海绵窦综合征

1引言 1.1背景 海绵窦综合征由各种蝶鞍旁损害累及海绵窦所致,肿瘤、颈动脉动脉瘤、颈动脉-海绵窦瘘(CCF)和炎症是海绵窦综合征的主要病因.     

Foix综合征32例分析

对32例Foix综合征患者的临床资料进行回顾性分析,结果海绵窦区、鞍区或眶上裂肿瘤12例,痛性眼肌麻痹10例（2例出现海绵窦区占位性软组织影,其中1例第12周复查时占位性软组织消失）,海绵窦动静脉瘘5例,动脉瘤2例,海绵窦血栓形成1例,结核性肉芽肿1例,糖尿病性神经病1例。经采用不同方法治疗,预后较好。表明Foix综合征以肿瘤、痛性眼肌麻痹为主要病因。影像学检查具有重要诊断价值。确诊后应根据病因采取不同的治疗手段。     

48例糖尿病合并腕管综合征的临床及神经电生理分析

目的糖尿病合并腕管综合征的治疗方法与单纯腕管综合征或糖尿病多发性周围神经病的治疗方法均有所区别,通过总结分析糖尿病合并腕管综合征的临床资料,早期识别及诊断,有利于改善预后。方法对该院2015年4月—2016年8月住院治疗的691例糖尿病患者均进行神经传导速度测定。结果 48例检测出腕管综合征,临床症状主要表现为一侧或双手麻木,以双侧发病多见,感觉纤维损害较运动纤维损害为重。结论神经电生理技术是诊断腕管综合征的重要辅助检查,糖尿病合并腕管综合征,是糖尿病性周围神经病的一种表现形式,由于其发病机制的复杂性,其治疗方法与单纯腕管综合征或糖尿病多发性周围神经病的治疗方法均有所不同,早期识别,早期治疗,有利于改善患者的生活质量。     

急性多发性神经根神经炎的特殊类型(附3例报告)

急性多发性神经根神经炎的临床表现是颅神经及脊神经的损害,亦可出现脑、脑干、小脑及脊髓损害,分别称为急性脑多发性神经根神经炎,急性脑干多发性神经根神经炎、急性小脑多发性神经根神经炎及急性脊髓多发性神经根神经炎、此即急性多发性神经根神经炎的特殊类型。现举3例,并对急性多发性神经根神经炎的特殊类型作一讨论。     

以四肢麻木和无力首诊的原发性干燥综合征一例

原发性干燥综合征(primary sjogren's syndrome,pSS)合并神经系统损害发生率为10%～60%,90%～95%累及周围神经系统(PNS).表现主要有单神经病、感觉性神经病和远端对称性多发性神经病三种类型,以感觉性神经病变和(或)多神经炎最常见,但以感觉运动性神经病为首诊者少见.我院成功诊治1例,现报道如下.     

颅内海绵状血管瘤的显微外科治疗

海绵状血管瘤多位于脑内,少数生长在脑外.脑外海绵状血管瘤多位于鞍区,与海绵窦关系密切,常累及周围颅神经.常规手术因病变和周围结构显露欠清,术中易出现大出血迫使手术中断,降低血管瘤切除率,且易损伤海绵窦周围的颅神经.采用显微外科操作技术可以清楚显露病变和周围结构关系,最大程度减少术中出血,脑外型海绵状血管瘤患者可最大程度的避免损伤颈内动脉及海绵窦区的颅神经,大大提高肿瘤切除率.本文对25例颅内海绵状血管瘤患者的临床资料进行分析,探讨颅内海绵状血管瘤患者其临床特点及治疗方法.     

痛性糖尿病神经病诊断和治疗

痛性糖尿病神经病诊断和治疗郭玉璞糖尿病周围神经病的临床表现多种多样，其病因和发病机制亦难以用单一病因和发病机制解释。无论是对称性远端型多发性神经病或非对称的神经根、神经干和多发性单神经病，除有各自特有的症状和体征外，疼痛症状可出现于任何类型糖尿病神经...     

