重症肌无力患者认知功能特征

目的探讨重症肌无力(myasthenia gravis,MG)患者的认知功能特征。方法纳入83例MG患者和39例健康对照者,应用简易智能状态检查量表(mini-mental status examination,MMSE)、加利福尼亚语言学习测验(California verbal learning test,CVLT)、简易视觉空间记忆测验(brief visuospatial memory test-revised,BVMT-R)、符号数字模式测验(symbol digit modalities test,SDMT)、Benton线方向判断测验(benton judgment of line orientation test,BJLOT)、定步调听觉连续加法测验(paced auditory serial addition test,PASAT)、言语流畅性测验(verbal fluency test,VFT)进行认知功能评估,应用贝克抑郁测验(Beck depression inventory,BDI)评估抑郁状态,对两组资料进行对比分析,并进一步评估疾病分型、合并症情况、病程、疾病严重程度、药物治疗情况等临床特征对MG组认知损害的影响。结果MG组MMSE[28(26,29)分,29(28,30)分]、CVLT、BVMT-R、SDMT[(37.06±12.18)分,(47.54±14.91)分]、PASAT[(32.86±10.23)分,(37.00±8.82)分]评分低于对照组,差异具有统计学意义(P<0.05),两组间BJLOT、VFT、BDI评分差异无统计学意义(均P>0.05)。Spearman相关分析显示重症肌无力患者疾病严重程度定量评分(quantitative MG score,QMG)与SDMT得分存在负相关(r=-0.234,P<0.05);MG日常生活质量量表(MG-activities of daily living profile,MG-ADL)评分与BVMT-R 3试总和得分存在负相关(r=-0.283,P<0.05)。结论重症肌无力存在认知功能损害,主要表现在记忆力、注意力、信息处理速度、视觉记忆,提示重症肌无力可能存在中枢神经系统受累。部分认知领域的损害可能与疾病严重程度相关。临床医生在工作中应加强对MG患者认知障碍的认识,早期评估,密切随访,适当干预。     

Pilates for rehabilitation in patients with multiple sclerosis: A systematic review of effects on cognition,health-related physical fitness,general symptoms and quality of life

ObjectiveThe aims of this systematic review is to analyze the effectiveness of Pilates intervention programs on cognitive function, health-related physical fitness, general symptoms, physical function, quality of life, and the impact Pilates can have on activities of daily living in Multiple Sclerosis (MS) patients. This review also aims to provide a synthesis of the most commonly used protocols regarding exercise parameters, such as periodicity and treatment duration.Data sourcesSystematic review of the literature was carried out following the PRISMA guidelines (PROSPERO no. CRD42017070004). A literature search was undertaken for studies that investigated the effects of Pilates training on MS patients, using databases included PubMed, Medline, Scopus and the Physiotherapy Evidence Database (PEDro) up to May 2018.Study selectionThree researchers independently reviewed the titles and abstract of each article to screen the papers in relation to the inclusion criteria.Data extractionData were extracted by three researchers independently. The eligible articles were read in full and their levels of evidence were evaluated using the PEDro scale.Data synthesisForty-two papers were found during the research phase. Duplicated (n = 23) or incomplete articles (n = 1) were excluded. Studies were also eliminated from the sample based on methodological approach (study design) quality assessment (n = 6). Twelve studies were ultimately selected and analyzed.ConclusionsThe majority of the studies analyzed showed positive results after Pilates training intervention and concluded that Pilates intervention is safe and effective for the treatment of dysfunction of balance, strength, quality of life, cognition, physical performance, walking and posture parameters on MS patients. The articles investigated in this review provide a scientific basis to support Pilates as an option for rehabilitation programs for MS patients.     

中药治疗卒中后认知障碍疗效和安全性的系统评价

目的:评价中药治疗卒中后认知障碍(PSCI)的疗效与安全性。方法:计算机检索中国知网数据库,万方数据库,维普数据库,中国生物医学文献数据库,PubMed,The Cochrane library和ClinicalTrials.gov共7个数据库,纳入中药治疗PSCI的随机对照试验(RCT)。使用Cochrane偏倚风险评估工具对纳入研究的方法学质量进行评价,并对纳入研究进行描述性分析,应用RevMan 5.3软件进行Meta定量分析。结果:共纳入16项RCT,1296例受试者,干预组649例,对照组647例。研究结果显示中药联合西药组和中药组干预在改善PSCI患者蒙特利尔认知评估量表,简易精神状态检查量表,Barthel指数评定量表,日常生活能力量表,中国脑卒中临床神经功能缺损程度评分量表和美国国立卫生研究院卒中量表的评分上均优于西药组,且未见严重的不良反应。结论:中药在改善PSCI患者的认知功能水平方面具有潜在优势,对改善日常生活能力及神经功能缺损症状也有一定疗效,并未观察到严重不良事件。本文纳入研究的方法学质量不高,建议后续开展高质量的RCT来研究中药治疗PSCI的疗效和安全性,为该疾病的临床诊疗提供决策依据。     

