肝豆状核变性病患者构音障碍的临床特点

目的探讨肝豆状核变性病(WD)患者构音障碍的临床特点。方法对112例WD构音障碍患者进行改良Frenchay量表评分,对轻度、中度患者构音障碍进行单词检查,对重度、极重度患者进行构音器官检查。结果重度和极重度构音障碍患者改良Frenchay评分与最长发音时间呈正相关(r=0.154,r=0.0526;均P0.05)。极重度构音障碍患者改良Frenchay评分与构音器官功能评分呈正相关(r=0.0426,P0.05)。中度构音障碍患者的舌根音发音异常与改良Frenchay评分呈正相关(r=0.0234,P0.05);送气音发音异常与改良Frenchay评分呈正相关(r=0.0372,P0.05)。结论 WD重度、极重度构音障碍患者发音时间较短,不能形成有效言语;极重度患者构音器官功能较差;中度构音障碍患者舌根音和送气音的发音异常更多见。     

MTHFR C677T基因多态性与脑小血管病认知功能障碍的相关性研究

目的探讨MTHFR C677T基因多态性与脑小血管病(cerebral small vessel disease,CSVD)患者认知功能障碍的相关性。方法选取2017年12月-2019年3月在潍坊市人民医院神经内科就诊的门诊及住院CSVD患者,参照蒙特利尔认知评估量表(Montreal cognitive assessment,MoCA)和临床痴呆评定量表评分,将患者分为3组:认知功能正常组,轻度认识功能障碍组和痴呆组。所有患者均取晨起空腹静脉血测定Hcy水平及MTHFR C677T基因型,分析Hcy水平、MTHFR C677T基因型与CVSD患者认知功能障碍的相关性。结果共纳入130例CSVD患者,其中男性87例(66.9%)。认知功能正常组63例,轻度认识功能障碍组35例,痴呆组32例。痴呆组患者血清Hcy水平高于轻度认知功能障碍组(P0.001)和认知功能正常组(P0.001),轻度认知功能障碍组血清Hcy水平高于认知功能正常组(P0.001)。Hcy水平与CSVD患者认知功能下降程度正相关(r=0.626,P0.001)。MTHFR677C→T基因突变与CSVD患者认知功能下降程度正相关(r=0.359,P=0.001)。T等位基因频率与CSVD患者认知功能障碍弱正相关(r=0.273,P0.001)。结论 MTHFR 677 C→T基因突变与CSVD患者认知功能障碍的发生及进展正相关,可作为早期预测认知功能障碍的血清学标志物之一。     

轻度认知损害患者脑血流动力学与认知功能相关分析

目的探讨轻度认知损害(MCI)患者脑血流动力学特点及其与认知功能的相关性。方法采用简易智能状态检查量表(MMSE)和蒙特利尔认知评价量表(MoCA)评价40例轻度认知损害患者及性别和年龄相匹配的40例正常对照者的认知功能,经颅多普勒超声检测双侧大脑中动脉和基底动脉平均血流速度、搏动指数、阻力指数、收缩期峰值流速与舒张末期流速比值。结果 MCI组患者大脑中动脉搏动指数(P=0.023)和阻力指数(P=0.035)高于对照组,而平均血流速度和收缩期峰值流速与舒张末期流速比值以及基底动脉各项血流动力学指标差异均无统计学意义(P0.05)。MCI组患者大脑中动脉搏动指数异常率高于对照组[45%(18/40)对20%(8/40);χ~2=4.615,P=0.032]。Pearson相关分析显示,MCI组大脑中动脉搏动指数与MoCA评分呈负相关(r=-0.382,P=0.036),与MMSE评分无关联性(P0.05);而平均血流速度、阻力指数和收缩期峰值流速与舒张末期流速比值以及基底动脉各项血流动力学指标与MMSE和Mo CA评分均无关联性(P0.05)。根据搏动指数将轻度认知损害患者进一步分组,搏动指数异常亚组MoCA评分低于搏动指数正常亚组[(18.57±3.02)分对(23.41±2.78)分;t=3.914,P=0.015]。结论轻度认知损害患者存在脑血流动力学改变,尤以大脑中动脉搏动指数升高与认知功能障碍的关系最为密切。     