1例合并慢性吉兰-巴雷综合征的糖尿病周围神经病变病例

慢性吉兰-巴雷综合征, 又称为慢性炎性脱髓鞘性多发性神经根神经病(chronic inflammatory demyelinating polyradiculoneuropathy, CIDP), 是一类由免疫介导的脱髓鞘性周围神经病。本研究分析1例以四肢麻木、疼痛伴无力为主要症状的CIDP患者的临床资料, 结合文献回顾, 从发病机制、临床表现、实验室检查及治疗方面探讨CIDP和糖尿病周围神经病变的鉴别。     

老年人周围神经疾病的特点

周围神经疾病是神经系统常见的疾病,目前尚无统一的分类,临床上常见的分类包括:按病理分为轴索变性周围神经病和脱髓鞘性周围神经病;按病因分为感染性多发性周围神经病、中毒性周围神经病、营养缺乏和代谢性周围神经病、遗传性周围神经病、副肿瘤综合征、免疫介导的周围神经病及结缔组织病所致的周围神经病等;按受损神经的功能分为感觉性周围神经病、运动性周围神经病和自主神经病;按受累神经分布形式或范围分为单神经病和多发单神经病.各种分类之间有明显的重叠.周围神经疾病的诊断除了依靠临床表现以外,主要的客观检查手段是肌电图和神经传导测定以及周围神经病理检查.     

原发性干燥综合征合并POEMS综合征一例

POEMS综合征组以多发性感觉运动性周围神经病为突出表现，常常伴有多系统损害以及与浆细胞瘤相关的临床症候群。目前，国内外报道已超过百例，但合并干燥综合征从未见报道。现就诊治过的原发性干燥综合征合并POEMS综合征患者1例报告如下。     

Distant metastasis of hepatocellular carcinoma to Meckel’s cave and cranial nerves: A case report and review of literature

BACKGROUNDMetastasis occurs as a late event in the natural history of hepatocellular carcinoma (HCC), and most patients die of liver failure attributed to the tumor supplanting the liver. Conversely, the brain is a less common metastatic site.CASE SUMMARYWe describe a rare case of hepatitis C virus-related multiple HCC metastasizing to the cavernous sinus, Meckel’s cave, and the petrous bone involving multiple cranial nerves in an 82-year-old woman. At admission imaging studies including Gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging (MRI) revealed multiple HCC nodules in both right and left lobes. Ultrasound guided biopsy of the left lobe revealed moderately differentiated HCC. Molecular targeted therapy with Lenvatinib (8 mg/d for 94 d, per os) and Ramucirumab (340 mg/d and 320 mg/d, two times by intravenous injection) were administered for 4 mo, resulting in progression of the disease. Three months after the start of molecular target therapy, the patient presented with symptoms of hyperalgesia of the right face and limited abduction of the right eye, indicating disturbances in the right trigeminal and abducens nerves. Brain MRI disclosed a mass involving the cavernous sinus, Meckel’s cave and the petrous bone. Contrast-enhanced MRI with gadolinium-chelated contrast medium revealed a well-defined mass with abnormal enhancement around the right cavernous sinus and the right Meckel’s cave.CONCLUSIONThe diagnosis of metastatic HCC to the cavernous sinus, Meckel’s cave, and the petrous bone was made based on neurological findings and imaging studies including MRI, but not on histological examinations. Further studies may provide insights into various methods for diagnosing HCC metastasizing to the craniospinal area.     

脑出血合并重症格林-巴利综合征一例报道并文献复习

格林-巴利综合征(GBS)是一种免疫介导的周围神经病,常累及脑神经。脑出血是脑卒中最严重的亚型之一,每年发病近200万人,但脑出血后合并GBS极为罕见。因此本文主要分析1例脑出血合并重症GBS的临床资料,并结合既往文献探讨脑出血合并重症GBS患者的临床特点、发病机制、诊断、治疗及预后,以提高临床医生对脑出血合并重症GBS的认识。     

Cavernous sinus syndrome: a series of 126 patients

The etiology of cavernous sinus syndrome (CSS) remains difficult to determine in spite of the development of neuroimaging techniques. We conducted the current study to identify clinical and imaging features that allow a reliable approach to the etiologic diagnosis of patients with CSS. We studied a consecutive series of 126 patients with CSS, defined as involvement of 2 or more of the third, fourth, fifth (V1, V2), or sixth cranial nerves, or involvement of only 1 of them in combination with a neuroimaging-confirmed lesion in the cavernous sinus.Tumors were the most common cause of CSS (80 patients). All patients with optic nerve involvement had a tumor. No patient with a normal MRI had a tumor. The lack of pain during the course of the disease (odds ratio [OR], 0.58; 95% confidence intervals [CI], 0.06-0.40), V2 involvement (OR, 12.17; 95% CI, 2.98-49.71), and male sex (OR, 3.2; 95% CI, 1.31-8.14) were independently associated with the presence of a tumor. Pain at the onset of disease (OR, 12.09; 95% CI, 3.14-46.50) and third cranial nerve involvement (OR, 4.9; 95% CI, 1.01-24.60) were independently associated with Tolosa-Hunt syndrome.     

