癫痫患儿的免疫功能及免疫球蛋白辅助治疗的初步研究

目的：探讨癫痫患儿的免疫功能状态及免疫球蛋白辅助治疗的疗效。方法：测定５４例癫痫患儿血中ＩｇＧ、ＩｇＡ、ＩｇＭ、Ｓ３及Ｔ细胞亚群，与正常对照组比较；对１４例常规抗癫痫治疗效果不佳者加用免疫球蛋白辅助治疗，观察疗效。结果：癫痫患儿血清ＩｇＧ降低（Ｐ＜０．０５），ＩｇＡ降低（Ｐ＜０．０１），ＩｇＭ及Ｃ３正常，ＣＤ３、ＣＤ４降低（Ｐ＜０．０５），ＣＤ８升高（Ｐ＜０．０１），ＣＤ４／ＣＤ８降低（Ｐ＜０．０５），免疫球蛋白辅助治疗有效率５７．２％。结论：癫痫患儿存在免疫功能紊乱，提示免疫机制参与了癫痫发病机制；免疫球蛋白辅助治疗癫痫有效。     

呼吸道病毒和格林－巴利综合征

用ＥＬＩＳＡ法检测了４５例ＧＢＳ患儿ＣＳＦ中ＲＳＶ－ＩｇＧ和ＰＩＶ－ＩｇＧ，发现其阳性率分别为３７。８％、２８．９％，与对照组有显著性差异。测定６例６ＢＳ患儿的血清和ＣＳＦ中ＲＳＶ－ＩｇＧ和ＰＩＶ－ＩｇＧ，并设立Ｓ／ＣＳＦ值，发现ＧＢＳ患儿Ｓ／ＣＳＦ值变化时，可能有三种情况：（１）ＢＢＢ受到损害，神经系统未发生病毒感染；（２）ＢＢＢ的完整无损，但ＣＮＳ内存在病毒感染；（３）ＢＢＢ的损害和病毒感染同时存在。     

格林—巴利综合征患者血清和脑脊液中的抗硫脂抗体

探讨抗硫脂抗体与格林－巴利综合征（ＧＢＳ）的关系。方法采用固相酶联免疫吸附法对急性期ＧＢＳ患者血清和脑脊液（ＣＳＦ）中抗硫脂ＩｇＧ和ＩｇＭ抗体进行检测。结果ＧＢＳ患者血清和ＣＳＦ中抗硫脂ＩｇＧ及ＩｇＭ抗体的阳性率均明显高于正常对照组；血清中抗硫脂ＩｇＭ抗体滴度与标本收集时患者发病天数呈负相关（Ｐ＜０．０５），而血清中抗硫脂ＩｇＧ抗体滴度与临床分级（Ｐ＜０．０１）、ＣＳＦ中抗硫脂ＩｇＧ抗体滴度（Ｐ＜０．０１）呈正相关；血清中抗硫脂ＩｇＧ或ＩｇＭ阳性的ＧＢＳ患者，体检时有不同程度的感觉障碍患者为５６％，而血清中抗硫脂抗体阴性患者仅为１６％，两者之间差异有显著意义（Ｐ＜０．０５）。结论抗硫脂抗体可能在ＧＢＳ的病理过程中起重要作用     

格林-巴利综合征患者脑脊液抗P2蛋白IgG抗体增高

用ＥＬＩＳＡ方法，检测３６例格林－巴利综合征（ＧＢＳ）患者和４０例其他神经病（ＯＮＤ）患者的血清和脑脊液及４０例健康对照（ＮＣ）血清标本的抗Ｐ２蛋白ＩｇＧ和ＩｇＭ抗体。结果发现：ＧＢＳ和ＯＮＤ血清抗Ｐ２Ｉｇ６和ＩｇＭ抗体与ＮＣ血清比较无明显差异（Ｐ＞０．０５）。ＧＢＳ脑脊液抗Ｐ２ＩｇＧ抗体明显高于ＯＮＤ（Ｐ＜０．０５），而抗Ｐ２ＩｇＭ抗体则两者无显著差异（Ｐ＞０．０５）。     