脑白质病变与轻度认知功能障碍的关系

目的 探讨脑白质病变(WML)与轻度认知功能障碍(MCI)的关系.方法 71例WML患者根据头颅MRI检查分为轻度组(27例)、中度组(21例)、重度组(23例),39例无WML的对照者为对照组.对入组者进行神经心理学量表检查;比较各组MCI的患病率,分析WML与MCI的相关性.结果 WML轻、中、重度组的MCI患病率明显高于对照组(均P＜0.01);WML中、重度组简易精神状态检查(MMSE)及蒙特利尔认知评估量表(MoCA)评分显著低于WML轻度组和对照组(均P＜0.01);随着WML程度的加重,除了抽象能力评分,MoCA其他各认知领域的评分均显著降低(均P＜0.05).多元线性相关分析显示,WML程度与MMSE、MoCA总分及除抽象思维能力的各认知域评分呈负相关(r=-0.252 ～-0.782,均P＜0.01).结论 WML可导致MCI,其对认知功能障碍的影响与WML的程度有关.     

伴自主神经功能障碍的帕金森病患者的睡眠结构及其与快速眼动睡眠障碍的相关性

目的探讨伴发自主神经功能障碍的帕金森病(PD)患者的睡眠结构及其与快速眼动睡眠障碍(RBD)的关系。方法收集48例PD患者的一般资料。根据皮肤交感反应(SSR)检查,将患者分为SSR正常组及SSR障碍组。应用统一PD评定量表第三部分(UPDRS-Ⅲ)、Hoehn-Yahr(H-Y)分级、汉密尔顿焦虑量表(HAMA_(14))、汉密尔顿抑郁量表(HAMD_(24))、匹兹堡睡眠指数量表(PSQI)、MMSE、蒙特利尔认知评估量表(MoCA)、自主神经症状量表(SCOPA-AUT)评估患者的运动、非运动症状及自主神经功能;RBD筛查量表(RBDSQ)评定患者RBD症状;多导睡眠监测(PSG)记录睡眠参数。结果与SSR正常组比较,SSR障碍组病程显著延长,H-Y分级及HAMA_(14)、HAMD_(24)、PSQI、SCOPA-AUT、RBDSQ量表评分显著升高(P0.05～0.01)。与SSR正常组相比,SSR障碍组患者睡眠效率及非快速眼动期(NREM)-Ⅱ期睡眠百分比显著下降,觉醒指数、NREM-Ⅰ期睡眠百分比、周期性腿动指数(PLMI)显著增高(P0.05～0.01)。SSR障碍组周期性腿动和RBD比率均显著高于SSR正常组(χ~2=4.463,P=0.035;χ~2=8.889,P=0.003)。RBDSQ与SCOPA-AUT评分、NREM-Ⅰ期睡眠百分比、REM期睡眠百分比、呼吸暂停-低通气指数、PLMI和觉醒指数呈正相关(均P0.01),与总睡眠时间、睡眠潜伏期、REM潜伏期、睡眠效率、NREM-Ⅱ期睡眠百分比、NREM-Ⅲ期睡眠百分比呈负相关(均P0.01),与总觉醒时间无相关性。结论伴自主神经功能障碍的PD患者病程长,病情重,容易存在焦虑、抑郁情绪,客观存在睡眠结构紊乱,RBD发生率高。PD患者RBD的严重程度与自主神经功能障碍程度密切相关。     

动脉瘤性蛛网膜下腔出血后综合康复195例临床分析

目的 探讨动脉瘤性蛛网膜下腔出血患者的综合康复治疗效果,了解不同部位动脉瘤对患者功能障碍的影响。方法 回顾性分析2011-10—2021-10中国康复研究中心收治的195例动脉瘤性蛛网膜下腔出血患者的一般资料、手术方法、量表评分、主要功能障碍。结果 经过康复治疗,患者简易精神状态检查量表评分(t=—8.239,P<0.001)、Fugl-Meyer评定量表运动评分(t=—7.360,P<0.001)和Fugl-Meyer平衡评分(t=—10.096,P<0.001)、ADL明显改善(t=—12.392,P<0.001),NIHSS评分下降(t=8.257,P<0.001)。不同位置动脉瘤手术方法选择不同,前交通动脉瘤、颈内动脉瘤行介入栓塞术较高分别达56.2%、73.3%,差异有统计学意义(χ²=4.844,P=0.028;χ²=5.229,P=0.022),大脑中动脉瘤行夹闭术组更高,达77.8%,差异有统计学意义(χ²=16.167,P<0.001)。动脉瘤的位置对于运动功能障碍单...     