Speech-in-noise perception ability can be related to auditory efferent pathway function: a comparative study in reading impaired and normal reading children

IntroductionDeficient auditory processing can cause problems with speech perception and affect the development and evolution of reading skills. The efferent auditory pathway has an important role in normal auditory system functions like speech-in-noise perception, but there is still no general agreement on this.ObjectiveTo study the performance of the efferent auditory system in a group of children with reading impairment in comparison with normal reading and evaluation of its relationship with speech-in-noise perception.MethodsA total of 53 children between the ages of 8–12 years were selected for the study of which 27 were with reading impairment and 26 were normal reading children. Transient evoked otoacoustic emissions suppression and auditory recognition of words-in-noise test were performed for all the children.ResultsThe average amplitude of transient evoked otoacoustic emissions suppression showed a significant difference between the two groups in the right (p = 0.004) and in the left ear (p = 0.028). Assessment of the relationship between transient evoked otoacoustic emissions suppression and monaural auditory recognition of words-in-noise scores showed a significant moderate negative relationship only in the right ear (p = 0.034, r = −0.41) of the normal reading children. Binaural auditory recognition of words-in-noise scores were significantly correlated with the amplitude of transient evoked otoacoustic emissions suppression in the right ear (p < 0.001, r = −0.75) and in the left ear (p < 0.001, r = −0.64) of normal reading children. In the reading impaired group, ?a weaker correlation was observed between binaural auditory recognition of words-in-noise scores and transient evoked otoacoustic emissions suppression in the right (p = 0.003, r = −0.55) and in the left ear (p = 0.012, r = −0.47).ConclusionsTransient evoked otoacoustic emissions suppression pattern in the reading impaired group was different compared with normal reading children, and this difference could be related to efferent system performance. Words-in-noise scores in children with impaired reading were lower than in normal reading children. In addition, a relationship was found between transient evoked otoacoustic emissions suppression and words-in-noise scores in both normal and impaired reading children.     

Pretarsal blepharospasm: Clinical and electromyographic characteristics

ObjectiveTo describe the clinical and electromyographic characteristics of blepharospasm caused by selective involvement of the pars pretarsalis of the orbicularis oculi muscle.MethodsClinical assessment and simultaneous electromyographic recordings from levator palpebrae superioris and pars orbitaria and pretarsalis of orbicularis oculi muscles were performed in patients with blepharospasm and primary failure to botulinum toxin injections. Patients with selective abnormal electromyographic activity of the pars pretarsalis of the orbicularis oculi muscle were identified and treated with selective pretarsal injections of botulinum toxin.ResultsWe found 24 patients with pretarsal blepharospasm confirmed by the electromyographic assessment. All of them were functionally blind. Three clinical-electromyographic patterns were identified: (a) Impairment of eyelid opening; (b) Increased blinking; (c) Spasms of eye closure combined with varying degrees of excessive blinking and impairment of eye-opening. Pretarsal injections of botulinum toxin induced a significant improvement in all patients and 50 % regained normal or near-normal vision. The clinical improvement was sustained after repeated pretarsal injections.ConclusionsPretarsal blepharospasm can be suspected on clinical grounds and it can be confirmed by electromyographic recordings.SignificanceRecognition of this type of blepharospasm is important because of its excellent response to botulinum toxin injections applied into the pretarsal part of the orbicularis oculi muscle.     

