平山病8例肌电图与影像特征分析

目的探讨平山病临床表现,肌电图及影像学特点。方法对温州医学院附属第一医院2003年6月至2009年6月间确诊的8例平山病患者的临床表现,肌电图及影像学资料进行回顾性分析。结果 8例患者双侧上肢远端肌肉均呈神经源性损害,提示受损节段多在下颈髓前角细胞。8例颈部自然位MRI发现低位颈髓萎缩,屈曲位均发现颈髓前移,硬脊膜后壁前移,硬脊膜外间隙增宽。增强扫描中均可见不同程度的硬膜后间隙内异常强化影。结论根据平山病临床电生理及影像学检查显示平山病可能为下颈段脊髓病变,神经电生理检查与屈颈位MRI在平山病的诊断中有重要作用。     

妊娠期子宫颈上皮内瘤变及子宫颈癌的诊治

关于子宫颈（宫颈）癌合并妊娠的确切定义尚未统一。国际上比较认可的是将妊娠期、分娩期和产后6个月内发现的富颈癌，称为妊娠相关性宫颈癌。国外报道妊娠相关性富颈癌发病率为0．01％～0．1％，妊娠期富颈上皮内瘤变（CIN）发病率为0．93％～5％。86％的妊娠期宫颈病变属于低度鳞状上皮内病变（LSIL），其中大部分为人乳头瘤病毒（HPV）感染，14％为高度鳞状上皮内病变（HSIL）。     

平山病MRI特征分析

比较12例无颈椎病史的正常人(对照组)和6例平山病患者(观察组),颈椎中立位及过屈位MRI资料.结果观察组患者C6水平颈髓正中前后径中立位(Dn)及过屈位(Df)分别为(0.562±0.066)cm、(0.408±0.077)cm,对照组分别为(0.650±0.043)cm、(0.565±0.057)cm,两组相比,P均<0.01.观察组患者过屈位颈髓前后径较正常人变小更为明显,且以一侧变扁为著.认为平山病患者屈颈位MRI有下颈髓及其硬膜囊的异常表现,可作为诊断平山病的重要依据.     

平山病4例临床分析

目的讨论平山病的临床特征、影像学特点及发病机制。方法对4例患者的临床、神经电生理、影像学改变进行分析。结果4例患者有3例为青春期男性，1例为青春期女性，主要表现为局限于手和前臂的肌萎缩，尺侧肌肉萎缩较重，上肢呈斜坡样。4例手指伸展时出现震颤；3例伴寒冷麻痹。常规颈MRI3例可见颈髓下段轻度萎缩，1例无明显异常，均未发现髓内异常信号。屈颈MR均可见下段颈髓前移、变平，硬脊膜向前移位；硬脊膜外间隙增宽；硬脊膜外间隙内半月形信号影，在T1加权像与脊髓的信号相同，在T2加权像呈高信号，其中有一些弧线形或圆形的血管流空信号。结论当遇到青少年（尤其是男性）出现手及前臂不对称的肌无力和萎缩而无感觉障碍时，应首先考虑有平山病的可能，可进一步行屈颈MR检查，出现下颈髓及其硬膜囊的特征性表现可确诊。     

3种尿蛋白检测方法对糖尿病性肾脏疾病诊断敏感性和特异性的比较

目的比较尿蛋白的3种评价方法对于糖尿病性肾脏疾病（DKD）临床诊断的敏感性和特异性。方法对349例2型糖尿病患者进行24小时尿总蛋白（UTP）、尿白蛋白排泄率（UAER）、尿白蛋白／肌酐比值（ACR）测定及眼底检查,行工作特征曲线（ROC）分析,并观察3种方法与视网膜病变（DR）的相关性。结果ACR的ROC曲线下面积（AUC）是0．71,大于UAER的0．61和UTP的0．61（P〈0．01）,ACR诊断DKD的敏感性是68．42％（男）、60．0％（女）,特异性是71．7％（男）、73．73％（女）,均高于UTP和UAER。ROC曲线得出的UAER最佳临界点是54．95μg／min。ACR-DR相关系数r为0．43,高于UTP—DR、UAER-DR的相关系数。结论初步研究显示ACR对于DKD诊断的准确性高于UTP和UAER,且ACR的敏感性和特异性高。     

