肌萎缩侧索硬化伴认知功能障碍的研究进展

认知功能障碍在肌萎缩侧索硬化（ALS）患者中是普遍存在的，ALS伴认知功能障碍常提示预后不良。近年来对ALS患者认知功能障碍的研究取得了一定的进展，本文对ALS伴认知功能障碍的临床特点、生物标志物、遗传学特征和最新治疗进展进行综述，以期为ALS伴认知功能障碍的临床诊疗提供新思路。     

Enlarged high frequency oscillations of the median nerve somatosensory evoked potential and survival in amyotrophic lateral sclerosis

ObjectiveA large N20 and P25 of the median nerve somatosensory evoked potential (SEP) predicts short survival in amyotrophic lateral sclerosis (ALS). We investigated whether high frequency oscillations (HFOs) over N20 are enlarged and associated with survival in ALS.MethodsA total of 145 patients with ALS and 57 healthy subjects were studied. We recorded the median nerve SEP and measured the onset-to-peak amplitude of N20 (N20o-p), and peak-to-peak amplitude between N20 and P25 (N20p-P25p). We obtained early and late HFO potentials by filtering SEP between 500 and 1 kHz, and measured the peak-to-peak amplitude. We followed up patients until endpoints (death or tracheostomy) and analyzed the relationship between SEP or HFO amplitudes and survival using a Cox analysis.ResultsPatients showed larger N20o-p, N20p-P25p, and early and late HFO amplitudes than the control values. N20p-P25p was associated with survival periods (p = 0.0004), while early and late HFO amplitudes showed no significant association with survival (p = 0.4307, and p = 0.6858, respectively).ConclusionsThe HFO amplitude in ALS is increased, but does not predict survival.SignificanceThe enlarged HFOs in ALS might be a compensatory phenomenon to the hyperexcitability of the sensory cortex pyramidal neurons.     

Relationship between EMG-detected and ultrasound-detected fasciculations in amyotrophic lateral sclerosis: A prospective cohort study

ObjectivesFasciculation potentials (FP) are an important consideration in the electrophysiological diagnosis of ALS. Muscle ultrasonography (MUS) has a higher sensitivity in detecting fasciculations than electromyography (EMG), while in some cases, it is unable to detect EMG-detected fasciculations. We aimed to investigate the differences of FP between the muscles with and without MUS-detected fasciculations (MUS-fas).MethodsThirty-one consecutive patients with sporadic ALS were prospectively recruited and in those, both needle EMG and MUS were performed. Analyses of the amplitude, duration, and number of phases of EMG-detected FPs were performed for seven muscles per patient, and results were compared between the muscles with and without MUS-fas in the total cohort.ResultsThe mean amplitude and phase number of FP were significantly lower in patients with EMG-detected FP alone (0.39 ± 0.25 mV and 3.21 ± 0.88, respectively) than in those with both FP and MUS-fas (1.22 ± 0.92 mV and 3.74 ± 1.39, respectively; p < 0.0001 and p = 0.017, Welch’s t-test).ConclusionSmall FP may be undetectable with MUS. MUS cannot replace EMG in the diagnostic approach for ALS.SignificanceClinicians should use a combination of EMG and MUS for the detection and quantitative analysis of fasciculation in ALS.     

颞下颌关节强直动物模型中最大张口度与强直严重程度关系研究

目的　研究主、被动最大张口度（AMMO、PMMO）与颞下颌关节强直严重程度的关系。方法　选取28只健康雄性绵羊随机分为实验组和对照组，每组各14只。实验组绵羊双侧颞下颌关节模拟髁突矢状骨折，其中左侧翼外肌被切断以阻断其功能；对照组绵羊未进行手术。于术前及术后12、24周对所有绵羊体重、AMMO、PMMO、颞下颌关节形态学特点进行测量评估。结果　实验组只有右侧保留翼外肌功能的颞下颌关节发生了骨强直。术后12、24周，实验组绵羊AMMO、PMMO、极限距离均显著低于对照组，差异均有统计学意义（均P < 0.05）。实验组绵羊AMMO和PMMO与骨融合区宽度、长度、面积及钙化程度均呈负相关（均P < 0.05），其中骨融合区面积为主要影响因素（术后12、24周相关系数r分别为-0.94、-0.95）。结论　颞下颌关节强直动物模型中阻断翼外肌功能可阻止骨强直的发生；对于早期髁突矢状骨折，可通过牙合垫或牙合板进行张口训练，进而阻断翼外肌功能。当颞下颌关节发生骨强直时，骨融合区面积越大，张口受限越明显。     

In Vivo Kinematics of Functional Ankle Instability Patients and Lateral Ankle Sprain Copers During Stair Descent

Patients with mechanic ankle instability experience increased tibiotalar and subtalar joint laxity. However, in vivo joint kinematics in functional ankle instability (FAI) patients and lateral ankle sprain (LAS) copers, especially during dynamic activities, are poorly understood. Ten FAI patients, 10 LAS copers, and 10 healthy controls were included in this study. A dual fluoroscopic imaging system was used to analyze the tibiotalar and subtalar joint kinematics during stair descent. Five key poses of stair descent were analyzed. Kinematic data from six degrees of freedom were calculated utilizing a solid modeling software. The range of motion and joint positions in each degree of freedom were compared among the three groups. The tibiotalar joints of FAI patients and LAS copers were significantly more inverted than those of healthy controls during the foot strike (p = 0.016, = 0.264). The subtalar joints of FAI patients were significantly more anteriorly translated (pose 2, p = 0.003, = 0.352; pose 3, p < 0.001, = 0.454; pose 4, p = 0.004, = 0.334), inverted (pose 4, p = 0.027, = 0.234; pose 5,p = 0.034, = 0.221), and externally rotated (pose 4, p = 0.037, = 0.217; pose 5; p = 0.004, = 0.331) than those of healthy controls during the mid‐stance and the heel off. The FAI patients showed excessive tibiotalar inversion and subtalar joint hypermobility during stair descent. Meanwhile, the LAS copers maintained subtalar joint stability, and only showed excessive tibiotalar inversion in foot strike. These data provide insight into the mechanisms behind the development of FAI after initial LAS. © 2019 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 37:1860–1867, 2019     

