中枢神经系统淋巴瘤二例

目的探讨中枢神经系统淋巴瘤的临床、脑脊液细胞学、影像学和病理学。方法对两例中枢神经系统淋巴瘤患者进行腰椎穿刺术,行脑脊液测压、常规、生化、细胞学检查及磁共振(MRI)扫描。结果例1为原发性中枢神经系统非霍奇金淋巴瘤脑病变,例2为继发性中枢神经系统非霍奇金淋巴瘤脊髓病变。例1患者临床表现主要为肢体无力、记忆力下降及脑神经损伤。脑脊液压力125mm H2O,氯化物130.0mmol·L-1,葡萄糖4.97 mmol·L-1,蛋白0.46g·L-1。头颅MRI T1WI呈低信号,T2WI呈高信号,增强后强化。病理切片可见少量小圆形肿瘤细胞,免疫组化显示为CD20阳性。例2患者临床主要表现为肢体无力,脑脊液压力180mm H2O,氯化物124.6mmol·L-1,葡萄糖3.77 mmol·L-1,蛋白1.73g·L-1。椎体MRI示T1WI低信号,T2WI呈高信号,增强后均匀强化。病理诊断为弥漫性大B细胞淋巴瘤。结论中枢神经系统淋巴瘤的临床表现和其侵犯部位有关,脑脊液中可找到肿瘤细胞,MRI表现为低T1WI信号,高T2WI信号,增强后强化。病理多显示为B细胞来源的淋巴瘤。     

头颅CT扫描与脑脊液细胞学检查对诊断蛛网膜下腔出血的价值

目的 :探讨头颅CT扫描与脑脊液检查对诊断蛛网膜下腔出血的价值。方法 :对 30例经头颅CT及腰穿脑脊液检查诊断为原发性SAH者行脑脊液细胞学检查 ,检查时间为病程中的 3、7、15、2 3、35d。结果 :30例原发性SAH ,CT阳性者 18例 ,阳性率 6 0 % ;腰穿脑脊液外观均匀血性 18例 ,黄变 7例 ,清 5例 ,血性和黄变占 83 % ;找到SAH特征细胞 ,即红细胞吞噬细胞和含铁血黄素吞噬细胞 2 3例 ,占 76 6 %。结论 :CT阴性不能排除原发性SAH ,其可通过腰穿脑脊液外观、红细胞形态观察及脑脊液细胞学检查而 10 0 %确诊 ,远较CT阳性率 6 0 %高 (P <0 0 1)     

原发性中枢神经系统淋巴瘤临床影像和病理形态特征分析

目的 探讨原发性中枢神经系统淋巴瘤(PCNSL)的临床影像和病理特征.方法 回顾性分析15例原发性中枢神经系统淋巴瘤的影像学特征和应用CD20、CD79a、CD3、CD43、GFAP、Vimentin、CgA、Syn、Ki-67等进行免疫组化染色.结果 原发性中枢神经系统淋巴瘤临床表现为中枢神经系统受损表现,头颅CT和MRI检查肿瘤多侵及额叶与颞叶,病理组织学特征为B细胞占大多数,特点是弥漫一致的小圆细胞,呈浸润性生长,有围管现象.结论 原发性中枢神经系统淋巴瘤是高度恶性肿瘤,预后差,临床影像表现没有特异表现,病理形态和免疫组化是诊断最可靠的依据.     

MRI与CT在诊断原发性脑淋巴瘤中的特点及应用

目的探讨MRI与CT在临床上检查诊断原发性脑淋巴瘤的特点及应用。方法对笔者所在医院近期的13例经病理证实的原发性脑淋巴瘤的MRI、CT资料进行回顾性分析。结果 13例患者中,9例单发者,4例多发者,一共21个病灶。CT平扫多表现为等或稍高密度,而MR平扫时T1WI等或稍低信号,T2WI呈高信号。结论原发性脑淋巴瘤的CT和MRI表现具有一定的特征,对CT与MRI影像资料进行综合分析,对提高早期诊断的正确性具有一定临床意义。     

头颅CT扫描与脑脊液细胞学检查对诊断蛛网膜下腔出血的价值

目的：探讨头颅CT扫描与脑脊液检查对诊断蛛网膜下腔出血的价值。方法：对30例经头颅CT及腰穿脑脊液检查诊断为原发性SAH者行脑脊液细胞学检查,检查时间为病程中的3、7、15、23、35d。结果：30例原发性SAH,CT阳性者18例,阳性率60％;腰穿脑脊液外观均匀血性18例,黄变7例,清5例,血性和黄变占83％;找到SAH 征细胞,即红细胞吞噬细胞和含铁血黄素吞噬细胞23例,占76．6％。结论：CT阴性不能排除原发性SAH,其可通过腰穿脑脊液外观、红细胞形态观察及脑脊液细胞学检查而100％确诊,远较CT阳性率60％高（P＜0．01）。     