慢性胃炎患者胃内pH值相关因素分析

背景：传统观点认为胃酸分泌随年龄增加和胃黏膜萎缩而减少,但也有研究持不同观点。目的：研究慢性胃炎患者胃内pH值与性别、年龄、糖尿病、萎缩和幽门螺杆菌（H.pylori）感染之间的相关性。方法：共纳入67例慢性胃炎患者,采用pH试纸测定胃液pH值,病理切片结合快速尿素酶试验或^13C-尿素呼气试验检测H.pylori感染情况。分析不同因素与慢性胃炎患者胃内pH值的相关性。结果：67例患者中,慢性非萎缩性胃炎患者35例（52．2％）,慢性萎缩性胃炎32例（47．8％）;H.pylori阳性21例（31．3％）,H.pylori阴性46例（68．7％）。平均胃内pH值为2．86,胃内pH值随年龄增长呈升高的趋势（P=0．15）。Logistic回归分析显示胃内pH值与性别（P=0．17）、年龄（P=0．06）、糖尿病（P=0．75）、萎缩（P=0．67）和H.pylori感染（P=0．11）均无相关性。结论：性别、年龄、糖尿病、萎缩和H.pylori感染不是影响慢性胃炎患者胃内pH值的重要因素。     

特异性小干扰RNA对晚期糖基化终产物受体介导肝星状细胞激活和胶原生成的抑制作用

目的 探讨特异性小分子干扰RNA对晚期糖基化终产物受体（RAGE）介导肝星状细胞（HSC）激活和胶原生成的影响。方法 构建RAGE特异性siRNA表达载体，经脂质体转染入HSC—T6细胞，以空白和转染非特异性siRNA表达载体pGCsi-C为对照，分别用实时荧光定量PCR和Western印迹法检测各组HSC—T6细胞RAGE、α平滑肌肌动蛋白（α—SMA）和Ⅰ型胶原基因及蛋白的表达。结果 与空白对照组和pGCsi—C组相比，转染特异性siRNA表达载体pGCsi—R1的HSC—T6细胞RAGE mRNA和相对分子质量50×10^3、46×10^3的RAGE蛋白表达分别下调79．65％、78．04％（F=26．005，P〈0．01），56．09％、54．93％（F=365．185，P〈0．01）和63．67％、59．67％（F=386．07，P〈0．01），同时α—SMAmRNA和蛋白、Ⅰ型胶原mRNA和蛋白的表达也受到明显抑制，分别为空白对照组和pGCsi—C组的57．53％、65．50％（F=20．913，P〈0．05）、58．48％、62．80％（F=56．592，P〈0．05）、71．16％、74．23％（F=18．091，P〈0．05）和71．91％、76．09％（F=17．299，P〈0．05）。结论 RAGE特异性siRNA可在细胞内稳定表达，并能有效抑制HSC的激活和Ⅰ型胶原生成。     

TB-SA抗体检测、PPD试验在结核性脑膜炎诊断中的应用价值

目的评价TB-SA抗体检测、结核菌素试验（PPD）对结核性脑膜炎（结脑）的诊断价值。方法对69例结脑患者和69例非结核病患者同时进行血清、脑脊液TB-SA抗体检测和PPD,对两种检测方法的结果进行比较。结果TB—SA抗体在结脑诊断中的敏感性为40．5％,特异性为73．9％;PPD在结脑诊断中的敏感性为45．7％,特异性为45．0％。结脑组TB—SA抗体检测和PPD的阳性率分别为37．7％和44．9％（P=0．487）;非结核组TB-SA抗体检测和PPD的阴性率分别为73．6％和45．8％（P=0．001）;结脑组脑脊液、血清TB—SA抗体检测阳性率分别为30．4％和37．7％（P=0．405）。结论TB—SA抗体、PPD不是诊断结脑有价值的参考指标,两者的诊断敏感性无统计学差异;血清TB—SA抗体特异性高于PPD,在排除结脑诊断中具有显著优势;与血清相比,脑脊液TB-SA抗体检测在诊断结脑中元显著优势。     

