样发作为主要表现,伴有内分泌、循环系统等多系统症状。患者头颅影像学有异常表现,肌肉活检示破碎红纤维。结论MELAS的初步诊断主要依据临床表现和影像学特点,肌肉活检可以为进一步明确诊断提供重要的依据。     

可逆性脑血管收缩综合征的特点

可逆性脑血管收缩综合征(reversible cerebral vasoconstriction syndrome,RCVS)是一组以剧烈头痛(典型者为雷击样头痛)为特征性临床表现,伴或不伴有局灶性神经功能缺损或癫癎发作的临床综合征,其病理基础是大脑动脉的可逆性收缩,多于发病后1～3个月恢复[1].     

脑内脱髓鞘性假瘤的诊断与治疗（附3例报告）

目的探讨脑内脱髓鞘性假瘤（DPT）的临床表现、影像学特点、病理特征及治疗效果。方法回顾性分析3例DPT的临床资料,并复习相关文献。结果脑内DPT以急性起病为主;影像学多表现为皮质下单一病灶,其强化呈非闭合性、斑片样或垂直于侧脑室分布的火焰样;病理检查发现病灶呈炎性脱髓鞘样改变;皮质类固醇激素治疗后症状均得到明显改善。结论脑内DPT的临床表现与大多颅内肿瘤类似,易误诊为脑胶质瘤;其影像学具有一定的特征性表现;类固醇激素治疗可获得满意疗效。     

4例NOTCH3基因突变的遗传性脑小血管病患者临床及认知特点

目的对4例NOTCH3基因突变的遗传性脑小血管病患者的临床及认知特点进行分析比较。方法回顾性收集2015年5月-2017年9月天津市环湖医院神经内科收治的4例NOTCH3基因突变的遗传性脑小血管病患者的临床资料,分析其首发症状、临床表现、影像学检查、辅助检查、基因检测及相关神经心理学评估等方面的特点。结果 4例NOTCH3基因突变的患者中2例以急性缺血性卒中,1例以认知功能障碍,1例以头晕头痛就诊,其中1例患者除了有急性卒中症状外还伴有家族性秃头和腰痛。4例患者中2例伴有脑血管病危险因素。4例患者头颅MRI均可见不同程度皮质下脑白质损害,但部位不完全相同。4例患者基因检测分别在19号染色体NOTCH3基因3号,4号,20号,11号外显子发生杂合突变。4例患者神经心理学评估均提示存在认知功能障碍,主要在执行功能、语言、抽象能力及延迟回忆方面分值降低,而在定向力、计算力、命名、注意力等方面降低不明显。结论 NOTCH3基因突变的遗传性脑小血管病患者在临床特征方面有各自不同的特点,个体间存在异质性。     

A pediatric case of reversible cerebral vasoconstriction syndrome with cortical subarachnoid hemorrhage

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare disorder characterized by acute onset, severe headache, with reversible vasoconstriction of cerebral arteries often accompanied by additional neurological symptoms. This syndrome is seen mainly in middle-aged adults, predominantly women. Herein, we report on a pediatric case of RCVS with cortical subarachnoid hemorrhage (SAH). A 12-year-old boy developed acute, severe headache with paralysis of lower extremities causing gait disturbance after administration of eletriptan. Brain magnetic resonance angiography (MRA) revealed multifocal narrowing of the cerebral arteries, whereas magnetic resonance imaging (MRI) demonstrated sulcal hyperintensity on fluid-attenuated inversion recovery, consistent with cortical SAH. The patient's clinical symptoms resolved spontaneously after a few days and the MRI and MRA findings disappeared 3months later, suggesting a diagnosis of RCVS. Eletriptan might cause vasoconstriction of cerebral arteries. Although most patients with RCVS are adults and pediatric cases are rare, RCVS should be considered in a child complaining of severe headache.     

大脑胶质瘤病的分型探讨(附13例报告)

目的 探讨大脑胶质瘤病(gliomatosis cerebri,GC)的分型及影像和病理学特征.方法 对本院神经外科2010年3月至2012年11月间收治的13例GC进行分型对比研究.结果 GC的临床表现以头痛(8/13)和癫痫(3/13)常见.影像学检查显示弥漫性病灶累及大脑半球3个或3个以上脑叶及中枢结构,MRI呈长T1、长T2异常信号,增强扫描通常无强化,MRS也具有诊断价值.光镜和电镜结果显示肿瘤组织多以星形胶质细胞瘤多见(9例),也可为少突胶质细胞瘤(2例).依据组织学表现,GC 1型以低级别星形胶质细胞瘤多见.结论 可将GC分为两型,并且各具有不同的影像和病理学特征,1型GC以星形细胞瘤常见,临床上可依据分型的不同选择个体化的治疗方法.     

