Tolosa-Hunt综合征

摘要： Miller-Fisher综合征是一种自身免疫学介导的疾病,通常急性起病,病前常有呼吸道或胃肠道感染病史,可发生于任何年龄段或任何季节。临床上主要表现为经典的三联征:眼肌麻痹、共济失调、腱反射减弱或消失。本文回顾性分析了2021年10月29日就诊于陕西省人民医院的1例以头痛及单侧眼肌麻痹为主要表现的GQ1b阴性的Miller-Fisher综合征,现将其临床资料及诊疗经过报道如下,以为临床提供指导意义。

摘要： 目的 探讨Tolosa-Hunt综合征的临床特点,提高对该病的认识.方法 回顾性分析浙江省金华市中心医院2018-2019年18例诊断为Tolosa-Hunt综合征患者的临床资料,总结分析其临床特点.结果 18例Tolosa-Hunt综合征患者中13例(72.2％)以头痛或眶周疼痛为首发症状,同时伴眼肌麻痹,13例(72.2％)动眼神经受累.血常规及血沉均正常,仅3例脑脊液蛋白轻度升高,9例(50％)单侧海绵窦影像学有异常改变.激素治疗后所有患者疼痛均在3天内缓解,眼肌麻痹在治疗后1周-3个月内好转或消失,但仍有小部分复发或有后遗症.结论 Tolosa-Hunt综合征患者的疼痛可先于眼肌麻痹或同时发生,血常规和脑脊液检查无特异性改变.头颅增强MRI见海绵窦区异常强化,为其影像学诊断的客观依据,激素治疗效果明显,眼肌麻痹恢复比疼痛缓解慢.

摘要： 目的 分析Tolosa-Hunt综合征患者的临床特点及预后情况.方法 收集2008年6月至2018年6月山东省立医院神经内科收治的62例Tolosa-Hunt综合征患者的临床资料,对其进行分析总结.结果 62例患者中,男性34例,女性28例,83.9％的患者发病年龄在40～79(中位数为61.5)岁.66.1％患者以头面痛或头痛伴随颅神经麻痹症状起病,常见临床表现依次为头面痛61例(98.4％)、眼球运动障碍50例(80.7％)、视物重影47例(75.8％)、眼睑下垂43例(69.4％).在头痛及颅神经麻痹症状的预后方面,完全缓解组与未完全缓解组在性别、年龄、吸烟、饮酒、住院时间,及有无高血压、糖尿病等方面均无统计学差异.回归分析结果显示,白细胞计数、起始激素治疗剂量及静脉激素治疗时间与患者预后无关.结论 Tolosa-Hunt综合征的特征性表现为头面痛和眼肌麻痹,诊断应仔细询问病史、进行全面查体并结合影像学检查综合分析.

