Glossopharyngeal Neuralgia

Glossopharyngeal neuralgia (GPN) is a rare disease that must be differentiated from trigeminal neuralgia. The purpose of this article is to provide a comprehensive review of anatomy, pathophysiology, diagnostic criteria, and several options of treatment for GPN. Lessons learned through our experience of treating GPN are presented in detail, as well as cases of misdiagnosis and diagnostic pitfalls. Microvascular decompression (MVD) should be primarily considered for medically intractable GPN. Techniques employed in MVD for GPN are categorized and described. Especially, we underscore the advantages of the ‘transposition’ technique where insulating material is positioned ‘off’ the root entry zone (REZ), instead of ‘on’ it. We believe this ‘off-the-REZ’ technique can fundamentally prevent recurrence, if applicable. In addition, Gamma Knife radiosurgery can be an alternative option when a patient is ineligible for MVD, though it is categorized as a destructive procedure.     

Primary Intracranial Squamous Cell Carcinoma in the Brain Stem with a Cerebellopontine Angle Epidermoid Cyst

Primary intracranial squamous cell carcinoma is extremely rare, with most cases arising from a preexisting benign epidermoid cyst. We report a rare case of primary intracranial squamous cell carcinoma in the brain stem with a cerebellopontine angle (CPA) epidermoid cyst. A 72-year-old female suffered from progressive left hemiparesis, difficulty in swallowing, and right hemifacial numbness. Diffusion-weighted magnetic resonance imaging revealed a high signal intensity (SI) lesion in the CPA region and an intra-axially ring-enhanced cystic mass in the right brain stem with low SI. Whole-body positron emission tomography showed no evidence of metastatic disease. The histological findings revealed a typical epidermoid cyst in the CPA region and a squamous cell carcinoma in the brain stem. We speculate that the squamous cell carcinoma may have been developed due to a chronic inflammatory response by the adjacent epidermoid cyst. The patient underwent a surgical resection and radiotherapy. After 12 months, she had no evidence of recurrence.     

原发性舌咽神经痛最微外科手术治疗的并发症

目的探讨显微神经外科手术治疗原发性舌咽神经痛的并发症的防治。方法1986年5月至2005年7月对50例舌咽神经痛患者进行了显微神经外科手术治疗,均采用枕下乙状窦后锁孔入路,包括显微血管减压术17例,舌咽神经根及迷走神经根上部1～2根丝切断术16例,显微血管减压术同时加行舌咽神经根及迷走神经根上部1～2根丝切断术17例。结果100%患者术后即刻疼痛全部消失。44例患者获平均6.1年的随访,随访期间治愈率为100%。无1例疼痛复发。并发症包括吞咽困难、饮水呛咳、声嘶6例,阵发性干咳4例,耳鸣1例,化脓性脑膜炎1例,无菌性脑膜炎2例。结论枕下乙状窦后锁孔入路显微神经外科手术治疗舌咽神经痛是有效、安全的,合理选择术式、熟悉局部显微解剖、掌握娴熟显微手术技巧和积累丰富手术经验是降低并发症发生率的前提。     

Lipoma Causing Glossopharyngeal Neuralgia: A Case Report and Review of Literature

The cerebello-pontine angle lipomas causing trigeminal neuralgia or hemifacial spasm are rare. A lipoma causing glossopharyngel neuralgia is also very rare. A 46-year-old woman complained of 2-year history of severe right throat pain, with ipsilateral episodic otalgic pain. The throat pain was described as an episodic lancinating character confined to the throat. Computed tomography and magnetic resonance imaging revealed a suspicious offending posterior inferior cerebellar artery (PICA) compressing lower cranial nerves including glossopharyngeal nerve. At surgery, a soft, yellowish mass (2×3×3 mm in size) was found incorporating the lateral aspect of proximal portion of 9th and 10th cranial nerves. Only microvascular decompression of the offending PICA was performed. Additional procedure was not performed. Her severe lancinating pain remained unchanged, immediate postoperatively. The neuralgic pain disappeared over a period of several weeks. In this particular patient with a fatty neurovascular lump causing glossopharyngeal neuralgia, microvascular decompression of offending vessel alone was enough to control the neuralgic pain.     

