特发性颅内压增高的研究进展

特发性颅内压增高也称做假性脑瘤,是一种以无明确原因的颅内压升高为特点的临床综合征。此类患者的神经影像学检查和脑脊液分析正常,但颅内压增高及其相关症状和体征却存在,如果延误治疗,可能会导致患者并发严重的视觉障碍。目前临床上对该病的认识产生了一些新的观点。本文主要综述特发性颅内高压的病理机制、诊断及治疗的研究进展。     

Pseudotumor cerebri secondary to lithium carbonate

Three patients were initially seen with headache, blurred vision, and papilledema while taking lithium carbonate for their respective bipolar affective disorder. A diagnosis of pseudotumor cerebri was made in each case when a thorough evaluation revealed only elevated intracranial pressure. Two of the patients had complete resolution of their symptoms and papilledema after discontinuing use of the drug. Increased intracranial pressure with papilledema persisted in the third patient when she failed to adjust psychiatrically, necessitating continuance of the lithium carbonate therapy. A history of lithium carbonate ingestion should be sought in patients with the syndrome of pseudotumor cerebri. All patients receiving this drug should have a regular funduscopic examination.     

Benign Intracranial Hypertension with Particular Reference to Its Occurrence in Fat Young Women

Benign intracranial hypertension (pseudotumor cerebri), a syndrome common to a number of disorders, is characterized by headaches and blurred vision. The patient is alert and has papilledema without localizing signs. Air studies show normal ventricles under increased pressure. The authors describe 61 consecutive cases of this pseudotumour, 48 of which were in fat young women, and propose that this group represents a clinical entity that has hitherto received little attention.

In these 61 patients, 40 complete-exchange pneumoencephalograms showed normal ventricles, normal fluid volume and prominent cortical sulci. In 32, subtemporal decompression resulted in prompt and lasting relief. Three patients had late convulsive seizures after surgery. Seven patients had nasal quadrantanopsias, the implications of which are discussed. The authors believe that the high intracranial pressure in this condition is due to cerebral hyperemia, not brain edema. Further investigation will perhaps demonstrate a relationship between obesity, vascular dilatation and increased intracranial pressure.

     

原发性低颅压综合征的临床及影像学特点

目的:探讨原发性低颅压综合征的临床特点和影像学改变。方法:回顾性分析10例原发性低颅压综合征(SIH)患者的临床表现、脑脊液检查及影像资料。结果:10例患者体位性头痛的发生率为100%,可伴有头晕、恶心、呕吐、耳鸣、复视、颈抵抗、行走不稳;腰穿脑脊液(CSF)压力均低于70 mmH2O,50%患者蛋白升高,40%白细胞升高,20%红细胞升高。影像学检查可见硬脑膜增厚、弥漫性强化、硬膜下积液和硬膜下血肿等表现。经补液治疗后均痊愈。结论:体位性头痛是原发性低颅压综合征的特征性改变,腰椎穿刺及头颅MR I检查具有诊断价值;本病误诊率较高,经治疗后预后良好。     

Pseudotumour cerebri in association with polyarthritis, urticaria and cryoglobulinemia.

Pseudotumour cerebri is a disorder characterized by elevated intracranial pressure in the absence of hydrocephalus and intracranial mass lesions. The cause of this disease remains obscure but has been related to diverse underlying conditions. We report a patient with pseudotumour cerebri, polyarthritis, urticaria, hypocomplementemia and cryoglobulinemia. Serial lumbar punctures were unsuccessful in lowering the cerebrospinal fluid (CSF) pressure. A trial of prednisone, 40 mg daily for 4 weeks, resulted in clinical improvement but was ineffective in lowering the CSF pressure. Circulating immune complexes and complement activation may have played a role in the development of increased intracranial pressure. Cryoglobulinemia should be added to the list of disorders associated with pseudotumour cerebri.     

