脑膜癌病的临床特点

目的 探讨脑膜癌病的临床特点.方法 回顾性分析25例脑膜癌病患者的临床资料.结果 本组中既往有恶性肿瘤病史7例;临床表现为头痛24例,恶心呕吐21例,意识障碍及四肢无力各6例,癫痫发作、精神症状及视力减退各5例等;脑膜刺激征阳性20例,视乳头水肿12例.CSF压力升高13例,蛋白升高16例,糖、氯化物降低14例;23例CSF细胞学检查发现异型细胞,17例CSF细胞免疫组化染色为阳性.头颅MRI平扫均未发现脑膜异常信号,5例头颅MRI增强扫描2例出现脑膜强化.结论 脑膜癌病临床表现以颅内压升高症候群为主,CSF细胞学及细胞免疫组化染色可确诊并发现癌细胞的来源.     

脑膜癌病59例临床分析

目的 探讨脑膜癌病（MC）的临床和脑脊液（CSF）细胞学特征。方法 回顾性分析59例确诊为MC患者的临床资料。结果 本组中老年患者占91．5％（54例）;呈亚急性或急性起病,病情进展较快;疾病早期多不发热（55例,93．2％）;头痛（57例）、呕吐（45例）及视盘水肿（21例）等颅内压增高症状为突出表现;32例（54．2％）出现脑神经受累,以视神经、展神经受累多见,其次为动眼神经、面神经及前庭蜗神经等;59例均有脑膜刺激征,其中31例（52．5％）较明显;59例CSF细胞学检查均找到癌细胞。结论 MC的临床表现具有一定的特征,确诊依赖于CSF细胞学检查找到癌细胞。     

神经白塞综合征的临床和脑脊液细胞学特点

目的回顾性分析神经白塞综合征(NBS)临床和脑脊液(CSF)细胞学资料,探讨该病的临床和CSF细胞学特点。方法回顾1991-2010年间北京协和医院NBS住院患者共27例,分析其临床表现、神经影像学和CSF细胞学特点。结果 27例患者中,神经科症状急性病程19例,亚急性病程6例,慢性病程2例。临床表现:头痛9例,偏瘫8例,复视6例,偏身感觉障碍5例,意识障碍5例,失语3例,癫痫发作3例,精神行为异常、智能减退6例,截瘫2例,共济失调2例,呛咳、吞咽困难2例,帕金森综合征1例。头MRI异常16例,多发片状T1低或较低信号,T2高信号,病灶分布于基底节、脑干、丘脑等处,9例行增强MRI均有强化;2例颈段脊髓MRI可见强化病灶。磁共振静脉成像(MRV):1例符合上矢状窦血栓形成。CSF检查:19例行腰穿查CSF,其中8例压力升高,17例蛋白升高(0.48～1.08g/L);行CSF细胞学检查(沉淀池法制片,迈-格-姬染色)的11例中7例表现为淋巴细胞为主的炎性反应,其中6例伴有中性粒细胞比例升高,余4例中3例为淋巴细胞与中性粒细胞为主的混合性炎性反应,1例为中性粒细胞性炎性反应,8例可见激活淋巴细胞,4例可见浆细胞。结论 NBS临床表现多样,神经影像学改变以基底节、脑干和丘脑等邻近中线的结构受累为主,CSF细胞学呈炎性反应表现。CSF细胞学炎性反应类型,特别是CSF中性粒细胞比例升高对NBS的诊断具有一定意义。     

脑膜癌病的脑脊液细胞学诊断相关因素研究

目的 探讨影响脑膜癌病脑脊液细胞学诊断的相关因素。方法 回顾性分析46例脑膜癌病患者的临床资料。结果 46例脑膜癌病的脑脊液中，38例发现瘤细胞(约82．6％)；40例腰穿脑脊液中，35例检到瘤细胞；6例脑室穿刺脑脊液中，3例检到瘤细胞；35例腰穿脑脊液检到瘤细胞中，26例为第一次检到瘤细胞，5例第2次检到瘤细胞，3例第3次检到瘤细胞，l例3次以上检到瘤细胞；6例硬脑膜-蛛网膜强化者仅l例脑脊液细胞学检到瘤细胞；4例软脑膜-蛛网膜强化者脑脊液细胞学均检到瘤细胞；38例原发肿瘤中，19例血液系统肿瘤(白血病、淋巴瘤)均检到瘤细胞，7例肺癌检出4例，5例胃癌检出3例，4例乳腺癌检出2例，1例室管膜瘤全部检出，1例脑淋巴瘤全部检出。结论常规脑脊液细胞学检查具有较高的特异性，脑脊液的来源、腰穿检查的次数、脑膜癌病病变的位置、原发肿瘤及组织类型均为影响其敏感性的重要因素。     

