The purpose of this retrospective analysis was to evaluate the clinical presentations, radiological characteristics, patient outcomes, and therapeutic approaches among individuals diagnosed with primary central nervous system lymphoma (PCNSL), high-grade glioma (HGG), and metastatic brain tumors (METS). We assembled a cohort of brain tumor patients from two medical centers, with two oncologists independently reviewing their clinical profiles. A retrospective examination of 87 PCNSL, 87 HGG, and 71 METS cases was performed to assess the aforementioned parameters. Notable variations were identified in the incidence of epileptic seizures and cognitive impairments between PCNSL and METS patients. Cerebral hemisphere involvement was predominantly observed in HGG and METS cases. PCNSL cases exhibited a higher likelihood of multiple lesions, whereas HGG showed a greater tendency for recurrence. The median survival times were established at 24.3 months for PCNSL, 44.5 months for HGG, and 27.1 months for METS patients. In PCNSL cases, the number of lesions was identified as a significant predictor of mortality (P = 0.008). Our findings highlight the importance of clinical and imaging features in diagnosing PCNSL, which may present distinct features compared to HGG and METS.

目的 探讨自身免疫性脑炎的临床特点及治疗预后.方法 收集2016年6月至2019年10月于徐州医科大学附属医院确诊的19例自身免疫性脑炎患者的临床资料,回顾性分析其临床表现、脑脊液检查结果、影像学表现、脑电图表现及治疗预后.结果 19例患者中男14例,女5例,年龄18～68岁,平均年龄(42.1±16.0)岁.起病形式多呈急性或亚急性,临床表现为癫痫发作14例,精神行为异常10例,认知障碍9例,意识水平下降或昏迷4例,言语障碍3例,自主神经功能障碍3例,睡眠障碍3例,腹泻1例,低钠血症1例.脑脊液检查:白细胞数增高者18例,蛋白增高者11例,免疫球蛋白IgG增高者11例,液基细胞学见淋巴细胞增多者7例.影像学:头颅MRI检查主要表现为额叶、颞叶、海马、岛叶等部位T2加权像(T2WI)、液体衰减反转恢复(FLAIR)序列呈高信号,部分患者弥散加权成像(DWI)呈高信号,增强扫描伴强化者少见.脑电图:正常者6例,轻度异常者5例,中度异常者4例,重度异常者1例.治疗预后:多数患者使用糖皮质激素、免疫球蛋白等免疫治疗后改良Rankin量表(MRS)评分较入院时降低1～3分,症状完全恢复者6例,放弃治疗者1例.结论 自身免疫性脑炎临床表现复杂多样,以癫痫发作、精神行为异常、认知障碍急性或亚急性起病的患者,应考虑自身免疫性脑炎可能,需完善脑脊液、头颅MRI等相关检查,尽早启动免疫治疗.

神经系统副肿瘤综合征(paraneoplastic neurological syndrome,PNS)是指机体各系统的恶性肿瘤或潜在的恶性肿瘤,在非浸润、压迫或转移的情况下,产生"远隔"效应而导致神经系统临床症状[1].现报告1例确诊PNS患者的临床资料,结合相关文献探讨该病的病因、临床表现、病理及影像学特征.

Objective: To investigate the clinical features and imaging characteristics of mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). Methods: Seventeen patients with MELAS diagnosed in the Affiliated Hospital of Xuzhou Medical University from July 2014 to August 2018 were enrolled in this study and their clinical manifestations, imaging and histopathological features were retrospectively analysed. We also discussed and summarised the related literature. Results: All of the 12 patients had seizures; stroke-like episodes in 12 cases; audio-visual impairment in 12 cases; headache in six cases; dysplasia in four cases; mental retardation in three cases; ataxia in two cases. On cranial magnetic resonance (MR) scans, the most common manifestations were in temporal-occipital-parietal lobe, cortical or subcortical areas as well as frontal lobe, thalamus, and basal ganglia showing long or equal T1 signals, long T2 signals, and hyperintense or iso-intense diffusion-weighted imaging (DWI) signals accompanied by ventricular enlargement and brain atrophy. MR spectroscopy showed that lactic acid peaks could be found in lesion sites, normal brain tissues, and cerebrospinal fluid. Muscle biopsy and genetic testing are the gold standard for diagnosing MELAS, muscle biopsy revealed COX-negative muscle fibres and SDH-stained red ragged fibres (RRF) under the sarcolemma. Mutations of mtDNA A3243G locus were common on gene testing. Improvement of mitochondrial function was observed after symptomatic and supportive treatment. Conclusion: MELAS should be considered for patients with epileptic seizures, headache, stroke-like episodes, extraocular palsy, cognitive decline and other clinical manifestations with the lesion located in the temporal-occipital-parietal lobe regardless of the distribution of blood vessels, and further examinations including muscle biopsy and gene testing should be performed to confirm the diagnosis.