Spinal Extradural Arachnoid Cyst

Spinal extradural arachnoid cyst (SEAC) is a rare disease and uncommon cause of compressive myelopathy. The etiology remains still unclear. We experienced 2 cases of SEACs and reviewed the cases and previous literatures. A 59-year-old man complained of both leg radiating pain and paresthesia for 4 years. His MRI showed an extradural cyst from T12 to L3 and we performed cyst fenestration and repaired the dural defect with tailored laminectomy. Another 51-year-old female patient visited our clinical with left buttock pain and paresthesia for 3 years. A large extradural cyst was found at T1-L2 level on MRI and a communication between the cyst and subarachnoid space was illustrated by CT-myelography. We performed cyst fenestration with primary repair of dural defect. Both patients'' symptoms gradually subsided and follow up images taken 1-2 months postoperatively showed nearly disappeared cysts. There has been no documented recurrence in these two cases so far. Tailored laminotomy with cyst fenestration can be a safe and effective alternative choice in treating SEACs compared to traditional complete resection of cyst wall with multi-level laminectomy.     

蛛网膜囊肿伴癫痫的外科治疗

目的 探讨颅内网膜囊肿（arachnoid cyst,AC)与癫痫的关系以及外科治疗方法。方法 手术治疗23例颅内大脑凸面蛛网膜囊肿（AC）伴癫痫病人,采用AC和致痫灶切除21例,AC－腹腔分流术1例,胼胝体切开术1例。结果 23例病人中,完全不发作有5例,显改善有11例,无变化7例。结论 颅内AC可引起癫痫,采用AC和致痫灶切除效果较为理想。     

颅内蛛网膜囊肿手术治疗方式探讨

目的探讨颅内蛛网膜囊肿的手术治疗方法及其适应证。方法回顾分析我院51例手术治疗患者临床资料。囊肿壁部分切除并脑池开放术26例，其中12例同时行癫痫手术；囊肿一腹腔分流术11例，神经内窥镜下囊肿造瘘术14例。结果患者术后均恢复良好，术后3～6个月复查头颅CT，囊肿消失或明显缩小者41例，无改变10例。随访3个月～8年，症状消失或改善45例，无改变5例，加重1例。12例行致痫灶切除术患者症状控制满意。结论神经内镜囊肿一脑池或脑室造瘘术，是治疗颅内蛛网膜囊肿的有效方法。对于伴有癫痫的患耆，采取开颅囊肿壁切除并脑池开放同时行致痫灶切除术能提高患者术后生活质量。     

Syringomyelia Associated with a Spinal Arachnoid Cyst

While syringomyelia is not a rare spinal disorder, syringomyelia associated with a spinal arachnoid cyst is very unusual. Here, we report a 62-year-old man who suffered from gait disturbance and numbness of bilateral lower extremities. Spinal magnetic resonance imaging (MRI) showed the presence of a spinal arachnoid cyst between the 7th cervical and 3rd thoracic vertebral segment and syringomyelia extending between the 6th cervical and 1st thoracic vertebral segment. The cyst had compressed the spinal cord anteriorly. Syringomyelia usually results from lesions that partially obstruct cerebrospinal fluid flow. Therefore, we concluded that the spinal arachnoid cyst was causing the syringomyelia. After simple excision of the arachnoid cyst, the symptoms were relieved. A follow-up MRI demonstrated that the syringomyelia had significantly decreased in size after removal of the arachnoid cyst. This report presents an unusual case of gait disturbance caused by syringomyelia associated with a spinal arachnoid cyst.     