炎症性肠病患者外周血单个核细胞和血浆中肿瘤坏死因子受体相关因子-6的激活

目的探讨肿瘤坏死因子受体相关因子-6（tumor necrosis factor receptor—associated factor-6,TRAF-6）在炎症性肠病患者外周血单个核细胞和血浆的表达,及其与内镜下疾病活动性的相关性。方法应用酶联免疫吸附试验（ELISA）分析炎症性肠病患者和正常对照者血浆中TRAF-6蛋白的表达,SYBR—green real time PCR方法分析炎症性肠病患者和正常对照者外周血单个核细胞中TRAF-6 mRNA的表达。数据处理使用Graph Pad Prism 5。结果克罗恩病患者（P=0．000）和溃疡性结肠炎患者（P=0．000）血浆TRAF-6水平显著高于正常对照者,但是克罗恩病患者（r=-0．114,P=0．377）以及溃疡性结肠炎患者（r=0．044,P=0．727）血浆TRAF-6表达水平和内镜下疾病活动指数均无显著的相关性。克罗恩病患者（P=0．000）和溃疡性结肠炎（P=0．000）患者外周血单个核细胞TRAF-6 mRNA表达均显著高于正常对照者外周血单个核细胞中mRNA的表达。结论炎症性肠病患者血浆和外周血单个核细胞中均存在TRAF-6的激活,但血浆TRAF-6的高表达不能反映内镜下的疾病活动程度。     

涂层可降解雷帕霉素洗脱支架术后6个月双联抗血小板治疗的长期疗效与安全性: CREATE研究4年随访结果分析

目的探讨涂层可降解雷帕霉素药物洗脱支架（DES）术后应用6个月双联抗血小板治疗（DAPT）的长期疗效和安全性。方法多中心、前瞻性CREATE研究中术后6个月随访时未发生主要不良心脏事件（MACE）的存活患者共2034例,全部接受Excel涂层可降解雷帕霉素DES治疗,其中1626例（79．9％）于术后6个月内停用氯吡格雷（DAPT≤6个月）,408例（20．1％）于6个月后继续服用氯吡格雷（DAPT〉6个月）。对比两组患者在6个月至4年随访期间的MACE和支架内血栓发生率。结果DAPT〉6个月者与≤6个月者相比,心性死亡（2．0％VS2．2％,P：0．753）、心肌梗死（0．2％VSO．4％,P=1．0）和靶病变血运重建（1．7％vs2．4％,P=0．407）发生率差异均无统计学意义。两组总的MACE发生率差异亦无统计学意义（3．7％VS3．4％,P=0．819）。两组总的血栓事件发生率分别为1．5％和0．7％,差异无统计学意义（P=0．128）。两组明确的及很可能的血栓累计发生率差异亦无统计学意义（0．7％VS0．5％,P=0．469）。多因素分析结果表明,陈旧性心肌梗死是支架内血栓的独立危险因素（OR=15-313,95％CI：4．02-58．25,P〈0．001）。DAPT疗程≤6个月与MACE（OR=0．987,95％CI=0．5451．787,P=0．965）及支架血栓事件发生率（OR=0．847,95％CI=0．2083．439,P=0．816）无显著相关性。结论4年临床随访结果表明,冠心病患者置入涂层可降解DES术后使用6个月DAPT安全、有效,但还需随机、对照研究进一步证实。     

平山病的临床研究

目的 分析平山病的临床特点.方法 总结近9年29例平山病患者的临床资料.结果 29例均为男性,发病年龄12～25岁.其中22例只有单侧卜肢症状,7例有双上肢症状,右侧多见或是症状明显,仪有1例双上肢症状儿乎对称.部分肌萎缩使前臂呈现"斜坡"样的特殊形状.肌电图检查示局限于上肢相应阶段的对称件脊髓前角细胞的损害.颈段MRI检杳示为C5-7椎体水平脊髓萎缩,症状偏重侧萎缩明显.1例在C5-6节段的脊髓前角位置出现长T2信号.29例均应用颈托治疗,随访1-5年,预后良好.结论 平山病男性多见,一般20岁以前隐袭起病,多数患者是在青春期身高快速增长时期后的2年左右逐渐发现手部症状.颈段MRI示下颈段脊髓萎缩,左右不对称,其萎缩轻重与肢体症状轻重相一致.肌电图表现为双侧下颈段脊髓的前角病变.极个别患者可以出现脊髓内的异常信号,但无下肢的锥体束征.     

