Hemorrhage predisposing to cerebral infarction in children with moyamoya disease

Two children with a new diagnosis of hemorrhagic type moyamoya disease experienced cerebral infarction after intraventricular hemorrhage. The patients developed ischemia 15 and 2 days after the initial diagnosis of hemorrhage. No provocative factor such as hypoperfusion was identified, but both patients exhibited signs of increased intracranial pressure on computed tomographic scan before infarction. Increased intracranial pressure in the setting of hemorrhage may be a risk factor for ischemic complications in patients with moyamoya disease. The optimal acute management of these patients requires further study.     

Intraventricular Hemorrhage Long after Successful Encephaloduroarterio Synangiosis in Moyamoya Patient

Intraventricular hemorrhage long after successful encephaloduroarterio synangiosis (EDAS) is very rare. The effect of revascularization surgery for preventing hemorrhagic event of moyamoya disease remains controversial. We report a 17-year-old female with intracerebral hemorrhage and intraventricular hemorrahge 10 years after successful EDAS. Even though cerebral vessels angiography showed good collateral circulations without specific weak points, a cerebral hemorrhage could occur in patient with ischemic type of moyamoya disease long after successful indirect bypass operations. Good collateralization of cerebral angiography or magnetic resonance perfusion image after indirect bypass surgery would ensure against ischemic symptoms, not a hemorrhage. And, thus a life-time follow-up strategy might be necessary even if a good collateral circulation has been established.     

Efficacy of superficial temporal artery–middle cerebral artery anastomosis with routine postoperative cerebral blood flow measurement during the acute stage in childhood moyamoya disease

OBJECT: Surgical revascularization for moyamoya disease prevents cerebral ischemic attacks by improving cerebral blood flow (CBF). It is undetermined, however, how rapid increase in CBF affects chronic ischemic brain during the acute stage in childhood moyamoya disease. MATERIALS AND METHODS: The present study includes nine consecutive cases of patients with childhood moyamoya disease (2~8 years old, 6.2 in average), who underwent superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis on 17 hemispheres. We prospectively performed single-photon emission computed tomography 1 and 7 days after 17 surgeries. The follow-up period ranged from 12 to 37 months (24.9 in average). RESULTS: The outcome of 17 surgeries was excellent (disappearance of transient ischemic attack) in 14 hemispheres (82.4%) and good (reduction of transient ischemic attack) in three hemispheres (17.6%). No patient suffered peri-operative infarction, except for one (5.9%) manifesting as pseudolaminar necrosis in a part of the cerebral cortex supplied by STA-MCA bypass at the subacute stage, which did not affect his long-term neurological status. One patient (5.9%) presented with transient facial palsy due to hyperperfusion, which resolved within several days. No patient manifested permanent neurological deterioration during the follow-up period. CONCLUSION: The STA-MCA anastomosis is a safe and effective treatment for childhood moyamoya disease. We recommend routine CBF measurement for avoiding surgical complications including both cerebral ischemia and hyperperfusion.     

Symptomatic hyperperfusion after superficial temporal artery–middle cerebral artery anastomosis in a child with moyamoya disease

Object Surgical revascularization for moyamoya disease prevents cerebral ischemic attacks by improving cerebral blood flow (CBF). It is undetermined, however, how rapid increase in CBF affects ischemic brain at acute stage, especially in children. Case report A 4-year-old girl with moyamoya disease underwent right superficial temporal artery–middle cerebral artery (STA–MCA) anastomosis. She suffered temporary left facial palsy 5 days after surgery. Postoperative N-isopropyl-p-[¹²³I]iodoamphetamine single-photon emission computed tomography (¹²³I-IMP-SPECT) revealed focal intense increase in CBF at the sites of anastomosis. Magnetic resonance imaging/angiography showed the apparently patent STA–MCA anastomosis as a thick high signal without ischemic changes. Her symptom improved 9 days after surgery, and single-photon emission computed tomography (SPECT) 2 months later showed normalization of CBF. Surgical revascularization completely relieved the transient ischemic attack on her left hand that was seen before surgery. Conclusion We demonstrated, for the first time, that delayed focal neurological deficit after STA–MCA anastomosis can be caused by focal hyperperfusion in childhood moyamoya disease.     

