神经系统副肿瘤综合征误诊为前列腺炎1例分析

本文将神经系统副肿瘤综合征误诊为前列腺炎1例分析如下. 1 病历摘要 男,51岁.主因下腹伴骶尾部疼痛2个月余于2011-06-27入院.2011-04出现下腹持续性疼痛,向骶尾部放散,伴前胸后背疼痛,程度较轻,就诊于多家医院查前列腺液见白细胞满视野,诊断“前列腺炎”,治疗效果欠佳.2011-06-20发现左锁骨上淋巴结和腹股沟淋巴结肿大,抗炎治疗后腹股沟淋巴结消失,左锁骨上淋巴结变小.2011-06-20左右就诊于广安门医院查胸片示右下肺多发结节影,前列腺液示白细胞40～50/HP,口服汤药治疗,为进一步治疗收入我科.     

神经系统副肿瘤综合征诊断及免疫治疗研究进展

神经系统副肿瘤综合征(PNS)是一种少见的临床综合征,在临床上容易误诊。目前在主要为排他性诊断,其手段包括免疫相关抗体及磁共振、(FDG)PET/CT等影像学检查、肌电图、肌肉活检等。PNS的治疗,应在积极治疗原发肿瘤的同时,给予免疫调节治疗,缓解患者神经系统症状,改善生存质量。     

以神经系统副肿瘤综合征为首发症状的会厌癌1例

患者男,61岁,主因言语不清、行走困难1年半余,伴吞咽困难、偶有饮水咳呛,就诊于我院针灸门诊.既往:支气管扩张病史1月余;疑似副肿瘤综合征2年,未系统治疗;成人still病6年.查体除左颌下淋巴结肿大明显外,余项均正常.神经系统检查:言语含糊不清,眼球位置居中、活动不受限,双眼侧视现水平眼震,垂直视现垂直眼震,舌体卷曲不能外伸;站立不稳,宽基底步态,四肢肌容量正常,双上肢肌力Ⅴ级、双下肢肌力Ⅴ-级,肌张力正常,膝腱反射亢进;双侧指鼻不准、跟膝胫试验(+),Romberg征(-);深浅感觉无异常;右Babinski(±).实验室检查:肿瘤全项:甲胎蛋白9.0 ng/mL;纤维蛋白原4.45 g/L;血小板聚集率:72.26,余项大致正常.辅助检查:头颅MRI、胸腺CT未见异常;副鼻窦CT示右筛窦炎;腹部CT示胆囊炎;新斯的明试验阴性;视诱发正常;电生理示BAEP正常,重频电刺激无异常;上下肢体感觉诱发电位正常,双正中、双尺运动感觉神经传导速度正常;抗神经元细胞核自身抗体(抗Hu)(+).     

抗磷脂抗体综合征合并中枢神经系统疾病5例报道及文献复习

目的:探讨抗磷脂抗体综合征(APS)中枢神经系统损害的临床表现及诊治特点。方法:回顾性分析APS合并中枢神经系统疾病患者5例的临床资料,结合文献进行分析。结果:5例抗磷脂抗体(aPL)均阳性,合并脑梗死2例,合并舞蹈病2例,合并癫痫1例,合并僵人综合征1例;治疗予以抗血小板、抗凝、抑制炎症、免疫抑制等,患者症状均得到改善。结论:神经系统受累的APS的诊断主要依据相关临床表现及a PL的检测,未有明确病因的神经系统疾病,可行a PL检测,有助于APS的诊治。     

2例血友病患者的获得性兔疫缺陷综合征

获得性细胞免疫系统功能障碍综合征的特征是淋巴细胞减少,T淋巴细胞功能缺陷和辅助T细胞/抑制T细胞比率倒转。并伴有机会性感染和恶性肿瘤的危险性增加。这种获得性免疫缺陷综合征(AIDS)的致病因子尚未确定。本文报告2例无症状血友病患者发生弥漫性淋巴结肿大,其性质与AIDS非常相似。例1:男性、32岁,患严重血友病甲,因子Ⅷ浓度<0.01u/ml,1980年～1981年间共用5,680u因子Ⅷ浓缩物治疗。1982年4月发现有淋巴结肿大。观察数月而无症状。抗HBs和抗HBc阳性,但无肝炎临床表现。与AIDS患者无接触史。体检发现双侧颈、耳后、枕、锁骨上、腋下和腹股沟区均有淋巴结     