Rhino-Orbital-Cerebral Mucormycosis

This review focuses on sinus, sino-orbital, and rhinocerebral infection caused by the Mucorales. As the traditional term of "rhinocerebral" mucormycosis omits the critical involvement of the eye, the more comprehensive term as rhino-orbital-cerebral mucormycosis (ROCM) is used. The most common underlying illnesses of ROCM are diabetes mellitus, hematological malignancies, hematopoietic stem cell transplantation, and solid organ transplantation. Sporangiospores are deposited in the nasal turbinates and paranasal sinuses in immunocompromised patients. Qualitative and quantitative abnormalities of neutrophils, monocytes and macrophages increase the risk for development of mucormycosis. Altered iron metabolism also is a critical factor in the pathogenesis of patients with diabetes mellitus who are at risk for ROCM. Angioinvasion with thrombosis and tissue necrosis is a key pathophysiological feature of human Mucorales infection. The ethmoid sinus is a critical site from which sinus mucormycosis may extend through the lamina papyracea into the orbit, extraocular muscles, and optic nerve. The brain may be seeded by invasion of the ethmoidal and orbital veins, which drain into the cavernous sinuses. Diplopia and ophthalmoplegia may be the earliest manifestations of cavernous sinus syndrome before changes are apparent on diagnostic imaging modalities. Negative diagnostic imaging does not exclude cavernous sinus mucormycosis. Mucormycosis of the maxillary sinus has a constellation of clinical features that are different from that of ethmoid sinus mucormycosis. A painful black necrotic ulceration may develop on the hard palate, indicating extension from the maxillary sinus into the oral cavity. Orbital apex syndrome is an ominous complication of mucormycosis of the orbit. Once within the orbital compartment, organisms may extend posteriorly to the optic foramen, where the ophthalmic artery, ophthalmic nerve and optic nerve are threatened by invasion, edema, inflammation and necrosis. Early diagnosis of sinus mucormycosis is critical for prevention of extension to orbital and cerebral tissues. Optimal therapy requires a multidisciplinary approach that relies on prompt institution of appropriate antifungal therapy with amphotericin B, reversal of underlying predisposing conditions, and, where possible, surgical debridement of devitalized tissue. Outcomes are highly dependent upon the degree of immunosuppression, site and extent of infection, timeliness of therapy, and type of treatment provided. New modalities for early diagnosis and therapeutic intervention are critically needed for improved outcome of patients with ROCM.     

Successful treatment of invasive sinus aspergillosis with micafungin and itraconazole

BACKGROUND: We report a case of invasive sinus aspergillosis that extended to the orbital cavity and cavernous sinus and was improved by treatment with micafungin and itraconazole. CASE REPORT: A 83-year-old woman was referred to our hospital because of headache and impaired of eye movement on the right side. Physical examination revealed impaired function of cranial nerves, II, II, IV, and VI on the right side. MRI showed evidence of inflammation of the right sphenoid sinus and ethmoidal sinus and an enhancing mass in the right cavernous sinus and orbit. Because a culture of a specimen from the right sphenoid sinus extracted during endoscopic sinus surgery, yielede Aspergillus fumigatus, a diagnosed of invasive sinus aspergillosis complicated by cavernous sinus symdrome and orbital apex symdrome was made. It was difficult to completely remove the mass in the sinuses surgically and drug therapy with micafungin was started and then itraconazole was added. The clinical manifestations and the impaired function of cranial nerves II, III, IV, and VI improved, and MRI showed regression of the mass in the sinuses temporary in response to drug therapy. CONCLUSION: Invasive sinus aspergillosis often progresses rapidly in the absence of surgery. Our case is valuable, because invasive sinus aspergillosis was improved by drug therapy alone, and combined treatment with micafungin and itraconazole was effective.     

The dangers of the “Head Down” position in patients with untreated pituitary macroadenomas: case series and review of literature

Purpose

Cavernous sinus syndrome is a rare phenomenon, characterized by simultaneous neuropathies of cranial nerves III–VI. Various pathological processes have been reported as precipitating etiologies, including infection, inflammation, vascular lesions, and neoplasms.

Purpose

We report a unique case series of cavernous sinus syndrome attributable to prolonged Trendelenburg or prone positioning during non-cranial procedures and review the pertinent literature to enlighten on this rare but catastrophic phenomenon.

Methods

Retrospective case series.

Results

In the past year we encountered two patients who presented with acute cavernous sinus syndrome upon awakening from non-cranial operations. One patient underwent an extensive urologic resection of a bladder malignancy positioned in Trendelenburg for approximately 4 h. The second patient underwent a lumbar laminectomy and discectomy in prone position. Both patients were discovered to have infarcted large pituitary macroadenomas as the etiology of their acute ophthalmoplegias, and transnasal, transsphenoidal resection was performed acutely to decompress the cavernous sinus contents. Pathologic analysis of the resected specimens in each case confirmed necrotic, infarcted pituitary adenoma. Both patients made a complete recovery with no evidence of residual or recurrent tumor in short term follow-up.

