低颅压综合征合并颅内静脉血栓形成1例及文献回顾

目的 总结分析低颅压综合征合并颅内静脉系统血栓形成的临床特征、诊断方法及治疗原则.方法 报道1例低颅压综合征合并颅内静脉系统血栓形成病例,并复习相关文献.结果 患者以体位性头痛起病,经腰椎穿刺及影像学检查诊断为低颅压综合征,继发硬膜下积液及颅内静脉系统血栓形成.头颅CT、MRI均可提供低颅压综合征的表现,MRV及全脑血管造影术(DSA)是静脉系统血栓形成的重要诊断手段.结论 低颅压综合征是静脉系统血栓形成的危险因素之一,了解两者的临床特征有利于早期诊断及治疗.     

立体定向活检术在颅内无强化效应病变中的诊断价值

目的 探讨立体定向活检术在颅内无强化效应病变中的定性诊断价值.方法 对47例在MRI增强扫描中未见明显强化的颅内病变行立体定向活检,其中MRI引导有框架立体定向活检38例,无框架神经导航定向活检9例.结果 获得明确病理诊断42例,未获得阳性病理结果5例,活检病理诊断阳性率89.4％.对颅内非肿瘤性病变,MRI诊断与病理诊断相符合9例;对肿瘤性病变,MRI诊断与病理诊断相符合14例;MRI诊断与病理诊断的符合率为48.9％.活检术后穿刺道少量出血1例,无颅内感染和死亡病例.结论 对颅内无强化效应的病变,立体定向活检是获得定性诊断安全、可靠的方法.     

伴代谢综合征的急性脑梗死患者颅内血管狭窄差异分析

目的：探讨伴代谢综合征的急性脑梗死患者颅内动脉血管的差异。方法2010‐07—2013‐12在我院住院治疗患者1500例,其中符合纳入标准的1072例,其中正常健康人群255例,急性脑梗死患者（病例组）817例。另将所有研究对象中有颅内血管狭窄者作为颅内病变组。通过头部CTA或核磁共振血管成像或全脑血管造影术检查,明确有无颅内动脉血管狭窄性病变。通过单因素及多因素的Logistic回归,比较分析颅内血管病变的危险因素。结果脑梗死患者中代谢综合征发病率46.7％,其中59％存在颅内动脉狭窄,OR值为4.11。颅内动脉病变组代谢综合征发病率为50.8％。代谢综合征与脑梗死及颅内动脉病变呈正相关。糖耐量减低（IG T ）及糖尿病（DM ）与颅内动脉血管狭窄有相关性,其危险度为无糖尿病患者的2.44及2.6倍。多因素Logistic回归分析中发现颅内动脉病变与高血压及TG呈正相关性。结论急性脑梗死患者中代谢综合征是颅内动脉血管狭窄的独立危险因素。代谢综合征的五个组分中高血压与颅内血管病变的相关性最强。     

结节性硬化症的临床和CT诊断（附20例报告）

结节性硬化症的临床和ＣＴ诊断（附２０例报告）周晓鸥高万本唐克新李建勋周小东结节性硬化（ｔｕｂｅｒｏｕｓｓｃｌｅｒｏｕｓｉｓ）属神经皮肤综合征之一，是一种少见的遗传性疾病，其发病率约为１１０万，占低智能儿的０５％［１］。典型病例不多。病变可累及全身多...     

原发颅内恶性畸胎瘤一例

目的探讨原发颅内恶性畸胎瘤的临床特点及诊断治疗方案,提高对该疾病的认识。方法报告1例颅内三脑室恶性畸胎瘤病例临床资料,结合文献进行分析。结果患者行手术切除,病理为恶性畸胎瘤,术后行全中枢照射+瘤床推量放疗,放疗后全身化疗4个周期。治疗后随访56个月,未出现肿瘤复发及脊髓内或远处转移。结论颅内恶性畸胎瘤为罕见颅内肿瘤,肿瘤切除术后辅以放疗、化疗是安全、有效的治疗措施之一。     

提高颅内病变立体定向活检准确率的体会

CT与立体定向术相结合进行颅内病变活检．具有定位准确、创伤小、成功率高以及诊断准确率高的优点。我们应用瑞典Electa公司生产的Leksell定向系统行颅内病变活检33例．现诊断准确率为96．97％．无手术并发症、无死亡。报告如下。     

颅脑损伤后脑性盐耗综合征

颅脑损伤后有时可发生脑性盐耗综合征( cerebral salt wasting syndrome,CSWS),它是因为颅内病变导致的肾性失钠,同时伴随水份的丢失,临床表现低血钠、高尿钠,同时可伴有低血容量及多尿,这些表现与抗利尿激素不适当分泌综合征( syndrome of inappropriate antidiuretic hormone secretion,SIADH)及尿崩症易混淆,容易引起误诊误治.     