目的 探讨平山病的临床表现、神经电生理及影像学特征.方法 回顾性分析2011年1月至2017年12月徐州医科大学附属医院13例确诊为平山病的住院患者的临床、神经电生理及影像学资料.结果 13例患者中男性12例,女性1例;年龄15～26岁,平均(19.6±3.2)岁.所有患者均有肌肉无力和萎缩(其中6例单侧受累,7例双侧均受累),手指震颤10例,肌束颤动2例,“寒冷麻痹”1例.神经电生理:13例患者感觉传导、运动传导均未见阻滞,复合肌肉动作电位的波幅下降,均表现为慢性失神经性改变.影像学:颈椎MRI平扫(直立位)表现为生理曲度变直、颈髓变扁平,部分出现颈髓萎缩或异常信号.屈曲位均出现颈髓变扁平、硬膜外新月形信号影,增强后部分患者出现硬膜外异常信号强化.均予以营养神经治疗,其中11例患者使用颈托治疗,1例患者予颈前路关节融合术和颈托联合疗法,所有患者肌无力症状均得到一定程度的改善.结论 青少年男性如出现单侧上肢肌肉无力萎缩伴手指震颤或肌束颤动,需考虑平山病可能,应进一步完善神经电生理及颈椎MRI检查(尤其是屈曲位)以确诊.

目的 调查原发性干燥综合征患者睡眠质量及影响睡眠质量的相关因素,为改善干燥综合征患者睡眠质量提供依据.方法 采用匹兹堡睡眠质量指数(Pittsburgh sleep quality index,PSQI)、 医院焦虑抑郁量表(hospital anxiety and depression scale,HADS)、 疲劳严重度量表(ftigue se-verity scale,FSS)、 欧洲干燥综合征疾病活动指数(EULAR Sj?gren's syndrome disease activity index,ESSDAI)、 欧洲干燥综合征患者报告指数(EULAR Sj?gren's syndrome patient reported index,ESSPRI)及自制一般情况调查表对门诊的68例原发性干燥综合征患者进行问卷调查,分析睡眠障碍与人口学、临床资料、 实验室检查、 疾病活动性的相关性.结果 患者PSQI总分(7.7±3.6)分,明显高于健康人群(3.9±2.5);不同性别患者睡眠障碍的检出率差异无统计学意义(P>0.05).伴睡眠障碍组在年龄、HADS、FSS、ESSPRI、ESSDAI、IL-6、IgG、 血沉方面与不伴睡眠障碍组差异具有统计学意义(P<0.05).睡眠障碍与年龄、HADS、FSS、ESSPRI、IL-6水平均呈正相关(r=0.521、0.756、0.503、0.294、0.915,P<0.05),与ESSDAI、 血沉、IgG水平负相关(r=-0.345、-0.343、-0.429,P<0.05),与CRP无明显相关性(P=0.834).结论 原发性干燥综合征的睡眠障碍明显高于健康人群,相关的因素有年龄、 焦虑抑郁状态、 疲劳、 高IL-6水平、 高ESSPRI等.与系统损害的程度呈负相关,提示较高的针对原发病的治疗强度可能改善患者的睡眠情况.

目的 探讨结节性硬化症(TSC)的临床及影像学特点.方法 回顾性分析徐州医科大学附属医院收治的TSC患者的临床资料,总结临床和影像学特点.结果 所有患者中,颜面部血管纤维瘤8例、色素脱失斑9例、癫痫发作11例、智能减退7例.头颅CT可见室管膜下多发钙化结节;头颅MRI可见皮质及皮质下结节,T2WI、FLAIR序列均呈高信号.偶可见侧脑室占位,在头颅MRI上信号混杂,增强扫描强化明显,病理结果提示为室管膜下巨细胞星形细胞瘤.肾脏检查可发现多发结节、多发肾囊肿、血管平滑肌脂肪瘤.所有癫痫患者均使用抗癫痫药物,半数疗效不佳,其中3例行癫痫病灶切除术,病理提示为局灶皮质发育不良.结论 表现为皮肤损害和癫痫发作的患儿,无论有无家族史,均需高度怀疑TSC,需进一步完善头颅CT/MRI、肾脏超声/CT以便确诊.药物治疗无效的部分癫痫患者可行手术治疗.

