Isaacs综合征5例报告并文献回顾

目的探讨Isaacs综合征潜在的发病机制、临床表现、诊断及治疗。方法回顾性分析2017年3月至2019年3月收治的5例Isaacs综合征患者临床资料,并进行相关文献复习。结果 5例患者均为男性,主要表现为肌肉颤搐、痉挛、多汗,4例予以卡马西平和泼尼松口服治疗,效果良好。结论 Isaacs综合征临床罕见,对症治疗和免疫治疗不可或缺。     

Isaacs综合征1例报道和文献复习

目的：探讨Isaacs综合征的发病机制、临床特征、诊断和治疗。方法：回顾性分析中国医科大学附属第一医院神经内科收治的1例Isaacs综合征患者的临床表现、辅助检查、治疗和预后,结合文献进行分析。结果：患者男性,71岁。主要表现为自发性肌肉颤搐,睡眠时症状不消失。肌电图可见颤搐电位和轻度运动神经传导速度减慢。卡马西平治疗后症状控制良好。结论：Isaacs综合征是一种自身免疫性疾病,病因尚不明确,肌肉颤搐及典型肌电图表现是诊断关键,卡马西平和苯妥英钠为首选治疗药物。     

神经性肌强直5例报告并文献复习

目的 探讨Isaacs综合征临床表现、肌电图特点及临床转归.方法 分析5例神经性肌强直病例的临床症候、肌电图表现、诊断及治疗情况,并进行文献回顾.结果 5例病例临床主要表现为肌肉颤搐、多汗等；肌电图表现为持续快速的二联、三联或多联的运动单位放电活动；应用卡马西平可缓解临床症状.结论 神经性肌强直是一种少见的周围神经病变,白发性连续性肌肉活动为其主要表现；肌电图特点为持续快速的二联、三联或多联运动单位放点活动；应用卡马西平治疗有效,但仍需定期随访.     

45例Isaacs综合征临床与肌电图

目的分析少见的Isaacs综合征的临床特点厦肌电图表现并探讨其可能的发病机制。方法将1995～2005年10年间收治的45例Isaacs综合征的发病谤因、临床表现、电生理表现进行分析。结果45例患者中发病年龄多为青壮年,女性多见,主要表现为自发性连续性肌肉颤搐。睡眠时不消失并伴有多汗,少数伴发重症肌无力,胸腺瘤。免疫治疗有效。肌电图表现为持续的自发运动电位发放。结论Isaacs综舍征是一种自身免疫性疾病,病变部位可能为周围神经运动纤维、神经末梢运动终板厦运动纤维的末端。诊断主要靠临床表现结合肌电图。     

Isaacs综合征5例报道

Isaacs综合征是一种少见的自发性连续性肌肉颤动.现将我院近期收治的5例报道如下. 1临床资料5例患者男3例,女2例,年龄28～47岁,平均年龄38.6岁,病程2～24个月,平均10.4个月.3例以四肢肌肉抽动、疼痛为主诉,2例以双下肢肌肉抽动、疼痛为主诉.5例均有易出汗,休息后肌痛不适可缓解的特点,均无家族史,1例伴有肾病综合征.神经系统检查:颅神经无异常,受累肢体肌力3～5级,肌张力增高,呈板样强直者1例,余轻度增高或正常;腱反射消失者1例,减弱者3例,正常者1例,无病理征,四肢无感觉障碍.肌肉抽动波及全身者1例,波及四肢者4例.     

Isaacs综合征的临床病理研究

目的探讨Isaacs综合征的临床特点、病理、诊断及治疗方法。方法回顾性分析14例Isaacs综合征的临床表现、实验室检查及治疗预后。结果14例均表现为连续性自发性肌肉颤搐,睡眠时不消失。肌电图可见持续的自发的运动电位发放。肌肉呈神经源性病理改变。卡马西平或苯妥英钠控制症状较好。结论肌肉颤搐及典型肌电图表现和病理可诊断本病,卡马西平或苯妥英钠可以控制症状,也可进行免疫治疗。     

艾萨克(Isaacs)综合征3例诊治分析

目的 :探讨罕见的艾萨克 (Isaacs)综合征的临床特点、诊断及治疗方法。方法 :分析 3例艾萨克综合征临床表现、肌电图特点及治疗预后。结果 :3例均为青壮年女性 ,主要表现为自发性连续性肌肉颤搐 ,睡眠不消失 ,均伴多汗。肌电图发现持续的自发运动电位发放。卡马西平控制症状较好 ,免疫治疗有效。结论 :本综合征可能在女性中多见 ,颤搐表现结合肌电图是诊断关键 ,控制症状时用卡马西平可能优于苯妥英钠 ,并需进行免疫治疗     

Isaacs综合征(附一例报告并文献复习)

<正> Isaacs综合征又称获得性神经肌强直、神经性肌强直、连续性肌纤维活动、运动单位持续活动综合征。国内报告较少。现报告我院诊治一例并结合文献讨论如下。     

Isaacs综合征并病毒性脑炎1例报道

<正>Isaacs综合征又名肌颤动、肌纤维颤动一多汗综合征、持续性肌纤维兴奋综合征(syndyome of continuous muscle fibreac- tivity)或神经性肌强直(neruomyotonia)、连续性肌纤维活动等,是一种非常罕见的病因尚未明确的综合征,是由周围神经病变引起的临床少见的自发性连续性肌肉活动综合征,国内鲜见报道,现将我院收治一例报告如下。     