大剂量免疫球蛋白及肾上腺皮质激素治疗重症肌无力比较

目的为研究免疫球蛋白对重症肌无力（ＭＧ）的治疗效果,对６２例ＭＧ患者进行了免疫球蛋白及肾上腺皮质激素治疗的对比研究。方法３２例ＭＧ患者静脉注射大剂量免疫球蛋白（ＩＶＩｇ）,３０例ＭＧ患者应用了肾上腺皮质激素（ＡＣＳ）冲击治疗。临床绝对评分及相对评分作为治疗前后疗效判定标准。结果６２例ＭＧ患者治疗前后评分有明显差异（Ｐ＜０．０１）,ＡＣＳ治疗前后差值较ＩＶＩｇ明显加大（Ｐ＜０．０１）。结论ＩＶＩｇ治疗ＭＧ有效,ＡＣＳ治疗ＭＧ效果优于ＩＶＩｇ。ＩＶＩｇ可作为治疗ＭＧ的二线药物。     

多发性硬化患者脑脊液血清配对标本蛋白质系列指标的分析

目的　分析蛋白系列指标,判断血脑屏障通透性,明确 Ｉｇ Ｇ 来源。方法　用考马斯亮蓝 Ｇ２５０ 法测定总蛋白,聚丙烯酰胺凝胶电泳银染色法测定寡克隆区带,火箭免疫电泳法同时测定 Ｉｇ Ｇ 和白蛋白, Ｃ Ｓ Ｆ Ａｌｂ／ Ｓｅｒｕｍ Ａｌｂ 比值判断血脑屏障通透性。结果　３０ 例多发性硬化患者 ２２ 例血脑屏障通透性正常,其蛋白系列指标阳性率： Ｏ Ｃ Ｂ 为 ５４．５％ , Ｉｇ Ｇ 合成率为 ６３．６％ , Ｃ Ｓ Ｆ Ｉｇ Ｇ／ Ｃ Ｓ Ｆ Ａｌｂ 比值为 ４０．９％ , Ｃ Ｓ Ｆ Ｉｇ Ｇ／ Ｃ Ｓ Ｆ Ｔ Ｐ 为 ４５．５％ , Ｃ Ｓ Ｆ Ｉｇ Ｇ 为 ４５．５％ , Ｏ Ｃ Ｂ＋ Ｉｇ Ｇ 合成率为 ８１．８％ 。结论　在判断清楚血脑屏障通透性是正常还是异常的前提下再分析蛋白系列指标,并且以寡克隆区带和 Ｉｇ Ｇ 合成率为主要指标,只有血脑屏障通透性正常而寡克隆区带和 Ｉｇ Ｇ 合成率又异常时,才能肯定的认为有中枢鞘内 Ｉｇ Ｇ（抗体）的合成。     

胸苷激酶基因治疗人多形性胶质母细胞瘤BT325并诱导BT325和9L肿瘤细胞的凋亡

目的：了解转单纯疱疹病毒胸苷激酶基因（ＨＳＶ－ＴＫ）前后胶质瘤细胞对更昔洛韦（ＧＣＶ）的药物敏感性；钙通道阻滞剂尼莫地平是否增强ＨＳＶ－ＴＫ／ＧＣＶ基因治疗的肿瘤杀伤作用，以及该治疗方法能否诱导胶质瘤细胞凋亡。方法：用带有ＨＳＶ－ＴＫ／ＧＣＶ基因的重组逆转录病毒载体，转染人多形性胶质母细胞瘤株ＢＴ３２５。用ＭＴＴ法测定ＧＣＶ对转染ＨＳＶ－ＴＫ基因ＢＴ３２５瘤细胞的杀伤作用。结果：０．１ｕｇ／ｍｌ的ＧＣＶ对转ＨＳＶ－ＴＫ基因的ＢＴ３２５瘤细胞有杀伤作用，加入尼莫地平后，０．０１ｕｇ／ｍｌ的ＧＣＶ即有杀伤作用。同时发现该治疗方法可以引起胶质瘤细胞的凋亡。结论：ＨＳＶ－ＴＫ／ＧＣＶ基因治疗对胶质瘤有很强的杀伤作用，并可以引起肿瘤细胞的凋亡；尼莫地平有协同作用，这可能使尼莫地平做为一种增敏剂在脑肿瘤该治疗中使用。     

