37例烟雾病的临床特点及影像学分析

该文回顾性分析了15例儿童烟雾病和22例成人烟雾病患者首发症状、临床表现及影像学,其中儿童患者以缺血型为主(80%),成年患者以出血型(81.8%)为主。37例均行数字减影脑血管造影(DSA),其中14例同时行头颅磁共振血管成像(MRA)检查,结果显示,成人患者的血管狭窄程度重于儿童。烟雾病临床表现多样,儿童和成人烟雾病在临床表现、发病类型、影像学表现上均有一定差别。DSA和磁共振血管成像(MRA)是诊断烟雾病有效的检查方法。     

匹罗卡品致(癎)大鼠海马中表达生长抑素的中间神经元数目变化及其轴突出芽

Objective To investigate the roles of somatostatin(SS)positive intemeurons in the development and compensation of temporal lobe epilepsy.Methods Piloearpine-induced epilepsy rat model was established.Immunohistochemistry method was used to detect number changes and axonal sprouting of SS positive intemeurons in different domains of the hippocampus at difierent time points.Degeneration of SS positive interneurons and their neurophils were detected by the double immunofluorescence staining with SS and Fluoro-Jade B(FJB)at 7 and 60 days after status epilepticus (SE).Results In the exoerimental rat group,the number of SS positive neurons decreased in each hippocampal domain,and it reached the lowest at 7 days post-SE(There were 11.1±3.3 in hilus,2.8±0.9 in CA1region and 1.8±0.7 in CA1region,t=13.519,9.644 and 8.808,all P<0.01).In chronic phase,the number of SS neurons gradually recovered,and exceeded the control group in CA1 area at 60 days post-SE(12.8±1.5 vs 8.8±1.3,t=-4.506,P<0.01),however,the number of SS neurons in the hilus(25.5±4.6)and CA1 area(4.8±0.8)remained significantly less than normal levels(t value were 4.691 and 3.953.both P<0.01).Increased SS positive fibers were found in the lacunosum-molecular (1m)layer and outer molecular layer of dentate gyrus after 30 days post-SE,and numerous SS positive fibers were seen threnghout the layers of area CA1 at 60 days post-SE.Double immunofluuorescence revealed that a few SS positive interneurons and fibers were also labeled by FJB in area CA1 at 7 days post-SE and in CA domain/hilus at 60 days post-SE.Conclusions SS intemeurons loss plays an important role in the development of temporal lobe epilepsy.The loss is partially caIlsed by the degeneration and death of neurons;SS positive neurophils increase within area CA1 in chronic phase may play a significant role in the generation and compensation of temporal lobe epilepsy.     

匹罗卡品致(癎)大鼠海马中表达生长抑素的中间神经元数目变化及其轴突出芽

Objective To investigate the roles of somatostatin(SS)positive intemeurons in the development and compensation of temporal lobe epilepsy.Methods Piloearpine-induced epilepsy rat model was established.Immunohistochemistry method was used to detect number changes and axonal sprouting of SS positive intemeurons in different domains of the hippocampus at difierent time points.Degeneration of SS positive interneurons and their neurophils were detected by the double immunofluorescence staining with SS and Fluoro-Jade B(FJB)at 7 and 60 days after status epilepticus (SE).Results In the exoerimental rat group,the number of SS positive neurons decreased in each hippocampal domain,and it reached the lowest at 7 days post-SE(There were 11.1±3.3 in hilus,2.8±0.9 in CA1region and 1.8±0.7 in CA1region,t=13.519,9.644 and 8.808,all P<0.01).In chronic phase,the number of SS neurons gradually recovered,and exceeded the control group in CA1 area at 60 days post-SE(12.8±1.5 vs 8.8±1.3,t=-4.506,P<0.01),however,the number of SS neurons in the hilus(25.5±4.6)and CA1 area(4.8±0.8)remained significantly less than normal levels(t value were 4.691 and 3.953.both P<0.01).Increased SS positive fibers were found in the lacunosum-molecular (1m)layer and outer molecular layer of dentate gyrus after 30 days post-SE,and numerous SS positive fibers were seen threnghout the layers of area CA1 at 60 days post-SE.Double immunofluuorescence revealed that a few SS positive interneurons and fibers were also labeled by FJB in area CA1 at 7 days post-SE and in CA domain/hilus at 60 days post-SE.Conclusions SS intemeurons loss plays an important role in the development of temporal lobe epilepsy.The loss is partially caIlsed by the degeneration and death of neurons;SS positive neurophils increase within area CA1 in chronic phase may play a significant role in the generation and compensation of temporal lobe epilepsy.     

匹罗卡品致(癎)大鼠海马中表达生长抑素的中间神经元数目变化及其轴突出芽

Objective To investigate the roles of somatostatin(SS)positive intemeurons in the development and compensation of temporal lobe epilepsy.Methods Piloearpine-induced epilepsy rat model was established.Immunohistochemistry method was used to detect number changes and axonal sprouting of SS positive intemeurons in different domains of the hippocampus at difierent time points.Degeneration of SS positive interneurons and their neurophils were detected by the double immunofluorescence staining with SS and Fluoro-Jade B(FJB)at 7 and 60 days after status epilepticus (SE).Results In the exoerimental rat group,the number of SS positive neurons decreased in each hippocampal domain,and it reached the lowest at 7 days post-SE(There were 11.1±3.3 in hilus,2.8±0.9 in CA1region and 1.8±0.7 in CA1region,t=13.519,9.644 and 8.808,all P<0.01).In chronic phase,the number of SS neurons gradually recovered,and exceeded the control group in CA1 area at 60 days post-SE(12.8±1.5 vs 8.8±1.3,t=-4.506,P<0.01),however,the number of SS neurons in the hilus(25.5±4.6)and CA1 area(4.8±0.8)remained significantly less than normal levels(t value were 4.691 and 3.953.both P<0.01).Increased SS positive fibers were found in the lacunosum-molecular (1m)layer and outer molecular layer of dentate gyrus after 30 days post-SE,and numerous SS positive fibers were seen threnghout the layers of area CA1 at 60 days post-SE.Double immunofluuorescence revealed that a few SS positive interneurons and fibers were also labeled by FJB in area CA1 at 7 days post-SE and in CA domain/hilus at 60 days post-SE.Conclusions SS intemeurons loss plays an important role in the development of temporal lobe epilepsy.The loss is partially caIlsed by the degeneration and death of neurons;SS positive neurophils increase within area CA1 in chronic phase may play a significant role in the generation and compensation of temporal lobe epilepsy.     