原发性低颅压综合征的临床及影像学特点

目的探讨原发性低颅压综合征的临床及影像学特点。方法回顾性分析24例原发性低颅压综合征患者的临床资料。结果本组男8例,女16例;多以急性或亚急性起病。所有患者均表现为体位相关性头痛,即座位或站立位时头痛,平卧时减轻或明显缓解;头痛一般位于前额,也可位于后枕部及颞部;头痛性质均为胀痛。24例患者腰穿脑脊液检查示压力均≤60 mmH2O(1 mmH2O=0.0098 kPa)。本组24例患者行头颅CT检查发现,21例正常,2例脑肿胀,1例脑肿胀并蛛网膜下腔出血。14例患者行头颅MRI检查发现,10例脑下坠,8例静脉窦扩张(以上矢状窦扩张为主),6例脑室、脑池变小,9例硬膜下积液;4例行增强扫描检查发现,3例弥漫性硬脑膜强化。结论原发性低颅压综合征临床表现为急性或亚急性起病的体位性头痛,常伴有恶心、呕吐,脑脊液压力常≤60 mmH2O。头颅CT、MRI检查可有助诊断。     

肺癌转移致脑膜癌病12例临床分析

目的 分析12例肺癌致脑膜癌病患者的临床特征.方法 对12例经脑脊液细胞学证实的肺癌致脑膜癌病患者的临床资料进行分析.结果 首发症状多为头痛、呕吐,头颅CT或MRI检查多无异常发现,脑脊液检查颅内压均有不同程度增高,脑脊液生化检查蛋白升高者多见,细胞学检查均见癌细胞,形态与原发肿瘤相似,经治疗后25%的患者获得缓解.结论 对以头痛、呕吐发病,伴颅内压增高及脑膜刺激征,且CT及MRI检查无明显异常者,应高度警惕脑膜癌病的可能,鞘内注射加全身化疗是有效的治疗手段.     

原发性脑淋巴瘤CT与MRI诊断

目的分析原发性脑淋巴瘤的CT和MRI表现,以提高对其的认识,减少误诊。方法回顾性分析17例经手术病理证实的原发性脑淋巴瘤的CT和MRI资料。结果CT和MRI主要表现为：（1）CT平扫示脑内单发或多发的等密度或较高密度瘤灶,肿瘤可沿脑脊液播散;MRI T1WI呈等或稍低信号,T2WI呈等或稍高信号;1例出现坏死及出血;②少部分病例瘤周水肿及占位效应轻至中度;（3）MRI增强扫描大多呈团块状均匀强化;1例环形强化。④除病变主体位于胼胝体膝部和压部外,瘤灶长轴多与颅脑前后径走行平行或大致相同。结论原发性脑淋巴瘤多在大脑深部,明显强化,CT和MRI表现具有一定的特异性,结合CT与MRI影像特点和临床资料综合分析,有可能在术前作出正确诊断。     

室管膜型原发性中枢神经系统淋巴瘤四例:临床表现及脑脊液细胞学分析

目的分析室管膜型原发性中枢神经系统淋巴瘤(PCNSL)的临床及影像学特点,探讨脑脊液细胞学检测方法在诊断中的价值。方法回顾4例室管膜型PCNSL患者临床诊断与治疗经过,分析其临床特点、影像学改变、脑脊液细胞学和免疫细胞化学染色结果。结果 4例患者平均发病年龄为44岁,发病至就诊时间13 d～5个月。临床表现为头痛、脑膜刺激征,分别伴有脊神经根征(2例)、多组脑神经麻痹(1例)和偏瘫(1例);病程中有间断低热(3例)。其中2例于发病2和4个月时死于脑疝。腰椎穿刺检查脑脊液压力(2例)、白细胞计数(4例)和蛋白定量(4例)升高,葡萄糖降低(3例)。4例患者脑脊液细胞学检测均发现淋巴瘤细胞或异形淋巴细胞,免疫细胞化学染色大多数细胞呈现B细胞标记物阳性。头部MRI增强扫描第三和第四脑室、侧脑室壁异常强化,合并脑室周围强化病灶(2例)和鞍区病变(1例)。PET扫描和骨髓穿刺检查未发现颅外或脊髓受累证据。结论脑脊液细胞学和免疫细胞化学检测是诊断室管膜型PCNSL的重要方法。室管膜及脑室周围病变应考虑PCNSL的可能,尤其MRI增强扫描发现结节样强化者更应提高警惕。     