脊髓型多发性硬化

报告５例脊髓型多发性硬化（ＭＳ），其临床表现为脊髓损害的症状和体征，病程中均有缓解与复发。其中１例做了全身尸检，为国内首例尸检报告。病理特点：①脊髓白质广泛脱髓鞘改变；②枕叶白质有两处陈旧性软化灶而无临床症状与体征；③伴有周围神经脱髓鞘病变，并且周围神经病变与临床表现及电生理学检查相符。作者结合文献对ＭＳ的诊断、病理特点进行了讨论     

伴严重失写的皮质下失语综合征

近年来关于皮质下病变引起的言语行为变化已得到广泛注意。最近我们收治1名皮质下失语综合征,临床上伴有严重失写等特殊症状,本文从神经语言学角度进行分析和讨论。 病例报告 邢某,男,44岁,潜在左利手,文化程度大学毕业,患者于1月前某晚21时突然头痛,无     

原发性颅内间变性血管周细胞瘤伴全身多发转移1例

本文报告1例原发性颅内间变性血管周细胞瘤（haemangiopericytomas,HPC）伴全身多发转移的患者，患者为52岁男性，14年前以头痛起病，头颅磁共振提示后枕叶占位，行开颅病变切除术。术后随访期间发现原位肿瘤多次复发，并伴肝脏、髂骨、胸椎等多处转移。手术全切（gross total resection,GTR）是该病例的主要治疗方式，术后通过多次放疗、介入栓塞、高强度聚焦超声等多种综合治疗方案，使病变得到有效控制。原发性颅内血管周细胞瘤是一种罕见的神经系统肿瘤，间变性HPC具有高复发及颅内外转移的特点，综合性治疗是主要手段。     

The moyamoya syndrome associated with irradiation of an optic glioma in children: Report of two cases and review of the literature

We report two cases of the moyamoya syndrome which became clinically apparent after irradiation of an optic glioma during childhood. A summary of 14 cases of this syndrome following irradiation of intracranial tumors is also presented. Nine of these cases were optic gliomas; five were found in children with neurofibromatosis, another disorder that has a strong association with the moyamoya syndrome. The effectiveness of irradiation of optic gliomas in childhood is not definitely established. The possibility of inducing serious vascular disease is a further reason for caution when considering irradiating these tumors.     

老年颅内肿瘤的治疗体会

目的 探讨老年人颅内肿瘤的临床特点和治疗方法。方法 本组65岁以上老年颅内肿瘤126例，分别采用手术，X-刀和非手术治疗。结果 手术治疗65例，良性肿瘤肉眼或显微镜下全切除32例。次全切除6例，恶性肿瘤全切除8例，次全切除10例，大部切除8例。1例囊性颅咽管瘤行立体定向穿刺内放疗。X-刀治疗32例，肿瘤直径在3cm以内，行单次治疗20例；直径在5cm以内。行分次治疗12例，非手术29例。结论 老年人颅内肿瘤有其临床特点，应根据患情况及肿瘤性质，采取不同的治疗方法。     

颅内原发性多细胞源性肿瘤(附八例报告)

目的 探讨颅内原发性多细胞源性肿瘤的临床特点与诊治方法.方法 同顾性分析我院收治的8例颅内原发性多细胞源性肿瘤患者的临床资料,总结其临床表现、影像特点及治疗方法,复习文献并探讨其可能发生机制.结果 8例患者共发现16个肿瘤.包括脑膜瘤6个,垂体瘤3个,听神经瘤2个,星型细胞瘤2个,松果体细胞瘤2个,黑色素母细胞瘤1个.一次手术切除5例,分次切除3例,共全切肿瘤12个,次全切除3个,大部切除1个.结论 MR对颅内原发性多细胞源性肿瘤具有重要的诊断价值,一次或分次手术是其主要治疗手段.     

Germinoma in cerebral hemisphere associated with Down syndrome

A Down syndrome patient with germinoma developing in the cerebral hemisphere is reported. A review of the literature yielded only 14 cases of Down syndrome with brain tumors, including our case. This finding of brain tumors in patients with Down syndrome may reflect chance occurrence. However, it is of interest in this regard that in 6 of the 14 (43%) reported cases the lesions were intracranial germ cell tumors. Received: 15 March 1996 Revised: 20 May 1996     

低颅压综合征合并颅内静脉血栓2例并文献回顾分析

目的总结分析低颅压综合征(SIH)合并颅内静脉血栓形成(CVT)的临床表现,诊断和治疗方法。方法报道2例低颅压综合征合并颅内静脉血栓形成,并回顾文献报道15例同类病例,分析其临床特征、影像学表现、治疗和病理生理机制。结果17例患者均以亚急性起病,其中14例患者表现为体位性头痛。常见的伴随症状有恶心、呕吐、复视、耳鸣等。所有患者的影像学检查都存在SIH和CVT的表现,头部核磁共振(MRI)加静脉成像(MRV)是诊断颅内静脉血栓形成的主要手段。结论低颅压综合征是颅内静脉血栓形成的危险因素之一,了解两者内在的病理生理联系和临床特征有利于对该病的早期诊断和及时治疗。     

Coexisting lipomatous meningioma and glioblastoma in Cowden syndrome: A unique tumor association

