脑血管肌纤维发育不良的临床和影像学特点(附4例报告)

目的 探讨脑血管肌纤维发育不良(FMD)的临床和影像学特点.方法 对4例经DSA及MR血管成像(MRA)检查确诊的脑血管FMD患者的临床资料进行回顾性分析.结果 4例患者中,3例为青年人(18～27岁),1例为中年女性(48岁),均无卒中史.所有患者均表现为缺血性脑血管病,3例为脑梗死,1例为短暂性脑缺血发作.影像学检查发现2例颈内动脉颅外段及2例右侧大脑后动脉(PCA)呈串珠样改变.1例颈动脉FMD患者合并大脑中动脉夹层及大脑前动脉微小动脉瘤,1例大脑后动脉FMD患者合并颈内动脉颅外段夹层.结论 脑血管FMD常导致无卒中史的中青年人出现缺血性脑血管病的发生,影像学特点为受累血管呈串珠样改变.     

脑血管病患者的脑血流自身调节潜力分析

目的 应用TCD和倾斜试验评价脑血管病患者的脑血流自身调节.方法 脑血管狭窄患者32例(A组),无血管狭窄的脑梗死患者28例(B组),健康对照26名.通过呼吸试验计算呼吸抑制指数(BHI)及血管运动反应性(VMR)评价脑小血管CO2反应性;头高位70°倾斜试验改变体位,记录卧立位脑血流速度(CBFV)及血压、心率,评价脑小血管的血压-自动调节.结果 与对照组比较,A组、B组VMR(分别为0.18±0.02和0.26±0.04,对照组为0.43±0.06)、BHI(分别为0.76±0.15和1.05±0.15,对照组为1.52±0.19)显著降低(P＜0.05).A组患侧与对侧VMR(分别为0.10±0.01、0.22±0.02)及BHI(分别为0.51±0.14、0.94±0.16),差异有统计学意义(P＜0.05).A组患侧BHI值小于0.69(P＜0.05).Logistic回归分析显示BHI下降与脑梗死相关(B=2.234,P=0.016).直立位时血压、心率增加,CBFV下降.3例直立性低血压患者直立位血压和CBFV均显著下降,二者之间相关(r=0.430,P=0.004).结论 脑梗死及脑血管狭窄患者脑小血管CO2反应性受损.自身调节能力受损,卒中风险可能增加.直立位时,机体通过提高周围血压和心率,参与颅内自动调节.     

MRI/~1H-MRS在MELAS综合征与卒中鉴别诊断中的应用研究

目的探讨线粒体脑肌病伴高乳酸血症及卒中样发作(MELAS)综合征头颅磁共振(MRI)及头颅磁共振波谱(~1H-MRS)特点与其早期诊断价值。方法对8例诊断为MELAS综合征患者的MRI及MRS结果进行分析。结果 8例MELAS综合征患者的头颅MRI结果示病变累及多个脑叶,主要包括额叶、颞叶、顶叶、枕叶皮质及双侧基底核、放射冠区,其中4例患者可见不同程度的脑萎缩;头颅磁共振血管造影(MRA)检测均未见颅内动脉异常,且病灶区域与主要供血动脉分布无明显相关性;4例患者头颅~1H-MRS可见病变区域显著Lac峰,以及病灶区NAA峰显著降低。结论头颅~1H-MRS能够为MELAS综合征患者提供无创性检查,分析脑组织能量代谢变化,对MELAS综合征的早期诊断具有重要价值。     

吸入自体CO2 对脑动脉狭窄患者的颅内血流自身调节潜力分析

目的通过经颅多普勒超声(transcranial doppler,TCD)观察血液CO2浓度改变研究脑动脉狭窄患者的颅内小血管调节潜力,建立实用的评价脑血流自身调节潜力的检查方法。方法选择具有颅内外血管狭窄的患者20例为脑血管狭窄组,并选择年龄配比的无血管狭窄者18名为对照组,行双侧MCA连续监测脑血流速度变化,通过屏气改变血液CO2浓度,吸入自体CO2以旁流监测仪监测CO2浓度的变化,分别记录脑血流速度、及血压、心率等,计算出呼吸抑制指数(breath-holding index,BHI),血管运动反应性(vascularmotor reactivity,VMR)及脑血管运动储备(cerebrovascular reserve capacity,CRC)数值评价脑小血管CO2反应性。结果通过呼吸抑制试验,自体CO2吸入试验结合TCD连续监测脑血流速度的检查方法。呼吸试验结果显示血管狭窄患者VMR(0.23±0.09vs 0.40±0.10,P<0.001)及BHI值(0.46±0.18 vs 0.81±0.20,P<0.001)与对照组比较均有降低。血管狭窄组血管狭窄侧BHI值小于0.69(P=0.014)...     