Scapular winging as a symptom of cervical flexion myelopathy

A 23-year-old man complained of weakness of the right arm that he first noted six years prior to his visit. Neurological examination revealed atrophy and weakness of the triceps and serratus anterior muscle on the right side, which resulted in scapular winging on that side. MRI with neck flexion revealed compression of the cervical cord enabling a diagnosis of flexion myelopathy. Proximal muscle weakness and atrophy in flexion myelopathies including Hirayama disease are extremely rare. Here, we report a case of unilateral, proximal upper limb atrophy with scapular winging, attributed to middle cervical flexion myelopathy.     

Atopy and neural damage

The occurrence of myelitis with atopic diathesis (atopic myelitis) affecting young adults has recently been noted in Japan. The disease preferentially affects the posterior column of the cervical spinal cord, as shown clinically and by MRI. It is characterized by hyperIgEaemia and the presence of mite antigen-specific IgE. The spinal cord lesions have been shown to be eosinophilic inflammation on biopsy and thus an allergic mechanism is thought to be operative in this condition. In addition, we also found that Hirayama disease, juvenile muscular atrophy of the distal upper extremity, is also associated with airway allergy such as allergic rhinitis and atopic asthma. In children, poliomyelitislike illness after acute asthma attacks is well known as Hopkins syndrome. Moreover, by the prospective study of the history of allergic disorders in common neurologic diseases, an association between spinal progressive muscular atrophy (SPMA) and asthma as well as between myelitis and atopic dermatitis has been demonstrated. These observations strongly suggest a link between atopic diathesis and spinal cord damage. Central nervous system damage associated with atopic diathesis may be classified into two types; eosinophilic myelitis preferentially affecting the cervical spinal cord and lower motor neuron damage, such as Hopkins syndrome, Hirayama disease and SPMA. The former is typically associated with atopic dermatitis while the latter, with airway allergy.     

Cervical myelopathy due to a "tight dural canal in flexion" with a posterior epidural cavity

A 41-year-old man noticed weakness and atrophy in his right hand and forearm resembling the non-progressive juvenile muscular atrophy of unilateral upper extremity (Hirayama's disease). MRI showed an abnormal cavity in the posterior epidural space which appeared on neck flexion communicating with the subarachnoid space in addition to the flattening of the lower cervical spinal cord on neck flexion. When evaluating atypical cases of Hirayama's disease, the pathomechanism demonstrated in the present case should be taken into consideration.     

Advanced Hirayama disease with successful improvement of activities of daily living by operative reconstruction

Juvenile muscular atrophy of the distal upper extremity (Hirayama disease) is a benign and non-progressive motor neuron disease. Application of a cervical collar is believed to prevent progression of symptoms in the early stages, but there is no effective therapy for the advanced disease. We found that tendon transfer improved the activities of daily living (ADL) of a patient with advanced Hirayama disease. An operative reconstruction can be valuable, even in patients with Hirayama disease who have developed impaired ADL due to extensive intrinsic hand muscle atrophy.     

Endomysium antibodies in coeliac disease: an improved method.

The ultra structural binding sites of endomysium antibodies have been studied on human umbilical cord tissue. The sensitivity and specificity of IgA endomysium antibodies were compared with recently described methods using basement membrane of smooth muscle of monkey oesophagus. Thirty adults affected by coeliac disease (10 in remission) and 75 healthy adult controls with normal intestinal mucosa (35 false antigliadin positive) were investigated. Sensitivity and correlation of endomysium antibodies with total villous atrophy in untreated coeliac disease patients were 100% on the human umbilical cord smooth muscles, and only 90% on the muscular layer of primate oesophagus. Indirect immunofluorescence was superior to peroxidase staining in detecting these IgA antibodies. The easy availability and enhanced testing sensitivity of the umbilical cord is an advance towards a better diagnostic tool for coeliac disease.     