Cerebral blood flow findings in moyamoya disease in adults

Three adult patients with moyamoya disease are described. They presented with intracerebral hematoma, cerebral infarction and subarachnoid hemorrhage, respectively. Subarachnoid hemorrhage is rare in moyamoya and is usually the result of aneurysm rupture. No aneurysm was found in our patient. Regional cerebral blood flow (rCBF) and the cerebral perfusion reserve assessed by the acetazolamide test, were significantly reduced in all three patients. The areas with most reduced baseline rCBF and most impaired vasoreactivity did not always correspond to the site of the vascular accident, indicating that these CBF changes were at least in part due to the moyamoya disease and/or its underlying causes, and not solely to the vascular accidents.     

急性脑卒中患者血清神经元特异性烯醇化酶、S-100B蛋白、髓鞘碱性蛋白水平的变化

目的探讨急性脑卒中患者血清神经元特异性烯醇化酶(NSE)、S-100B蛋白、髓鞘碱性蛋白(MBP)水平变化及其临床意义。方法回顾性对照分析发病在48 h内的45例急性脑卒中患者(其中脑梗死组19例,脑出血组15例,短暂性脑缺血发作组11例)血清NSE、S-100B蛋白、MBP水平变化及其与脑梗死、脑出血患者神经功能缺损程度的相关性。结果脑梗死组和脑出血组患者血清NSE、S-100B蛋白、MBP水平均较对照组有不同程度增高(P<0.05),而短暂性脑缺血发作组与正常对照组比较均无明显变化。脑梗死组和脑出血组患者中神经功能缺损较重的中重型亚组患者血清NSE、S-100B蛋白、MBP水平较轻型亚组水平高(P<0.05)。结论血清NSE、S-100B蛋白、MBP水平可作为急性脑卒中患者病情判断、预后评估的指标之一,对早期脑梗死与短暂性脑缺血发作也有鉴别诊断价值,尤其适用于无法进行影像学检查的脑卒中患者。     

Intracerebral hemorrhage versus infarction: Stroke severity,risk factors,and prognosis

The purpose of this study was to compare stroke severity, risk factors, and prognosis in patients with intracerebral hemorrhage versus infarction. We prospectively studied 1,000 unselected patients with acute stroke of a verified type in the Copenhagen Stroke Study. Neurological deficits and functional disabilities were evaluated weekly from the time of acute admission throughout the rehabilitation period. Eighty-eight (9%) had intracerebral hemorrhage. The relative frequency of intracerebral hemorrhage rose exponentially with increasing stroke severity. In multivariate analyses, stroke type had no influence on mortality, neurological outcome, functional outcome, or the time course of recovery. Initial stroke severity was the all-important prognostic factor. The relative importance of hypertension and blood pressure on admission was not greater for intracerebral hemorrhage than for infarction. No preponderance was found between type of stroke and sex, age, and smoking. Diabetes, ischemic heart disease, and elevated serum total cholesterol level all favored cerebral infarction as opposed to intracerebral hemorrhage. We conclude that the type of stroke per se has no influence on stroke prognosis in general; the extent of the injury is decisive. The poorer prognosis in patients with intracerebral hemorrhage is due to the increase in frequency of intracerebral hemorrhage with increasing stroke severity. The likelihood of cerebral infarction occuring as opposed to intracerebral hemorrhage seems increased fivefold in stroke patients with diabetes. Hypertension and blood pressure on admission were not predictors of stroke type.     

