自发性低颅压综合征31例临床特征和影像学分析

目的 探讨自发性低颅压综合征（SIH）的临床特点和影像学改变.方法 回顾性分析31例SIH患者的临床及影像资料.结果 所有患者均有体位性头痛,可伴有恶心、呕吐、头晕、耳鸣、听力丧失、行走不稳、一过性视物模糊、复视、颈肩部疼痛、颈强直 CSF压力均＜70 mmH2O,蛋白升高12例,WBC增多11例,RBC增多7例 全部患者行头颅CT检查,8例见脑肿胀,脑沟变浅,脑室变窄,临床症状消失后复查均恢复正常 15例行头颅MRI检查,8例行增强检查示硬脑膜弥漫性强化,头颅CT及MRI检查见4例硬膜下积液、2例硬膜下血肿 全部患者采用内科综合疗法,1例行硬膜下血肿清除术,均痊愈.结论 SIH的临床表现多样,体位性头痛是特征性症状,腰穿CSF及头颅MRI检查具有诊断价值 本病预后良好,但少数并发硬膜下血肿.     

原发性低颅压综合征15例临床分析及文献复习

目的探讨原发性低颅压再综合征（PIH）的临床特点、发病机制、脑脊液（CSF）及影像学改变。方法回顾性分析15例PIH患者的临床资料并复习文献。结果15例患者均有体位性头痛；CSF压力均小于70mmH2O，细胞数增高2例，蛋白增高9例；13例行头颅CT平扫，9例正常，2例双侧脑室系统缩小，1例慢性硬膜下血肿，1例硬膜下积液；9例行头颅MRI检查，4例硬脑膜增厚并明显强化，1例慢性硬膜下血肿，1例“脑下沉”改变。结论认识PIH的临床特点、发生机制、CSF及影像学特征，可提高PIH的确诊率；本病预后一般较好。     

原发性低颅压综合征13例分析

目的探讨原发性低颅压综合征(PIH)的临床特点,脑脊液(CSF)及影像学改变,以了解PIH诊断及预后,以提高对本病的认识。方法分析13例PIH患者临床资料。结果 13例PIH患者均有体位性头痛;脑脊液压力均<70mmH2O,细胞数增高者2例,蛋白增高者6例;11例行头颅CT平扫8例正常,2例(例4、9)硬膜下积液,1例(例7)例双侧侧脑室缩少;6例行头颅MRI 2例(例1、5)硬脑膜增厚,并均匀强化,1例(例9)硬膜下积液,1例(例13)"脑下沉"。结论 PIH患者以体位性头痛为主要症状,脑脊液压力<70mmH2O,头颅CT/MRI可提示硬膜下积液、硬脑膜增厚及"脑下垂"等改变。经大量补液及鞘内注入生理盐水,口服尼莫地平等,治疗效果良好。     

13例自发性低颅压综合征的临床分析

目的 探讨自发性低颅压综合征(SIHS)的临床特点、诊断及鉴别诊断。方法 回顾性分析13例SIHS患者的临床表现、CSF和头颅影像学的特征。结果 所有患者均有体位性头痛，卧位时缓解，可伴有恶心、头晕、耳鸣、复视、听力下降和颈项强直；CSF压力均低于60mmH2O，轻中度白细胞增多和蛋白增高，黄变3例；4例行头颅MRI检查，均显示弥漫性硬脑膜增厚；13例行头颅CT检查，11例正常，1例硬膜下血肿，1例双侧硬膜下积液。硬膜外保留置管注入生理盐水治疗4例，全部迅速治愈。结论 认识SIHS的临床特点、CSF改变和MRI的特征，对及时诊断有重要价值；硬膜外保留置管注入生理盐水治疗SIHS效果较好。     

原发性低颅压综合征16例临床分析和影像学特点

目的 探讨原发性低颅压综合征(SIH)的临床特点、发病机制、脑脊液(CSF)及影像学改变.方法 回顾性分析16例SIH患者的临床资料.结果 16例患者均有体位性头痛;CSF压力均＜70mmH2O,细胞数增高2例,蛋白增高9例;16例行头颅CT平扫,11例正常,2例脑肿胀,2例慢性硬膜下血肿,1例硬膜下积液;9例行头颅MRI检查,4例硬脑膜增厚并明显强化,2例慢性硬膜下血肿,1例脑下沉改变.结论 认识SIH的临床特点、发病机制、CSF及影像学特征,可提高SIH的确诊率;本病预后一般较好.     

