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Ultra-early amplitude decrement after repetitive nerve stimulation supports early neuromuscular junction injury in amyotrophic lateral sclerosis:a prospective cross-sectional study

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Neural Regeneration Research 2022年第3期17卷 655-660页

作者： Jing-Yue Ma Xiang-Yi Liu Shuo Zhang dong-sheng fan Department of Neurology Peking University Third HospitalBeijingChina Beijing Municipal Key Laboratory of Biomarker and Translational Research in Neurodegenerative Diseases BeijingChina

The dying-back hypothesis holds that the damage to neuromuscular junctions and distal axons in amyotrophic lateral sclerosis occurs at the earliest stage of the *** basic studies have confirmed early damage to neuromuscular junctions,but it is difficult to obtain such evidence directly in clinical *** this prospective cross-sectional study,we recruited 22 patients with early amyotrophic lateral sclerosis with disease duration < 12 months and with clinical symptoms limited to the upper *** also recruited 32 healthy *** nerve stimulation was performed,and patients were followed for 12 *** found a significant change in the response to repetitive nerve stimulation in amyotrophic lateral sclerosis patients without spontaneous electromyographic *** that were prone to denervation had an increased decrement response of target muscles after repetitive nerve *** results suggest that changes in response to repetitive nerve stimulation may occur before denervation in amyotrophic lateral sclerosis *** damage to lower motor neurons is more obvious in patients with a higher percentage of repetitive never stimulation-related amplitude *** study was approved by the Institutional Ethics Committee of Peking University Third Hospital(approval No.M2017198) on August 24,2017.

关键词： amplitude decremental response amyotrophic lateral sclerosis dying-back hypothesis motor neuron disease nerve electrophysiology physiopathology prognosis repetitive nerve stimulation

Better survival in female SOD1-mutant patients with ALS:a study of SOD1-related natural history

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Translational Neurodegeneration 2019年第1期8卷 14-23页

作者： Lu Tang Yan Ma Xiao-lu Liu Lu Chen dong-sheng fan Department of Neurology Peking University Third Hospital49 North Garden RoadHaidian DistrictBeijing 100191People’s Republic of China

Background:SOD1 mutations are the most common cause of amyotrophic lateral sclerosis(ALS)in non-Caucasian *** natural history profiles of SOD1-mutant patients will be beneficial for the strategy and interpretation of future SOD1-targeted clinical ***:Mutational distribution,age at onset(AAO),site of onset,diagnostic delay,disease progression(rate of ALSFRS-R decrease,ΔFS)and survival were *** comparisons between heredity of disease,gender,and mutations were ***:Sixty-six cases with 43 SOD1 mutations were included and analysed,with ***47Arg as the leading mutation and seven novel variants *** mean(SD)AAO was 43.92 years(9.24)for all subjects,with a significant difference between patients carrying mutations in exon 2(n=24,46.83,8.31)and exon 4(n=18,37.75,7.67)(p=0.002).The median(IQR)diagnostic delay from symptom onset was 14.50(6.00–36.50)months for all SOD1-mutant patients,9.50(4.75–24.25)months for males and 24.00(9.50–47.50)months for females,revealing a gender difference(p=0.009).Similar advantages in median(IQR)ΔFS[male:female,0.55(0.24–0.94)vs 0.19(0.06–0.90),p=0.041]and mean(95%CI)survival[57.4(38.90–75.90)months vs 125.6(99.80–151.50)months,p=0.006]were also observed in females,both of which existed in sporadic ALS only when stratified by familiar or sporadic ***:The results highlight a distinct mutational distribution and natural history spectrum in ALS patients carrying SOD1 mutations in China.A prominent mild disease progression was observed in female patients,which had rarely been reported in the previous *** finding,together with the detailed analysis of natural history among each mutation,can have important implications for future genetic counselling and SOD1-targeted clinical trials.

关键词： Amyotrophic lateral sclerosis Natural history studies SOD1 mutations Gender difference

Promising application of a new ulnar nerve compound muscle action potential measurement montage in amyotrophic lateral sclerosis:a prospective cross-sectional study

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Neural Regeneration Research 2023年第4期18卷 908-912页

作者： Yi-Xuan Zhang Jing-Yue Ma Xiang-Yi Liu Shuo Zhang Zhou Yu dong-sheng fan Department of Neurology Peking University Third HospitalBeijingChina Beijing Municipal Key Laboratory of Biomarker and Translational Research in Neurodegenerative Diseases BeijingChina Key Laboratory for Neuroscience National Health Commission/Ministry of EducationPeking UniversityBeijingChina