Fetal posterior cerebral artery in pediatric and adult moyamoya disease: A single-center experience of 480 patients

PurposeIn past pediatric and adult cohort studies of moyamoya disease, the fetal posterior cerebral artery has received less attention. Its relationship with the clinical manifestations and collateral circulation of moyamoya disease or ipsilateral cerebral hemispheres remains unclear.MethodWe summarize the clinical features of patients with and without fetal posterior cerebral artery moyamoya disease from consecutive cases.We explored the relationship between fetal posterior cerebral arteries and collateral circulation in the ipsilateral cerebral hemispheres, as well as differences among different subgroups of patients.According to the morphology, the fetal posterior cerebral artery is divided into complete fetal posterior cerebral artery and partial fetal posterior cerebral artery. Clinical features were classified as: infarction,hemorrhage,and non-stroke in unilateral/bilateral cerebral hemispheres. Collateral circulation is divided into extracranial vascular compensation and leptomeningeal collateral circulation. Digital subtraction angiography and CT/MR were used to evaluate the blood flow status and clinical characteristics of patients with moyamoya disease.ResultA total of 960 cerebral hemispheres from 142 pediatric patients and 338 adult patients were included in the study. A total of 273 (56.9%) patients had 399 cerebral hemispheres (41.6%) with fetal posterior cerebral arteries. Adults with fetal posterior cerebral arteries had lower rates of infarction (24.6%vs37.3%, P =0.005) and were less likely to have bilateral stroke (8.4%vs11.5%, P =0.038). Cerebral hemispheres with fetal posterior cerebral artery were more likely to have anterior cerebral artery and middle cerebral artery stenosis and less likely to have occlusion (P =0.002, 0.001), and less likely to involve the posterior circulation (P < 0.001). The cerebral hemispheres of the fetal posterior cerebral artery had higher leptomeningeal collateral circulation scores. There are significant differences in extracranial vascular compensation between cerebral hemispheres with and without fetal posterior cerebral artery. Adult patients with fetal posterior cerebral artery were more advanced in Suzuki stage (P =0.017).ConclusionsOur results suggest that fetal posterior cerebral artery is associated with infarct manifestations in pediatric and adult moyamoya disease. In the cerebral hemispheres, the fetal posterior cerebral artery is associated with ipsilateral hemispheric anterior and posterior circulation artery injury, extracranial vascular compensation, leptomeningeal collateral circulation compensation, and infarction. Adult patients with fetal posterior cerebral artery were more advanced in Suzuki stage.     

能谱计算机断层扫描容积螺旋穿梭扫描技术在烟雾病中的初步应用研究

目的 探讨能谱计算机断层扫描（computed tomography,CT）容积螺旋穿梭扫描技术在烟雾病患者血管和灌注成像中的应用价值。 方法 对4例术前、11例术后的烟雾病患者行容积螺旋穿梭扫描检查,获得四维CT血管成像及全脑CT灌注成像。采用CT平扫分型、颈内动脉分期、侧支循环分类、烟雾状血管丰富程度分期、CT灌注异常分期及Alberta卒中早期CT评分的评估标准,分左右大脑半球综合评价烟雾病的脑实质和脑血管病变。 结果 术前4例烟雾病患者,8个半球中的5个半球（62.5%）处于颈内动脉分期的III期;本组侧支循环主要来源于颅底血管网（46.1%）;梗死分期中,8个半球（100%）出现异常,其中7个半球（87.5%）处于梗死前期的Ⅱ期,且Alberta卒中早期CT评分均在10分以上。术后11例（22个半球）患者均行颞浅动脉-大脑中动脉搭桥术,其中左侧6例,右侧4例,双侧1例,吻合血管通畅率为100%;梗死分期中19个半球（86.4%）出现异常,其中10个半球（45.5%）处于II期、8个半球（36.4%）处于Ⅲ期。 结论 能谱CT容积螺旋穿梭技术可以同时获得四维CT血管成像及全脑CT灌注成像,能对判断烟雾病患者的动脉分期、侧支循环、脑灌注情况、术后血管吻合等影像学信息发挥重要作用。     

国人烟雾病临床特征探讨

目的探讨中国烟雾病人的临床特征。方法将54例中国人烟雾病的发病年龄、性别、临床症状、影像及治疗的特点，与日韩及欧美烟雾病患者进行了分析对比：结果中国人烟雾病的发病年龄似有日韩一样的两个高峰，但成人及男性患者发生率高于日韩，脑梗塞发生率也较日本高，脑短暂缺血性发作及癫痫的发生率却低于日本，脑出血的发生率与日本相近且以女性患者多见，两国患者的治疗效果无明显差异：结论中国人烟雾病的临床特征与日韩欧美患者不完全相同，其发病机理可能存在一些差异。     