2型糖尿病患者皮肤交感反应异常情况及其相关因素

目的探讨2型糖尿病患者皮肤交感反应(SSR)异常情况及其相关因素。方法对354例2型糖尿病患者及65名健康体检者进行SSR检测。根据SSR参考值将2型糖尿病患者分为SSR正常组和SSR异常组,比较两组的临床资料。采用Logistic回归分析2型糖尿病患者SSR异常的相关因素。结果 SSR正常组为238例(67.23%),SSR异常组为116例(32.77%)。SSR异常组中上肢异常率(16.95%)显著低于下肢异常率(27.68%)(P=0.001)。与SSR正常组比较,SSR异常组病程显著延长,神经传导速度(NCV)异常及糖尿病视网膜病变比率显著增高(均P0.01),其余各指标差异均无统计学意义。Logistic回归分析显示,视网膜病变(OR=1.588,95%CI:0.991~2.545,P=0.045)及NCV异常(OR=3.052,95%CI:1.847~5.042,P=0.000)是2型糖尿病患者SSR异常的独立相关因素。结论糖尿病患者SSR的异常率为32.77%;下肢异常率明显高于上肢。糖尿病病程越长,越容易发生SSR异常。糖尿病视网膜病变和NCV异常是2型糖尿病患者SSR异常相关的独立相关因素。     

原发性震颤患者的认知功能及抑郁情绪的研究

目的探讨原发性震颤(essential tremor,ET)患者的认知功能和情绪障碍及其相互关系。方法采用简易智能量表(MMSE)、汉语失语症检查法口语表达分测验及阅读分测验、Rey复杂图形测验、修订韦氏成人智力量表中的数字广度分测验、相似性分测验、符号-数字分测验、威斯康星卡片分类测验、连线测验、词语流畅性测验和画钟测验,对40例ET患者和40名年龄、文化程度、利手等相匹配的健康对照进行认知评定。同时,采用汉密尔顿抑郁量表(HAMD)测评患者的抑郁情绪,HAMD评分8分为伴有抑郁症状。结果ET组的Rey复杂图形测验(t=6.66,P0.001)、数字广度分测验(顺)(t=3.32,P0.001)、符号-数字分测验(t=4.18,P0.001)、威斯康星卡片分类测验各项(P0.05)、连线测验(t=3.59,P0.001)、词语流畅性测验(t=-3.98,P0.001)和画钟测验(t=-4.18,P0.001)等各项成绩均较正常对照组低;而在汉语失语症检查法口语表达分测验及阅读分测验、相似性测验中两组间得分差异无统计学意义(P0.05);患者组各项认知测验成绩与震颤强度和病程无关(P0.05);52.5%(21/40)ET患者合并抑郁症状,但多为轻度抑郁(71.4%);抑郁症状的发生与年龄有关(2=27.03,P0.001),多出现在50岁以下的患者(85.7%);是否伴有抑郁的ET组之间各项认知测验得分差异无统计学意义(P0.05)。结论ET患者存在多认知域的功能障碍,尤以执行功能、注意力及记忆受损较为明显;ET患者中抑郁症状的发生率较高。     

卒中后抑郁患者交感神经皮肤反应特点及临床意义

目的探讨卒中后抑郁(PSD)患者交感神经皮肤反应(SSR)的特点及临床意义。方法共采集脑卒中患者88例,根据汉密尔顿抑郁量表(HAMD)、抑郁自评量表(SDS)评分将脑卒中患者分为无抑郁组、轻中度抑郁组、重度抑郁组,选取22名健康人群作为健康对照组。各组患者分别进行SSR测定,记录首次达峰时间及SSR波幅并进行比较分析。结果本组脑卒中患者PSD发生率为67.05%,PSD组与健康对照组相比,SSR首次达峰时间延长;波幅减低(均P0.05)。PSD组与无抑郁组相比,SSR首次达峰时间延长;SSR波幅减低(P0.05)。重度抑郁组与轻中度抑郁组相比,SSR首次达峰时间延长;SSR波幅减低(均P0.05)。结论 PSD患者SSR明显异常,抑郁越重,其异常程度越明显,SSR检测可有助于PSD诊断。     