强化认知训练在改善2型糖尿病患者认知功能障碍和日常生活活动能力中的作用

目的 探讨强化认知训练在改善2型糖尿病(T2DM)患者认知功能障碍和日常生活活动能力中的作用。方法选取2017年1月～2018年12月该院内科就诊T2DM患者92例。采用抛银币法将其分为强化组与常规组,各46例。两组均在口服降糖药或皮下注射胰岛素等控制血糖基础治疗上予以口服尼莫地平片30 mg/次,3次/d。常规组予以常规干预措施,强化组在常规组基础上加用强化认知训练。两组均干预12周。评估两组干预前与干预12周后认知功能及日常生活活动指标的变化,并比较临床效果。结果 干预12周后,两组HDS评分、MOCA评分和WAISE评分较前均显著上升,FAQ评分和ADL评分较前显著下降(P0.05或P0.01),且强化组上升或下降幅度较常规组更明显(P0.05);同时强化组患者临床总有效率(95.65%)较常规组(82.61%)的效果更佳(χ2=4.04,P0.05)。结论 强化认知训练用于T2DM患者效果较肯定,不仅能显著改善患者的认知功能,而且有利于提高患者日常生活活动能力。     

右美托咪定对老年机械通气患者睡眠与认知的影响

目的 探讨老年机械通气患者使用右美托咪定对睡眠和认知功能的影响。方法 自2018年1月～2019年12月,将我院ICU老年呼吸机患者随机分为右美托咪定组和咪达唑仑组,每组30例。用脑电双频指数监测镇静深度,以多导睡眠检测仪记录睡眠脑电图。记录两组呼吸机使用期间不良反应的发生次数。通过ICU模糊评估方法(CAM-ICU)评估两组患者在7 d内出现谵妄的病例数。使用PSQI和MMSE量表追踪患者3个月的睡眠质量和认知功能状态。结果 右美托咪定组的睡眠效率显著更高(P0.05)。7 d内谵妄发生率右美托咪定组较咪达唑仑组患者显著减少(P0.05)。两组患者在ICU期间发生呼吸抑制不良事件发生率有显著差异(P0.05),但意外脱管和心血管事件的不良事件发生率无显著差异(P0.05)。两组在3个月时的MMSE评分有显着差异(P0.05),但PSQI检查无统计学差异(P0.05)。结论 对于有短期机械通气的老年患者,右美托咪定的镇静作用是理想的。     

Cognitive and behavioral profile of Perry syndrome in two families

ObjectivePerry syndrome (PS) is a rare neurodegenerative disorder with autosomal dominant inheritance caused by point mutations in DCTN1 and characterized by parkinsonism, hypoventilation, weight loss, and psychiatric symptoms. Even though behavioral manifestation is a main feature of PS, detailed neuropsychological assessment was not performed in this cohort. In this study, the neuropsychological profile of individuals from one Polish and one Colombian family are presented.MethodsDetailed clinical and neuropsychological data were obtained from Polish and Colombian families. Clinical and neuropsychological examinations on the proband from the Polish family were performed 6 times over 11 years. Each of 3 individuals from the Colombian family received a clinical and neuropsychological assessment.ResultsThe neurologic examination showed severe parkinsonism, levodopa-induced motor fluctuations, and dyskinesias in all cases. Respiratory insufficiency was observed in 2 patients and weight loss in 1 individual. Neuropsychological assessment revealed predominant deterioration of working memory and learning capacity in the Polish patient. He also demonstrated compulsive behaviors, such as excessive shopping and eating, but only in the “on” phase. In the Colombian family, attentional deficits were present in 2 out of 3 cases. Out of 4 reported cases apathy and depressed mood were present in 2 individuals. Two cases demonstrated impulsivity and one had episodes of hypomania.ConclusionsBoth of these families revealed relatively similar neurologic and neuropsychological profiles. The Polish patient's behavioral and neuropsychological profile was mostly compatible with a behavioral variant of frontotemporal dementia. Of note, not only depression and apathy, but also impulsivity can occur in PS.     

Have outcomes following extremely preterm birth improved over time?

Increased survival of infants born preterm, especially those born extremely preterm (<28 weeks’ gestation), has meant that more are reaching later childhood and adulthood. As preterm birth is associated with a higher risk of neurodevelopmental deficits, the aim of this review was to determine whether or not the advances in perinatal care that led to improved survival have also had a positive impact on long-term neurodevelopment. Studies examining temporal changes in neurodevelopment are limited, and only from high-income countries. However, based on available published data, there is no definite trend of improved neurodevelopment at school age for neurosensory, cognitive, academic achievement, motor or executive function with time. Cerebral palsy rates, however, may be decreasing. More research is needed into the potential contributors for the trends observed, and also for other outcomes such as mental health and behavior.