17例烟雾病的临床特点与DSA分析

目的:研究烟雾病的临床特点、DSA表现。方法对17例经DSA检查确诊的烟雾病患者,分析其临床表现及DSA检查结果。方法:脑缺血表现14例,出血表现3例,DSA上表现为双侧病变6例,单侧病变11例,异常血管网是本病的特征,部分病例的临床表现、CT表现与DSA显示的血管狭窄和闭塞有不一致性。结论:青壮年发生脑血管病, 血压不高,反复出现脑梗死或出现脑室出血或蛛网膜下腔出血者,应考虑MMD的可能,及早做DSA检查。     

Pathological and clinical features of primary ventricular hemorrhage due to moyamoya disease]

The clinical and pathological features of 20 cases of moyamoya disease complicated with primary intraventricular hemorrhage (PIVH) and verified by CT scanning, cerebral angiography and autopsy were reported. It seemed to the author that moyamoya disease appeared to be prone to cause PIVH, this hemorrhage being likely due to ischemic malacia in the ventricular wall. The tortuous, abnormal network vessels ruptured and bled as a result of infarction and damage of the walls of the miliary aneurysms. PIVH might be divided into two types: the panventricular hemorrhage and partial ventricular hemorrhage, according to the amount of blood that filled into the ventricles. 6 cases of the panventricular type were fatal. The symptoms were in conformity with the traditional concept of the most critical, intraventricular hemorrhage. The external ventricular drainage in combination with lumber puncture drainage would be the better way of treatment for these patients. 14 cases of the partial ventricular hemorrhage type showed the clinical features of acute subarachnoid hemorrhage (SAH). The treatment was similar to SAH too, and the prognosis was much better. It would be worth noting that moyamoya disease should be a factor not to be neglected in causing PIVH.     

单侧烟雾病的临床特征

【摘要】
目的 探讨单侧烟雾病的临床特征及预后。
方法 回顾性分析2003年1月～2010年1月在中国人民解放军第307医院神经外科就诊的单侧烟雾病患者,对患者的发病情况、影像学检查结果、治疗结果及预后情况进行分析。
结果 78例患者中男52例,女26例,成人52例,儿童26例,其中2例患儿合并家族性烟雾病。首发症状中24例患者表现为脑梗死,短暂性脑缺血发作（transient ischemic attack,TIA）18例,头痛17例,9例表现为脑出血（8例为成人）,其他表现共10例。46例患者为右侧病变,32例患者为左侧病变。73例患者接受手术,69例患者接受随访。术后6例出现TIA,2例出现新发脑梗死,9例脑出血患者未再发生脑出血。32例患者接受血管造影复查,29例显示颅内外已建立良好的侧支循环。2例患者术后对侧血管出现狭窄或闭塞,按复查血管病变是否存在对侧进展,分为进展组和非进展组,其中进展比例在本组为6.3%（2/32）。在进展组儿童及伴有家族史所占比例均为100%,与非进展组这两项比较P值分别为0.043和0.002,差异具有显著性（P<0.05）。
结论 具有家族史的儿童患者可能是对侧进展的预测因素;手术后能建立明确的侧支循环,改善临床症状。     

Heparin therapy for stroke: hemorrhagic complications and risk factors for intracerebral hemorrhage

Hemorrhages occurred in 16 (3.1%) of 510 patients treated with continuous intravenous heparin for acute cerebral infarction (269), reversible ischemic neurologic deficit (81), or transient ischemic attack (160). Three patients (0.6%) had intracerebral hematomas. Risk factors included abnormal CT within 24 hours of onset of symptoms (3.2%), severe neurologic deficit (2.8%), two acute infarcts by CT (2.1%), known source of embolus (1.3%), and final diagnosis of cerebral infarction (1.1%). The only identifiable risk factor for systemic hemorrhage (GI 1.0%, GU 0.8%, muscle 0.4%, skin 0.1%) was age over 60 years. The incidence of intraspinal hematoma was 0.6%. Two of the intracerebral hematomas were fatal, and mortality was 31% in patients with hemorrhagic complications; however, the risk of CNS hemorrhage was only 0.8%.     