酒依赖患者自主神经及感觉功能的电生理研究

目的探讨酒依赖(AD)患者自主神经及感觉功能的电生理特征。方法对56例AD患者和30例健康对照者进行交感神经皮肤反应(SSR)体感诱发电位(SEP)及感觉神经传导速度(SCV)测定,分析SSR反应波及SEP上肢N20、下肢P40电位波幅、潜伏期和正中神经、胫神经感觉传导速度。于酒精戒断2个月时随访AD组患者的SSR。结果与正常对照组比较,AD组SSR异常率及反应波缺失率均显著升高(χ~2=7.860,P=0.005;χ~2=64.655,P=0.000)。与正常对照组比较,AD组入组时及酒精戒断后SSR波幅均显著降低,潜伏期显著延长(均P0.01)。与入组时比较,AD组酒精戒断后波幅、潜伏期差异无统计学意义(均P0.05)。AD组与正常对照组SEP上肢N20电位和下肢P40电位波幅、潜伏期及上肢正中神经、下肢胫神经SCV差异均无统计学意义(均P0.05)。AD患者酒依赖持续时间和日饮酒量分别与SSR潜伏期呈正相关(r=0.335,P=0.017;r=0.369,P=0.008),与SSR波幅呈负相关(r=-0.294,P=0.038;r=-0.310,P=0.028)。结论 AD患者存在周围神经损害,以C类交感神经节后纤维和传导痛温觉Aδ纤维功能障碍等小纤维损害为主,深感觉传导路尚无明显影响,SSR可为AD提供周围神经早期损害的客观指标。     

Stroke induces disturbances of autonomic system function

BACKGROUND AND PURPOSE: Autonomic dysfunction in patients with stroke worsens their health status and can induce life-threatening complications. The purpose of the study was to evaluate the prevalence of autonomic disturbances in post-stroke patients using selected methods and to analyse the correlation between their results. MATERIAL AND METHODS: The examined group comprised 22 patients (mean age 64.6+/-8 years) with ischaemic stroke treated at the Department of Neurology in Zabrze. The evaluation of the autonomic nervous system was conducted using a questionnaire, cardiovascular reflex tests (Ewing battery) and a neurophysiological method (sympathetic skin response, SSR). RESULTS: All the tests demonstrated coexistence of dysautonomia in most patients. Abnormal score of the questionnaire was present in 72.7% of patients with ischaemic stroke. Cardiovascular autonomic dysfunction (>or=2 abnormal cardiovascular tests) was diagnosed in 31.8% of patients. Abnormal SSR was found in 81.8% of patients. No association was found among results of the autonomic tests and age, sex, disease duration, aetiology and localization of the stroke focus. The results of the Ewing battery significantly correlated with the questionnaire (p=0.05) and SSR (p=0.03). CONCLUSIONS: The study confirmed the presence of autonomic disturbances of both the sympathetic and parasympathetic system in post-stroke patients. SSR is the most sensitive method and is useful in patients with various degrees of disability. The observed correlation between SSR and Ewing battery confirms the clinical usefulness of the neurophysiological method for the evaluation of the autonomic nervous system.     

帕金森病患者自主神经功能障碍相关研究

目的 评估交感神经皮肤反应(sympathetic skin response,SSR)和PD自主神经症状量表(the scale for outcomes in PD for autonomic symptoms,SCOPA-AUT)对帕金森病(Parkinson disease,PD)患者自主神经功能损害的诊断价值及自主神经功能障碍影响因素.方法 应用SSR、SCOPA-AUT和统一帕金森病评分量表(unified Parkinson disease rating scale,UPDRS)对50例PD患者进行评估;应用SSR及SCOPA-AUT量表对33例对照组进行评估.结果 PD组四肢SSR的潜伏期及波幅与对照组相比差异有显著统计学意义(P<0.01或0.05).SCOPA-AUT总分(23.54±12.48)、消化系统症状(8.00±3.77)及泌尿系统症状评分(6.28±3.51)均高于对照组,差异有统计学意义(P<0.01).SCOPA.AUT总分与UPDRS评分、H&Y分级、Schwab和England日常生活得分显著相关,差异有统计学意义(P<0.01或0.05),与病程、年龄无明显的相关性(P>0.05).结论 PD患者自主神经功能障碍随着病情进展而加重,PD患者SCOPA-AUT评分与上、下肢SSR波幅呈负相关,SSR和SCOPA-AUT可作为评价PD患者自主神经功能障碍的客观量化临床指标.