16例MELAS临床、神经影像与肌肉病理研究

目的探讨线粒体脑肌病并乳酸血症与卒中样发作(MELAS)的临床、影像学及肌肉病理特点。方法对16例MELAS患者的临床表现、影像学及肌肉病理特点进行分析。结果患者主要临床表现为卒中样癫发作(87.5%),其次为头痛、呕吐和智能减退等;头颅MRI提示脑实质片状异常信号,不按血管供应区分布;肌肉活检见破碎红纤维(RRF)。结论MELAS是一种具有特殊临床、影像学表现的线粒体脑肌病,其诊断依赖于肌肉活检病理和基因诊断。     

阻塞性呼吸睡眠暂停综合征与高血压并存患者脑血管功能状态的TCD初探

目的 研究分析阻塞性呼吸睡眠暂停综合征(OSAS)与高血压并存患者脑血管功能状态.方法 采用EME公司的COMPIONEⅡ型号经颅多普勒检查仪(TCD),对85例OSAS与高血压并存患者进行TCD检查,判断血管状态,采用屏气试验评价脑血管反应性,记录大脑中动脉(MCA)的平均血流速度(Vm)、搏动指数(PI),屏气指数(BHI)并与对照组比较,进行统计学分析.结果 两组间血管异常情况经χ~2检验,χ~2=4.1,P<0.05,差异有统计学意义.两组间的Vm经配对t检验,t=1.80,P>0.05差异无统计学意义.两组间的PI经配对t检验,t=1.62,P>0.05,差异无统计学意义.两组间BHI经配对t检验,t=4.75,P<0.01,差异有统计学意义.结论 OS-AS与高血压并存患者的脑血管功能状态与对照组比较脑血管反应性较差,更易发生动脉硬化,形成管腔狭窄.     

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(附1例报告)

目的分析典型线粒体脑肌病伴高乳酸血症和卒中样发作(MELAS)综合征的临床特点。方法回顾性分析1例典型MELAS综合征的临床资料。结果本例患者以言语混乱、行为异常等卒中样症状发病,有母系家族史,病程中有头痛及癫痫样发作。入院查体示听力下降,视野缺损,肌力下降。头颅CT及MRI提示卒中样病灶,且病灶不按血管走行分布。血及CSF检查排除病毒性、自身免疫性脑炎,最终经基因检测分析(mt DNA的A3243G位点AG突变)明确诊断为MELAS综合征。结论 MELAS综合征临床表现主要包括卒中样发作、癫痫、头痛、痴呆、听力损伤、周围神经病变、肌病、乳酸血症、糖尿病等,影像学表现主要包括卒中样病灶、基底节钙化及脑萎缩。     

脑分水岭梗死患者脑血管病变的研究

目的 研究脑分水岭梗死(CWI)患者脑血管病变的状况.方法 对48例幕上CWI患者行头颅MRI和MR血管成像(MRA)检查.根据梗死部位分为外侧型、内侧型和混合型CWI.梗死灶同侧供血动脉狭窄＞50％或中断为病变责任血管.结果 本组CWI患者MRA检查发现责任血管39例(81.3％),大血管病变80处;外侧型、内侧型和混合型CWI患者的责任血管检出率及其分布的差异无统计学意义.有串珠样梗死灶的患者大血管病变的比率(95.6％)显著高于无串珠样梗死灶患者(68.0％)(P＜0.05).结论 CWI患者颅内责任血管的检出率较高,各类型CWI之间无明显差异;有串珠样梗死灶的CWI患者多为颅内大血管病变所致.     