大剂量免疫球蛋白及肾上腺皮质激素治疗重症？…

目的 为研究免疫球蛋白对重症肌无力（ＭＧ）的治疗效果。对６２例ＭＧ患者进行了免疫球蛋白及肾上腺皮质激素治疗的对比研究,方法 ３２例ＭＧ患者静脉注射大剂量免疫球蛋白（ＩＶＩｇ）３０列ＭＧ患者应用了肾上腺皮质激素（ＡＣＳ）冲击治疗。临床绝对评分及相对评分作为治疗前后疗效判定标准,结果 ６２例ＭＧ患者治疗前后评分有明显差异（Ｐ〈０．０１）,ＡＣＳ治疗前后差值较ＩＶＩｇ明显加大（Ｐ〈０．０１）。结论 ＩＶ     

重症肌无力IgG型及IgM型抗突触前膜抗体检测

利用β－银环蛇毒素（β－ｂｕｎｇａｒｔｏｘｉｎβ－ＢＧＴ）特异性与突触前膜不可逆结合的特性提纯突触前膜相应蛋白受体为抗原，用ＥＬＩＳＡ检测４０例重症肌无力病人，发现重症肌无力病人血中存有抗突触前膜抗体。ＩｇＧ型阳性率（６０％）较ＩｇＭ型阳性率（２７．５％）高。提示重症肌无力病人突触前膜也受自身抗体的损伤。同时本实验还检测（抗乙酰胆碱受体ＡＣｈＲ抗体）作为对照     

机械心瓣膜患者双门深TCD微栓子信号检测

目的研究脑动脉微栓子信号（ＭＥＳ）的基本特征。方法采用双门深经颅多普勒（ＴＣＤ）检测了２３例机械心瓣膜患者的脑动脉ＭＥＳ。结果ＭＥＳ阳性发现率为８２．６％（１９／２３），ＭＥＳ出现频率为０～２２４（中位数３９）个／小时，ＭＥＳ相对强度为１１～３９（中位数２４．５）ｄＢ。８０．５％的ＭＥＳ在二个取样深度依次显示，时间延迟为０．７～３５．８（中位数６．３）ｍｓ，１９．５％的ＭＥＳ仅见于近端通道，而在远端通道未能显示。结论双门深ＴＣＤ是研究ＭＥＳ基本特征的有效方法     

Bilineal transmission in Tourette syndrome.

OBJECTIVE: To investigate the frequency and pattern of bilineal transmission in families of patients with Tourette syndrome (TS) compared with normal control subjects. METHODS: The study population consisted of two groups: 1) consecutive patients with TS with both parents (51 family sets; 153 individuals), and 2) normal control subjects randomly selected from public schools (20 family sets; 60 individuals). All patients with TS, normal control subjects, and their parents were evaluated for evidence of TS and associated features. Structured interviews and detailed questionnaires designed to assess tics, obsessive-compulsive behavior (OCB), and attention deficit disorder (ADD) were administered to all people in both groups. RESULTS: In addition to tics, 43 (84.3%) patients with TS had ADD, 33 (64.7%) had OCB, and 31 (60.8%) had both ADD and OCB. In 42 (82.4%) of the 51 patients, at least one parent exhibited features of TS; unilineal transmission (only one parent with tics, OCB, or ADD) was present in 29 (56.9%) TS families and an additional 13 (25.5%) TS families manifested evidence of bilineal transmission (both parents affected). More fathers than mothers of patients with TS had tics (31.4% versus 15.7%), whereas more mothers had OCB than did fathers (33.3% versus 15.7%). Features of ADD were equally distributed among fathers (35.3%) and mothers (33.3%) of patients with TS. Eight of 1,142 (0.7%) children in the general school population had some evidence of TS. One of 40 parents of the normal control subjects (2.5%) had symptoms of ADD, but none of the parents of normal control subjects manifested any features of TS or OCB. CONCLUSIONS: This study found evidence for bilineal transmission in one fourth of TS families. Features of the TS spectrum were rare in normal control families. Evidence of tics, OCB, and ADD should be investigated in both parents of patients with TS.     