室管膜型原发性中枢神经系统淋巴瘤四例：临床表现及脑脊液细胞学分析

目的分析室管膜型原发性中枢神经系统淋巴瘤（PCNSL）的临床及影像学特点,探讨脑脊液细胞学检测方法在诊断中的价值。方法回顾4例室管膜型PCNSL患者临床诊断与治疗经过,分析其临床特点、影像学改变、脑脊液细胞学和免疫细胞化学染色结果。结果4例患者平均发病年龄为44岁,发病至就诊时间13d-5个月。临床表现为头痛、脑膜刺激征,分别伴有脊神经根征（2例）、多组脑神经麻痹（1例）和偏瘫（1例）;病程中有间断低热（3例）。其中2例于发病2和4个月时死于脑疝。腰椎穿刺检查脑脊液压力（2例）、白细胞计数（4例）和蛋白定量（4例）升高,葡萄糖降低（3例）。4例患者脑脊液细胞学检测均发现淋巴瘤细胞或异形淋巴细胞,免疫细胞化学染色大多数细胞呈现B细胞标记物阳性。头部MRI增强扫描第三和第四脑室、侧脑室壁异常强化,合并脑室周围强化病灶（2例）和鞍区病变（1例）。PET扫描和骨髓穿刺检查未发现颅外或脊髓受累证据。结论脑脊液细胞学和免疫细胞化学检测是诊断室管膜型PCNSL的重要方法。室管膜及脑室周围病变应考虑PCNSL的可能,尤其MRI增强扫描发现结节样强化者更应提高警惕。     

Magnetic resonance imaging of the brain and spine

Summary The efficacy of magnetic resonance imaging (MRI) in the diagnosis of diseases of the central nervous system is reviewed. MRI, computed tomography (CT) and certain radionuclide studies are compared in the evaluation of intracranial tumours, cerebral vascular disease, multiple sclerosis and other white matter diseases, dementia, head injury, infection, epilepsy, spinal lesions and in paediatric central nervous system disorders. The measurement of cerebrospinal fluid volumes and dynamics by MRI is discussed. MRI most clearly has advantages where CT is degraded by bone hardening and streak artefacts (spine, skull base, posterior and temporal fossa, sella and parasellar regions) and in diseases in which the X-ray attenuation of the suspected lesion differs little from normal parenchyma (paediatric brain disorders, demyelination and dysmyelination, early oedema associated with infarction, infection or low-grade infiltrating neoplasm, subacute and chronic haemorrhage and lesions in the spinal subarachnoid space and cord). Elsewhere MRI and CT should be seen as complementary rather than competitive methods of imaging. In spite of an absence of information about the contribution of MRI to management decisions and a lack of rigorous, prospective controlled trials, MRI will play an increasing role in the diagnosis of diseases of the central nervous system.     

Isolated neurosarcoidosis presenting as headache and multiple brain and spinal cord lesions mimicking central nervous system metastases

Sarcoidosis is uncommon in children. Although isolated neurosarcoidosis has been seen in 15% adults with sarcoidosis, pediatric neurosarcoidosis is rarely reported. Neurosarcoidosis may present with cranial neuropathy, including facial palsy, optic nerve or other cranial nerve involvement, peripheral neuropathy, or manifestations of the central nervous system affecting the hypothalamus, pituitary gland, cerebral cortex, cerebellum, meninges, and spinal cord. The useful diagnostic investigations include magnetic resonance imaging of the brain and spinal cord, cerebrospinal fluid studies, brain and meningeal biopsy if feasible, chest radiography to reveal sarcoidosis, angiotensin-converting enzyme level in the serum or cerebrospinal fluid, and Kveim test when available. We herein report a case of isolated brain biopsy-confirmed neurosarcoidosis in a 17-year-old boy presenting with severe unilateral headache and multiple brain and spinal cord MRI lesions mimicking central nervous system metastases.     