系统性淋巴瘤软脑膜转移的脑脊液细胞学分析

目的 探讨CSF细胞学及免疫细胞化学对系统性淋巴瘤软脑膜转移的诊断价值。方法 回顾性分析我院5例系统性淋巴瘤转移至软脑膜的病例,分析其CSF细胞学及免疫细胞化学特点。结果 5例患者中男性3例,女性2例,年龄14～74岁,平均年龄45岁。患者主要临床症状为头痛及恶心呕吐。此外,1例伴有发热,1例伴有脑神经损害,表现为眼肌麻痹。患者MRI扫描显示脑实质内均未发现异常信号,仅2例有脑膜强化。常规CSF细胞学显示5例CSF中均含有大量异型淋巴样细胞。免疫细胞化学显示4例CSF中CD20和/或CD79α阳性的异型细胞比例显著增加,这4例患者之前有脑外B细胞淋巴瘤病史;1例CSF中CD3阳性异型细胞为主。此患者随后发现了乳腺肿瘤,最终诊断为间变性大细胞淋巴瘤。5例CSF中异型细胞均与原发部位肿瘤细胞免疫表型一致,因此系统性淋巴瘤转移至软脑膜诊断明确。结论 CSF细胞学对系统性淋巴瘤的软脑膜转移诊断有重要意义,联合免疫细胞化学检测可进一步明确诊断。少数无系统性淋巴瘤病史的患者需要积极检查寻找原发肿瘤的起源和部位。     

结核性脑膜炎脑脊液细胞学检查及临床意义

结核性脑膜炎(结脑)脑脊液(CSF)细胞学资料报告较少,现将我们42例结脑 CSF细胞学检查结果报告如下,并就有关问题略加讨论。 资料与方法 病例来源 本组检查对象为42例经病史、症状、体征、CSF 常规、生化检查确诊为结脑的住院病人。男性26例,女性16例。年龄19～59岁,平均26.9岁。病程1个月内者13例,1～3个月者17例,4～6个月者7例,6个月以上者5例。42例结脑中,并Ⅱ型肺结核者17例;Ⅲ型肺结核15例;伴高颅压者27例。全部病人均采用本院结脑治疗方案(略)。临床治愈     

Guillain-Barré综合征患者临床及脑脊液细胞学特点

目的探讨Guillain-Barré综合征(GBS)患者临床及CSF细胞学特点。方法回顾性分析37例GBS患者的临床资料。结果本组患者急性起病33例,亚急性起病4例。临床表现为四肢无力18例(48.6%),双下肢无力16例(43.2%),四肢麻木21例(56.8%),双侧面神经麻痹12例(32.4%),其他颅神经损害5例(13.5%),呼吸肌麻痹3例(8.1%),全自主神经功能障碍及Fisher综合征各1例(5.4%);变异型GBS 6例(16.2%)。腰穿CSF检查示4例(10.8%)患者压力高,11例(29.7%)细胞计数升高,29例(78.4%)蛋白升高,7例(18.9%)免疫球蛋白升高。34例(91.9%)CSF细胞学检查异常,22例(64.7%)患者表现为激活的淋巴细胞反应,12例(35.3%)以激活的单核细胞反应为主。感觉或运动神经传导异常25例(67.6%),F波或H反射异常33例(89.2%)。32例患者给予丙种球蛋白或血浆置换治疗,5例给予糖皮质激素治疗,均有效。结论 GBS多数表现为四肢对称性麻木、无力,部分为变异型。F波、H反射电生理检查早期异常率高。CSF细胞学检查早期以淋巴细胞反应为主,后期以单核细胞反应为主。     