Cerebellopontine angle lesions in children

Background Cerebellopontine angle (CPA) lesions are more commonly found in adults in which they account for 5–10% of all intracranial tumors. However, they are uncommon in children, with an incidence of only 1%. Materials and methods This is a review of the management of CPA lesions in children admitted to the Hospital Nacional de Pediatría “Profesor Doctor Juan P. Garrahan” (Argentine National Pediatrics Hospital “Professor Juan P. Garrahan”) between January 1988 and December 2003. Results The series included 30 children with 33 CPA lesions, 20 arising from the subarachnoid space of the CPA and 13 from the vicinity and growing mainly into the CPA. Twenty-seven tumors were located in the left CPA (82%) and six (12%), on the right. Ten of the 30 patients developed hydrocephalus, but only three of these required treatment. All patients underwent retrosigmoid suboccipital craniotomy and microsurgical resection. Gross total removal was achieved in 12 cases, subtotal in 18, and fenestration of the cyst wall in the three arachnoid cysts. Ten patients have no sequelae, ten have mild deficit, three have severe deficits, and seven have died. Conclusion The CPA is a rare location for lesions in children, with clear predominance on the left side. Benign lesions are more frequent. Even though schwannoma is the most frequently found lesion, the histology varies widely.     

Undetermined Fibrous Tumor with Calcification in the Cerebellopontine Angle

In this report, we introduce an undetermined fibrous tumor with calcification occurring in the cerebellopontine angle (CPA). A 51-year-old woman was admitted with a short history of dizziness. Computed tomography and magnetic resonance images revealed a 2×2×2 cm sized mass at the left CPA which was round and calcified. There was no dura or internal auditory canal involvement. At surgery, the tumor was located at the exit of 7th and 8th cranial nerve complex. It was very firm, bright yellow and well encapsulated. Histologic findings revealed that the tumor was predominantly composed of fibrous component, scant spindle cells and dystrophic calcification. Immunohistochemical staining demonstrated positive for vimentin and negative for epithelial membrane antigen (EMA), S-100 protein, CD34, factor XIIIa and smooth muscle actin. The diagnosis was not compatible with meningioma, schwannoma, metastatic brain tumors, and other fibrous tumors. Although the tumor was resected in total, long term follow-up monitoring is necessary due to the possibility of recurrence.     

Malignant Transformation of an Epidermoid Cyst in the Cerebellopontine Angle

Intracranial squamous cell carcinoma is extremely rare, with most of the cases arising from malignant transformation of an epidermoid or a dermoid cyst. The patient presented with facial weakness. Initial magnetic resonance imaging revealed a mass in the right cerebellopontine angle. A subtotal resection was performed via right retrosigmoid suboccipital approach. Histopathological findings were consistent with an epidermoid tumor. Five months later, the patient underwent gamma knife radiosurgery due to highly probable recurrent epidermoid tumor. Two years after, the patient''s neurological deficit had been newly developed, and follow-up magnetic resonance imaging demonstrated a large contrast-enhancing tumor in the left cerebellopontine angle, which compressed the brainstem. After resection of the tumor, histopathological examinations revealed a squamous cell carcinoma probably arising from an underlying epidermoid cyst. We report a case of an epidermoid tumor in the cerebellopontine angle that transformed into a squamous cell carcinoma.     

Arachnoid Cyst with Spontaneous Intracystic Hemorrhage and Chronic Subdural Hematoma

We report a case of arachnoid cyst in which subdural hematoma and intracystic hemorrhage developed spontaneously. Usually, arachnoid cysts are asymptomatic, but can become symptomatic because of cyst enlargement or hemorrhage, often after mild head trauma. Although they are sometimes combined with subdural hematoma, intracystic hemorrhage has rarely been observed. Our patient had a simultaneous subdural hematoma and intracystic hemorrhage without evidence of head trauma.     

面神经监测在小脑桥脑角肿瘤切除术中的应用

目的：探讨术中面神经监测在小脑桥脑角肿瘤手术中保护面神经的方法和效果。方法：将45例患随机分为监测组与对照组，然后比较分析两组患术前，术后7－10d及随访后的面神经功能。结果：术后7－10d面神经功能I－Ⅲ级两组分别为15例和9例，Ⅳ-Ⅴ级分别为7例和14例；随访后两组面神经功能Ⅰ-Ⅲ级分别为21例和14例，Ⅳ-Ⅴ级分别为1例和9例，统计学分析差异有显性。结论：术中面神经监测对小脑桥脑角肿瘤手术中保护面神经重要指导作用，能有效地改善术后面神经功能。     