摘要： 目的 分析表现为眼肌麻痹的海绵窦区病变的病因谱组成,观察相应临床及影像特点及其在病因学诊断时的预测价值.方法 回顾并随访2005年1月至2014年1月间,因眼肌麻痹来诊,确诊为海绵窦区病变的住院患者137例.依据临床特征、实验室检查、影像资料、病理结果等进行病因学分类,并对不同病因海绵窦病变的临床及影像资料进行统计学分析.结果 炎症性疾病[51例(37.2％)]是最常见的病因,早期疼痛(OR5.591,95％ CI 1.703～18.401)及动眼神经受累(OR4.902,95％CI 1.015～24.630)与炎症性疾病独立相关;肿瘤[43例(31.3％)]是本组海绵窦病变第二位病因,其中以累及海绵窦区脑膜瘤最常见,三叉神经第二支受累(OR 1.017,95％ CI 1.005～1.071)与肿瘤独立相关.海绵窦区血管性病变28例(20.4％),其与男性性别独立相关(OR 3.506,95％CI 1.362 ～8.765).海绵窦感染性疾病15例(10.9％),以真菌感染最常见,多继发于鼻窦感染.结论 表现为眼肌麻痹的海绵窦病变病因谱主要包括炎症性、肿瘤性、血管性、感染性疾病四大类,本组资料以炎症性疾病最常见.部分海绵窦病变的临床特征对病因学有一定的预测价值,病程早期疼痛、动眼神经受累与炎症性疾病独立相关,而显著面部感觉障碍、三叉神经第二支受累更多提示肿瘤性疾病可能.优化MRI检查方法可更好地识别海绵窦区病变.%Objective To classify different causes of cavernous sinus lesion in patients with ophthalmoplegia and analyze their clinical and imaging features.Methods We confirmed the etiological diagnosis of 137 hospitalized ophthalmoplegia patients with cavernous sinus lesion retrospectively from January 2005 to January 2014 in the Department of Neurology of Beijing Tongren Hospital.The diagnosis was made according to clinical feature,laboratory test,imaging studies and pathology.The clinical data of these patients were analyzed and compared among different groups.Results Fifty-one cases (37.2％) were confirmed as inflammatory diseases of cavernous sinus,which were the most common cause in 137 patients.Early stage pain (OR 5.591,95％ CI 1.703-18.401) and involvement of oculomotor nerve (OR 4.902,95％ CI 1.015-24.630) were independently associated with inflammatory diseases.Forty-three cases (31.3％) were confirmed to have tumor,which was the second cause of cavernous sinus lesions,and meningioma was the most common tumor.The second branch of trigeminal nerve involvement was independently correlated (OR 1.017,95％ CI 1.005-1.071) with tumor.The percentage of male (OR 3.506,95％ CI 1.362-8.765) was significantly higher in 28 cases (20.4％) with cavernous sinus vascular lesions.Cavernous sinus infectious diseases were seen in 15 cases (10.9％),and fungal infection was the most common,mostly secondary to sinus infection.Conclusions The common causes of cavernous sinus lesion include cavernous sinus inflammatory diseases,neoplastic diseases,vascular diseases and infectious diseases,and inflammatory diseases is the most commonly seen cause in this group of patients.Obvious facial sensory disturbances and the second branch of trigeminal nerve involvement are independent associated with tumor.The early course of pain and involvement of oculomotor nerve are associated with inflammatory diseases.Optimization of MRI examination method can better identify the cavernous sinus lesions.

摘要： 目的：分析复发性Tolosa-Hunt综合征（THS）的临床特征、复发特点。方法收集24例2006年1月至2016年5月确诊的复发性THS患者的临床资料。对其一般特征、临床表现、病程及复发特征、实验室及影像学检查、治疗转归等进行分析，并与同期69例初发性THS患者的临床资料进行比较。结果复发性THS占THS总数的25.8%（24/93）。复发组男性比例显著高于初发组[66.7%（16/24）比42.0%（29/69）]，差异有统计学意义（P＜0.05）；复发组三叉神经第一支受累率低于初发组[16.7%（4/24）比33.0%（23/69）]，差异有统计学意义（P＜0.05）；复发组总病程3个月至20年，总复发次数2～10次，同侧复发18例，对侧复发6例，平均复发间隔1.9年（3个月至6年），2例在激素减量时复发，22例在激素停用时复发。24例患者均接受MRI检查，其中19例（79.2%）显示海绵窦受累，单侧受累16例，双侧受累3例；复发后激素治疗仍有效。结论 THS具有一定的复发性，少数患者复发频繁，复发周期差异较大，可为数个月至数年，易于在同侧，也可在海绵窦两侧交替复发。复发症状常较初发者轻，男性患者及未经激素规律治疗者可能更易复发，复发性THS对激素治疗仍有良好的反应。%Objective To analyze the clinical features, recurrent characters in patients with recurrent Tolosa-Hunt syndrome (THS). Methods The clinical data of 24 hospitalized patients with recurrent THS from January 2006 to May 2016 were collected The general features, clinical manifestations, disease courses, recurrent features, lab and imaging studies, treatment measures and outcoming of recurrent THS patients was investigated , and compared with 69 patients with first attack THS in corresponding period. Results Recurrent THS patients were 25.8%(24/93) of total THS. The male rate in recurrent group was significantly higher than that in first attack group: 66.7%(16/24) vs. 42.0%(29/69), P<0.05. The involved rate of trigeminal nerves in recurrent group was significantly lower than that in first attack group:16.7%(4/24) vs. 33.0%(23/69), P<0.05. The disease courses were from 3 months to 20 years. The total recurrent frequencies were from 2 to 10 times. The recurrence occurred in the same side in 18 patients, and in contralateral in other 6 patients. The intervals were from 3 months to 6 years, and average intervals were 1.9 years. Two patients recurred in hormone reduction, and 22 patients recurred in hormone withdrawal. All cases received MRI examination. Nineteen patients (79.2%) of them had lesions in cavernous sinus. 16 patients had one side lesions and 3 patients had bilateral lesions. The recurrent patients still had good responds to corticosteroids treatment. Conclusions Recurrences in THS are common, taking place in about 26%total patients, and usually at an interval of months or years from the initial attack. These recurrences may be ipsilateral, contralateral, or rarely, bilateral. Corticosteroids are still effective to recurrent cases.