脑膜淋巴瘤的临床脑脊液细胞学研究

目的探讨脑脊液细胞学、免疫细胞化学和流式细胞分析在脑膜淋巴瘤诊断中的灵敏性和特异性。方法13例诊断为脑膜淋巴瘤的患者，其中原发性中枢神经系统淋巴瘤5例，均为非霍奇金淋巴瘤B细胞型（NHL-B）；继发性8例，包括NHL-B6例，非霍奇金淋巴瘤T细胞型（NHL-T）1例，mantle型1例。神经系统表现为头痛、视乳头水肿、脑脊膜刺激征、脊髓腰骶神经根受累和多脑神经麻痹等。腰穿脑脊液压力升高，细胞计数、蛋白均升高，糖减低。所有患者均采用沉淀池法，免疫细胞化学单克隆抗体CD19、CD20、CD79a、CD34和免疫球蛋白轻链等；其中3例脑脊液进行淋巴细胞亚群流式细胞分析。以4例病毒性脑炎和5例炎性脱髓鞘病患者的脑脊液为对照。结果脑脊液细胞学发现每例患者均有淋巴瘤细胞或异型淋巴细胞。免疫细胞化学发现5例患者多数细胞B细胞标记物阳性；例6多数细胞CD34阳性；例8CD20阳性细胞比例升高；例7CD19、CD20阴性。除例8外，其余脑脊液中细胞呈CD4和CD8阴性或极少数阳性。对照组CD4阳性细胞为主，伴有少量CD8阳性细胞，CD19和CD20阴性或极少数阳性。脑脊液流式细胞分析发现例9和例11的B淋巴细胞占85．9％～97．4％，提示异常的B细胞克隆。对照组以CD4、CD8阳性细胞为主，CD19阳性细胞低于4．0％。结论脑脊液细胞学和免疫细胞化学是诊断脑膜淋巴瘤的重要方法，脑脊液淋巴细胞亚群的流式分析能够对诊断脑膜淋巴瘤有所帮助。     

脑膜癌病的临床特点及脑脊液检查对其诊断的重要意义

目的探讨脑脊液检查对诊断脑膜癌病的重要意义。方法对22例脑膜癌病患者临床资料进行回顾性分析。结果22例脑膜癌病患者中男15例,女7例,45岁以下6例,平均年龄(53.21±8.67)岁,临术及病理学诊断11例为肺癌,3例为胃癌,2例乳腺癌,1例为恶性淋巴瘤,5例来源未明。结论脑膜癌病为恶性肿瘤颅内转移的特殊形式,多以头痛为首发症状,脑脊液细胞学检查找到癌细胞是诊断脑膜癌病的金标准,多次复查细胞学有助于早期确诊。     

脑多发性结核瘤11例的临床、脑脊液细胞学与病理特点

目的 探讨脑多发性结核瘤的临床、脑脊液细胞学和病理特点。方法 回顾性分析2000年11月至2005年5月我院诊治的脑多发性结核瘤11例。结果 患者临床上以发热和头痛起病，脑膜刺激征4例，意识障碍5例，偏瘫3例，抽搐2例。脑脊液压力升高8例，脑脊液蛋白升高10例，糖减低7例。脑脊液常规白细胞计数正常7例，升高4例；脑脊液细胞学见4例呈混合性细胞反应，2例呈淋巴-单核细胞反应，3例未见异常。11例头MRI均显示脑内多发结节或占位性病变，11例强化明显。5例开颅脑活检证实为结核性肉芽肿，抗酸染色阳性3例。结论 脑内多发性结核瘤是一种特殊类型的颅内结核感染，综合其临床表现、影像学和脑脊液细胞学等特点有助于临床诊断。     

Leptomeningeal carcinomatosis: Prognostic value of clinical,cerebrospinal fluid,and neuroimaging features

Introduction

Leptomeningeal carcinomatosis (LC) is a devastating complication occurring in 5% of all patients with cancer. To date there are no well-established prognostic markers in patients with LC, except for the presence of cerebrospinal fluid (CSF) blocks and the Karnofsky performance status scale (KPS). We aimed to identify clinical, neuroradiologic and CSF prognostic factors related to LC survival and to develop an easy-to-use Prognostic Scoring Scale (PSS) to identify patients who are more likely to benefit from receiving treatment.

Methods

Single-center retrospective study evaluating patients who had a diagnosis of LC during a 10-year period. Diagnosis was made by malignant cytology or imaging; suspicious cases treated as LC were also included.