遗传性痉挛性截瘫一家系的临床特点与spastin基因突变分析

目的　探讨常染色体显性遗传的遗传性痉挛性截瘫 (SPG)一家系的临床特点,并行spastin基因突变分析。方法　对整个家系进行详细的临床检查,先证者和另一例家系内患者进行了心肌酶学、头颅核磁共振成像(MRI)、胸髓MRI、肌电图、体感诱发电位检查。应用聚合酶链式反应 单链构象多态性(PCR SSCP)结合DNA序列分析方法,检测该家系中所有 5例患者和 4名有血缘关系的健康人及家系外 50名无血缘关系健康对照者spastin基因的突变情况。结果　先证者和家系内另一例SPG患者胸髓MRI显示胸髓萎缩;PCR SSCP检测发现家系内患者均出现异常SSCP电泳带,经测序证实为Leu378Gln突变。结论　该常染色体显性遗传SPG家系的患者具有典型的临床表现,为spastin基因Leu378Gln突变所致。     

咪唑类驱虫药致迟发性脑病45例分析

左旋咪唑和阿苯达唑属于咪唑类驱虫药,临床上广泛用来驱虫,有报道此药可引起变态反应性脑病.我们1994-10～2002-12收治口服咪唑类驱虫药致迟发性脑病45例分析如下.     

可逆性后部白质脑病综合征合并HELLP综合征的临床分析

目的分析可逆性后部白质脑病综合征(PRIS)合并HELLP综合征患者的临床特征,提高对该病的认识。方法回顾性分析我院诊治1例及国内文献报道4例.PRLS合并HELLP综合征的临床表现、实验室及影像学检查、治疗和转归。结果5例患者中出现严重高血压4例,头痛5例,癫痫发作5例,视觉异常5例,意识障碍5例,精神障碍2例,局灶性神经系统定位体征3例,肢体水肿1例。实验室检查均提示有溶血、肝酶升高、血小板减少。5例患者头部cT或MRI检查均显示双侧顶枕叶白质为主的对称性异常,部分累及额叶、颞叶,病灶在cT为低密度灶,MRI为长T_1长T_2信号灶。结论 RPLS同时合并HELLP综合征是妊高征患者罕见的并发症,充分认识其临床和影像学特点,有助于及时诊断和治疗。     

左旋咪唑致脱髓鞘脑病的临床与影像学特征

左旋咪唑致脱髓鞘脑病的临床与CT表现在国内已有报道,现将 1995年8月～2002年8月中南大学湘雅医院神经内科确诊的32例本病患者的临床及影像学资料分析如下.     

Arnold-Chiari Ⅰ型畸形的临床及误诊分析

目的 :研究 2 1例Arnold -ChiariⅠ型畸形患者的临床及误诊原因。方法 :回顾性分析 2 1例Arnold -ChiariⅠ型畸形患者的临床资料。结果 :本病大多合并脊髓空洞症 ,临床主要为枕骨大孔区、脊髓中央管及小脑受损征侯群。临床表现多样 ,缺乏特异性是误诊的主要原因。结论 :本病的诊断依靠其临床特点及磁共振检查。     

妥泰治疗成人部分性癫痫发作的临床研究

目的 :观察加用及单用妥泰二种方式治疗癫痫患者的临床疗效和副反应 ,探讨单用妥泰更快、更有效的给药方式。方法 :加用组 (A组 ) ,5 0例病人 ,在服用卡马西平或丙戊酸钠的基础上加用妥泰 2 5 mg/ d,增量 2 5 mg/ w至2 0 0 mg/ d。单药组 :按初始剂量及加量速度不同又分为 B1 组 ,B2 和 B3组。 B1 组妥泰 2 5 mg/ d,增量 2 5 mg/ w至 2 0 0 mg/d;B2 组妥泰初始剂量 5 0 mg/ d,增量 2 5 mg/ w至 2 0 0 mg/ d;B3组妥泰初始剂量 5 0 mg/ d,增量 2 5 mg/ 3d至 2 0 0 mg/ d。结果 :各组患者托吡脂总有效率分别为加用组 5 8% ,初始剂量 2 5 mg/ d组 6 4 .3% ,初始剂量 5 0 mg/ d,增量 2 5 mg/周组6 0 % ;初始剂量 5 0 mg/ d,增量 2 5 mg/ 3d组 6 5 % ,3组单用托吡脂总有效率比较无明显差异 (P>0 .0 5 )。出现率比较高的副作用为胃纳差 ,体重减轻和嗜睡、头晕 ,初始剂量 5 0 mg/ d的两组 (B2 和 B3组 )副作用出现率高于加用组及小剂量组 (P<0 .0 5 ) ,但都比较轻。结论 :本研究提示托吡脂可给予较大初始剂量 (5 0 mg/ d) ,以较快速度增量 (2 5 mg/ 3d)。