Is onset of Tourette syndrome influenced by life events?

The aim of this study is to investigate the possible relationship between stressful life events, personality, and onset of Tourette syndrome in children. The study group included 93 subjects aged 7-18 years: 41 with Tourette syndrome (TS), 28 with obsessive-compulsive disorder (OCD), and 24 healthy controls. Diagnoses were based on the Child Schedule for Schizophrenia and Affective Disorders (K-SADS). All children were tested with the Screen for Child Anxiety Related Emotional Disorders, Children's Yale Brown Obsessive Compulsive Scale, Beck Depression Inventory or Children's Depression Inventory, the Life Experience Survey, and the Junior Temperament and Character Inventory. The findings were compared among the groups. Subjects with Tourette syndrome and healthy controls had significantly less stressful life events than subjects with (OCD). There were no significant differences between the TS subjects and the healthy controls. This finding applied to total lifetime events, total lifetime negative events, and events in the year before and after illness onset. Subjects with TS and the healthy controls also showed a significantly lesser impact of life events than subjects with OCD. The Tourette syndrome group showed a significantly lesser impact of stressful life events than controls. Harm avoidance tended to be higher in the patients with Tourette syndrome and comorbid attention deficit hyperactivity disorder and obsessive-compulsive disorder than in patients with Tourette syndrome only. There seemed to be no association between life events, diagnosis, and personality. Although there is some research suggesting that tics can be influenced by the environment, the onset of Tourette syndrome does not seem to be related to stressful life events, nor to an interaction between stressful life events and personality.     

Cavum septi pellucidi in Tourette syndrome.

BACKGROUND: An enlarged cavum septum pellucidum (CSP) is a putative marker of disturbed brain development, and it has been associated with a variety of neuropsychiatric disorders. The goal of this study was to characterize systematically the CSP and the related cavum vergae in individuals with Tourette syndrome (TS). METHODS: The overall size and anteroposterior length of the CSP in 161 children (97 with TS and 64 normal pediatric control subjects) and 107 adults (43 with TS and 64 normal adult control subjects) were rated on high-resolution magnetic resonance images in the coronal view. The associations of CSP size with diagnosis and symptom severity scores were assessed using ordinal logistic regression. RESULTS: CSP size in TS children was significantly smaller than in normal control subjects, and it was inversely associated with attention-deficit/hyperactivity disorder symptom severity in the TS subjects. CSP size was not significantly associated with the comorbid diagnoses of OCD or ADHD. These results were replicated in the independent sample of adults with TS and their same-age control subjects. The presence of a cavum vergae was not significantly associated with a diagnosis of TS. CONCLUSIONS: These findings suggest that the pathophysiology of TS may involve abnormalities in the early development of the CSP or in the neighboring corpus callosum, septal nuclei, or limbic system.     

Tourette综合征患儿三种肺炎支原体特异性抗体的检测

目的 探讨肺炎支原体(MP)感染与Tourette综合征(TS)的关系.方法 根据美国耶鲁综合抽动严重程度量表(YCTSS)评分,将4l例TS患儿(TS组)分为轻度组和中重度组,以同期健康儿童60例作为对照组,采用酶联免疫吸附法测定2组血清中MP特异性抗体免疫球蛋白A(MP-IgA)、免疫球蛋白G(MP-IgG)、免疫球蛋白M(MP-IgM)的水平;观察TS组与对照组是否存在血清MP-IgA、MP-IgG、MP-IgM的差别.结果 TS组MP-IgA阳性率(24.39%)和MP-IgG阳性率(53.66%)均高于对照组(6.67%,28.33%),差异均有统计学意义(P均＜0.05);TS组MP-IgM阳性率与对照组比较,差异无统计学意义(P＞0.05).结论 MP感染与TS发病可能存在一定的联系.