神经系统副肿瘤综合征17例临床分析

目的：对神经系统副肿瘤综合征患者的临床基本特征进行分析和了解。方法：对17例神经系统肿瘤综合征患者的临床资料进行回顾性分析并加以总结。结果：17例副肿瘤综合征患者中3例在发现肿瘤后才出现神经症状,14例在出现副肿瘤症状后才发现肿瘤。诊断出副肿瘤综合征时5例已出现肿瘤转移。结论：副肿瘤综合征临床表现形式多样，可由多种病因引起。临床早期确诊对于治疗非常重要。     

抗Hu副肿瘤综合征病例2例分析并文献复习

目的:探讨抗Hu神经系统副肿瘤综合征的临床特点及预后。方法:报道我院2例抗Hu副肿瘤综合征并结合文献进行回顾性分析。结果:其中1例表现为严重的中枢性低通气,同时伴有周围神经病变,但全身PET-CT并未发现肿瘤;另1例表现为非特异性症状,肿瘤筛查中发现头颈部肿块,主要分布在鼻窦和腮腺,病理活检为小细胞癌。鼻窦肿块切除后短期内患者症状缓解明显。搜索既往报道的抗Hu相关副肿瘤综合征患者477例,最常见的表现是亚急性感觉性周围神经病,颅脑的MRI表现无特异性,最常见合并小细胞肺癌;在所有的病例中,有2.5%的患者表现为中枢性低通气。结论:抗Hu副肿瘤综合征应作为中枢性低通气的鉴别诊断之一。副肿瘤综合征相关抗体的检测,有利于更早发现肿瘤。     

ALK阳性梭形细胞间变性大细胞淋巴瘤1例

<正>患者女性,54岁,因畏寒、寒战2个月发热1个月余入院,体温最高达40 ℃,抗炎治疗后体温仍反复,近2个月体重下降约10 kg。全身多区域淋巴结肿大,胸部CT示纵隔淋巴结肿大,超声检查示双侧颈部、双侧锁骨上淋巴结异常肿大,全身PET-CT显示右侧咽旁、纵隔、双侧颈部、双侧锁骨区、双侧盆壁及双侧腹股沟多发淋巴结FDG代谢异常增高。实验室检查：WBC 9.05×109/L,中性粒细胞比例86.2%,血红蛋白102 g/L,     

1例神经系统副肿瘤综合征的护理

副肿瘤综合征(paraneoplastic syndrome,PS)为癌肿的远隔效应,是癌肿瘤对患者肢体远处组织、脏器非转移性损害的统称[1].神经系统副肿瘤综合征(paraneoplastic neurological syndromes,PNS)这一概念的提出,至今已有半个世纪.神经系统副肿瘤综合征是癌肿通过远隔作用引起的神经障碍,可不同程度地影响神经系统,而没有癌肿直接侵犯神经组织或代谢性、感染性及血管性并发症表现[2].恶性肿瘤发病率近年来升高,但早期诊断困难.约1%的癌肿患者出现神经系统副肿瘤综合征,神经系统出现明显的临床症状可在肿瘤发现前[3],因累及中枢神经系统、周围神经肌肉、血管等,使临床表现更加不典型、多样化,导致早期误诊.了解和认识"副肿瘤综合征"有助于肿瘤的早期发现,而症状护理在此病中减轻了患者的病痛,起到积极作用.     

1例神经系统副肿瘤综合征的护理

副肿瘤综合征（paraneoplastic syndrome,PS）为癌肿的远隔效应,是癌肿瘤对患者肢体远处组织、脏器非转移性损害的统称.神经系统副肿瘤综合征（paraneoplastic neurological syndromes,PNS）这一概念的提出,至今已有半个世纪.神经系统副肿瘤综合征是癌肿通过远隔作用引起的神经障碍,可不同程度地影响神经系统,而没有癌肿直接侵犯神经组织或代谢性、感染性及血管性并发症表现.恶性肿瘤发病率近年来升高,但早期诊断困难.约1%的癌肿患者出现神经系统副肿瘤综合征,神经系统出现明显的临床症状可在肿瘤发现前,因累及中枢神经系统、周围神经肌肉、血管等,使临床表现更加不典型、多样化,导致早期误诊.了解和认识“副肿瘤综合征”有助于肿瘤的早期发现,而症状护理在此病中减轻了患者的病痛,起到积极作用.     