Abstract：

Objective To investigate the diagnostic value of sympathetic skin response(SSR)and the scale for outcomes in PD for autonomic symptoms(SCOPA-AUT)in autonomic dysfunction and influencing factors of autonomic dysfunction in patients with parkinson's disease(PD).Methods SSR,SCOPA-AUT and unified Parkinson's disease rating scale(UPDRS)were used to evaluate 50 patients with PD.The results were taken from patients with PD and compared with those of 33 control subjects.Results It was significantly different to compare the SSR latency and amplitude of PD patients limbs with those of the normal controls.PD patients had more total SCOPA-AUT score(23.54 4±12.48)and domain scores in gastrointestinal(8.00 4±3.77)and urinary symptoms(6.28±3.51)compared to control subjects(P<0.01).There were significant correlations between the total score with UPDRS.Hoehn-Yabr scale or score of Schwab and England disability scale(P<0.01 or 0.05),and no correlation with duration or age(P>0.05).Conclusions Autonomic dysfunction of PD increased significantly with disease severity.There were significantly negative correlation between the total score of SCOPA-AUT with the limb SSR amplitude(P<0.05)in PD patients.SSR and SCOPA-AUT can act as an objective index to assess autonomic dysfunctions in patients with PD quantitatively.     

重症吉兰-巴雷综合征的预测因素分析

目的分析轻症与重症吉兰-巴雷综合征(Guillain-Barrésyndrome,GBS)患者临床及神经电生理检查特点,筛选出重症患者的预测因素。方法回顾性分析2006年1月至2015年11月在本院住院治疗的GBS患者101例。根据疾病高峰期Hughes评分划分为轻症组(0~2分)和重症组(3~6分)。分别统计两组患者发病年龄、性别、前驱感染史、首发症状、是否有延髓功能障碍、是否累及颅神经和自主神经、有无轴索损害等指标,分析两组之间差异,并筛选出重症患者的预测因素。结果以非感觉异常为首发症状(P0.001)、有延髓功能障碍(P0.001)、颅神经受累(P=0.025)、自主神经系统受累(P=0.018)、运动系统受累(P=0.004)以及轴索损害(P0.001)的患者容易发展为重型患者。多因素Logistic回归分析发现轴索损害(P=0.008,OR=4.632)、延髓受累(P=0.010,OR=10.420)、颅神经受累(P=0.047,OR=0.076)是发展为重型的独立危险因素。结论轴索损害、延髓受累和颅神经受累可能是GBS患者发展至重型的有意义的预测因素。     

Autonomic dysfunction in multiple sclerosis is related to disease activity and progression of disability.