以突聋为首发症状的脑梗死患者临床特点

目的探讨以突聋为首发症状的脑梗死患者的临床特点,提高对该类情况的认识,尽早做出正确诊断。方法报道我院2014年收治的2例以突聋为首发症状的脑梗死病例,均完善头颅MRI、HR-MRI、DSA、颈动脉超声、TCD检查,结合文献复习,总结临床特点。结果该2例患者首发症状表现为突聋合并头晕,DSA均发现基底动脉近段狭窄。病例1头颅MRI示双侧桥脑及左侧小脑新发梗死灶,予急诊动脉溶栓治疗,后续双联抗血小板聚集及他汀治疗,听力好转。病例2头颅MRI示右侧桥脑新发梗死灶,予双联抗血小板聚集及他汀治疗,遗留听力下降。结论以突聋为首发症状的脑梗死并不罕见,突聋患者应尽快完善脑血管检查,突聋患者具有以下临床特点时需要高度警惕脑梗死:伴随头晕症状、合并多种动脉粥样硬化危险因素、基底动脉狭窄。     

Atraumatic nonaneurysmal sulcal subarachnoid hemorrhages: a diagnostic workup based on a case series

Introduction: Atraumatic and nonaneurysmal sulcal subarachnoid hemorrhage (sSAH) is a rare type of cerebrovascular disease with various etiologies previously reported in small case reports. In this study, we propose to analyze clinical presentations, imaging patterns and etiologies in a large case series of such patients in order to propose a diagnostic workup. Methods: We retrospectively analyzed clinical and radiological data of consecutive patients with a diagnosis of atraumatic and nonaneurysmal sSAH, admitted to our institution between 2008 and 2011. All patients had both computed tomography (CT) and magnetic resonance imaging (MRI) as a part of their initial evaluation. Results: 30 patients (18 women and 12 men, mean age: 60 years) were identified. The main clinical symptoms at presentation were focal and transient neurological deficit (n = 22) and thunderclap headache (n = 10). Four patients had progressive headache and 4 other had partial or generalized epileptic seizures. MRI abnormalities associated with sSAH were prior hemorrhages, microbleeds, severe leukoencephalopathy and hemosiderosis suggesting cerebral amyloid angiopathy (CAA; n = 9), vasogenic edema in parieto-occipital areas compatible with a posterior reversible encephalopathy syndrome (PRES; n = 3), cortical venous thrombosis (n = 2) and concomitant acute cortical stroke (n = 3). Other underlying causes of sSAH, not diagnosed on MRI, were reversible cerebral vasoconstriction syndrome (RCVS) based on clinical criteria and conventional angiography (n = 4), angiitis diagnosed by skin biopsy (n = 1), vascular malformation diagnosed on CT and digital subtraction angiographies (n = 3), and overanticoagulation (n = 1). Four cases remained unresolved. Conclusion: This study confirmed that sSAH is a rare condition related to a wide spectrum of etiologies. Combination of brain MRI and magnetic resonance angiography and eventually digital subtraction angiography allowed the identification of an underlying etiology for 87% of patients. CAA, RCVS and PRES represented more than 50% of the etiological mechanisms. Among older patients, sSAH was mainly related to CAA while in younger patients, RCVS represented the most frequent etiology.     

Initial vasodilatation in a child with reversible cerebral vasoconstriction syndrome

We describe the case of a 10-year-old boy who developed reversible cerebral vasoconstriction syndrome (RCVS) after cerebellitis. He received intravenous immunoglobulin and methylprednisolone to treat the cerebellitis. However, he then presented with a sudden severe headache, vomiting, and generalized tonic-clonic seizure. Brain magnetic resonance angiography (MRA) initially revealed diffuse cerebral vasodilatations, and diffuse multifocal segmental vasoconstrictions developed several days later. His clinical symptoms gradually resolved after several days, in the absence of any specific therapy. MRA performed 46 days after symptom onset showed that the multifocal segmental vasoconstrictions had resolved, suggesting a diagnosis of RCVS. The imaging features of RCVS include multifocal segmental vasoconstriction. However, our case suggests that diffuse cerebral vasodilatation may in fact be evident during the early stage of disease.     