儿童出血型烟雾病的损伤模式

【摘要】
目的 分析儿童出血型烟雾病的损伤模式。
方法 回顾性分析中国人民解放军第307医院2002年12月～2011年12月收治的26例出血型烟雾病患儿,根据出血部位发生丘脑出血2例,非丘脑性脑实质出血（基底节和脑叶）3例,脑室出血21例。按照改良的Morioka分级对所有患者的脑血管造影的出血侧脉络膜前动脉和后交通动脉（anterior choroidal artery and posterior communicating artery,AChA-PComA）进行分级,并分析出血病灶分布模式与出血侧AChA-PComA分级及脉络膜后动脉分级的关系。
结果 所有患儿均发生1次出血。4例患儿合并动脉瘤,其中1例为大脑前动脉瘤,1例为脉络膜前动脉瘤,2例为脉络膜后动脉瘤;所对应出血类型均为脑室出血。AChA-PComA正常或轻度扩张阶段,非丘脑性脑实质出血比例最大（2/3,66.7%）,脉络膜后动脉均未显影;AChA-PComA极度扩张和延长阶段,脑室出血所占比例最大（16/17,94.1%）（P＜0.05）,脉络膜后动脉均未显影;AChA-PComA未显影阶段,脑室出血比例仍最大（4/6,66.7%）,其次为丘脑出血（2/6,33.3%）,脉络膜后动脉均表现为极度扩张和延长（P＜0.05）。
结论 儿童出血型烟雾病中出血类型主要为脑室出血;脑室出血患者相对其他出血类型AChA-PComA极度扩张和延长所占比例高;当颈内动脉在后交通动脉闭塞时即AChA-PComA未显影阶段,脉络膜后动脉均表现为扩张和延长。     

儿童缺血型烟雾病的临床和影像分析

【摘要】
目的 分析儿童缺血型烟雾病患者的临床和影像特点,探讨脑梗死的相关因素。
方法 回顾性分析2011年6月～2012年6月收治的27例儿童缺血型烟雾病患者的临床及影像学资料。
大脑半球分为无脑梗死半球32侧和脑梗死半球22侧,将患者性别、年龄、造影特点（包括54侧半球
的Suzuki分期、大脑后动脉分期,眼动脉、颞浅动脉、脑膜动脉、枕动脉、上颌动脉和后循环向前循
环代偿）与脑梗死的发生进行统计分析。
结果 27例患者的临床资料显示性别和年龄与脑梗死的发生差异不具有显著性（性别χ2=2.095,
P =0.148;年龄χ2=4.286,P =0.453）。根据半球计算：皮质脑梗死17侧（17/22）,额叶梗死14侧
（14/22）,皮质下脑梗死9侧（9/22）。无梗死侧半球和梗死侧半球Suzuki分期以及大脑后动脉分期
的差异具有显著性（Suzuki分期Z=－3.054,P =0.002;大脑后动脉分期Z=－4.557,P <0.001）。眼动脉、
颞浅动脉、脑膜动脉、枕动脉、上颌动脉和后循环向前循环代偿与脑梗死的发生差异不具有显著性。
结论 本研究显示儿童缺血型烟雾病皮质脑梗死较皮质下脑梗死常见。在皮质脑梗死中,额叶脑
梗死最常见。脑梗死的发生与Suzuki分期和大脑后动脉受累程度有关。     

S-100 protein and neuron-specific enolase in cerebrospinal fluid and serum: markers of cell damage in human central nervous system

The development of a radioimmunoassay for S-100 protein is described. This method was used in combination with a recently developed radioimmunoassay for neuron-specific enolase in cerebrospinal fluid and serum from 47 patients with cerebral infarction, transient ischemic attack, intracerebral hemorrhage, subarachnoid hemorrhage, and head injury. In cerebrospinal fluid, increased concentrations of both S-100 and neuron-specific enolase were found after large infarcts, whereas after small infarcts and transient ischemic attacks, only neuron-specific enolase increased. The increased concentrations of S-100 and/or neuron-specific enolase were noted 18 hours to 4 days after cerebral infarction and transient ischemic attacks. Cerebrospinal fluid concentrations of these proteins also reflected the severity of the disease in patients with intracerebral hematoma, subarachnoid hemorrhage, or head injury. Temporal changes in serum S-100 and neuron-specific enolase concentrations reflected the clinical course in 4 patients. In stroke patients, the S-100 and neuron-specific enolase concentrations may reflect the extent of brain damage and could be useful in selecting patients with major stroke for more aggressive treatment during the acute phase.     