Isaacs综合征临床分析

目的:探讨罕见的Isaacs综合征的临床特点、诊断及治疗方法。方法:对本院收治的9例lsaacs综合征的临床表现、实验室检查及治疗预后进行回顾性分析。结果:9例主要表现为自发性连续性肌肉颤搐,睡眠不消失。肌电图发现持续的自发运动电位发放。卡马西平或苯妥英钠控制症状较好。结论:颤搐表现结合肌电图是诊断本综合征关键,控制症状可用卡马西平或苯妥英钠,并可进行免疫治疗。     

Idiopathic Hypertrophic Spinal Pachymeningitis : Report of Two Cases and Review of the Literature

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory disease characterized by hypertrophic inflammation of the dura mater and various clinical courses that are from myelopathy. Although many associated diseases have been suggested, the etiology of IHSP is not well understood. The ideal treatment is controversial. In the first case, a 55-year-old woman presented back pain, progressive paraparesis, both leg numbness, and voiding difficulty. Initial magnetic resonance imaging (MRI) demonstrated an anterior epidural mass lesion involving from C6 to mid-thoracic spine area with low signal intensity on T1 and T2 weighted images. We performed decompressive laminectomy and lesional biopsy. After operation, she was subsequently treated with steroid and could walk unaided. In the second case, a 45-year-old woman presented with fever and quadriplegia after a spine fusion operation due to lumbar spinal stenosis and degenerative herniated lumbar disc. Initial MRI showed anterior and posterior epidural mass lesion from foramen magnum to C4 level. She underwent decompressive laminectomy and durotomy followed by steroid therapy. However, her conditions deteriorated gradually and medical complications occurred. In our cases, etiology was not found despite through investigations. Initial MRI showed dural thickening with mixed signal intensity on T1- and T2-weighted images. Pathologic examination revealed chronic nonspecific inflammation in both patients. Although one patient developed several complications, the other showed slow improvement of neurological symptoms with decompressive surgery and steroid therapy. In case of chronic compressive myelopathy due to the dural hypertrophic change, decompressive surgery such as laminectomy or laminoplasty may be helpful as well as postoperative steroid therapy.     

Sparganosis in the Lumbar Spine : Report of Two Cases and Review of the Literature

Sparganosis is a rare parasitic infection affecting various organs, including the central nervous system, especially the lumbar epidural space. This report describes the identification of disease and different strategies of treatments with preoperative information. A 42-year-old man presented with a 2-year history of urinary incontinence and impotence. He had a history of ingesting raw frogs 40 years ago. Magnetic resonance (MR) imaging showed an intramedullary nodular mass at conus medullaris and severe inflammation in the cauda equina. A 51-year-old woman was admitted with acute pain in the left inguinal area. We observed a lesion which seemed to be a tumor of the lumbar epidural space on MR imaging. She also had a history of ingesting inadequately cooked snakes 10 years ago. In the first patient, mass removal was attempted through laminectomy and parasite infection was identified during intra-operative frozen biopsy. Total removal could not be performed because of severe arachnoiditis and adhesion. We therefore decided to terminate the operation and final histology confirmed dead sparganum infection. We also concluded further surgical trial for total removal of the dead worm and inflammatory grannulation totally. However, after seeing another physician at different hospital, he was operated again which resulted in worsening of pain and neurological deficit. In the second patient, we totally removed dorsal epidural mass. Final histology and enzyme-linked immunosorbent assay (ELISA) confirmed living sparganum infection and her pain disappeared. Although the treatment of choice is surgical resection of living sparganum with inflammation, the attempt to remove dead worm and adhesive granulation tissue may cause unwanted complications to the patients. Therefore, the result of preoperative ELISA, as well as the information from image and history, must be considered as important factors to decide whether a surgery is necessary or not.     

Bowel Ischemia in Refractory Status Epilepticus: Report of Two Cases and Review of the Literature

Background

Prolonged systemic antibiotic prophylaxis for central nervous system (CNS) devices may be associated with increased risk of antimicrobial resistance. The primary objective of this study was to determine the impact of prolonged CNS device antibiotic prophylaxis on the growth of resistant microorganisms and Clostridium difficile.

Methods

This retrospective, observational, cohort study included patients admitted to intensive care units with traumatic brain injury or other neurocritical illness. Patients who received a CNS device and antibiotic prophylaxis for at least 72 h were compared to patients with similar neurologic injuries who did not receive a CNS device.