Conclusion

We report a brief case series of acute cavernous sinus syndrome resulting from dependent positioning during non-cranial operations in patients with pituitary macroadenoma. Although rare, this highlights a potential danger of “head down” positioning in patients with intracranial pathology—particularly in or around the sella and cavernous sinus. Despite multiple cranial neuropathies upon presentation, both patients made complete recovery following surgical decompression of the cavernous sinuses.

     

Hepatocellular carcinoma metastasizing to the skull base involving multiple cranial nerves

INTRODUCTION Hepatocellular carcinoma (HCC), the most common primary tumor of the liver, is estimated to cause more than a quarter of a million deaths throughout the world each year. Metastasis is one of the most significant factors affecting prognosis. I…     

Neuro-ophthalmologic vascular emergencies in the elderly

The significance, management and prognosis of several disorders affecting either vision or ocular motility are discussed. Transient and persistent visual loss are common problems in the elderly. Anterior ischemic optic neuropathy is extensively reviewed because of the importance of recognizing forms due to giant cell arteritis, which require urgent treatment with steroids to prevent further visual loss. The various pathologic conditions that can affect the ocular motor nerves in the cavernous sinus are mentioned. The problem of differentiating benign ischemic lesions causing a third nerve palsy from ominous compressive palsies due to aneurysms is addressed, with emphasis on the clues provided by the pupil.     

Diabetic ophthalmoplegia

Summary Diabetic neuropathy, and especially ocular paralysis (alone or associated with palsy of other cranial nerves) may be the first clinical manifestation of diabetes mellitus. The purpose of this work is to present the cases studied, which had an ocular paralysis as the first clinical manifestation, and which were affected by diabetes, unknown until that moment, clinical or latent, or were prediabetics. Thus, we believe that the existence of an ocular paralysis may be added to the other signs and symptoms mentioned as guiding elements for the diagnosis of the diabetic or prediabetic state. Our cases were carefully studied; they showed paralysis of III, IV and VI cranial nerves, isolated or, in 2 cases, associated with palsy of other cranial nerves. The existence of clinical or latent diabetes was confirmed by laboratory tests; the diagnosis of prediabetes was based on the usual criteria. Clinical evolution was identical to that observed in patients with clinically known diabetes. Some considerations on the pathogeny of diabetic ophthalmoplegia in relation to its sudden onset and to the rapid recovery of the cases studied are added.     

青少年颅内动脉瘤的影像学特征及治疗

目的探讨青少年颅内动脉瘤的影像学特征及治疗策略。方法对16例青少年（年龄为15—18岁;男10例,女6例）颅内动脉瘤的影像学特征、治疗方式及其结果进行回顾性分析。结果青少年颅内动脉瘤占同期颅内动脉瘤的0．9％。以蛛网膜下腔出血发病者8例,以肢体无力、动眼神经麻痹、外伤后鼻衄等发病者8例。共发现20个颅内动脉瘤,其中位于前循环15个,位于后循环5个;动脉瘤的部位：大脑中动脉5个,基底动脉及海绵窦内各3个,后交通动脉、脉络膜前动脉、颈内动脉各2个,眼动脉、大脑后动脉及椎动脉各1个。动脉瘤的直径〈15mm者13个,≥15mm者7个,其中4个〉25mm,属于巨大动脉瘤。3例患者为多发动脉瘤。16例患者中,12例接受治疗：①介入治疗8例：其中5例行动脉瘤内栓塞术治疗,2例行载瘤动脉闭塞,1例海绵窦段颈内动脉假性动脉瘤行支架置入术;②显微外科手术治疗4例：其中2例行动脉瘤颈夹闭术,2例大脑中动脉复杂动脉瘤行颅内外动脉旁路移植后行动脉瘤孤立术。未治疗4例：1例治疗前载瘤动脉闭塞,动脉瘤自愈;术前动脉瘤再破裂死亡1例,2例因治疗风险巨大未行治疗。9例治疗患者经平均45个月的随访,均恢复良好（格拉斯哥预后量表评分为4～5分）结论①大脑中动脉是青少年颅内动脉瘤的好发部位;巨大型、外伤性、多发性、梭形动脉瘤比例较高;②青少年颅内动脉瘤治疗难度较大,部分病例需采用载瘤动脉闭塞或血管旁路移植术后孤立动脉瘤的方法进行治疗,预后较好;⑧青少年颅内动脉瘤的发病方式、影像学特征及治疗特点与儿童颅内动脉瘤相似,而与成人区别较大。