特发性颅内压增高综合征的临床研究

特发性颅内压增高综合征(IIH)是一种相对少见、病因不明的慢性颅内压增高,易发生在育龄肥胖女性。临床以头痛、视盘水肿为主要表现,无脑结构和脑脊液成分的异常,视觉丧失是最重要的致残原因。病程早期的治疗强调减肥和服用碳酸酐酶抑制剂,若颅内高压导致进行性视力减退应提前施行视神经鞘开窗术。作者回顾了近年对该病发病机制、诊断和鉴别诊断以及治疗方面的研究进展。     

Intracranial hypertension with delayed puberty: a rare presentation of juvenile onset systemic lupus erythematosus

An adolescent boy presented with headache, bilateral papilloedema, growth retardation and absent secondary sexual characteristics. The diagnosis of intracranial hypertension was confirmed by increased intracranial pressure and normal neuroimaging of the brain except for partial empty sella and prominent perioptic cerebrospinal fluid (CSF) spaces. Evaluation showed an erythrocyte sedimentation rate of 150 mm/hr, positive antinuclear antibody, anti-dsDNA and antiribosomal P protein. Renal biopsy revealed diffuse segmental proliferative lupus nephritis (LN) class IV-S (A), which confirmed the diagnosis of systemic lupus erythematosus (SLE). Treatment of LN with intravenous pulse methylprednisolone and cyclophosphamide normalised the patient's CSF pressure and symptoms. In cases of intracranial hypertension, SLE must be considered. Growth retardation and absence of secondary sexual characteristics could coexist and may be presenting features of SLE. These manifestations point to advanced grades of LN, which could be asymptomatic and may be missed without a renal biopsy.     

首诊眼科的颅内病变患者临床分析

目的:分析首诊于眼科的颅内病变患者临床特征。方法:回顾性分析2009年6月至2012年4月因视力障碍或视野缺损首诊于我院眼科的13例患者,均以视神经病变、视乳头水肿收入院,最终经MRI、MRV、脑脊液检查等确诊为颅内病变。结果:13例患者单眼发病5例,双眼发病8例,11例以视力下降或一过性视物模糊为主要症状,2例以视野缺损为主要症状,行头颅MRI检查7例为颅内占位性病变,具体为:3例脑膜瘤,1例松果体瘤,1例颅咽管瘤,2例颈内动脉瘤,余6例头颅CT或MRI检查未见明显异常,腰穿脑脊液压力均大于250mmH2O,其中2例MRV或DSA检查提示颅内静脉窦血栓形成(CVST)。结论:以视神经病变、视乳头水肿为表现的患者应行头颅MRI检查以排除颅内占位性病变,有些视乳头水肿患者头颅MRI检查阴性时,需进一步行MRV、DSA等检查以排除颅内静脉窦血栓形成等疾病。     

介绍一种复制急性颅内高压动物模型的新方法

急性颅内高压动物模型一般系采用向脑室内定量注入生理盐水或人工脑脊液来形成。但用此法产生的颅内高压压力不稳定,随时间衰减。我们改用恒压灌注的方法形成颅内高压动物模型,方法简单,压力恒定。如果调节恒压灌注器的高度,还可获得准确的不同程度的颅内压。本方法是形成急性颅内高压动物模型较好的方法。     

自发性低颅内压综合征20例分析

目的探讨自发性低颅内压综合征(SIH)的临床表现、脑脊液、影像学的改变,诊治及预后,提高对本病的认识。方法对20例自发性低颅内压综合征患者的临床资料进行回顾性分析。结果 20例患者均有体位性头痛,卧位时缓解或消失,腰穿压力均<0.588 kPa(60 mm H2O),其中8例呈血性CSF,蛋白增高。6例白细胞升高。17例行头CT检查正常,1例硬膜下积液,1例硬膜下血肿,1例脑室变小。MRI检查7例显示弥漫性硬脑膜增厚强化。结论 SIH患者以体位性头痛为主要症状,脑脊液压力<60 mm H2O。治疗以补充生理盐水为主,疗效不佳时可给予激素及鞘内注入生理盐水。一般预后良好。认识自发性低颅内压综合征的临床表现、脑脊液、影像学的改变至关重要。注意与颅内高压、蛛网膜下腔出血,原发性、肥厚性硬脑膜炎等鉴别。     

改进侧脑室引流术在儿童结核性脑膜炎中的治疗价值

目的探讨治疗儿童结核性脑膜炎颅内高压的有效方法。方法66例儿童结核性脑膜炎颅内高压患儿分别以改进法(实验组)、传统法(对照组)行侧脑室穿刺引流术,比较术后患儿临床表现、颅内压水平、脑脊液性状、脑脊液外渗及颅内感染情况及存活率。结果两组在颅内压恢复正常例数、脑脊液恢复正常时间、脑脊液外渗、颅内感染及意外情况、存活率等方面有显著性差异(P<0.05)。结论改进侧脑室穿刺引流术治疗儿童结核性脑膜炎优于传统法,值得推广。     