Previous studies have shown that ulnar nerve compound muscle action potential recorded by the conventional“belly-tendon”montage does not accurately and completely reflect the action potential of the ulnar nerve dominating the abductor digiti minimi muscle due to the effects of far-field potentials of intrinsic hand muscles.A new method of ulnar nerve compound muscle action potential measurement was developed in 2020,which adjusts the E2 electrode from the distal tendon of the abductor digitorum to the middle of the back of the proximal *** new method may reduce the influence of the reference electrode and better reflect the actual ulnar nerve compound muscle action *** this prospective cross-sectional study,we included 64 patients with amyotrophic lateral sclerosis and 64 age-and sex-matched controls who underwent conventional and novel ulnar nerve compound muscle action potential measurement between April 2020 and May 2021 in Peking University Third *** compound muscle action potential waveforms recorded by the new montage were unimodal and more uniform than those recorded by traditional *** the controls,no significant difference in the compound muscle action potential waveforms was found between the traditional montage and new montage *** amyotrophic lateral sclerosis patients presenting with abductor digiti minimi spontaneous activity and muscular atrophy,the amplitude of compound muscle action potential-pE2 was significantly lower than that of compound muscle action potential-dE2(P<0.01).Using the new method,damaged axons were more likely to exhibit more severe amplitude decreases than those measured with the traditional method,in particular for patients in early stage amyotrophic lateral *** addition,the decline in compound muscle action potential amplitude measured by the new method was correlated with a decrease in Revised Amyotrophic Lateral Sclerosis Functional Rating Scale *** findings suggest tha

关键词： amyotrophic lateral sclerosis axonal degeneration biomarker compound muscle action potential distal E2 electrode early diagnosis far field potential nerve electrophysiology prognosis evaluation proximal E2 electrode ulnar motor nerve conduction

Risk Factors, Regional Disparity and Trends of Ischemic Stroke Etiologic Subtypes

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Chinese Medical Journal 2018年第2期131卷 127-129页

作者： Dan-Yang Tian, dong-sheng fan Department of Neurology Peking University ThLrd Hospital Beijing 100191 China

Stroke remains the leading cause of morbidity and mortality in the world. Unlike coronary heart disease, stroke can occur due to different etiologies, and the pathogenesis.

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Elimination Rate of Serum Lactate is Correlated with Amyotrophic Lateral Sclerosis Progression

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Chinese Medical Journal 2016年第1期129卷 28-32页

作者： Yuan-Jin Zhang dong-sheng fan Department of Neurology Peking University Third Hospital Beijing 100191 China

Background： Mitochondrial dysfunction plays an important role in the pathogenesis of amyotrophic lateral sclerosis （ALS）. We aimed to demonstrate mitochondrial dysfunction in ALS using a lactate stress test and to examine the relationship between mitochondrial dysfunction with motor deterioration. Methods： We enrolled 116 patients and observed clinical variables, including the survival state. Results： Patients with a rapid slope of revised ALS functional rating scales （ALSFRS-r）（〉20 U/year） exhibited the slowest elimination rate （median -4.67 × 10^-3 mmol·L ^-1min ^-1, coefficient of variation, 590.15%）, the shortest duration （0.63 ± 0.28 years） and the worst ALSFRS-r （32.59±4.93）. Patients with a moderate slope ofALSFRS-r （1~20 U/year） showed a moderate elimination rate （median -11.33 × 10^-3 mmol·L ^-1min ^-1, coefficient of variation, 309.89%）, duration （1.16± 0.45 years）, and ALSFRS-r （34.83 ± 6.11）. The slower progressing （〈10 U/year group） patients exhibited a rapid elimination rate （median： - 12.00 × 10^-3 mmol·L ^-1min ^-1, coefficient of variation： 143.08%）, longer duration （median： 3 years, coefficient of variation： 193.33%）, and adequate ALSFRS-r values （39.58 ± 9.44）. Advanced-phase ALS patients also showed slower elimination rate （ER, quartiles - 17.33, -5.67, 4.00） and worse ALSFRS-r （34.88 ± 9.27）, while early-phase patients showed a more rapid ER （quartiles -25.17, -11.33, -3.50） and better ALSFRS-r （39.28 ± 7.59）. These differences were statistically significant. Multiple linear regression analysis revealed strong direct associations among ER, ALSFRS-r slope （standard beta = 0.33, P = 0.007）, and forced vital capacity （predict %）（standard beta = -0.458, P = 0.006, adjusted for ALSFRS-r, course and onset region）. However, the data obtained from 3 years of follow-up showed no statistically significant difference in the survival rates between the most rapid and slowest ER groups. Conclusion： There is a potential linear relationship between ER and mot

关键词： Advanced Phase Amyotrophic Lateral Sclerosis Early Phase Elimination Rate of Serum Lactate

Diets for Patients with Amyotrophic Lateral Sclerosis： Pay Attention to Nutritional Intervention

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Chinese Medical Journal 2017年第15期130卷 1765-1767页

作者： Li-Peng Yang dong-sheng fan Department of Neurology Peking University Third Hospital Beijing 100191 China Department of Neurology Beijing Tsinghua Changgung Hospital Beijing 102218 China Department of Neurology Peking University Third Hospital Beijing 100191 China

INTRODUCTIONAmyotrophic lateral sclerosis （ALS） is characterized by the progressive degeneration of upper and lower motor neurons. Typical clinical features orALS are limb paralysis, muscle atrophy, dysphagia, dysar... 详细信息