Results

Fifty patients with LC were analyzed (58% women). Median age was 54.4 years, and KPS was 60%. The most common types of tumor were breast (35%), lung (24%), and hematologic malignancies (16%). Thirty-two percent of patients were diagnosed by imaging, 22% by cytology, and 40% by both. Median overall survival (OS) was 10 weeks (95% confidence interval 5.1–14.9). Median OS for patients who received specific treatment was 21.2 weeks vs. 6.38 weeks for patients receiving supportive care only (p < 0.001). In multivariate analysis, initial KPS, initial CSF protein level (<112 mg/dL) and time from diagnosis of primary tumor to diagnosis of LC (>67 weeks) were significant and independent predictors of increased survival.

Conclusions

Prognosis remains poor in LC. The predictive factors for patients with LC here identified could help to improve the selection of patients who are more likely to benefit from receiving treatment.     

脑脊液细胞学在脑生殖细胞瘤诊断中的应用及八例报告

目的 通过总结8例脑生殖细胞瘤患者的脑脊液细胞学结果,探讨脑脊液细胞学在脑生殖细胞瘤诊断中的价值.方法 总结2006年1月至2009年6月我院脑脊液细胞学发现肿瘤细胞的8例脑生殖细胞瘤患者,分析其临床特点、影像学和脑脊液细胞学结果.结果 8例患者中男性7例,女性1例.年龄13～25岁,分别以多饮、多尿和少汗等内分泌症状或神经科症状起病.神经科症状包括:头晕、头痛、智能减退、精神行为异常、复视、双下肢无力、尿便障碍等.神经影像学可见鞍上区和(或)松果体区占位、脑室扩张、室管膜和软脑膜强化、神经根增粗强化等.脑脊液绒毛膜促性腺激素3.2～1087.0 mIU/ml.脑脊液细胞学8例均见肿瘤细胞,呈中等大小的圆形,核大,核仁明显,胞质丰富,胞质内可见较多空泡.背景间有小淋巴细胞为主的炎性反应.过碘酸Schiff染色肿瘤细胞胞质中见阳性颗粒.4例行免疫细胞化学染色,其中2例抗胎盘碱性磷酸酶阳性;2例行Ki-67染色,阳性细胞分别占12%和20%.细胞角蛋白和癌胚抗原染色等阴性.结论 脑生殖细胞瘤患者的脑脊液细胞学结果特征明显,结合细胞免疫化学可以明确其类型及脑脊液播散,在诊断脑生殖细胞瘤过程中起着重要作用.

Abstract：

Objective To investigate the value of the cerebrospinal fluid ( CSF ) cytology in diagnosis of intracranial germinomas by reviewing the outcomes of CSF cytology of 8 patients with intracranial germinomas. Methods Eight patients with positive CSF cytology at our clinic from January 2006 to June 2009 were reviewed. Conventional cytology and immunocytochemistry of CSF were performed. The relevant literature on the subject was reviewed. Results The patients, including 7 male and 1 female, developed endocrinological or neurological symptoms at the age of 13 to 25, and the typical neurological presentation included vertigo, headache, mental and behavior disorders, double vision and weakness of legs. The CSF cell count ranged from 0 to 300 leukocytes per cubic and elevated in 7 cases, typically lymphocytic inflammation. CSF level of human chorionic gonadotropin was 3.2-1087.0 mIU/ml, higher than the individual serum level. On CSF cytology studies, typical tumor cells of germinima were found, which had positive particles in cytoplasm on periodic acid Schiff stain. All presents had lymphocyte inflammation ( small lymphocyte predominant ). On immunocytochemical studies of CSF, the tumor cells were positive on placental alkaline phosphatase and Ki-67 stains. Conclusions CSF cytology is clinically useful for diagnosis of primary intracranial germinoma. Further clinical and cytological studies will be necessary for a better understanding of the biology of these tumors.     