Cowden syndrome (CS) is a rare hereditary hamartoma-cancer disorder related to germline mutations in the tumor suppressor phosphatase and tensin homolog (PTEN) gene. Association of CS with intracranial tumors, apart from Lhermitte-Duclos disease (LDD), is not well recognized. We present an exceptional instance of concomitant meningioma and glioblastoma in CS, the first case ever reported. Following a new-onset seizure, a 62-year-old male harboring the PTEN gene germline mutation c.334C > G was diagnosed with multiple brain tumors, which were erroneously thought to correspond to metastases. Because no primary cancer was found, an operation was proposed for histopathological diagnosis. Examination of surgical specimens obtained from the two lesions removed, one extra-axial and the other intracerebral, demonstrated a metaplastic meningioma with a lipomatous appearance and an isocitrate dehydrogenase wild-type glioblastoma, respectively. Loss of the PTEN gene expression was demonstrated immunohistochemically in both lesions, a finding that supports their relation to CS. A thorough literature review revealed only 25 additional CS patients with intracranial tumors other than LDD. All of them corresponded to primary lesions, with meningiomas accounting for 76% of the cases (19 patients), followed by pituitary tumors (three cases) and glioblastomas (two patients from the same family). Our report and literature review highlight the association between CS and primary brain tumors rather than metastasis. For judicious management of a CS patient with multiple intracranial tumors, different primary brain pathological entities should also be suspected first before considering metastasis. Close neurological monitoring and brain magnetic resonance imaging are advocated as part of the cancer screening in CS patients, particularly in cases with a family history of intracranial tumors.     

立体定向活检联合陀螺刀治疗囊性脑转移瘤

目的探讨囊性肺癌脑转移瘤采用立体定向活检与陀螺刀相结合治疗的疗效。方法对58例囊性肺癌脑转移瘤在CT导向下立体定向穿刺活检,并穿刺引流囊液使囊腔缩小,再行陀螺刀治疗。结果 58例患者57例明确了病理类型,肿瘤体积缩小,患者临床症状改善,陀螺刀治疗效果提高。结论立体定向手术联合陀螺刀治疗囊性肺癌脑转移瘤具有微创、安全、并发症少、有明显的近期效果和有效的肿瘤控制率等优点。     

Familial adenomatous polyposis and benign intracranial tumors: a new variant of Gardner's syndrome

INTRODUCTION: Familial adenomatous polyposis (FAP) is associated with malignant tumors of the central nervous system, predominantly medulloblastomas and glioblastoma multiforme (Turcot's syndrome) and with craniofacial osteomas (Gardner's syndrome). This report details the occurrence of benign, intracranial tumors in two related individuals with Gardner's syndrome, an association not previously described. PATIENTS AND METHODS: A 57-year-old woman (the propositus), her sister, two of her nieces and one of her grandnephews were previously diagnosed with Gardner's syndrome. The propositus came to neurosurgical attention because of vertigo associated with what proved to be an epidermoid cyst of the cerebellopontine angle. Her unaffected children and her relatives with Gardner's syndrome were examined and underwent computed tomography or magnetic resonance imaging. RESULTS: A 39-year-old woman with Gardner's syndrome, the niece of the propositus, was found to harbor an asymptomatic left frontal meningioma. DISCUSSION: Familial adenomatous polyposis, Gardner's syndrome, and that variant of Turcot's syndrome in which medulloblastoma predominate, are associated with a mutation of the adenomatous polyposis coli gene. The demonstration that patients with Gardner's syndrome can also have benign, nonneuroglial, intracranial tumors adds to the previously known extracolonic lesions associated with FAP. The molecular characterization of our patients should reveal if benign intracranial tumors represent a pleiotropic manifestation of the adenomatous polyposis coli gene mutation or if other genes are implicated.     

锁孔（微骨孔）技术在517例颅脑手术中的应用

目的探索、研究锁孔（微骨孔）技术治疗颅脑深部肿瘤和动脉瘤,回顾性总结采用锁孔技术显微手术治疗517例颅内深部肿瘤和动脉瘤的经验。方法对439例颅内深部肿瘤,包括脑桥小脑角及小脑肿瘤,岩斜区肿瘤、颅前窝脑膜瘤、鞍区肿瘤、镰旁和镰幕区脑膜瘤经锁孔（微骨孔）技术切除,以及在导航指引下锁孔-显微手术切除脑内深部胶质瘤;锁孔手术治疗颅内动脉瘤78例。结果517例手术中死亡2例（占0.4%）,在439例颅脑肿瘤中全切肿瘤396例（占90.2%）,次全切除22例,大部切除21例。结论锁孔（微骨孔）技术是治疗颅内深部肿瘤和动脉瘤的良好方法,具有损伤少、恢复快等优点。     

Intracranial plasma cell granuloma

We report two rare cases of primary intracranial plasma cell granuloma. The tumors probably arose from the dura and involved the cerebral parenchyma. These patients presented with clinical features of raised intracranial pressure and there was focal neurological deficit. The management issues are discussed.