摘要： 目的：研究 Tolosa - Hunt 综合征（THS）的病因、诊断及治疗。方法收集2010年至2014年住院诊断10例 Tolosa - Hunt 综合征患者的临床表现、神经影像学、实验室检查及激素治疗情况并进行回顾性分析。结果10例患者的颅神经麻痹累及动眼神经、滑车神经、外展神经及三叉神经第1、2、3支，有1例为完全性动眼神经麻痹。10例患者神经影像学分类均为良性的，应用激素后疼痛快速减轻，仅有1例遗留轻度复视。对治疗反应无年龄及性别差异。应用激素后10例患者疼痛均较眼肌麻痹缓解快。结论 THS 的诊断为排他性，神经影像学对于鉴别诊断 THS 非常重要，对于 THS 病人应全面完善检查除外其他疾病引起的疼痛性眼肌麻痹，包括血常规、红细胞沉降率、C 反应蛋白、甲状腺、肝肾功能检查、胸片、头部及眼眶部 CT 及磁共振检查。%Objective To study the etiology,diagnosis and treatment of Tolosa - Hunt syndrome. Methods The data of clinical mani-festations,neuroimaging,laboratory examinations and the therapeutic effect by steroid from ten patients with Tolosa - Hunt syndrome,hospitalized from 2010 to 2015,were retrospectively analyzed. Results The ten patients of our study involve paresis of one or more of the third,fourth,fifth and sixth cranial(VI1,VI2 and VI3). One case showed complete oculomotor nerve paralysis. The ten cases of THS can be classified as benign according to neuroimaging[when no abnormal neuroimaging can be found]. Our study revealed that the rapid alleviation of pain by steroid treat-ment was a characteristic feature of THS patients. Only one patient essentially remained mild diplopia. No age and gender differences was found in response to the treatment. We noticed that the time course of symptom relief differed between pain and palsy,with pain relief occurring with a much faster time course. Conclusion THS essentially remained a diagnosis of exclusion. Neuroimaging was important in differential diagnosis. Also full blood count,ESR,C reactive protein,full biochemistry tests including thyroid and liver functional tests and Chest X ray were indicated. Brain and orbital imaging included MRI and CT scan.

摘要： 目的：对Tolosa-Hunt综合征的临床表现、影像学特点及治疗转归进行分析总结,以提高该病的诊治水平。方法收集海军总医院2006年4月—2014年1月收治的16例Tolosa-Hunt综合征患者的临床资料,总结其临床表现、实验室检查、影像学特点及预后,并结合文献资料进行分析。结果16例多为急性或亚急性起病,病程从3d至8个月不等。症状以头痛和同侧眼肌麻痹为主,多累及Ⅲ、Ⅳ、Ⅴ1、Ⅵ多组颅神经。激素冲击治疗后疼痛症状迅速好转,颅神经麻痹症状在随后的1周至3个月内恢复,1例病程较长患者遗留眼肌麻痹。头颅MRI发现海绵窦、眶尖区软组织影,在T1加权像呈低信号或等信号,T2加权像呈稍高或等信号。结论 Tolosa-Hunt综合征临床表现以头痛、眼肌麻痹为主,对激素治疗敏感,颅脑增强MRI可为诊断提供客观的影像学依据,对患者定期随诊并复查颅脑增强MRI,可以减少误诊及漏诊。%Objective To analyze the clinical manifestation, imaging, and outcome of Tolosa-Hunt syndrome for an insight into this syndrome. Methods Sixteen cases of Tolosa-Hunt syndrome were diagnosed in our hospital from April 2006 to January 2014. Clinical manifestation, laboratory examination, neuroimaging findings, treatment and outcome were reviewed and summarized. Results Most patients developed disease in acute or subacute mode. The most frequent sign was unilateral or-bital pain and oculomotor paresis with prompt relief of symptoms to steroid treatment. Cranial-nerve palsy were recovered in the following one to three months. One patient with long duration remained ophthalmoplegia. Conclusion The main clinical manifestations of painful ophthalmoplegia is hea-dache, ophthalmoplegia and good responses to steroid treatment. The MR imaging findings of brain can provide accordance in diagnosing the disease. Since diagnosis and differential diagnosis are diffi-cult for the patients with normal MR imaging, MRI enhancement of brain is needed for accurate diagnosis.