BACKGROUND: Autonomic dysfunction is frequently observed in patients with multiple sclerosis (MS) but the evolution over time and the relationship to clinical characteristics are not yet established. OBJECTIVES: We investigated the correlation of disease activity and progression of disability with composite scores of cardiovascular autonomic dysfunction and serum levels of catecholamines in a cross-sectional study of patients with clinically active and clinically stable MS. In a longitudinal study of clinically active MS patients, we performed cardiovascular reflex tests for up to 2 years. METHODS: Twenty-six patients with clinically active relapsing-remitting MS, age 33.0 +/- 7.3 years, and nine patients with clinically stable MS, age 41.3 +/- 10.9 were studied. Twenty-four healthy volunteers served as controls. Standard autonomic tests were repeated at 3, 6, 12, 18 and 24 months in 18 of the 26 active patients participating in a placebo-controlled trial with interferon-beta-1a. Parasympathetic dysfunction was assessed by heart rate response to the Valsalva manoeuvre, deep breathing and active change of posture, while sympathetic dysfunction was analysed by blood pressure response to active change of posture and to sustained handgrip, and by measuring levels of norepinephrine and epinephrine in serum obtained in the supine position. RESULTS: In the cross-sectional study, the number of patients with at least one abnormal sympathetic test was higher in the 'active' patient group (39%) than in healthy controls (8%, P< 0.02) or 'stable' patients (0%, P< 0.04), while no difference was seen in the parasympathetic score. Median catecholamine levels were significantly lower in 'active' MS patients than in those with stable disease (norepinephrine, 204 ng/l (interquartile range 158-310 ng/l) vs 363 ng/l (269-507 ng/l), P<0.02 and epinephrine, 23 ng/l (16-28 ng/l) vs 32 ng/l (24-107 ng/l), P<0.04). In the subgroup of patients studied longitudinally, parasympathetic but not sympathetic dysfunction increased slightly during the follow-up period, with a significant correlation to the increase in clinical disability (r=0.7, P<0.002). No difference was seen for any of the autonomic scores between patients treated with interferon-beta (n=12) and those receiving placebo (n=6). During acute exacerbations, only parasympathetic dysfunction tended to increase in parallel with a deterioration in the EDSS. CONCLUSIONS: Parasympathetic dysfunction was closely related to the progression of disability in patients with MS. In contrast, sympathetic dysfunction was associated to the clinical activity of MS. This is in line with previous observations suggesting that the autonomic nervous system may be intimately linked with the disordered immune regulation in MS.     

Measurement of autonomic dysregulation in multiple sclerosis

OBJECTIVE: Patients with definite multiple sclerosis (MS) were tested for autonomic nervous system (ANS) dysfunction using clinical symptomatology criteria and non-invasive laboratory testing. Exactly 45.45% of patients exhibited subjective symptoms of autonomic dysfunction and 42.42% of patients had abnormal laboratory tests results. METHODS: The sympathetic ANS tests were abnormal in 30.3% of MS patients and the parasympathetic ANS tests were abnormal in 18.18% of MS patients. The most sensitive test for the presence of autonomic dysfunction was the sympathetic skin response. CONCLUSION: Autonomic dysfunction was often subclinical and we conclude that it is preferable to combine several tests for a more thorough and accurate evaluation of the ANS impairment in MS.     

Tests of autonomic dysfunction in patients with multiple sclerosis

Autonomic dysfunction is frequent in patients with multiple sclerosis (MS). The sympathetic skin response (SSR) and the R-R interval variation (RRIV) are simple electrophysiologic tests for the assessment of central and peripheral autonomic disturbances. Both tests were performed in 60 patients with clinically definite MS and 30 controls. The SSR was recorded simultaneously from both upper and both lower limbs. In all volunteers normal responses were recorded from the four limbs, but 39 patients (65%) showed abnormal responses in at least one limb. The reduction in amplitude of the response was correlated with patients' EDSS. In individual limbs, the SSR amplitude correlated with weakness, spasticity and cerebellar dysfunction, but was not sufficiently related to the deep sensory loss. The RRIV was abnormal in 48 MS patients (80%), as compared to the controls, but showed no significant relationship either to the EDSS or to the SSR. The sensitivity of SSR and RRIV is high and comparable with that of visual and somatosensory evoked potentials.     

Autonomic dysfunction in multiple sclerosis: correlation with disease-related parameters

Cardiovascular autonomic functions were investigated in a prospective, controlled study of 22 consecutive relapsing-remitting multiple sclerosis (MS) patients and 22 healthy subjects using 5 simple noninvasive tests and sympathetic skin response testing. Tests included the heart rate response to deep breathing, valsalva maneuver and standing, blood pressure response to standing and sustained hand grip, and were graded according to the Ewing and Clark classification as early, definite or severe impairment. The relationship between autonomic dysfunction and disease-related parameters such as the expanded disability status scale (EDSS) and disease duration was studied. Ninety percent of the patients had symptoms related with autonomic dysfunction, and 45.5 % had abnormal results in cardiovascular autonomic function testing with 4 patients also having abnormal sympathetic skin responses. Statistical analysis indicated that patients with a long disease duration rather than high EDSS carried a risk of autonomic involvement in MS. Both parasympathetic and sympathetic functions were impaired and this could have been easily overlooked by a standard EDSS follow-up. In this regard, autonomic function testing seems necessary in order to detect subclinical changes in MS patients and should be considered in outcome measures.     