Neuropathology of malignant lymphoma and its related disorders]

Primary central nervous system lymphoma are characterized by macroscopically the tumor mass formation in the cerebral hemisphere including basal ganglia and corpus callosum and diffuse invasion particularly in the periventricular region, and microscopically lymphoma cells proliferation around the vessels and diffuse invasion into the brain parenchyma. Secondary involvement of central nervous system by systemic lymphoma are characterized by tumor cells invasion to the meninges and cranial and spinal nerve roots and tumor cells invade to the brain parenchyma along the perivascular space. Tumor nodule formation are very rare and tumor cells are not found in the lumen of the vessels. In the cases of extradural metastasis of systemic lymphoma, in addition to spinal cord compression, secondary circulatory disturbance, particularly venous congestion are also important factor for the spinal cord damages. In intravascular malignant lymphoma, not only tumor cells proliferation in the lumen of small sized vessels, but also secondary vasculitis and fresh and old thrombus formation are important for the development of multiple infarction of central nervous system. And spinal cords particularly in the levels of lumbosacral spinal cord are dominantly involved. Recently, according to the longness of clinical course of intravascular malignant lymphomatosis, cases with tumor mass are reported and these tumor mass are very similar to that of primary central nervous system lymphoma.     

A rare case of nonenhancing primary central nervous system lymphoma mimic multiple sclerosis

Primary central nervous system lymphoma (PCNSL) is reported to have increased in the last decades. Early diagnosis is crucial for proper management of this tumor. We report a case of a 48-year-old man who was initially diagnosed with multiple sclerosis. Magnetic resonance imaging of the brain revealed multiple lesions with hypersignals in the bilateral basal ganglia and brain stem in T2-weighted image and non-enhancement, while positron emission tomography showed a low uptake of ¹⁸F-fluorodeoxyglucose in the affected brain, indicative of demyelination. However, this individual was correctly diagnosed with PCNSL after biopsy and further histological analysis. Primary central nervous system lymphoma must be considered even when nonenhancing, diffuse lesions are seen on MRI. A visible tumor on imaging is essential to ensure an early brain biopsy and histological diagnosis.Primary central nervous system lymphoma (PCNSL) accounts for 3.3% of all brain tumors.1 Primary central nervous system lymphoma in immunocompetent patients is non-Hodgkin lymphomas of germinal B-cell origin in the vast majority of cases, which arise from the brain, spinal cord, cerebrospinal fluid (CSF), or eyes in the absence of systemic disease.1,2 Because of its ambiguous clinical manifestation, neuroradiographic, and CSF cytology, PCNSL can be easily misdiagnosed. Our objective in presenting this particular case is to highlight that PCNSL should be considered even when non-enhancing, diffuse lesions are seen on MRI.     

原发中枢神经系统自然杀伤/T细胞脑膜淋巴瘤一例临床分析

目的 探讨原发中枢神经系统自然杀伤/T(NK/T)细胞脑膜淋巴瘤的临床特点和诊断方法.方法 报道1例原发中枢神经系统NK/T细胞脑膜淋巴瘤患者,并结合文献总结其临床特征.结果 患者青年男性,急性起病,表现为视物成双、头痛、呕吐,口角歪斜,短期内出现全身疼痛、四肢无力,腰穿白细胞和蛋白显著升高,脑脊液细胞学曙红亚甲基蓝Ⅱ和迈-格-姬染色可见大量异型淋巴细胞,核异型性明显,可见核分裂相;免疫细胞化学染色可见多数淋巴样细胞Ki-67阳性,提示为增殖活跃的淋巴瘤.脑脊液流式细胞学:97.98%的细胞表达细胞膜型CD3、CD7、CD56、CD2和CD5,部分表达CDs,免疫分型为恶性NK/T细胞;头颅MRI+强化提示双侧三叉神经增粗,软脑膜强化,增粗的三叉神经轻度强化;骨髓检查未见异常,胸腹CT及强化未见异常.依据患者的病史、体格检查、临床检测和疾病经过,诊断为原发中枢神经系统NK/T细胞脑膜淋巴瘤.结论 原发中枢神经系统NK/T细胞脑膜淋巴瘤是罕见的中枢神经系统淋巴瘤,具有特殊的免疫分型,临床表现为恶性进展的高颅压、脑神经和脊神经受累,脑脊液细胞学和流式细胞分型对确定诊断起关键作用,放化疗效果欠佳,预后差.