摘要： 目的：探究Tolosa-Hunt综合征的MRI表现及诊断价值。方法选取笔者所在医院2014年8月~2015年8月收治的60例Tolosa-Hunt综合征患者，依据诊断方式，将这些患者分为对照组(n=30)和研究组(n=30)两组。给予对照组应用CT诊断，给予研究组患者应用MRI诊断，分析Tolosa-Hunt综合征的MRI表现，对比观察两组患者临床诊断价值。结果在两组患者中，研究组患者中MRI能够清晰显示Tolosa-Hunt综合征患者，均为单侧发病，其MRI表现为病变侧海绵窦形态改变，T1WI上呈等或稍低信号，T2WI呈稍高信号，边缘清楚，颈内动脉被包绕，管腔不同程度变窄，增强扫描病灶明显强化；应用MRI诊断效果高于CT诊断，疾病检出率较对照组高，MRI的检出率为83.3%；对照组CT诊断检出率为66.7%，两组之间差异具有统计学意义(P<0.05)。结论临床中，对于临床究Tolosa-Hunt综合征诊断中，MRI诊断中，可以清楚反映病灶内成分，提高临床疾病检出率，值得在实际中推广。%Objective To discuss the MRI manifestations and diagnostic value of Tolosa-Hunt Syndrome. Methods 60 patients with Tolosa-Hunt Syndrome were selected from August 2014 to August 2015 in our Hospitals. According to the diagnosis way,all patients were divided into control group (n=30) and research group (n=30). control group used CT diagnosis,research group used at the application of MRI in the diagnosis of patients,analyzed MRI manifestation of Tolosa-Hunt Syndrome,compared two groups of patients with clinical diagnostic value.Results In two groups of patients,the team in patients with MRI could clearly show Tolosa-Hunt Syndrome patients,were unilateral onset,the MRI manifestation of cavernous sinus lesions side form change,showed or slightly low signal on T1WI,T2WI were slightly high signal,edge was clear,internal carotid artery wrapped around,lumen narrowing,different level enhancement scan lesions significantly improved;Application effect was higher than that of CT,MRI diagnosis of disease high detection rate was higher than the control group,MRI detection rate was 83.3%;Control group CT detection rate was 66.7%,statistically significant difference between the two groups (P<0.05).Conclusions In clinical,for clinical investigate Tolosa-Hunt Syndrome diagnosis,MRI in the diagnosis,can clearly reflect lesions in composition,improve clinical disease detection,it’s worth for further clinical promotion.

摘要： 目的 探讨Tolosa-Hunt综合征(THS)的临床、影像学表现及治疗,提高对该病的诊治水平.方法 对12例THS患者的临床表现、实验室检查、神经影像学结果、治疗方法及转归进行回顾性分析.结果 12例患者多为亚急性起病,2例眼肌麻痹与头痛同时发生、10例在头痛后14 d内出现;9例动眼神经麻痹、6例外展神经麻痹、2例滑车神经麻痹、2例视神经受累;血常规、血沉、脑脊液压力、常规、生化检查基本正常;MRI检查显示12例患侧海绵窦区软组织增厚、4例患侧眶上裂软组织增厚,病灶呈均匀性强化.所有患者激素治疗后10 ～72 h内疼痛缓解,11例用药后4 ～60(29.7±18.0)d体征完全恢复,1例遗留脑神经麻痹.结论 THS的头痛可先于眼肌麻痹2w或同时发生,血常规、血沉和脑脊液检查对诊断缺乏特异性提示,海绵窦MRI对诊断有重要价值,激素治疗效果明显,但体征恢复比疼痛缓解需要更长时间.