抑郁症患者血管内皮功能的异常改变

目的:探讨抑郁症患者血管内皮功能的改变。方法:运用日立-图腾彩色多普勒超声诊断仪高频探头检测24例抑郁症患者(抑郁症组)和20名健康对照者(健康对照组)肱动脉反应性充血前后血管内径的变化(即血流介导的内皮依赖性血管扩张,FMD)及舌下含服硝酸甘油后的内径变化(即非内皮依赖性舒张功能,NMD);同时测定两组血浆一氧化氮水平(NO);采用汉密尔顿抑郁量表(HAMD)和Beck抑郁量表(BDI)评定抑郁症组患者抑郁的严重程度。结果:与健康对照组相比,抑郁症组FMD较健康对照组明显降低(t=3.62,P0.01);两组NMD比较差异无统计学意义(t=1.55,P0.05)。抑郁症组的血浆NO水平明显低于健康对照组(t=3.63,P0.01)。相关分析显示,抑郁症组HAMD及BDI得分与FMD负相关(r=-0.687,r=-0.621;P均0.05);两组血浆NO水平与FMD正相关(r=0.726,r=0.743;P均0.05)。结论:抑郁症患者血管内皮功能受损可能与血浆NO水平减低及抑郁严重程度有关。     

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。     

Customer, Client, Consumer, Recipient, or Patient

Since the arrival of managed care, there has been a trend toward changing the basic terminology used to address clinicians and patients. Instead of the term patient, third party payors frequently use terms such as customer, client, consumer or recipient. One study demonstrated that patients prefer to be called patients. To investigate the preferred term to refer to patients and to be referred to by patients, we mailed a questionnaire to 100 physicians in four medical specialties each and to 100 psychologists. The overall response rate was 61%. Physicians overwhelmingly preferred to refer to patients by the patient's last name, their second preference was the patient's first name. Psychologists preferred to refer to the patients by first name, their second preference was the patient's last name. No group favored using terms such as client, customer, consumer, or recipient. Most physicians and psychologists preferred being referred to as doctors and nobody favored the term provider.     

Fasciitis, perimyositis, myositis, polymyositis, and eosinophilia

Several groups of cases of fasciitis and myositis with eosinophilia are reported. The common features are inflammation into fascia and/or perimysium, and/or muscle fibers; eosinophilia in blood and/or in muscle biopsy. The following classification of 24 cases is suggested: at one end of the spectrum are fasciitis with eosinophilia: diffuse fasciitis (Shulman syndrome): 10 cases (3 with hematological complications); 2 cases of diffuse fasciitis with muscle atrophy; 3 cases of restricted fasciitis. Relapsing perimyositis with eosinophilia belong to the same spectrum, either diffuse (5 cases) with myalgias, or localized (2 cases). Other cases are focal myositis or multiple myositis, polymyositis with eosinophilia. The relationship among these cases is discussed. There is a continuum among the different groups. The pathophysiology remains unknown.     

高海拔地区缺血性卒中患者单核 细胞/HDL-C比值与脑动脉粥样硬化性狭窄的相关性

目的 研究高海拔地区缺血性卒中患者单核细胞/HDL-C比值（monocyte/HDL-C ratio,MHR）与颅内动脉粥样硬化性狭窄（intracranial atherosclerotic stenosis,ICSA）程度的相关性。 方法 回顾性连续纳入2017年6月-2021年6月在青海省人民医院住院治疗的高海拔地区（海拔2260～4080?m）的急性缺血性卒中患者,依据DSA上脑血管狭窄程度（以狭窄最严重的动脉为准）分为无狭窄组、轻度狭窄（狭窄率≤50%）组、中度狭窄（狭窄率50%～70%）组、重度狭窄（狭窄率≥70%）组及闭塞（100%）组。比较5组患者的临床资料、实验室检查指标和MHR,并采用logistic回归模型计算不同程度血管狭窄的独立危险因素。 结果 共纳入349例患者,其中无狭窄组69例、轻度狭窄组78例、中度狭窄组41例、重度狭窄组84例、闭塞组77例。5组中年龄、性别分布、吸烟、饮酒、高血压、糖尿病比例方面差异均有统计学意义,实验室检查中白细胞、单核细胞、中性粒细胞、血小板计数以及血红蛋白、HDL-C水平和MHR差异也有统计学意义。多因素logistic回归分析显示,相对于无动脉狭窄,高龄为脑血管轻度狭窄（OR?1.061,95%CI?1.027～1.097,P＜0.001）,中度狭窄（OR?1.057,95%CI?1.017～1.099,P＝0.005）,重度狭窄（OR?1.096,95%CI?1.057～1.137,P＜0.001）,闭塞（OR?1.036,95%CI?1.001～1.072,P＝0.046）的独立危险因素;相对于无动脉狭窄,高MHR为轻度狭窄（OR?1.041,95%CI?1.009～1.074,P＝0.011）,中度狭窄（OR?1.082,95%CI?1.045～1.119,P＜0.001）,重度狭窄（OR?1.096,95%CI?1.062～1.131,P＜0.001）,闭塞（OR?1.101,95%CI?1.067～1.136,P＜0.001）的独立危险因素;相对于无动脉狭窄,单核细胞计数升高是中度狭窄（OR?1.684,95%CI?1.569～2.725,P=0.027）、重度狭窄（OR?3.529,95%CI?1.541～5.766,P=0.002 ）和闭塞（OR?5.446,95%CI?4.453～6.917,P=0.002）的独立危险因素。 结论 高龄、高MHR和单核细胞计数升高在高海拔地区对急性缺血性卒中患者的脑动脉粥样硬化性狭窄程度具有一定预测价值。     