Results

Study (n = 116) and control (n = 557) patients had mean APACHE II scores of 17.7 ± 9.2 and 15.1 ± 10.6 (p = 0.004) with 53.4 and 24.6 % receiving craniotomies (p < 0.001), respectively. Mean CNS device duration was 9.9 days, and 73 % of patients received cefuroxime for prophylaxis. The study cohort had a higher absolute incidence of resistant organisms compared with the control cohort (15.5 vs 4.1 %; odds ratio 1.93, 95 % CI 0.93–4.03, p = 0.078), though the study was underpowered to show statistical significance in multivariate analysis. C. difficile incidence was similar between groups (2.6 vs 2.0 %; odds ratio 1.45, 95 % CI 0.35–6.12, p = 0.61).

Conclusion

We found a higher incidence of resistant organisms in patients receiving prolonged antibiotic prophylaxis with a CNS device, but similar incidence of C. difficile compared to controls. Lack of data supporting prolonged antibiotic prophylaxis for CNS devices and the risk of nosocomial infections with resistant organisms encourage limiting prophylactic antibiotics to a short periprocedural course.

     

面神经瘤5例报告并文献复习

目的探讨面神经瘤手术方法、面神经保留及重建方法的选择与面神经功能恢复的关系。方法5例面神经瘤均行肿瘤切除术,2例神经纤维瘤和1例神经鞘瘤术后复发再次手术;1例面神经保留,3例面神经重建,1例面神经成形术。结果5例面神经瘤中神经鞘瘤3例和神经纤维瘤2例,均手术全切除。5例面神经功能按House分级法评估:Ⅰ级1例,Ⅱ-Ⅲ级2例,Ⅲ级1例,V级1例。结论面神经重建应取积极态度,争取一期手术,延期手术应在面肌萎缩前6～8个月内进行。     

Congenital Absence of a Cervical Spine Pedicle : Report of Two Cases and Review of the Literature

Congenital absence of a cervical spine pedicle is a rare clinical entity, and it is usually found incidentally on radiological studies performed after trauma in patients with cervical pain. We report two cases of congenital absence of a cervical spine pedicle and present a review of the literature.     

硬脑膜动静脉瘘3例报告及文献复习

正1病例介绍1.1病例1患者女性,53岁,因"反复头痛伴头昏、胡言乱语1月余"于2018年1月10日入院。患者1个月前开始无明显诱因出现头痛,以左侧额部、头顶部为主,呈阵发性针刺样,每次发作时间数小时不等,程度可耐受,且伴有头昏沉,并多次出现发作性胡言乱语、答非所问、行走缓慢,无恶心呕吐、视物模糊、肢体抽搐等。既往近30年来有反复发作头痛病史,平时涂抹风油精或清凉油可稍缓解,未诊治。有青     

颅内静脉窦血栓形成（9例临床报道）

目的:探讨脑静脉血栓形成(CVT)的临床特点与影像学表现。方法:回顾性分析本院9例CVT的临床及影像学资料。结果:①临床表现:头痛(77%)、癫样抽搐(66%)、局灶性神经功能缺失(66%)、视乳头水肿(44%)、不同程度意识障碍(22%)。②影像学资料及治疗转归:1例头颅CT示弥漫性脑水肿,蛛网膜下隙出血合并多发出血性脑梗死,因颅内压持续增高,予外科手术行去骨板减压术,见大脑表面浅静脉怒张呈绳索状并多数点片状出血灶。8例行头颅MRI和MRV,显示直接征像8例、间接征像6例。其中2例同时行DSA,均显示多个静脉和静脉窦充盈缺损。静脉血栓部位:上矢状窦合并浅静脉血栓1例,上矢状窦合并侧窦及多处静脉受累7例,直窦血栓1例。7例行肝素抗凝等对症治疗、1例行血管内局部溶栓、1例行矢状窦手术取栓并抗凝治疗。症状缓解至痊愈7例,病死2例。结论:首诊时诊断不明确,可先作头颅CT,排除颅内非CVT性病变,临床已拟诊CVT时,应首选MRI+MRV,进行综合判断是目前诊断和随访CVT的最好方法,婴幼儿CVT常表现为癫发作起病,提示CVT是婴幼儿重要的未被识别的性发作原因之一。     

The Posterior Fossa and Foreign Accent Syndrome: Report of Two New Cases and Review of the Literature

Foreign accent syndrome is a rare motor speech disorder that causes patients to speak their language with a non-native accent. In the neurogenic condition, the disorder develops after lesions in the language dominant hemisphere, often affecting Broca’s area, the insula, the supplementary motor area and the primary motor cortex. Here, we present two new cases of FAS after posterior fossa lesions. The first case is a 44-year-old, right-handed, Dutch-speaking man who suffered motor speech disturbances and a left hemiplegia after a pontine infarction. Quantified SPECT showed a bilateral hypoperfusion in the inferior lateral prefrontal and medial inferior frontal regions as well as a significant left cerebellar hypoperfusion. Further clinical investigations led to an additional diagnosis of brainstem cognitive affective syndrome which closely relates to Schmahmann’s syndrome. The second patient was a 72-year-old right-handed polyglot English man who suffered a stroke in the vascular territory of the left posterior inferior cerebellar artery (PICA) and developed a foreign accent in his mother tongue (English) and in a later learnt language (Dutch). In this paper, we discuss how the occurrence of this peculiar motor speech disorder can be related to a lesion affecting the posterior fossa structures.