Cannabis derivatives therapy for a seronegative stiff‐person syndrome: a case report

What is known and objective: Stiff‐person syndrome (SPS) is an uncommon and disabling disorder characterized by progressive rigidity and episodic painful spasms involving axial and limb musculature. SPS treatment is mostly based on benzodiazepines, baclofen, immunosuppressants and intravenous immunoglobulin. Cannabis derivatives [tetrahydrocannabinol (THC) and cannabidiol (CBD)] are available as an oromucosal spray (Sativex^®), indicated as add‐on treatment, for symptom improvement in patients with moderate to severe spasticity because of multiple sclerosis (MS). Our objective is to report a case of seronegative SPS successfully treated with THC‐CBD oromucosal spray. Case summary: We report a case of a 40‐year‐old man presenting with progressive muscle stiffness and intermittent spasms for 6‐years. The diagnosis of stiff‐person syndrome was based on the clinical features and neuroelectrophysiologic findings of continuous motor unit activity. Glutamic acid decarboxylase autoantibodies was absent in our patient, in both serum and cerebrospinal fluid (CSF). Cannabis derivatives oromucosal spray was introduced after a series of unsatisfactory traditional medical treatments. After 14 months treated with THC‐CBD oromucosal spray, improvement was verified in the eight dimensions of the scale of SF‐36 quality of life questionnaire. What is new and conclusion: Clinical experience with cannabis derivatives in patients with multiple sclerosis is accumulating steadily, but there is no current literature about its efficacy for SPS. Because MS and SPS share some neurological symptoms such as spasticity and rigidity, it is thought that THC‐CBC can be an option for SPS patient. Our case report suggests that THC‐CBD oromucosal spray is an alternative treatment for patients with refractory SPS, and further validation is appropriate.     

Usefulness of growth chart in children and adolescents with chronic fatigue syndrome

We presented three sheets of growth chart in children with chronic fatigue syndrome. The growth chart in 14-year-old boy (patient 1) showed decreased weight gain because of too much exercise. After that he complained nausea, abdominal pain, sleep disturbance and debilitating fatigue. The growth chart in 12-year-old girl (patient 2) revealed increased weight gain because of overeating due to the divorce of her parents. She developed syncope, sleep disturbance, and fatigue during overeating. The growth chart in 13-year-old girl (patient 3) showed decreased weight gain after she developed lymph node enlargement. We diagnosed her as autoimmune fatigue syndrome because of persistent positive antinuclear antibody. Although growth chart will not be able to detect childhood chronic fatigue syndrome prospectively, the chart may be useful for detecting some life events in these children.     

Synchronized early gastric cancer occurred in a patient with serrated polyposis syndrome: A case report

BACKGROUNDSerrated polyposis syndrome (SPS) is a relatively rare disease that is characterized by multiple serrated lesions/polyps. Very little is known regarding the extracolonic cancers associated with SPS. The genetic basis of the process remains unknown.CASE SUMMARYA 67-year-old male patient initially presented with belching and abdominal distension for a year as well as diarrhea for over 2 mo. The patient underwent colonoscopy and was diagnosed with serrated polyposis syndrome. Half a year later, a gastroscopy was performed during the postoperative re-examination to screen for other lesions of the upper gastrointestinal tract. An elevated lesion was detected in the anterior wall of the gastric antrum. Curative en bloc resection of the lesion was achieved via endoscopic submucosal dissection. The pathological result was high-grade dysplasia with focal intramucosal carcinoma. Exome sequencing was performed for the patient and five gastric cancer-associated variants (methylenetetrahydrofolate reductase, metaxin 1, coiled-coil domain containing 6, glutamate ionotropic receptor delta type subunit 1, and aldehyde dehydrogenase 1) were identified. CONCLUSIONThis paper reports a case that presented with both SPS and early gastric cancer. Genetic mutations that were potentially responsible for this condition were sought by exome sequencing.     

Heavily T2-weighted MR myelography in patients with spontaneous intracranial hypotension: a case-control study

We performed whole-spine heavily T2-weighted magnetic resonance (MR) myelography using a single-shot fast spin-echo pulse sequence in 17 patients (8 M/9 F) with spontaneous intracranial hypotension (SIH) to detect abnormal cerebrospinal fluid (CSF) collections. In addition, a group of age- and sex-matched controls were recruited. Follow-up MR myelography was also done at 3 weeks. MR myelography showed three kinds of abnormal CSF collections in 15 patients with SIH (88%): epidural fluid collection (n = 15, 88%), C1-2 extraspinal collections (n = 6, 35%) and CSF collections along nerve roots in the lower cervical or upper thoracic spines (n = 6, 35%). One patient (6%) showed a meningeal diverticulum. In contrast, none of the controls showed these findings. Overall, MR myelography results helped in early diagnosis of SIH in four (24%) patients whose initial brain MRIs failed to show typical SIH findings. Follow-up MR myelography results were compatible with the clinical changes with kappa statistics of 0.52 and an agreement rate of 76%. Our study showed heavily T2-weighted MR myelography provided a rapid, non-invasive and high yield method to diagnose and follow-up patients with SIH. Whether the CSF collections along the nerve roots represent the ongoing leakage sites warrants further study.     