Pregabalin effect on steady-state pharmacokinetics of carbamazepine, lamotrigine, phenobarbital, phenytoin, topiramate, valproate, and tiagabine

By reducing neuronal excitability through selective binding to the α(2)δ subunit of voltage-dependent calcium channels, pregabalin effectively treats epilepsy, chronic pain, and anxiety disorders. To evaluate if pregabalin coadministration affects pharmacokinetics of other antiepileptic drugs, population pharmacokinetic analyses using NONMEM software were performed on data from three epilepsy trials involving seven antiepileptic drugs with pregabalin as add-on therapy. Results demonstrated that pregabalin did not alter the steady-state plasma concentrations of carbamazepine, lamotrigine, phenobarbital, phenytoin, tiagabine, topiramate, and valproate. Furthermore, the small percent change in the population estimate of antiepileptic drug plasma clearance values (-2% to +7%) suggests that pregabalin coadministration exerted no significant effect on the pharmacokinetics of these antiepileptic drugs, with the possible exception of tiagabine (+34.9%). These findings are in agreement with those of previously published reports. A further clarification study is necessary for tiagabine. In conclusion, it appears that pregabalin can be coadministered with other antiepileptic drugs without concern for significantly altering their pharmacokinetic profiles.     

Clobazam, Oxcarbazepine, Tiagabine, Topiramate, and Other New Antiepileptic Drugs

Summary: Clinical investigators recently have studied at least 21 new antiepileptic drugs (AEDs) in people with epilepsy. This review briefly examines 15 of these new AEDs: clobazam (CLB), dezinamide, flunarizine (FNR), loreclezole, milacemide (MLM), MK-801, nafimidone, ORG-6370, oxcarbazepine (OCBZ), progabide (PGB), ralitoline, stiripentol, tiagabine (TGB), topiramate (TPM), and zonisamide (ZNS). CLB, PGB, and TGB represent agents that act on the GABA system, and MLM acts on the glycine system. MK-801 and ZNS (in part) are excitatory amino acid antagonists, and FNR is a calcium-channel antagonist. OCBZ is a keto analogue of carbam-azepine, which is not metabolized to the epoxide and may have fewer side effects. The remaining agents are novel compounds with a variety of suspected mechanisms. TPM appears especially effective for intractable partial seizures but has a high incidence of cognitive side effects. None of these new AEDs is useful for all patients with inadequate seizure control or ongoing toxicity. The role of each will require further clinical study and experience.     

Consecutive Gas Chromatographic Determination of Phenytoin, Phenobarbital, Primidone, Phenylethylmalondiamide, Carbamazepine, Trimethadione, Dimethadione, Ethosuximide, and Valproate from the Same Serum Specimen

A quantitative gas-liquid chromatographic procedure is described for the consecutive determination of phenytoin, phenobarbital, primidone, phenylethylmalondiamide, carbamazepine, trimethadione, dimethadione, ethosuximide and valproate from a single serum specimen of 1.2 ml. After extraction from serum by two different procedures, the anticonvulsants are chromatographed without further purification on a 3% OV 17 column either with or without derivative formation by means of "on-column" methylation. Multiple internal standards are employed in order to enhance the reproducibility of drug-concentration measurement.