Sympathetic skin response (SSR) is abnormal in multiple sclerosis

As the function of the autonomic nervous system is often compromised in multiple sclerosis (MS), different standardized tests are used to detect disseminated abnormalities in cardiovascular autonomic functions. Sympathetic skin response (SSR), a slow wave generated in deep layers of the skin, is induced by reflex activation of sudomotor sympathetic efferent fibers. SSR was studied in 70 patients classified into different categories according to the diagnostic criteria for MS. We also obtained pattern reversal visually evoked potentials and brainstem auditory evoked potentials as well as somatosensory evoked potentials (by median and posterior tibial nerve stimulation). SSR was abnormal in 66 patients (94.2%), including abnormal foot latency with normal hand latency in 30 (42.8%), delayed foot and hand latencies in 30 (42.8%), and no response in 6 (8.6%). The percentages of SSR abnormalities were similar in the different patient categories. Pathological SSR were more common than abnormal evoked potentials in suspected and probable MS. SSR appears to be a simple and effective means of assessing sympathetic sudomotor outflow disturbances in MS, providing a valuable addition to current electrophysiological procedures for the detection of MS lesions.© 1995 John Wiley &Sons, Inc.     

多发性硬化患者生活质量评估及其影响因素探讨

目的评估多发性硬化患者生活质量水平,并探讨其影响因素。方法收集2012年7月~2016年12月就诊于河南省人民医院复发缓解型多发性硬化患者(relapsing-remitting MS,RRMS)36例,继发进展性多发性硬化患者(secondary progressive MS,SPMS)21例作为研究对象。采用多发性硬化生活质量54项评分(Multiple Sclerosis Quality of Life-54 instrument,MSQo L-54)测试MS患者的生活质量;对所有研究对象进行认知功能、抑郁状态、疲劳、睡眠质量及日常生活能力评估。结果 (1)RRMS组及SPMS组患者躯体生活质量(RRMS组58.62±16.32;SPMS组28.77±15.99,P=0.000)、精神生活质量(RRMS组57.33±16.72;SPMS组36.27±23.50,P=0.000)均有下降,SPMS组下降更明显。多元逐步回归法分析,与MS生活质量相关因素中,Hamilton抑郁量表评分处于第一位(β=-0.516,P<0.001),其次为反应躯体化残疾程度的EDSS评分(β=-0.372,P<0.001),第三位为疲劳评分(β=-0.250,P=0.002)。结论 MS患者有不同程度的生活质量下降,SPMS患者更明显。EDSS评分、抑郁、疲劳影响患者生活质量,早期干预抑郁及疲劳,对于改善MS患者生活质量有益。     

Autonomic instability,as measured by pupillary unrest,is not associated with multiple sclerosis fatigue severity

Multiple sclerosis (MS) fatigue is one of the most common symptoms in MS, but its pathophysiology is still not understood Sympathovagal imbalance was suggested as a reason for fatigue in chronic fatigue syndrome. We examined the role of an imbalance in the central autonomic nervous system (ANS) as a cause of MS fatigue in 51 MS patients and a control group of 22 healthy volunteers. Fatigue was assessed with the revised MS Fatigue Severity Scale (FSS) and the Modified Fatigue Impact Scale (MFIS). Depression was evaluated with the Beck Depression Inventory (BDI). Disintegration of the central ANS expressed by pupillary fatigue waves was measured with pupillography and documented in the pupillary unrest index (PUI). All subjects had less than five points on the seven-point Stanford Sleepiness Scale and were therefore not sleepy. MS patients had significant higher mean FSS scores (p=0.001) and mean MFIS scores (p=0.003) than our control group. Mean BDI scores were significant higher (p=0.001) in the MS group, but were in the lowest score range (0-10 points) in both groups. Surprisingly, we found a statistically significant inverse correlation between PUI values and either FSS scores (p=0.001; r=-0.521) or MFIS scores (p=0.002; r=-0.423) in the MS group, but not in healthy participants. We therefore conclude that autonomic instability, as measured by pupillary unrest is not associated with MS fatigue severity.