桥脑小脑角区神经内镜应用解剖学的初步研究

目的 研究桥脑小脑角区神经内镜“锁孔”入路的神经和血管等解剖结构,探索神经内镜到达该区的可行性及相应的解剖定位标志。方法 成人尸头8例,新鲜少年尸头2例,经双侧颈总动脉、椎动脉灌注10％乳胶 红色染料 硫酸钡粉剂。用0°、30°硬镜交替配合使用,对桥脑小脑角区的解剖结构进行观察。结果 当枕下“锁孔”入路骨窗直径为15mm时,神经内镜在桥脑小脑角区操作自如,能暴露全部的桥小脑角区,包括桥脑的腹侧区、斜坡以及对侧的解剖结构。结论 ①硬膜下腔中,内镜准确到位的要点是内镜按照定位标志或沿途定位路标前进。②桥脑小脑角区的神经从头侧至尾侧和从内侧到外侧把桥脑小脑角区分成三个腔隙:头侧腔隙、中间腔隙和尾侧腔隙,每个腔隙均有主要血管和一组颅神经通过。     

Asymptomatic Motor Cortex Displacement due to a Giant Arachnoid Cyst

Cerebral lesions are held to induce plastic changes of the brain. Less well established, however, is how much space-occupying brain lesions may only displace functional representations. In a 66-year-old man we show, by means of functional magnetic resonance imaging and transcranial magnetic stimulation, a profound displacement of the motor cortex due to a large asymptomatic arachnoid cyst. Thus, the chronically compressed brain is capable of sustaining normal brain function without utilizing the potential of cortical plasticity.     

大型和巨大型桥脑小脑角脑膜瘤的显微手术治疗

目的 探讨大型及巨大型桥脑小脑角(CPA)脑膜瘤显微手术切除、操作要点及术后治疗。方法 对25例大型及巨大型CPA脑膜瘤采用扩大枕下入路实施手术,行肿瘤全切18例,大部分或部分切除7例。结果 24例存活,死亡1例。经7月到6年随访,显示症状明显改善17例,保持术前状态3例,颅神经损害加重4例。肿瘤复发3例。结论 经病变侧扩大枕下入路,改进操作方式及加强术后治疗,能比较理想地切除肿瘤和提高患者生存质量。     

A Case of an Arachnoid Cyst Masquerading as Corticobasal Degeneration

We present an individual, “JD”, a 69-year-old Caucasian, married female with symptoms that included progressive right arm stiffness, tremor, and clumsiness; increasing gait and balance disturbance; increased fatigue and emotionality. Neuropsychological evaluation revealed compromised semantics and language-associated functions; impaired visual constructional ability; markedly reduced cognitive and visuomotor processing speed; low average to average working memory; variable praxis performance; variable abstract reasoning, problem solving, and set shifting; and lower overall intellectual functioning compared to premorbid estimates. Overall, her neuropsychological profile indicated marked compromise of the frontal and left parietal regions. The data coupled with her symptom pattern and demographics partially fit corticobasal degeneration diagnostic criteria. Neuroimaging, however, performed 2 years prior to the assessment and again during the current workup revealed an enlarging arachnoid cyst compressing the left parietal and posterior frontal lobe and a small portion of the right medial frontal-parietal region. We discuss the neuroanatomical substrates involved in her cognitive presentation and how two very distinct pathological processes (corticobasal degeneration, arachnoid cyst) can result in two similar symptom presentations. We summarize how multidisciplinary assessment assists with differential diagnosis and treatment planning.     

桥小脑角肿瘤术中面、听神经监护

目的研究术中监护下桥小脑角肿瘤显微镜下切除的临床疗效。方法在41例桥小脑角肿瘤切除手术中,均进行脑干听觉诱发电位和面神经肌电图监护。结果肿瘤全切除者33例,次全切除者7例,大部切除者1例。41例患者均达到了面神经解剖保留,27例(65.9%,27/41)患者保留听力,其中14例为听神经瘤,占听神经瘤的56.0%(14/25)。结论术中脑干听觉诱发电位和面神经肌电图监护对于桥小脑角肿瘤切除术有重要的临床价值。