Abstract：

Objective To explore the association between mycoplasma pneumoniae infection and Tourette syndrome. Methods The serum mycoplasma pneumoniae-IgA, -IgG, -IgM in 41 children with Tourette syndrome and 60 healthy children were measured with ELISA method. Results The positive rate of mycoplasma pneumoniae IgA( 24. 39% )and IgG( 53.66% ) in Tourette syndrome group were higher than that of control group( 6. 67% and 28. 33%)( P ＜0. 05 ). The positive rate of mycoplasma pneumoniae-IgM was not different between patient and healthy children. Conclusion Mycoplasma pneumoniae infection may be associated with Tourette syndrome.     

伴与不伴注意缺陷多动障碍的抽动秽语综合征患儿动态脑电图的对照研究

目的　探讨伴与不伴注意缺陷多动障碍（ＡＤＨＤ） 的抽动秽语综合征（ＴＳ） 患儿间脑电图的差异。方法　对８６ 例伴与不伴ＡＤＨＤ 的ＴＳ患儿进行２４ ｈ 动态脑电图（ＡＥＥＧ） 监测。结果　伴ＡＤＨＤ的ＴＳ组（４０ 例）ＡＥＥＧ异常率为７５％ ,单纯ＴＳ组（４６ 例） 异常率为３０％ ,２ 组差异有显著性（ Ｐ＜０－０５） ;ＡＥＥＧ异常的主要表现为慢波异常以及癫痫样波。伴ＡＤＨＤ的ＴＳ组的ＡＥＥＧ 局部异常多于广泛异常,且以额叶受累多见;而单纯ＴＳ组则广泛异常多于局部异常。结论　伴与不伴ＡＤＨＤ的ＴＳ患儿在脑电生理学上存在异质性     

Decreased platelet imipramine binding in Tourette syndrome children with obsessive-compulsive disorder.

[3H]Imipramine binding to blood platelets was assessed in eight untreated Tourette syndrome (TS) children, nine drug-free TS children with obsessive-compulsive disorder (OCD), and nine age-matched and gender-matched control subjects. The density of [3H]imipramine binding sites in TS + OCD patients was significantly lower compared with TS-OCD patients (28%) as well as when compared with controls (31%). This alteration was not accompanied by differences in the affinity of the binding site to the ligand. The decreased density of the platelet serotonin "transporter" might implicate the involvement of the serotonergic system in the pathophysiology of OCD in TS patients, but not in TS per se.     

Antibodies against a neuron-like (HTB-10 neuroblastoma) cell in children with Tourette syndrome.

BACKGROUND: Similar to the model for Sydenham's chorea, antineuronal antibodies (ANAb), which develop in response to a preceding streptococcal infection, have been speculated to have a role in the development of Tourette syndrome (TS). METHODS: Serum antibodies against the neuron-like HTB-10 neuroblastoma cell were assayed by ELISA methods and Western blot analysis on 41 children with TS (mean age 11.3 years) and 39 control subjects (mean age 12.1 years). RESULTS: Group comparisons of ELISA assay optical density (OD) showed that mean OD values for serum antibodies were not different [control (mean +/- SEM), .506 +/- .076; and TS, .584 +/- .053 (p = .38)]. In contrast, median values [.353 in control subjects and .477 in TS subjects (p = .012)] were significantly different. Western blots identified numerous bands in all TS and control sera with no difference in identified HTB-10 antigens. There was no relationship between the presence of ANAb and age of tic onset, family history, tic severity, attention deficit hyperactivity disorder, or obsessive compulsive disorder. No relationship existed between positive strep titers (ASO > or = 166 and/or antiDNAaseB > or = 170) and ANAb determinations or the severity of tics. CONCLUSIONS: Children with TS have higher median, but not mean, levels of ANAb, as measured by the HTB-10 neuroblastoma cell membrane assay. This assay system identified antibodies in both control and clinical groups and failed to identify a relationship between antibodies and clinical phenotype or one-time markers for streptococcal infection. Further studies are required to define a possible immune-mediated hypothesis for TS.     