螺旋CT 血管生长技术及脑血管造影在烟雾病诊断中的价值

目的 探讨使用多排螺旋CT 血管生成技术(MSCTA)及脑数字减影(DSA)血管造影在烟雾病(MMD)中的诊断价值.方法 采用8 排螺旋CT 进行头部螺旋容积扫描,分析23 例烟雾病的CT 平扫及增强的影像表现,利用"血管生长(add vessel)"法对图像进行后处理,采用多平面体积重组及容积重建图像对脑血管进行三维重建并和DSA 结果进行比较分析.结果 CT 平扫13 例患者显示不同部位的脑出血,5 例患者表现为多发性的脑梗死,3 例患者仅见局限性的脑萎缩,2 例患者CT 平扫未发现明显异常.MSCTA 均清晰显示不同程度的颈内动脉颅内段和(或)大脑前动脉、大脑中动脉狭窄或闭塞,23 例均显示纤细迂曲的"烟雾"状异常血管网.其中15 例经股动脉插管行全脑数字血管减影(DSA)证实.结论 采用血管生成、容积重建技术,MSCTA 是明确诊断烟雾病的有效非创伤性首选检查方法,可替代DSA 进行烟雾病的筛选、诊断和随访观察.     

成人烟雾病的临床特点及影像分析

目的　研究成人型烟雾病的临床特点、影像表现及其两者的关系。方法　 6 1例经常规血管造影 (包括 DSA)和 MRA确诊的成人型烟雾病患者 (>16岁 )。分析其临床表现及血管造影结果。结果　主要临床表现为突发剧烈头痛、意识障碍 ,伴呕吐、失语及一侧肢体瘫痪。在 CT和 MRI上 ,脑出血 4 1例 (6 7.2 % ) ,脑梗死 15例(2 4 .6 % ) ,脑萎缩 2例 ,无异常表现 3例。在 DSA和 MRA上 ,双侧病变者 32例 ,单侧病变 2 9例 ,5 1例可见到烟雾状异常血管网 ,尤其在出血型患者中。后交通动脉增粗 38支、眼动脉增粗 31支及软脑膜吻合支增多 4 4侧。 5例大面积脑梗死侧支循环很差或缺乏。结论　成人型烟雾病以脑出血多见 ,在发病率和血管造影上和儿童无明显差异。治疗应首选血管重建手术。 DSA为诊断金标准 ,为术前必要检查 ;MRA无创伤性 ,为本病筛选、诊断、随访的首选方法。     

阿司匹林在成人缺血型烟雾病血管重建术后的应用

目的 探讨阿司匹林在成人缺血型烟雾病血管重建术后的应用效果。方法 回顾性分析2017年10月至2019年10月收治的符合入选标准的81例成人缺血型烟雾病的临床资料。47例术后应用阿司匹林（观察组）,34例未使用阿司匹林（对照组）。结果 观察组吻合口通畅率（97.87%,46/47）与对照组（94.12%,32/34）无统计学差异（P>0.05）。术后3个月,观察组改良Rankin量表评分[（1.38±1.05）分]明显低于对照组[（1.88±1.01）分;P<0.05]。观察组术后短暂性脑缺血发作发生率（4.26%）、脑梗死发生率（6.38%）均明显低于对照组（分别为23.53%、26.47%;P<0.05）。两组颅内出血、硬膜下血肿发生率均无统计学差异（P>0.05）。结论 阿司匹林可减少成人缺血型烟雾病血管重建术后脑缺血事件,同时改善病人预后,并且不增加颅内出血风险