Juvenile muscular atrophy of distal upper extremity (Hirayama disease)

This disease is characterized by initially progressive muscular weakness and wasting of the distal upper limb(s) in young people predominantly in men, followed by a spontaneous arrest within several years. This disease has been thought to be separate from motor neuron diseases, yet some authors still consider the illness a variant of motor neuron disease. However, the pathological evidence of ischemic changes in the lower cervical anterior horn should facilitate differentiation of the disorder from degenerative motor neuron disease. Recent radiological investigations proved compressive flattening of the lower cervical cord due to forward displacement of the cervical dural sac and spinal cord induced by neck flexion. These findings suggest that sustained or repeated neck flexion may cause ischemic changes in the cervical anterior horn. Application of a cervical collar to minimize neck flexion prevents progressive muscular weakness in an early stage of the disease.     

Whiplash: a review of a commonly misunderstood injury

Whiplash injury is a relatively common occurrence, but its mechanism and optimal treatment remain poorly understood. It is estimated that the incidence of whiplash injury is approximately 4 per 1,000 persons. The most common radiographic findings include either preexisting degenerative changes or a slight flattening of the normal lordotic curvature of the cervical spine. Computed tomography and magnetic resonance imaging are generally reserved for cases of neurologic deficit, suspected disc or spinal cord damage, fracture, or ligamentous damage. Biomechanics studies have determined that after rear impact C6 is rotated back into extension before movement of the upper cervical vertebrae. Thus, the lower cervical vertebrae were in extension while the upper vertebrae were in a position of relative flexion, producing an S shape in the cervical spine. It is believed that this abnormal motion pattern might play a role in the development of whiplash injuries. Historically, a soft cervical collar has been used early after the injury in an attempt to restrict cervical range of motion and limit the chances of further injury. More recent studies report rest and restriction of motion to be detrimental and to slow the healing process.     

老年性颈椎病的MRI诊断及表现

目的了解老年性颈椎病的ＭＲＩ表现在诊断方面的意义,并初步探讨一般病理过程。方法１００例老年性颈椎病均经用飞利蒲公司生产的０．５ＴＭＲＩ扫描系统,使用颈椎表面线圈行自旋回波及梯度回波序扫描。结果在１００例老年性颈椎病中,生理曲度发生改变占７８％;椎体骨质增生占椎体总数的６８％;间盘变性及间盘突出或膨出分别占间盘总数的５３％及４７％;后纵韧带骨化或肥厚占病例总数的１５％;椎体滑脱占病例总数的４％;而硬膜囊及脊髓受压占病例总数的１００％。结论ＭＲＩ能清楚显示颈椎病各部位的病变程度及病理变化。因此ＭＲＩ是诊断老年性颈椎病最理想的检查手段,对临床治疗及预后的判断具有重大意义。     

Gluteal tendinopathy in refractory greater trochanter pain syndrome: diagnostic value of two clinical tests

OBJECTIVE: To evaluate the value (sensitivity and specificity) of 2 modified physical tests for the diagnosis of gluteal tendinopathy in patients with refractory greater trochanter pain syndrome (GTPS). METHODS: The 2 tests were prospectively evaluated by a single physician in all consecutive patients with persistent (> or =4 months) GTPS and no hip joint arthropathy seen on radiography between 2002 and 2006. The 2 tests evaluated the occurrence of pain similar to spontaneous pain during a single-leg stance held for 30 seconds and resisted external derotation in a supine position (hip flexed 90 degrees ) then prone position (hip extended). A matched control population without hip pain was examined similarly. Tendinitis, tendon tear, and associated bursitis in the target group were documented by magnetic resonance imaging (MRI) in transverse, coronal, and sagittal planes, with MRI serving as the gold standard. RESULTS: Seventeen patients completed the study (mean +/- SD age 68.1 +/- 10.8 years, mean duration of symptoms 13 months). MRI revealed tendinopathy and/or bursitis of the gluteus medius and/or minimus tendons in all patients, with evidence of tearing in 15. Sensitivity and specificity were 100% and 97.3%, respectively, for the single-leg stance test and 88% and 97.3%, respectively, for the resisted external derotation test in the supine position. CONCLUSION: The 30-second single-leg stance and resisted external derotation tests had very good sensitivity and specificity for the diagnosis of tendinous lesion and bursitis in patients with MRI-documented refractory GTPS.