针刺干预对肌萎缩侧索硬化症模型小鼠脑及脊髓中IBA-1和TNF-α表达的影响

目的:探讨电针治疗肌萎缩侧索硬化症(ALS)的机制。方法:将SOD1^G93A转基因小鼠随机分为正常组、模型组、针刺组及利鲁唑治疗组,每组各8-10只。小鼠日龄30 d后,针刺组选取双侧天枢穴(ST25)及足三里穴(ST36)予以电针治疗。利鲁唑治疗组按照30 mg/(kg·d)的剂量予以90 d日龄小鼠灌胃治疗,1次/d。模型组及对照组常规饲养,不予干预。于小鼠电针治疗前及治疗后分别对4组进行体质量记录及行为学观察。采用免疫组化法检测各组小鼠脑干及脊髓中IBA-1和TNF-α表达变化。结果:正常组小鼠精神状态及运动功能良好,针刺组及利鲁唑组小鼠较模型组精神状态及运动功能明显改善; 转棒实验结果显示针刺组及利鲁唑组小鼠潜伏期较模型组延长; 免疫组化检测结果表明在模型组脑干IBA-1阳性细胞表达高于正常组(P<0.05); 而利鲁唑组IBA-1阳性细胞表达虽有降低,但组间无统计学差异(P>0.05)。该结果与IBA-1阳性细胞在脊髓L_4-5节段表达趋于一致(P<0.05)。针刺治疗后脊髓中促炎因子TNF-α表达明显减少(P<0.05),但利鲁唑组较模型组无统计学差异(P>0.05)。结论:电针治疗及利鲁唑灌胃治疗均可以改善小鼠运动功能及生存质量,但早期针刺干预明显减少脑干及脊髓中小胶质细胞的活化,抑制促炎因子TNF-α释放,表明电针治疗ALS有效,其机制可能与针刺抑制神经炎症产生有关。     

原发癌灶定位与甲状腺微小癌颈侧区淋巴结转移关系研究

目的 探究原发灶定位与甲状腺乳头状微小癌（PTMC）颈侧区淋巴结转移的关系，评估超声检查预测颈侧区淋巴结转移的准确率。方法 回顾性分析2014年1月至2015年12月天津医科大学肿瘤医院收治的134例PTMC病人的临床资料，均行中央区淋巴结清扫+改良颈侧区淋巴结清扫。依据超声定位分组，分析癌灶位置与颈侧区淋巴结转移的关系。结果 颈侧各分区淋巴结转移发生率分别为：Ⅱ区30.6%、Ⅲ区50.7%、Ⅳ区57.5%、Ⅴ区11.3%。癌灶位于中上极者颈侧区淋巴结转移发生率高于癌灶位于下极者（89.7% vs. 75.7%，P=0.038），靠近外侧者较内侧者更易出现颈侧区淋巴结转移（93.7% vs. 81.4%，P=0.049）。超声检查判定Ⅱ、Ⅲ、Ⅳ、Ⅴ区淋巴结转移的敏感度分别为43.9%、85.3%、85.7%、14.3%；特异度为91.4%、57.6%、35.1%、99.1%。超声预测Ⅲ、Ⅳ区淋巴结转移敏感度较高，Ⅱ、Ⅴ区淋巴结转移特异度较高。结论 癌灶位置与甲状腺微小癌颈侧区淋巴结转移密切相关，超声可为临床确定颈侧区淋巴结的清扫范围提供依据。     

MRI图像融合技术在肛瘘评估中的初步应用

目的采用图像融合技术获得T2WI与T2WI-FS的融合图像，评估其在肛瘘及肛周结构显示中的优势。 方法2016年6月至2018年6月，前瞻性选择中山大学附属第一医院29例肛瘘患者进行肛管磁共振（MR）检查，采用图像融合技术获取T2WI与T2WI-FS的融合图像T2WI-Fusion，利用Fisher score算法计算瘘管及肛门括约肌的组织间分辨力Fisher值、脂肪与肛门括约肌间的Fisher值，评估融合图像中瘘管及肛周结构的显示情况。采用改进的双刺激连续质量量表（DSCQS）对T2WI-FS、T2WI、增强3D-VIBE和T2WI-Fusion序列图像进行主观图像质量评价。 结果29例患者均成功获得T2WI与T2WI-FS的融合图像T2WI-Fusion。T2WI-Fusion、T2WI瘘管与括约肌间Fisher均值分别为6.46、3.31，T2WI-Fusion图像对瘘管的显示优于T2WI序列图像（P<0.001）。T2WI-Fusion、T2WI-FS脂肪与括约肌间Fisher均值分别为10.61、2.45，T2WI-Fusion图像对括约肌的显示优于T2WI-FS序列图像（P<0.001）。T2WI-Fusion对瘘管与括约肌的图像质量评价总评分均高于T2WI-FS、T2WI、增强3D-VIBE序列（P<0.001）。 结论MRI图像融合技术同时具备T2WI及T2WI-FS的优势，无需增加扫描序列及扫描时间，且操作简单，花费时间短，显著提高病变及肛周解剖结构的对比度和图像质量。