Abstract：

Objective To explore the clinical features and diagnostic method of primary natural killer( NK)/T cell meningeal lymphoma. Methods An unusual case of a 19-year-old male with primary NK/T cell meningeal lymphoma was reported. His clinical presentation and laboratory findings were discussed. The related literatures have been reviewed. Results The patient presented with diplopia,headache, vomiting and facial drooping at the onset, followed by progressive pain and weakness of the four limbs. Cerebrospinal fluid showed significant increase in pressure, leukocytes number, levels of protein,normal glucose and adenosine deaminase, negative tuberculosis antibody and sterile staining. In cerebrospinal fluid cytological analysis, May-Grunwald-Gimsa staining showed large number of atypical lymphocytes with irregular nucleus and nuclear fission, Ki-67 immunostaining showed extensive proliferative activity of the lymphoid cells. Flow cytometric immunophenotypic analysis of cerebrospinal fluid indicated 97. 98 percent of cells expressed surface CD3, CD7, CD56, CD2, CD5, and partially expressed CD8. This was a rare immunophenotype for NK/T-cell. Cranial MRI with gadolinium showed thickening of the trigeminal nerve with slight enhancement and diffuse leptomeningeal enhancement. CT of the chest and abdomen and bone marrow biopsies were negative. He was diagnosed as primary NK/T cell meningeal lymphoma based on the clinical features and related examination. Conclusions Primary NK/T cell meningeal lymphoma is a rare type of primary central nervous lymphomas which has special immunophenotype. The clinical features include progressive raised intra-cranial pressure, multiple cranial and spinal nerve involvements. Cerebrospinal fluid cytological analysis and flow cytometric immunophenotypic analysis are key work-up for diagnosis. It has poor response to chemotherapy and radiotherapy.     

Lymphomatosis cerebri presenting as a rapidly progressive dementia: clinical, neuroimaging and pathologic findings

Primary central nervous system lymphoma (PCNSL) usually presents with clinical and neuroimaging findings consistent with single or multiple intracranial mass lesions. On cranial magnetic resonance imaging (MRI), such lesions are nearly always contrast enhancing, reflecting disruption of the blood-brain barrier at the site of tumor nodules. We describe 2 cases from the UCLA Medical Center who developed a rapidly progressive dementia due to extensive gray and white matter cerebral lesions involving much of the brain. In the patient who came to autopsy, widely infiltrating, focally necrotic B-cell plasmacytoid lymphoma was noted throughout the cerebral neuraxis. MRI findings in case 2 were consistent with diffuse lymphomatous brain infiltration without mass lesions, which was biopsy proven. We conclude that PCNSL may occur in a diffusely infiltrating form which may occur without MRI evidence of mass lesions or blood-brain barrier compromise. We refer to this entity as 'lymphomatosis cerebri' and add it to the differential diagnosis of a rapidly progressive dementia.     

中枢神经系统血管内淋巴瘤病一例报告

目的通过对１例中枢神经系统血管内淋巴瘤病患者的临床、影像学和脑组织病理检查,探讨此病的诊断规律。方法对患者的临床症状及影像学改变进行系统观察,对脑手术标本进行组织学和免疫组织化学染色。结果患者,５０岁,男性。表现为亚急性脑病,病情迅速恶化,于病后４６天死亡。辅助检查发现血沉加快,脑脊液仅出现蛋白轻度升高。影像学检查显示大脑白质多灶性损害,随病程的延长,病灶迅速增大、增多。脑病理特点为广泛的血管内恶性Ｂ淋巴瘤细胞增殖,导致血管堵塞,白质内出现多发性陈旧及新鲜的缺血病灶。结论此病的诊断只能依靠脑病理学检查。临床鉴别诊断包括恶性胶质瘤、进行性多灶性白质脑病、多发性硬化和颅内血管炎。     

中枢神经系统结核误诊分析(附35例临床病理报告)

目的探讨中枢神经系统结核诊断及误诊原因.方法分析了35例生前被误诊、尸解病理证实的中枢神经系统结核.结果误诊为化脓性脑膜炎9例,病毒性脑炎7例,乙型脑炎4例,脑肿瘤及脑积水各3例,视神经脊髓炎、脑脓肿、败血症各2例,麻疹、多发性脑脊髓瘤、淋巴瘤各1例.临床表现除脑膜刺激征、颅高压外,可有中枢神经系统局灶损害症状.脑脊液改变为压力升高,细胞计数与蛋白含量增高,糖与氯化物降低.尸检发现脑膜病变为主,脑实质、脑室、脑血管、脊髓均有结核病变.结论中枢神经系统结核临床表现与结核病变性质、部位密切相关,认识其病理类型、临床症状多样性以及动态脑脊液观察有助于提高诊断率.