摘要： 本文为一误诊病例,将痛性眼肌麻痹伴慢性闭角型青光眼误诊为:1.左眼动眼神经麻痹;2.左眼青光眼,文中讨论了痛性眼肌麻痹,分析了误诊原因.

摘要： 目的：探讨Tolosa-Hunt综合征(THS)的128层CT表现.方法：回顾性分析临床确诊的3例THS的128层CT资料,结合有关文献,分析其128层CT表现.结果：3例THS 128CT平扫均表现为一侧海绵窦增宽,2例伴眶上裂软组织密度肿块,1例邻近硬脑膜增厚、密度增高,同时伴双侧颈内动脉海绵窦段钙化;增强扫描3例均示患侧海绵窦增宽、明显强化,2例累及眶上裂,1例累及视神经孔,同时伴有邻近硬膜强化,1例显示眶上裂肿块推移血管伴同侧海绵窦引流静脉增多、增粗、扩张、迂曲.结论：128层CT可以显示THS病变的部位、范围、强化程度,以及累及血管病变,有助于THS的诊断与鉴别诊断.

摘要： 目的：探讨Tolosa-Hunt综合征(THS)的128层CT表现.方法：回顾性分析临床确诊的3例THS的128层CT资料,结合有关文献,分析其128层CT表现.结果：3例THS 128CT平扫均表现为一侧海绵窦增宽,2例伴眶上裂软组织密度肿块,1例邻近硬脑膜增厚、密度增高,同时伴双侧颈内动脉海绵窦段钙化;增强扫描3例均示患侧海绵窦增宽、明显强化,2例累及眶上裂,1例累及视神经孔,同时伴有邻近硬膜强化,1例显示眶上裂肿块推移血管伴同侧海绵窦引流静脉增多、增粗、扩张、迂曲.结论：128层CT可以显示THS病变的部位、范围、强化程度,以及累及血管病变,有助于THS的诊断与鉴别诊断.

摘要： 目的：探讨Tolosa-Hunt综合征(THS)的128层CT表现.方法：回顾性分析临床确诊的3例THS的128层CT资料,结合有关文献,分析其128层CT表现.结果：3例THS 128CT平扫均表现为一侧海绵窦增宽,2例伴眶上裂软组织密度肿块,1例邻近硬脑膜增厚、密度增高,同时伴双侧颈内动脉海绵窦段钙化;增强扫描3例均示患侧海绵窦增宽、明显强化,2例累及眶上裂,1例累及视神经孔,同时伴有邻近硬膜强化,1例显示眶上裂肿块推移血管伴同侧海绵窦引流静脉增多、增粗、扩张、迂曲.结论：128层CT可以显示THS病变的部位、范围、强化程度,以及累及血管病变,有助于THS的诊断与鉴别诊断.

摘要： 目的：探讨Tolosa-Hunt综合征(THS)的128层CT表现.方法：回顾性分析临床确诊的3例THS的128层CT资料,结合有关文献,分析其128层CT表现.结果：3例THS 128CT平扫均表现为一侧海绵窦增宽,2例伴眶上裂软组织密度肿块,1例邻近硬脑膜增厚、密度增高,同时伴双侧颈内动脉海绵窦段钙化;增强扫描3例均示患侧海绵窦增宽、明显强化,2例累及眶上裂,1例累及视神经孔,同时伴有邻近硬膜强化,1例显示眶上裂肿块推移血管伴同侧海绵窦引流静脉增多、增粗、扩张、迂曲.结论：128层CT可以显示THS病变的部位、范围、强化程度,以及累及血管病变,有助于THS的诊断与鉴别诊断.