老年人原发性低颅压综合征的临床与影像学分析

目的 研究老年人原发性低颅压综合征(PIH)的临床及影像学特点,提高对本病的认识.方法 19例老年人PIH患者,观察其临床特点、脑脊液(CSF)压力、生化、头颅CT及MRI扫描.结果 起立性头痛和呕吐是其显著的特征,CSF压力均＜7cmH2O;部分病人脑膜刺激征阳性.CT可见脑室系统、脑池及脑沟变窄、缩小,部分患者可见硬膜下积液;MRI增强示硬脑膜强化、硬膜下积液、桥脑扁平、桥池变窄及小脑扁桃体疝.结论 老年人PIH发生率低,容易误诊,了解老年人PIH的临床、CSF、CT及MRI的特异性改变,对正确诊断PIH至关重要.     

低颅压综合征12例临床分析

目的 探讨低颅压综合征的临床特点、诊断、治疗及鉴别诊断。方法 回顾性分析12例低颅压综合征患者的临床资料。结果 所有患者均有体位性头痛，卧位时缓解，可伴有恶心、呕吐、耳鸣、眩晕、颈强直；CSF压力均低于60mm H2O，3例患者CSF中有轻中度白细胞增多和蛋白增高；1例患者为血性CSF；12例行头颅CT检查正常；2例头颅MRI示弥漫性硬脑膜增厚。治疗以大剂量补充生理盐水为主，2例腰穿时注入生理盐水治愈。结论 认识低颅压综合征的临床、CSF和MRI表现至关重要，因其极易与蛛网膜下腔出血等相混淆。     

自发性低颅压综合征临床表现、影像学特点及预后分析

目的探讨自发性低颅压综合征的临床、预后及影像学特点。方法北京协和医院2009年至2014年收治的自发性低颅压综合征患者16例,对其临床表现、实验室检测、影像学特点及预后进行分析。结果所有患者均表现为体位性头痛。13例脑脊液压力<60mm H2O,3例脑脊液压力为0。6例头颅CT提示硬膜下积液/积血,2例脑叶出血,5例存在双侧侧脑室体积减小,8例未见明显异常。头颅增强MRI检查示硬脑膜弥漫强化9例,硬膜下出血3例,硬膜下积液3例,脑组织下移4例,垂体增大2例。结论体位性头痛是自发性低颅压综合征相对典型的症状之一,多数患者预后良好。头颅增强MRI可显示弥漫性硬脑膜强化等典型的低颅压改变,头颅CT亦可提示部分改变。在详细询问病史的基础上,重视对头颅CT检查的判读,避免对头颅增强MRI检查的过度依赖,有助于该病的早期诊断。     

原发性低颅压综合征30例临床分析

目的 探讨原发性低颅压综合征(SIH)临床特点、发病机制、脑脊液(CSF) 及治疗方法.方法 回顾分析30例原发性低颅内压综合征患者的临床资料.结果 所有患者均有直立性头痛,部分病例伴有恶心、呕吐、耳鸣、头晕、颈强直;脑脊液压力测定,20.4～40.8 mmH2O为8例,41.8～51.0 mmH2O为11例,52.0～68.34 mmH2O为6例,测不出压力5例.CSF红细胞(440～600)×109/L 13例.白细胞(52.02～68.34)×109/L 9例,蛋白400～600 mg/L 8例,压颈实验无阻塞.影像学检查:本组全部行头颅CT检查,正常8例,双额颞硬膜下积液6例,脑室变小、脑组织肿胀9例,8例头颅CT检查正常者行头颅MRI检查,2例弥漫性硬脑膜增厚.给予大量补液、对症、支持治疗,颅压≤35 mmH2O者予以生理盐水鞘内注射,效果良好.结果 认识原发性低颅内压综合征的临床特点、发病机制、CSF和影像学表现至关重要,本病应警惕硬膜下血肿的发生,并注意与与蛛网膜下腔出血等的鉴别.结论 正确了解SIH的临床表现、脑脊液、头颅CT和MRI特点,预后良好.     