Differences in clinical characteristics between Tourette syndrome patients with and without 'generalized tics' or coprolalia

Abstract  The purpose of this study is to examine whether there are differences in clinical characteristics between Tourette syndrome (TS) patients with and without 'generalized tics' (GT) which involve the entire body, and/or coprolalia. Subjects were 64 patients (55 males and 9 females, mean age, 17.4 ± 7.2 years) who visited Tokyo University's outpatient clinic of neuropsychiatry from 1974 to 1993 and who met criteria for Tourette's disorder of DSM-III-R. Data on clinical characteristics, including tic symptoms and courses of their development, complications and developmental histories, treatment and severity, were collected by systematic chart review of all subjects. Tourette syndrome patients with 'generalized tics' tended to show multiple complex vocal tics more frequently than TS patients without GT. Tourette syndrome patients with coprolalia tended to show significantly higher rates of copropraxia, echolalia, and 'cleaning/washing' compulsion than did the TS patients without coprolalia. Tourette syndrome patients with both GT and coprolalia were classified as the severest group in terms of tic symptoms and social impairment. Tourette syndrome patients who had neither of these morbidities were classified into the mildest group in all aspects. Generalized tics and coprolalia seemed to indicate the severest end of the TS spectrum and seemed to be related with a need of intensive treatment.     

Locus of control,perceived parenting style,and symptoms of anxiety and depression in children with Tourette’s syndrome

AIM: This study explored the contribution of two psychosocial factors, locus of control (LOC) and perceived parenting style, to symptoms of internalizing disorders in children with Tourette syndrome (TS). This contribution was further evaluated in relation to TS severity. METHODS: Sixty-five children (53 boys, 12 girls) ages 9.0-16.9 years, of normal intelligence, completed questionnaires evaluating their depression and anxiety symptoms, LOC, and maternal parenting style. Their mothers rated TS severity, determined by tic severity, symptoms of attention-deficit hypreactivity disorder (ADHD) and obsessive compulsive symptoms (OCS). RESULTS: Higher rates of symptoms of anxiety and depression were associated with a more external LOC and a more rejecting and controlling parenting style. Additionally, depression correlated significantly with tic severity, ADHD and OCS, whereas anxiety correlated only with ADHD symptoms and OCS, but not with tics. Regression analyses showed that LOC, OCS and ADHD symptoms each significantly contributed to predicting anxiety level, whereas LOC and ADHD symptoms significantly contributed to predicting depression symptoms. CONCLUSIONS: Rates of symptoms of anxiety and depression in children with TS are markedly influenced by psychosocial factors, extending beyond the influence of ADHD and OCD, both common comorbid disorders in TS. An internal LOC, which is associated with an accepting and autonomy-granting parenting style, appears to be a protective factor against anxiety and depression.     

Aggressive behaviour in children with Tourette syndrome and comorbid attention-deficit hyperactivity disorder and obsessive-compulsive disorder.

BACKGROUND: Aggressive behaviour, defined as sudden, explosive outbursts of rage, has been reported as a clinical problem in approximately 23% to 40% of Tourette syndrome (TS) patients (1-5). Attention-deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD) are also reported in 50% to 70% of TS patients (6). OBJECTIVE: To investigate whether aggressive behaviour was associated with TS directly or found primarily in TS with comorbid ADHD or OCD. METHOD: Aggressive behaviour in 33 nonmedicated patients with TS (ages 6 to 14 years) and 6 healthy control subjects (ages 7 to 12 years) was examined by semistructured interview and multiinformant questionnaires. RESULTS: Aggression subscales on Achenbach's Child Behavior Checklist (CBCL) completed by parents and Teacher's Report Form (TRF) completed by teachers distinguished the TS-only and control groups from the group with TS + Comorbidity (P < 0.046, and P < 0.016) after adjusting for tic severity and age. The conduct disorder subscale on the Conners Parent Rating Scale (CPRS) was also significantly higher (P < 0.005) in the TS + comorbidity group than in the TS-only or control groups, with more problems reported in the older children. CONCLUSIONS: These findings provide additional evidence that aggressive behaviour observed in children with TS may be associated with comorbid ADHD or OCD (6), independent of tic severity or age. This is consistent with the clinical observation that most TS patients have only minimal symptoms, which do not interfere with their daily functioning.