Malignant cells in cerebrospinal fluid (CSF): the meaning of a positive CSF cytology

We reviewed the correlation between malignant cells in cerebrospinal fluid (CSF) (positive cytology) and pathologic findings at autopsy. The purpose was to discover: (1) the incidence of negative CSF cytology in patients with CNS malignancy, (2) the incidence of false-positive cytology, and (3) the relationship between a true-positive cytology and the distribution of malignant tumor at autopsy. Of 117 patients with CNS tumor and premortem cytologic examination of the CSF, 31 (26 percent) were positive and 86 (74 percent) were negative. Only 1 of 66 patients with tumor that did not reach the leptomeninges had a positive cytology. Of 51 patients with leptomeningeal tumor at autopsy, cytology was positive in 30 (59 percent) and negative in 21 (41 percent). Five potentially "false-positive" cytologies were encountered: three patients were treated, and tumor may have been eradicated; in two patients with lymphoma, inflammatory cells associated with infection were apparently mistaken for malignant cells. These data indicate that a positive CSF cytology is a reliable indicator of CNS malignancy and almost always reflects leptomeningeal tumor.     

Meningeal gliomatosis: a review of 12 cases

Diffuse or multifocal invasion of the leptomeninges by malignant glioma (meningeal gliomatosis) is believed to be rare. From 1971 through 1977, 11 of 52 patients with intracranial malignant gliomas examined at autopsy were found to have meningeal gliomatosis, and 1 additional patient was diagnosed clinically without autopsy (12 cases total). Eight of the 12 patients were diagnosed antemortem with positive cerebrospinal fluid (CSF) cytology, while the other 4 patients were diagnosed at autopsy only. All 11 autopsied patients had multifocal or diffuse meningeal tumor distant from the primary site; 8 patients had spinal subarachnoid seeding with tumor encroachment of cauda equina and spinal nerve roots, and 9 patients had tumor invasion into the lateral ventricles. Three patients had symptomatic spinal cord compression at the thoracic or lumbar level, and 10 patients had hydrocephalus. These 12 patients with meningeal gliomatosis were compared with the other 41 autopsied malignant glioma patients without the complication (controls); the patients with meningeal gliomatosis were significantly younger (mean age, 40 versus 57 years; p less than 0.005). Patients with meningeal gliomatosis lived somewhat longer (median, 49 weeks) compared to controls (35 weeks), but the difference was not statistically significant. With the advance of chemotherapy, patients with malignant glioma are living longer and the incidence of meningeal gliomatosis may rise. The diagnosis of meningeal gliomatosis can be suspected, especially if hydrocephalus is present, and can often be confirmed by CSF cytology.     

Leptomeningeal carcinomatosis as primary manifestation of pancreatic cancer

Leptomeningeal carcinomatosis (LMC) is a rare complication of cancer that often presents at an advanced stage after obvious metastasis of a primary cancer or locally advanced disease. We present an uncommon case of LMC secondary to pancreatic carcinoma presenting with headache, unilateral VII nerve palsy, and lower extremity weakness. Initial cerebrospinal fluid (CSF) studies were concerning for chronic aseptic meningitis but negative for malignant cells; the diagnosis of tuberculous meningitis was erroneously evoked. Three lumbar punctures were required to capture malignant cells. The diagnosis of LMC was based on CSF examination with cytology/immunohistochemistry and leptomeningeal enhancement on MRI. Post mortem autopsy revealed advanced and diffusely metastatic pancreatic adenocarcinoma. This patient demonstrates that solid tumors can present with leptomeningeal spread that often confuses the treating physician. Fungal or tuberculous meningitis can mimic LMC in the absence of neoplastic signs and negative CSF cytology. This event is exceedingly rare in pancreatic cancer. If the index of suspicion is high, repeat CSF sampling can increase the sensitivity of detection of malignant cells and thus result in the correct diagnosis.     

Primary leptomeningeal sarcomatosis

6 autopsy cases of primary leptomeningeal sarcomatosis are presented as a distinct nosological entity with a variable clinical picture and morphology in 5 males and 1 female. The clinical course from onset of symptoms till death ran for only a few weeks in most cases. 2 infants showed brain tumor symptoms and signs. 2 patients of advanced age presented a polyradiculoneuritic syndrome and 2 young adults had spinal cord compression symptoms and a mixed clinical form. In almost all cases, clinical symptoms and signs were for most of the course confined to one part of the neuraxis. The CSF was distinctly abnormal in all cases, showing elevated protein, depressed glucose and pleocytosis of variable extent. CSF sediment was investigated in 3 cases in all of which malignant tumor cells were found so a diagnosis of malignant meningeal tumor was made during life. Electron microscopy of CSF cells in 1 case confirmed the primitive character of the tumor cells. Complete autopsies revealed absence of any neoplasm outside of the CNS. Gross meningeal involvement was visible in all cases. Histologically, 3 tumor types were distinguished: polymorphic cell sarcoma, an undifferentiated form, and fibrosarcomatosis. Clinical data are analyzed in order to distinguish the condition from other neoplasms or infectious, especially tuberculous meningeal infiltrations. CSF cytology studies are considered the most useful step in clinical diagnosis. Neuropathological features are reviewed with stress on differentiation from malignant lymphomas of the CNS, diffusely spreading medulloblastoma, meningeal melanoblastosis and gliomatosis. The origin of meningeal sarcomatosis cells is briefly discussed. The use of the term "meningeal meningiomatosis" for this condition is deprecated.     