儿童蛛网膜囊肿外伤后硬膜下出血或积液的诊治

目的总结儿童蛛网膜囊肿外伤后硬膜下出血或积液的治疗经验。方法2012年收治5例男性儿童蛛网膜囊肿患者,外伤后发生硬膜下出血或积液,其中2例出血患者均行开颅血肿清除加囊壁切除术;3例硬膜下积液患者中,1例行蛛网膜囊肿一腹腔分流术,2例行神经内镜造瘘术。结果治疗1月后,5例均行CT或MRI检查随访,示硬膜下血肿吸收,硬膜下积液较前明显减少,头痛症状消失,恢复正常生活。结论儿童蛛网膜囊肿外伤后硬膜下出血或积液患者采取积极的手术治疗,可获得满意效果。     

原发性低颅压综合征18例临床、脑脊液和影像学特点分析

目的探讨原发性低颅压综合征的临床、脑脊液和影像学特点。方法回顾性分析18例原发性低颅压综合征的临床资料。结果原发性低颅压综合征以体位性头痛、恶心、呕吐、头晕、耳鸣为主要表现,部分伴有神经系统体征,腰穿脑脊液压力均低于70mmH2O,27%患者红细胞增多,33%脑脊液蛋白增高,影像学检查可见硬膜增厚、强化、硬膜下积液和硬膜下血肿等表现,给予大量补液治疗及对症支持治疗,效果良好。结论体位性头痛、腰穿脑脊液压力低为原发性低颅压综合征的主要临床特点,影像学可呈现硬膜增厚强化等特征性改变,治疗以大量补液为主。     

Syndrome of spontaneous cerebrospinal fluid hypovolemia: report of six cases

Syndrome of spontaneous cerebrospinal fluid hypovolemia (SCH) is a rare cause of new onset headache. We report six cases of SCH presenting with new onset headache. All six cases were females. Acute onset orthostatic headache and neck pain were the chief characteristics of SCH in our cases. The MRI brain showed pachymeningeal gadolinium enhancement in all patients. Spinal extradural CSF collection was demonstrable on MRI in three cases. All cases improved with conservative therapy. High index of clinical suspicion and contrast enhanced MRI brain is the key to accurate diagnosis in the majority of cases.     

原发性低颅压综合征的影像学特点

目的 分析原发性低颅压综合征病人影像学特点并探讨其发生的机制。方法 回顾分析了12例临床诊断明确的原发性低颅压综合征病人的临床及影像资料。结果 腰穿结果提示10例病人脑脊液压力低于60mmH2O，11例蛋白增高，3例细胞数增高。头MRI示硬脑膜弥漫性增强6例，硬膜下出血2例，硬膜下积液1例，下垂脑2例，垂体增大2例。核素脑池造影1例显示腰骶部脑脊液漏，1例显示核素不能到达大脑半球面。结论 直立性头疼是原发性低颅压综合征病人最典型的临床症状，硬脑膜弥漫性增强是最常见的影像学表现，脑脊液容量减低是原发性低颅压综合征病人临床及影像表现的病理生理基础。     

Spontaneous intracranial hypotension in the absence of magnetic resonance imaging abnormalities

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a neurologic syndrome of unknown etiology, characterized by features of low cerebral spinal fluid (CSF) pressure, postural headache and magnetic resonance imaging (MRI) abnormalities. METHODS: Four symptomatic cases of SIH presented to our institution over a six-month period. Magnetic resonance imaging studies were performed in all four cases. Diagnostic lumbar puncture was done in all except one case. RESULTS: All of the patients on whom lumbar punctures were performed demonstrated low CSF pressure and CSF protein elevation with negative cultures and cytology. Three out of the four patients exhibited MRI findings of diffuse spinal and intracranial pachymeningeal gadolinium enhancement and extradural or subdural fluid collections. One patient had no MRI abnormalities despite prominent postural headache and reduced CSF pressure at lumbar puncture. All patients recovered with intravenous fluids and conservative treatment. CONCLUSIONS: Magnetic resonance imaging abnormalities are found in most, but not all patients, with SIH. Cerebral spinal fluid abnormalities can be detected even in patients with normal MRI studies. It is important to recognize the variability of imaging results in this usually benign disorder.     

Pneumocephalus in Patients With Orthostatic Headache

Cerebrospinal fluid (CSF) leak or shunt overdrainage is a well-known cause of orthostatic headaches and low CSF pressures. We report two cases of orthostatic headache with pneumocephalus on brain imaging. The orthostatic headache developed after drainage of spinal operation site and epidural block. Brain MRI revealed characteristic findings of CSF hypovolemia including pachymeningeal enhancement and mild subdural fluid collections. Air was also observed in the ventricular or subarachnoid space in both patients, which might enter the subarachnoid or ventricular space during a procedure via the pressure gradient or an injection.     