AIDS合并颈部淋巴结结核在不同CD4＋T细胞水平的超声表现特征

目的探讨AIDS合并颈部淋巴结结核在不同CD4^＋T细胞水平的超声表现特征。 方法选取AIDS合并颈部淋巴结结核的患者42例,所有患者均经超声引导下粗针穿刺活检病理证实。依据穿刺前24 h内的CD4^＋T细胞计数值,将42例患者分为<50个/μl组,50~<100个/μl组,100~<200个/μl组,≥200个/μl组;并在颈部病灶穿刺前24 h内行常规二维超声及超声造影检查,详细记录病灶超声声像学特征,统计分析不同CD4^＋T细胞组间淋巴结的超声表现特征。 结果42例患者中,CD4^＋T细胞≥200个/μl组9例,100~<200个/μl组9例,50~<100个/μl组9例,<50个/μl组15例。CD4^＋T细胞≥200个/μl组患者,淋巴结最大直径主要集中在2~<3 cm,淋巴结钙化比例高于其他3组,无淋巴结融合及窦道,超声造影增强模式表现为均匀增强及不均匀增强。CD4^＋T细胞100~<200个/μl组患者淋巴结直径在2~<3 cm及≥3 cm均有分布,仅1例淋巴结表现为钙化,超声造影以不均匀增强表现为主。50~<100个/μl组患者中有7例淋巴结直径>3 cm,无钙化表现,5例有淋巴结融合,7例有窦道形成,超声造影表现为不均匀增强与环状增强。<50个/μl组患者淋巴结最大直径均>3 cm,无淋巴结钙化,病灶内部液化、淋巴结融合、窦道形成的比例均高于其他3组,超声造影以环状增强为主。随着CD4^＋T细胞减少,淋巴结直径越大,各组间差异具有统计学意义（χ²=30.89,P<0.01）;随着CD4^＋T细胞减少,淋巴结融合、窦道形成的概率增大,而钙化形成减少,各组间差异有统计学意义（χ²=62.63,P<0.01）;随着CD4^＋T细胞减少,超声造影增强模式逐步由均匀增强转变为环状增强,不同组间增强模式,差异有统计学意义（χ²=23.48,P<0.01）。 结论在不同CD4^＋T细胞水平,AIDS合并颈部淋巴结结核的超声表现存在差异,这为临床评估AIDS合并颈部淋巴结结核的病情进展提供了影像学依据。     

Spontaneous intracranial hypotension syndrome: report of twelve cases

PURPOSE: To investigate clinical, MRI, and radioisotope findings and therapeutic outcome of the syndrome of spontaneous intracranial hypotension (SIH). BACKGROUND: Spontaneous intracranial hypotension is characterized by orthostatic headache, low CSF pressure, and MRI findings of diffuse pachymeningeal gadolinium enhancement without previous history of head trauma or lumbar puncture. Spontaneous CSF leakage from a spinal dural tear has been suggested as the underlying pathogenic mechanism of SIH. Most patients recover without sequelae, but subdural collections have been described in a few. METHODS: Twelve consecutive patients (10 females, 2 males, mean age 39 years) with headache related to the syndrome of spontaneous intracranial hypotension were investigated. RESULTS: Eleven patients presented orthostatic headache, one patient had continuous nonpostural headache. Additional clinical symptoms included nausea, vomiting, tinnitus, diplopia, and back pain. All the patients had low CSF opening pressure, seven had increased CSF albumin, and four had pleocytosis. Brain MRI showed diffuse pachymeningeal gadolinium enhancement. Other features included subdural fluid collections (hematoma/hygroma) in four patients, downward displacement of the brain in four patients, and enlargement of the pituitary gland in one patient. Radioisotope cisternography results indicated, in two patients, a CSF leakage site in the cervico-thoracic region, and in one patient showed limited ascent of the tracer to the cerebral convexity and early appearance of radioisotope in the bladder. All the patients had complete resolution of headache with conservative treatment. CONCLUSIONS: Patients with SIH have distinct MRI and sometimes radioisotope cisternographic abnormalities and generally respond favorably to conservative management.     