38例可逆性后部白质脑病综合征的临床影像学特征及病因学分析

目的探讨可逆性后部白质脑病综合征(RPLS)的临床影像学特点以及病因学。方法回顾性分析2009年至2014年间在中南大学湘雅医院神经内科就诊的38例病人临床资料、影像学资料及治疗过程。结果 38例患者中女性23例,男性15例;高血压病史31例,有子痫或子痫前期病史6例;服用免疫抑制剂5例,肿瘤化疗史3例。30例(78.9%)患者首发表现为头痛头晕,此外血压增高、恶心呕吐、视力下降、痫性发作和意识障碍为主要临床表现。磁共振影像表现为大脑后半部对称性稍长T1、长T2信号,FLAIR序列为高信号,DWI低信号,并可发现皮质受累。结论 RPLS是一类预后良好的脑病综合征,病因复杂,目前主要依靠临床表现以及影像学明确诊断。     

脑出血恢复期内分泌激素异常的研究

目的 探讨脑出血恢复期患者内分泌激素变化情况.方法 用放射免疫法测定90例脑出血患者发病后3个月,6个月,12个月周围静脉血中FT3、FT4、TSH、GH、ACTH、Cor、PRL、T、E2、FSH、LH、P的浓度.分析内分泌异常的发生率及其与出血量、有无并发症及恢复时间的关系.结果 内分泌激素异常在发病后3个月时发生率11.1％,6个月时发生率8.9％,12个月时发生率7.8％.随着恢复时间的延长逐渐下降,出血量越大分泌异常发生率越高,发病当时有并发症者恢复期内分泌激素异常发病率高.结论 脑出血恢复期内分泌异常有一定的发生率,有必要对恢复期患者进行常规的内分泌激素水平检查,并用于指导治疗.     

丁苯酞软胶囊治疗椎动脉优势型眩晕疗效观察

目的观察丁苯酞软胶囊治疗椎动脉优势型后循环缺血性眩晕的疗效及安全性。方法收集首次发作眩晕并行MRA检查证实一侧椎动脉优势的后循环缺血患者76例为研究对象,随机分为治疗组和对照组,对照组给予阿司匹林加阿托伐他汀钙片基础治疗,治疗组在对照组的基础上加用丁苯酞软胶囊,治疗14 d后观察眩晕缓解程度、TCD血流异常率变化。结果治疗组总有效率92.1%,对照组总有效率65.8%,两组比较差异有统计学意义P0.05)。两组经治疗后基底动脉TCD异常率均有下降,其中治疗组(47.4%)与对照组(57.9%)相比较下降更明显,差异有统计学意义(P0.05)。结论丁苯酞软胶囊治疗椎动脉优势型眩晕有显著疗效且安全。     

Reversible cerebral vasoconstriction syndrome: rare or underrecognized in children?

Reversible cerebral vasoconstriction syndrome (RCVS) is a clinicoradiological diagnosis comprising ‘thunderclap’ headaches and reversible segmental vasoconstriction of cerebral arteries, occasionally complicated by ischaemic or haemorrhagic stroke. We report a case of RCVS in a 13‐year‐old male with severe thunderclap headaches and no focal neurological signs. Brain imaging showed multiple posterior circulation infarcts; cerebral computed tomography, magnetic resonance imaging, and catheter angiography showed multifocal irregularity and narrowing, but in different arterial segments. Laboratory studies did not support a diagnosis of vasculitis. Symptoms resolved over 3 weeks; magnetic resonance angiography 3 months later was normal and remained so after 2 years. We highlight the typical clinical features of RCVS in this case and suggest that the diagnosis should be considered in children with thunderclap headaches or stroke syndromes where headache is a prominent feature, especially if cerebrovascular imaging studies appear to be evolving or discrepant.     

Reversible cerebral vasoconstriction syndrome associated with hyperosmolar hyperglycaemic state: A case report and literature review

Reversible cerebral vasoconstriction syndrome (RCVS) is an uncommon disorder characterised by thunderclap headache and self-resolving angiographic vasospasm in the presence or absence of neurological deficit. We present the first case of RCVS likely precipitated by a complex array of confounding factors including a hyperosmolar hyperglycaemic state (HHS), induction chemotherapy with cyclophosphamide, non-Hodgkin’s lymphoma, pancytopenia and previous blood transfusions. However, the clinical presentation in this case of altered conscious state followed by thunderclap headache was highly suggestive of HHS being the crucial inciting factor. This report of RCVS associated with HHS lends unique insight into key underlying pathophysiological mechanisms, and warns of the need to maintain a high index of suspicion for this elusive condition given the dynamic and transient nature of its clinical and radiological features.