摘要： 本发明公开了一种抗病毒药物在制备治疗骨髓增生异常综合征药物中的应用及制得的骨髓增生异常综合征药物。该抗病毒药物为泛昔洛韦。本发明首次在斑马鱼模式生物中，明确泛昔洛韦对造血发育的影响，泛昔洛韦可能通过影响造血干祖细胞进而使整个血液系统发生缺陷。因此，本发明提出泛昔洛韦抗病毒药物在制备治疗骨髓增生异常综合征药物中的应用。

摘要： 本发明提供了一组可用于检测或诊断多囊卵巢综合征并发代谢综合征的生物标志物，并进一步提供包含该等生物标志物的用于检测多囊卵巢综合征并发代谢综合征的试剂盒及其使用方法，可用于对多囊卵巢综合征并发代谢综合征发病风险的快速高效诊断。

摘要： 本发明提供了向受试者立即或长期施用某些钾ATP(KATP)通道开放剂，任选地与生长激素组合，以在涉及KATP通道的疾病或病况的治疗中实现新型药效动力学、药物代谢动力学、治疗、生理学、代谢以及组成结果。还提供了在接受治疗的个体中实现这些结果并且降低不良作用的发生率的KATP通道开放剂的药物制剂、施用方法以及给药方法。还提供了共同施用KATP通道开放剂与其它药物(例如与生长激素组合)以治疗人类和动物的疾病(例如普拉德‑威利综合征(PWS)、史密斯‑马吉利综合征(SMS)等)的方法。

摘要： 本发明提供了一组可用于检测多囊卵巢综合征并发代谢综合征的生物标志物，并进一步提供包含该等生物标志物的用于检测多囊卵巢综合征并发代谢综合征的试剂盒及其使用方法，可用于对多囊卵巢综合征并发代谢综合征发病风险的快速高效诊断。

摘要： 在一个方面，本发明提供了抑制人患者中的MASP‑2依赖性补体激活的效应的方法，所述人患者患有或处于发展HSCT‑IPS的风险中，和/或患有或处于发展HSCT‑CLS的风险中，和/或患有或处于发展HSCT‑FO的风险中，和/或患有或处于发展HSCT‑ES的风险中。该方法包括向有此需要的受试者施用有效抑制MASP‑2依赖性补体激活的量的MASP‑2抑制剂的步骤。

摘要： 本发明涉及由化学式1表示的氨基甲酸酯化合物，或其药学上可接受的盐，溶剂合物或水合物用于减轻或治疗包括脆性X染色体综合征、Angelman综合征或Rett综合征的发育障碍的用途。

摘要： 本发明涉及神经激肽‑1(NK‑1)受体拮抗剂用于治疗脓毒症、脓毒症休克、全身炎性反应综合征(SIRS)、急性呼吸窘迫综合征(ARDS)或多器官功能障碍综合征(MODS)的新用途。本发明还涉及用于这样的用途的包含NK‑1受体拮抗剂和组合的一种或更多种治疗剂的药物组合物。

摘要： 本发明提供了向受试者立即或长期施用某些钾ATP(KATP)通道开放剂，任选地与生长激素组合，以在涉及KATP通道的疾病或病况的治疗中实现新型药效动力学、药物代谢动力学、治疗、生理学、代谢以及组成结果。还提供了在接受治疗的个体中实现这些结果并且降低不良作用的发生率的KATP通道开放剂的药物制剂、施用方法以及给药方法。还提供了共同施用KATP通道开放剂与其它药物(例如与生长激素组合)以治疗人类和动物的疾病(例如普拉德‑威利综合征(PWS)、史密斯‑马吉利综合征(SMS)等)的方法。

摘要： 本发明公开了一种抗病毒药物在制备治疗骨髓增生异常综合征药物中的应用及制得的骨髓增生异常综合征药物。该抗病毒药物为泛昔洛韦。本发明首次在斑马鱼模式生物中，明确泛昔洛韦对造血发育的影响，泛昔洛韦可能通过影响造血干祖细胞进而使整个血液系统发生缺陷。因此，本发明提出泛昔洛韦抗病毒药物在制备治疗骨髓增生异常综合征药物中的应用。

摘要： 本发明提供了一种用于早期诊断多囊卵巢综合征患者合并代谢综合征的标志物及用途。诊断试剂的用途，所述代谢标志物为血清氨基酸，包括：谷氨酸、亮氨酸和苯丙氨酸。本发明用于多囊卵巢综合征患者罹患代谢综合征的早期诊断，诊断准确度高，采用一次性检测血清中三个标志物浓度联合判断多囊卵巢综合征患者代谢综合征的发生情况，并且标准统一有利于临床疗效评价；应用测量方式简单方便有利于实际临床推广应用。