目的 探讨线粒体脑肌病的临床特征及影像学特点.方法 回顾性分析2014年7月至2018年8月在徐州医科大学附属医院临床确诊为线粒体脑肌病的31例住院患者的临床资料,并结合文献予以探讨与总结.结果 所有患者中癫痫发作19例,视听障碍17例,卒中样发作16例,头痛10例,发育异常8例,智力低下7例,共济失调3例,眼外肌麻痹1例,心慌胸闷1例.头颅MRI多表现为颞、枕、顶叶皮质或皮质下多发病灶,也可见于额叶及丘脑、基底节等处,呈长或等T1、长T2信号影,弥散加权成像(DWI)呈等或高信号,伴脑室扩大和脑萎缩,氢质子磁共振波谱(1H-MRS)显示病变部位、正常脑组织及脑脊液中均可出现乳酸峰.肌电图表现为神经源性或肌源性损害.肌肉活检可见细胞色素C氧化酶(COX)阴性肌纤维及琥珀酸脱氢酶(SDH)染色肌膜下出现的破碎红纤维.基因检测示线粒体DNA(mtDNA)点突变.予以改善线粒体功能及对症支持治疗.结论 具有癫痫发作、头痛、卒中样发作、眼外肌麻痹、智能下降等临床表现且病灶位于颞、枕、顶叶,不按血管分布者,应考虑为线粒体脑肌病可能,需进一步完善血乳酸测定、肌肉活检、基因检测等以确诊.

目的 观察T淋巴细胞亚群及血清趋化因子配体10(CXCL10)、趋化因子受体3(CXCR3)在干燥综合征患者中的水平变化,并探讨其临床意义.方法 选取75例干燥综合征患者作为观察组,并选取同期61例体检健康者为对照组,应用双色流式细胞法检测外周血T淋巴细胞亚群,使用ELISA法检测受试者血清CXCL10、CXCR3水平.采用Pearson相关分析血清CXCL10与CXCR3的相关性,应用受试者工作特征(ROC)曲线分析CXCL10、CXCR3的临床诊断价值,采用多因素Logistic回归分析影响干燥综合征发生的危险因素.结果 观察组CD3+CD8+、CD4+CD8+、CD8+CD28+T细胞低于对照组,血清CXCL10、CXCR3水平高于对照组(P均<0.05).观察组血清CXCL10与CXCR3水平呈正相关(r=0.391,P<0.05).CXCR3诊断干燥综合征的ROC曲线下面积(AUC)为0.782(95％CI:0.694～0.901,P=0.001),CXCL10诊断干燥综合征的AUC为0.891(95％CI:0.764～0.984,P=0.000).年龄、CD4+CD8+T细胞、CD8+CD28+T细胞及血清CXCL10、CXCR3水平是影响干燥综合征发生的危险因素(P均<0.05).结论 干燥综合征患者T淋巴细胞亚群发生异常,并且血清CXCL10及其受体CXCR3水平升高,血清CXCL10、CXCR3水平具有较高灵敏度和特异度,可作为诊断干燥综合征有效的生物标志物,其中CX-CL10诊断价值更高.

Objective: To investigate the anatomical characteristics, clinical manifestations, and imaging features of bilateral cerebral peduncular infarction. Methods: A retrospective analysis was performed on 11 patients diagnosed with bilateral cerebral peduncular infarction in the Affiliated Hospital of Xuzhou Medical University from December 2014 to December 2018. Their clinical and imaging features were analyzed and summarized in combination with the relevant national and international literature. Results: Among all the patients, there were eight cases with a history of hypertension, four cases with a history of diabetes mellitus, and four cases with a history of smoking. Conscious disturbance was observed in nine cases, quadriplegia in seven cases, pseudobulbar paralysis in three cases, and ataxia in one case. Brain magnetic resonance (MR) scans of bilateral cerebral peduncles showed patchy abnormal shadows with a hypointense signal on T1-weighted imaging (T1WI) and apparent diffusion coefficient (ADC) and hyperintense signal on T2-weighted imaging (T2WI), fluid-attenuated inversion recovery (FLAIR), and diffusion-weighted imaging (DWI). Computed tomography angiography (CTA) scans of head and neck showed severe stenosis or occlusion of vertebral artery, basilar artery, or posterior cerebral artery. All the patients received standardized treatment for cerebral infarction. Six patients died while five were left disabled. Conclusion: Bilateral cerebral peduncle infarction may be related to cerebral perfusion insufficiency caused by the stenosis or occlusion of vertebrobasilar artery and its branches. The main clinical manifestations are locked-in syndrome and persistent vegetative state. The specific imaging feature of "Mickey Mouse ear"-like infarction is associated with a poor prognosis.

目的:探讨云克对强直性脊柱炎性附着点炎的治疗效果.方法:34例经非甾体抗炎药物及传统抗风湿药物治疗失败的强直性脊柱炎性附着点炎患者使用云克治疗.观察治疗前后的血沉、CRP、血小板计数、MASES、附着点超声的变化.结果:云克治疗前后血沉、CRP、血小板计数变化具有统计学意义.MASES改善有效率为79％.结论:云克对于强直性脊柱炎性附着点炎的炎症指标下降及临床症状改善有效.