Isaacs syndrome

Although Isaacs syndrome is known by several names including neuromyotonia and quantal squander, it bears some resemblence to other syndromes characterized by muscular stiffness such as stiff-man syndrome. The purpose of this paper is to examine pertinent clinical findings about Isaacs syndrome. A patient who presented cramping pain in both thighs and difficulty releasing his grip is described. Physical examination revealed myokymia in all four extremities, hyporeflexia, and hypertrophy of the thenar musculature. Electromyographic studied show continuous motor unit activity at rest. The patient was treated with phenytoin and showed almost complete symptom resolution over a 24-hour period. Clinical and electromyographic examinations in conjunction with the rapid response to phenytoin are diagnostic characteristics of Isaacs syndrome.     

Intrathecal gadolinium-enhanced MR-cisternography in spontaneous intracranial hypotension associated with Behcet's syndrome

We report a 32-year-old woman diagnosed with spontaneous intracranial hypotension (SIH) and Behcet's Syndrome. Suspicion of SIH was based on cervical MRI findings, and magnetic resonance (MR)-cisternography revealed dural leaks bilaterally at lumbar region. MR-cisternography is a recently performed technique and gaining importance in demonstration of CSF fistula.     

磁共振胆管成像和动态十二指肠液检查鉴别诊断婴儿肝炎与胆道闭锁

目的探讨磁共振胆管成像(MRC)和十二指肠液引流对婴儿期胆道闭锁(EHBA)与婴儿肝炎综合征(IHS)的鉴别诊断价值。方法应用磁共振和婴儿十二指肠引流管对52例婴儿期持续性阻塞性黄疸行MRC和十二指肠液检查,以胆总管、肝总管显影和十二指肠液检查有胆汁(胆汁酸阳性)并经随访黄疸消退诊断为婴儿肝炎综合征;以胆总管、肝总管未显示,十二指肠液检查无胆汁(胆汁酸阴性)且经外科手术探查诊断为胆道闭锁;并将胆道闭锁MRC及十二指肠检查结果与手术、病理结果对比分析。结果52例患儿中最后有34例确诊为婴儿肝炎综合征,18例确诊为胆道闭锁。34例婴儿肝炎综合征中30例MRC能清楚显示胆总管、肝总管结构,2例显示较小胆囊,2例胆总管、肝总管未显示。首次十二指肠液检查有胆汁31例,3例无胆汁。18例胆道闭锁中13例胆囊、胆总管和肝总管未显示,4例仅显示较小胆囊,1例仅显示较大胆囊,十二指肠液检查均无胆汁。18例确诊为胆道闭锁的患儿中,17例与MRC及十二指肠引流诊断相符。结论十二指肠引流液检查能直接观察有无胆汁,诊断胆道闭锁的敏感性为100%,特异性为91.1%;MRC能显示肝外胆道结构,诊断胆道闭锁的敏感性为94.4%,特异性88.24%;十二指肠与MRC联合诊断胆道闭锁的敏感性94.4%,特异性97.06%,因此二者结合应用能较准确鉴别诊断婴儿肝炎与胆道闭锁。     

Chemical meningitis after cervical transforaminal epidural steroid injection: a case report

We herein report a case of chemical meningitis that developed after cervical transforaminal steroid injection. A 49-year-old man presented with symptoms of meningitis (severe headache and neck stiffness) after cervical transforaminal steroid injection at the right C5–6 level. The injection solution was a mixture of lidocaine (0.3 mL), hyaluronidase (1 mL), placenta hydrolysate (2 mL), and normal saline (1 mL). The patient developed symptoms of meningitis 2.5 hours after the cervical epidural injection. Cerebrospinal fluid (CSF) analysis was performed 1 day after the injection, and the results showed an elevated white blood cell count at 7106 cells/µL. The patient’s CSF analysis findings and symptoms did not differ from those of bacterial meningitis. However, considering that his symptoms developed 2.5 hours after the epidural injection, we believe that the patient developed chemical meningitis; therefore, he was symptomatically treated with an analgesic. Three days after the cervical transforaminal epidural injection, the patient experienced complete relief from the headache and neck stiffness. A Gram stain of the CSF revealed no organisms. Hence, the diagnosis of chemical meningitis was confirmed. Clinicians should be knowledgeable about the risk of this complication.