新型隐球菌脑膜炎45例临床分析

目的 探讨新型隐球菌脑膜炎（CNM）患者的临床特点,为诊断和治疗提供经验与参考。 方法 回顾性分析2010年1月—2019年6月海南省人民医院收治的45例CNM患者的临床表现及治疗情况。 结果 4.44%（2例）有明确鸽子接触史,22.22%（10例）合并有艾滋病,28.89%（13例）合并有肺结核、肝病等其他基础疾病。主要临床症状为头痛（36/45,80%）,发热（31/45,68.89%）,恶心、呕吐（15/45,33.33%）,复视、视力下降（3/45,6.67%）,意识障碍（4/45,8.89%）,抽搐（2/45,4.44%）,听力下降（1/45,2.22%）,脑膜刺激征阳性（11/45,24.44%）。80%(36例)出现高颅压,20例脑脊液压力大于300 mmH₂O。脑脊液检查显示颅内压升高,脑脊液蛋白、糖、氯化物呈现一高二低现象;20例患者脑脊液培养阳性,分离菌株对氟康唑、5-氟胞嘧啶(5-FC)、两性霉素B(AmB)和伏立康唑的敏感性较高;45例患者中,18例给予AmB联合5-FC治疗,12例接受AmB联合氟康唑治疗,4例接受氟康唑+5-FC治疗,8例单用AmB,3例单用氟康唑治疗,治愈24例,好转2例,有效率57.78%。 结论 CNM患者主要以颅内高压为特点,患者出现发热、头痛、脑膜刺激征阳性,应及时行腰穿,反复行脑脊液涂片及培养明确诊断,隐球菌对多种抗真菌药敏感,临床可个体化采用AmB、氟康唑（或）5-FC的联合方案治疗。     

氨茶碱治疗剖宫产术后不典型头痛1例

 硬脊膜穿破后头痛（postdural puncture headache,PDPH）是产科椎管内麻醉后常见并发症之一。通常认为是硬脊膜穿破后脑脊液漏导致颅内压降低而产生。典型的PDPH具有明显的体位特征。硬脊膜穿破后也可并发颅内硬膜下血肿等严重并发症,此时头痛的特征就变得不典型。本文报道1例在硬膜外阻滞下行剖宫产术后出现PDPH的病例,头痛的症状之后变得不典型,CT提示双侧额颞部硬膜下血肿,经氨茶碱治疗头痛明显缓解,且未再复发。     

早期腰大池置管治疗伴有恶性颅高压新型隐球菌性脑膜炎临床分析

目的：观察早期腰大池置管持续引流治疗伴有恶性颅高压的新型隐球菌性脑膜炎患者的临床效果。方法：对7例伴有恶性颅高压的新型隐球菌性脑膜炎患者采用早期腰大池置管持续引流（最长引流时间达4周）,同时给予两性霉索-B静脉、鞘内注射及5-氟胞嘧啶口服等常规治疗方案,以隐球菌计数、墨汁染色阴性时间及临床症状改善情况为观察指标。结果：有头痛、呕吐、意识障碍的患者经早期腰大池置管持续引流后症状即刻减轻,所有患者精神状态短期内明显好转;1例视力障碍的患者经引流处理2天后视力基本正常,仅有1例遗留有轻度视力障碍。经联合治疗最后痊愈4例,好转3例。治愈的4例患者随访至今均无复发。结论：早期腰大池置管持续引流能有效降低新型隐球菌性脑膜炎颅内压,改善临床症状,为抗真菌治疗赢取时间。     

单侧内耳Mondini畸形并发脑脊液耳漏1例报告及文献复习

目的：探讨单侧内耳Mondini畸形并发脑脊液耳漏患者的发病机制、临床表现、诊断和手术方法,提高对其临床特征的认识。方法：收集1例单侧内耳Mondini畸形并发脑脊液耳漏患者的临床资料,结合国内外相关文献,分析其临床特征、影像学表现、诊断和手术方法。结果：患者因"左侧鼻腔流水、头痛20年,加重1个月"就诊。查体见左耳鼓膜内陷、完整,左耳听力下降。鼻内镜下,颅内压增加时左侧咽鼓管咽口有脑脊液流出。结合患者相关检查结果和临床表现初步考虑左耳Mondini畸形并脑脊液耳漏。行左脑脊液耳漏修补术,患者术后听力良好,无鼻腔流液和头痛不适。随访1年,患者未复发脑膜炎。结论：反复发作不明原因的脑膜炎患者应考虑先天性内耳畸形的可能。颞骨薄层CT与MRI检查是确诊的主要手段,鼓室探查修补术是填补漏口的有效方法,手术是主要的治疗方法。     