关键词： Amyotrophic Lateral Sclerosis Diets Nutrition Therapy

Comparison of surgical intervention with functional treatment for acute ruptures of lateral ankle ligmant:a meta-analysis

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Asian Pacific Journal of Tropical Medicine 2012年第5期5卷 396-401页

作者： Zeng-Tao Hao Yu-Xia Ma Ting Hao Wei Feng Ji-Hong Wang dong-sheng fan Shu-Zheng Wen The Second Affiliated Hospital Inner Mongolia Medical College Women and Children Hospita of Inner Mongolia

Objective:To compare the effect of surgical intervention on functional ***: By searching the MEDLINE(1966 to October 2011),EMBASE(1980 to October 2011),the Chinese Biomedical Database Databases(1980 to October 2011),a total of 9 related RCT studies comparing surgical intervention with functional treatment were included in our *** software was taken to analyze the ***:These 9 studies Involved a total of 1 268 mostly young adults,including 580 patients with surgical treatment and 688 patients with functional *** results showed the stability of ankle activity in surgical treatment group was better than that in functional treatment group,with the OR and 95%CI of 0.72(0.52-0.99).No significant difference was found in the recurrence of the surgical and functional ***, the movement disorder in the surgical treatment suggested increased risk than that in functional group,with the OR and 95%CI of 2 39(0.98-5.85).Surgical group found more complication than the function group,such as deep vein thrombosis,deep venous thrombosis,tenderness of scar and sensory ***:In conclusion,our finding showed that surgical treatment could gain better efficacy than functional treatment,but may bring more ***,further large sample size RCT is warranted.

关键词： Acute ruptures Lateral ankle ligmant Surgical treatment Functional treatment

Clinical characteristics of patients with motor neuron disease and concurrent tumors

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Chinese Medical Journal 2021年第6期134卷 736-738页

作者： Yuan Li Lu Chen Nan Zhang dong-sheng fan Department of Neurology Peking University Third HospitalBeijing 100191China Beijing Municipal Key Laboratory of Biomarker and Translational Research in Neurodegenerative Diseases Beijing 100871China Key Laboratory for Neuroscience National Health Commission/Ministry of EducationPeking UniversityBeijing 100871China

To the Editor:Motor neuron disease(MND)is a kind of progressive,fatal neurodegenerative disease involving the upper and lower motor neurons and the pyramidal ***,the etiology of MND remains unknown,and there are limited treatment *** syndrome(PNS)is a rare form of MND-mimic *** have been reports of MND-like clinical symptoms in patients with different types of cancer,including breast cancer,lung cancer,and *** studies have drawn different conclusions,some have suggested that there is no relation between site-specific cancers and the risk of incident amyotrophic lateral sclerosis(ALS),while others have found that the overall risk of cancer of any site is significantly reduced in cases with ALS.[1]However,the prevalence of tumors in Chinese MND patients and the characteristics of patients with MND concomitant tumors are unclear.

关键词： patients tumors lung

Association Between Rectus Abdominis Denervation and Ventilation Dysfunction in Patients with Amyotrophic Lateral Sclerosis

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Chinese Medical Journal 2016年第17期129卷 2063-2066页

作者： Hua-Gang Zhang Shuo Zhang Ying-sheng Xu Nan Zhang dong-sheng fan Department of Neurology Peking University Third Hospital Beijing 100191 China

Background： Spontaneous potentials in electromyography （EMG） ofparaspinal muscles are associated with diaphragm denervation and, therefore, poor respiratory timction in amyotrophic lateral sclerosis （ALS） is understandable. EMG changes in the rectus abdominis （RA） display an effect similar to those in paraspinal muscles with respect to the function of lower motor neurons in the thoracic spinal cord. The RA denervation was examined to determine its association with ventilation dysfunction in ALS. Methods： We collected the clinical data of 128 patients with sporadic ALS in Department of Neurology of Peking University Third Hospital from 2009 to 2013. EMG, Revised ALS Functional Rating Scale （ALSFRS-R） and forced vital capacity （FVC） were perIbrmed in all patients and the differences in the EMG changes in RA between those with and without FVC 〉 80% were analysed. Results： The mean FVC value was 83.4% ~ 17.1% （range： 45%-I 31%） of the predicted value. A total of 79 patients displayed FVC _〉80%, and 49 patients displayed FVC 〈80%, Compared with the patients displaying a normal FVC （60/79, 75.9%）, spontaneous activity in RA was significantly different among those patients displaying an FVC 〈80% （47/49, 95.9%）. In addition, spontaneous potentials in RA were more frequently detected in patients exhibiting dyspnea （32/33, 97.0%） than in patients without dyspnea （75/95, 78.9%）. Conclusion： Spontaneous potentials in RA are associated with ventilation dysfunction and dyspnea in ALS patients.

关键词： Denervation Dyspnea Forced Vital Capacity Rectus Abdominis Ventilation Dysfunction