Presenting features and value of diagnostic procedures in leptomeningeal metastases.

OBJECTIVE: To study the presenting features and value of routine diagnostic procedures in patients with leptomeningeal metastases (LMM) related to the primary malignancy to improve diagnostic assessment. METHODS: The authors studied the presenting features and value of routine diagnostic procedures in relation to the histology of primary malignant disease in 45 patients with LMM of solid (n = 30) or hematologic (n = 15) malignancies. RESULTS: Patients with solid LMM present mostly with spinal or radicular symptoms (53%), whereas patients with hematologic LMM more often show cranial nerve dysfunction at presentation (53%). Multifocal neurologic symptoms were seen in 67% of patients. The first CSF cytology demonstrated malignant cells more frequently in solid LMM compared with hematologic LMM (73% versus 53%). Extralumbar punctures increased the sensitivity of cytology to a greater extent in hematologic LMM than in solid LMM (34% versus 10%). Abnormal neuroimaging findings were found more often in solid LMM than in hematologic LMM (67% versus 40%). Increased total CSF protein in combination with either multifocal neurologic symptoms or abnormal neuroimaging findings was found in 73% of patients with a negative first CSF cytology. CONCLUSIONS: Patients with LMM presented differently depending on the histology of the primary tumor. In patients with a negative first CSF cytologic examination, multiple lumbar punctures increased the diagnostic accuracy, especially in hematologic LMM. LMM could also be diagnosed in patients with known cancer if total CSF protein was increased in combination with either multifocal neurologic symptoms or abnormal neuroimaging findings, preferably MRI.     

Gd-DTPA enhancement of cranial nerves on MR imaging. Neoplastic lesions

This study describes a radiological finding - enhancement of cranial nerves and correlates patients' clinical findings and outcome. Seven patients with enhancement of cranial nerves on postcontrast MR were retrospectively reviewed. Cranial nerves having contrast enhancement were optic, oculomotor, trigeminal, facial, acoustic, glossopharyngeal, vagus and accessory nerves. The patients' underlying diseases were malignant lymphoma (3), leukemia (1 patient) and metastatic tumor (2 lung, 1 rectum cancer). Most of the cases (4 out of 7) developed parenchymatous lesion later. Seven patients had CSF cytology study, positive in 3 cases, negative in 4 cases at first spinal tap. In 1 case (case 5) of negative cytology, elevated CEA (carcinogen antibody) was noted. In 2 cases, initial symptoms were sudden hearing loss. Autopsy was done for 1 case of metastatic tumor involving cranial nerves. Contrast MR is a useful examination for depicting cranial nerve involvement with neoplastic change.     

脑膜转移瘤临床特征及脑脊液细胞学分析

目的探讨脑膜转移瘤的早期临床特点和脑脊液细胞学特征。方法对79例脑膜转移瘤患者的原发病、临床特点、脑MRI和脑脊液细胞学变化进行回顾性分析。结果79例患者中以原发于肺癌及乳腺癌多见,分别为肺癌30例(37.98%)、乳腺癌18例(22.79%)。早期多出现剧烈头痛(71例,89.87%)和脑膜刺激征(69例,87.34%)。MRI强化检查,52例(69.33%)出现脑膜增厚、结节强化。51例行脑脊液检查,颅内压均有不同程度增高,细胞学分析显示,出现异型细胞和肿瘤细胞30例(58.82%)。结论脑膜转移瘤患者早期多出现剧烈头痛和脑膜刺激征,MRI增强检查可明显提高其早期诊断率。脑脊液细胞学可为诊断提供确诊依据。