自发性低颅内压综合征七例报告

目的研究自发性低颅内压综合征（SIH）的临床、脑脊液（CSF）和影像学的改变。方法对1年8个月时间收治的7例SIH患者观察其临床表现、CSF压力、生化指标及头颅CT和MRI增强扫描。结果当患者起立时即出现头痛、呕吐及颈强直,卧位时症状消失。CSF压力均低于7cmH2O。其中5例呈血性CSF,蛋白增高似蛛网膜下腔出血（SAH）。CT示脑室缩小。增强MRI示硬脑膜强化、硬脑膜下积液、桥脑扁平、桥池变窄及小脑扁桃体下疝的特征性改变。当临床症状好转时,其CSF压力和生化指标恢复正常。结论了解SIH的临床、CSF和MRI表现至关重要,因其极易与SAH相混淆。     

Stroke and Death Due to Spontaneous Intracranial Hypotension

Background

Spontaneous intracranial hypotension has become a well-recognized cause of headaches and a wide variety of other manifestations have been reported. Recently, several patients with asymptomatic spontaneous intracranial hypotension were reported. I now report two patients with spontaneous intracranial hypotension who developed multiple arterial strokes associated with death in one patient, illustrating the spectrum of disease severity in spontaneous intracranial hypotension.

Methods

Medical records and radiologic imaging of the two patients were reviewed.

Results

Case 1. A 45-year-old man presented with an orthostatic headache. Neurologic examination was normal. MRI showed bilateral subdural fluid collections, brain sagging, and pachymeningeal enhancement. At lumbar puncture, the opening pressure was too low to record. He underwent two epidural blood patches with transient improvement of symptoms. His headaches progressed and a CT-myelogram showed a lower cervical CSF leak. Subsequently, periodic lethargy and confusion was noted and he then rapidly deteriorated. Examination showed coma (GCS: 4 [E1, M2, V1]), a fixed and dilated right pupil, and decerebrate posturing. Bilateral craniotomies were performed for the evacuation of chronic subdural hematomas. Immediate postoperative CT showed bilateral posterior cerebral artery infarcts and a recurrent right subdural hematoma, requiring re-evacuation. Postoperative examination was consistent with brain death and support was withdrawn.  Case 2. A 42-year-old man presented with a non-positional headache. Neurologic examination was normal. CT showed bilateral acute on chronic subdural hematomas and effacement of the basilar cisterns. MRI showed brain sagging, bilateral subdural hematomas, and pachymeningeal enhancement. Bilateral craniotomies were performed and subdural hematomas were evacuated. Postoperatively, the patient became progressively lethargic (GCS: 8 [E2, M4, V2]) and variable degrees of pupillary asymmetry and quadriparesis were noted. MRI now also showed multiple areas of restricted diffusion in the pons and midbrain, consistent with multiple infarcts. CT showed worsening subdural fluid collections with midline shift and increased effacement of the basilar cisterns. Repeat bilateral craniotomies were performed for evacuation of the subdural fluid collections. Neurologic examination was then noted to be fluctuating but clearly improved when lying flat (GCS: 10T [E4, M6, VT]). CT-myelography demonstrated an extensive cervico-thoracic CSF leak. An epidural blood patch was performed. The patient made a good, but incomplete, recovery with residual quadriparesis and dysphagia.

Conclusions

Arterial cerebral infarcts are rare, but potentially life-threatening complications of spontaneous intracranial hypotension. The strokes are due to downward displacement of the brain and can be precipitated by craniotomy for evacuation of associated subdural hematomas.     

Two cases of medically-refractory spontaneous orthostatic headaches with normal cerebrospinal fluid pressures responding to epidural blood patching: Intracranial hypotension versus hypovolemia and the need for clinical awareness

The diagnosis of spontaneous intracranial hypotension or cerebrospinal fluid (CSF) hypovolemia syndrome requires a high index of suspicion and meticulous history taking, demonstration of low CSF pressure and/or neuroimaging features. A 31-year-old male, presented with subacute onset moderate occipital and sub-occipital headaches precipitated by upright posture and relieved on recumbency and neck pain for 2 years. There was no trauma, cranial/spinal surgery. Clinical examination was normal and CSF opening pressure and laboratory study were normal. Magnetic resonance imaging (MRI) brain showed thin subdural hygroma. Another patient, 41-year-old male presented with 1 month of subacute onset severe bifrontal throbbing orthostatic headaches (OHs). CSF opening pressure was normal. Contrast MRI brain showed the presence of bilateral subdural hygromas, diffuse meningeal enhancement, venous distension, sagging of the brain, and tonsillar herniation. We report two cases of “spontaneous OHs” with normal CSF pressures who were successfully treated with epidural blood patching after poor response to conservative management.Key Words: Cerebrospinal fluid hypovolemia, epidural blood patch, pachymeningeal enhancement, spontaneous intracranial hypotension, spontaneous orthostatic headache