A patient with intracranial hypotension and co-existent normal-pressure glaucoma: The beijing intracranial and intraocular pressure (iCOP) study

Glaucoma is the second leading cause of blindness worldwide. Normal tension glaucoma(NTG) is becoming the primary type of glaucoma. Unfortunately, the pathogenesis of NTG is unclear. Recently, some reports suggested that lower intracranial pressure was an important risk factor of NTG. Here, we reported a 53-year old patient complaining about positional headache suffered from intracranial hypotension. At the same time of diagnosis, coexistent normal-tension glaucoma was detected. So, Normal-tension glaucoma may pathogenetically be associated with low cerebrospinal fluid pressure.     

老年蛛网膜下腔出血患者头痛的相关因素

目的了解老年人蛛网膜下腔出血(SAH)后头痛的相关因素,探讨其发病机制。方法分析82例老年SAH患者出血量、颅压、脑脊液改变与头痛程度的关系,并与84例非老年性SAH患者作对照。结果老年SAH组CT显示多为中等量以下出血(92.6%),颅压多在2.94 kPa以下(93.9%)。老年组中等量以上出血者所致头痛程度与阴性、少量出血者无明显差异(P>0.05),颅压>2.94 kPa时头痛明显加重(P<0.01),脑脊液为新鲜出血时头痛明显(P<0.05)。头痛程度较非老年SAH组轻(P<0.05)。结论发生血性变的CSF引起蛛网膜下腔广泛的炎性反应,其程度在老年人可能较轻,头痛程度较非老年人轻。     

Spontaneous intracranial hypotension: case reports and literature review

The clinical syndrome of spontaneous intracranial hypotension (SIH) was first proposed in 1938 and describes a headache syndrome virtually identical to the headaches, which may follow dural puncture. Orthostatic headache, low cerebrospinal fluid opening pressure, and diffuse meningeal enhancement on post-contrast T1-weighted MRI brain studies are the major features of this increasingly recognised syndrome. We describe a case series of patients diagnosed with SIH, their treatment and outcome, and a review of the literature. We propose that this is an important cause of new daily persistent headaches, which is usually relatively easy to diagnose, and if recognised early, is eminently treatable.     

内镜经鼻入路颅底软骨肉瘤切除术后铅黄肠球菌脑膜炎合并顽固性脑脊液漏一例

铅黄肠球菌脑膜炎十分罕见,它属于具有高致死率的肠球菌脑膜炎,但目前对其临床特点和预后了解很少,对其诊断和治疗也缺乏经验。我们在此报道一例特殊的术后铅黄肠球菌脑膜炎病例。这是一位50岁男性患者,罹患广泛累及中颅底的巨大软骨肉瘤,在内镜经鼻入路全切肿瘤的过程中,鞍结节区发生硬脑膜缺损,术中予人工硬脑膜修补,但患者术后发生反复发作的脑膜炎、难治性脑脊液漏和间断性颅高压,脑膜炎和颅高压导致两次经鼻内镜脑脊液漏修补术失败。在第二次脑膜炎发作时,多次脑脊液培养证实脑膜炎致病菌为万古霉素耐药性铅黄肠球菌。铅黄肠球菌脑膜炎最终通过敏感抗生素利奈唑胺治疗和反复腰穿引流治愈,随后,通过第三次内镜经鼻入路多层修补术和同时进行的腰大池腹腔分流术成功治愈脑脊液漏。在至今两年的随访过程中未见脑膜炎、脑脊液漏和颅高压复发。     

原发性低颅压综合征临床特征、脑脊液和影像学改变

目的：分析原发性低颅压综合征临床特征、脑脊液和影像学改变。方法方便选取该院2013年3月—2016年3月接诊的原发性低颅压综合征患者22例,对其临床资料进行回顾性分析。结果原发性低颅压综合征易发年龄为30~45岁,主要临床症状为体位性头痛、头晕、呕吐、耳鸣,部分患者伴有不同程度的神经系统体征,腰穿脑脊液压力﹤70 mmH2O,8例患者红细胞增多,9例患者脑脊液蛋白升高,经影像学检查可见患者硬膜强化、增厚,硬膜下可见积液及血肿,局部组织移位,基底池狭窄,给予患者补液及对症治疗,治疗效果显著。结论原发性低颅压综合征的主要临床特点为体位性头痛、腰穿脑脊液压力低,患者影像学可见硬膜强化、增厚,硬膜下可见积液及血肿,局部组织移位,基底池狭窄等改变。