{"title":"What advances in upstream medical therapies inform neurorestoratology","authors":"John R. Bach","doi":"10.1016/j.jnrt.2023.100049","DOIUrl":"10.1016/j.jnrt.2023.100049","url":null,"abstract":"<div><p>Recent advances in upstream medical therapies for neuromuscular disorders suggest that the best outcomes result from their administration in the pre-symptomatic, and perhaps, neonatal period. Currently available therapies, and many other extremely expensive therapies in the pipeline soon to be considered by the Food and Drug Administration, and suggest the importance of avoiding potential life-threatening disease complications for patients to continue to benefit from these. There is evidence that this is almost always possible with the use of respiratory muscle aids to avoid pneumonias and respiratory failure, but these are currently little understood and rarely offered by the medical community. However, restoring neuromuscular function necessitates keeping the patient alive and well.</p></div>","PeriodicalId":44709,"journal":{"name":"Journal of Neurorestoratology","volume":"11 2","pages":"Article 100049"},"PeriodicalIF":3.3,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45771161","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lu Diao , Xiaoyan Liu , Mingze Chai , Jianjun Li , Yu Wang , Wenyong Gao , Haisheng Liu , Mi Liu , Lin Chen
{"title":"Clinical trial of intrathecal injection of protein polymers for apoplexy: A protocol","authors":"Lu Diao , Xiaoyan Liu , Mingze Chai , Jianjun Li , Yu Wang , Wenyong Gao , Haisheng Liu , Mi Liu , Lin Chen","doi":"10.1016/j.jnrt.2023.100052","DOIUrl":"10.1016/j.jnrt.2023.100052","url":null,"abstract":"<div><p>Protein polymers derived from mesenchymal stem cells, especially human umbilical cord mesenchymal stem cells, showed promising potentials in treating ischemic stroke, thanks to the advantages of selective assembly, targeted delivery, efficient repair of damaged tissues, high safety, highly stable chemical properties, and being easy for storage. Herein, we introduced a clinical trial based on protein polymers derived from human umbilical cord mesenchymal stem cells. The main purpose of this clinical study is to preliminarily verify the safety and efficacy of intrathecal administration and application of protein polymers in clinical treatment of ischemic stroke cases. The summary of the program, the research plan, test protein polymer intrathecal injection method, experimental protein polymer management, patient evaluation index, data management and statistical analysis, technical features, trial schedule etc. are detailed described in the article. This clinical study provides information for other clinical trials based on protein polymers and medicines used to treat apoplexy.</p></div>","PeriodicalId":44709,"journal":{"name":"Journal of Neurorestoratology","volume":"11 2","pages":"Article 100052"},"PeriodicalIF":3.3,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44235480","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Experience of diagnosis and managements for patients with primary progressive freezing of gait","authors":"Li-Li Zhang , Ya-Jie Zhao , Liang Zhang , Xiao-Ping Wang","doi":"10.1016/j.jnrt.2022.100039","DOIUrl":"10.1016/j.jnrt.2022.100039","url":null,"abstract":"<div><h3>Objective</h3><p>The goal of this research was to evaluate the clinical characteristics of patients with primary progressive freezing of gait (PPFOG).</p></div><div><h3>Patients and methods</h3><p>This retrospective study enrolled 8 PPFOG patients and 10 age-matched Parkinson's disease with freezing of gait (PDFOG) patients. All patients underwent structured forms to document clinical manifestations and neuropsychological evaluations.</p></div><div><h3>Result</h3><p>PPFOG patients demonstrated later onset age than PDFOG patients (70.00 ± 9.97 years versus 53.30 ± 5.40 years, respectively; <em>P</em> < 0.05). Besides FOG (100%), the most prevalent concomitant symptoms of PPFOG patients were falling (<em>n</em> = 6, 75%), and fatigue (<em>n</em> = 5, 62.5%). More PPFOG patients had cerebrovascular risk factors and mild brain magnetic resonance imaging (MRI) abnormalities, which were constant with the manifestations of “cerebral small vessel disease” (<em>P</em> = 0.0001).</p></div><div><h3>Conclusion</h3><p>According to this study, the majority of PPFOG patients were linked with cerebrovascular disease risk factors and mild brain MRI abnormalities. More research on larger populations is needed to better understand the potential mechanisms underlying the link between PPFOG and cerebral small vessel disease.</p></div>","PeriodicalId":44709,"journal":{"name":"Journal of Neurorestoratology","volume":"11 1","pages":"Article 100039"},"PeriodicalIF":3.3,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48591532","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jianhua Zhao , Qiong Li , Xiantao Ouyang , Fan Wang , Qing Li , Zhixiu Xu , Danxia Ji , Qingwu Wu , Jian Zhang , Chengbiao Lu , Sibei Ji , Shaomin Li
{"title":"The effect of CX3CL1/ CX3CR1 signal axis on microglia in central nervous system diseases","authors":"Jianhua Zhao , Qiong Li , Xiantao Ouyang , Fan Wang , Qing Li , Zhixiu Xu , Danxia Ji , Qingwu Wu , Jian Zhang , Chengbiao Lu , Sibei Ji , Shaomin Li","doi":"10.1016/j.jnrt.2023.100042","DOIUrl":"10.1016/j.jnrt.2023.100042","url":null,"abstract":"<div><p>CX3C chemokine ligand 1 (CX3CL1), a unique member of the CX3C family, is most widely expressed in endothelial cells and neurons. Its specific receptor is CX3CR1, which is mainly expressed in central nervous system (CNS) microglia. CX3CL1 has effects on the brain and neurological system. CX3CL1 and its specific receptor CX3CR1 can inhibit calcium influx in neurons, promote the activation of protein kinases and the activation of nuclear transcription factor кB, etc., thereby reducing the release of inflammatory factors and achieving small stable glue in the state of plasmocytes and inhibiting the inflammatory response in CNS. This phenomenon limits neuronal death to a certain extent and has a particular neuroprotective effect. Currently, the CX3CL1/CX3CR1 signal axis impact is of particular interest in a variety of CNS disorders, such as Alzheimer's disease, stroke, cerebral small vessel disease, Parkinson's disease, epilepsy, and other diseases. A wealth of studies has reported CX3CL1/CX3CR1 signal axis as a functional regulator of microglia, thereby affecting the occurrence and development of CNS diseases. The current article briefly enlightens the role of the CX3CL1/CX3CR1 signal axis and microglia and its effect on CNS diseases.</p></div>","PeriodicalId":44709,"journal":{"name":"Journal of Neurorestoratology","volume":"11 1","pages":"Article 100042"},"PeriodicalIF":3.3,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42290785","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Mechanical insufflation–exsufflation to facilitate ventilator weaning and possible decannulation for patients with encephalopathic conditions","authors":"John R. Bach , Daniel Wang","doi":"10.1016/j.jnrt.2022.100031","DOIUrl":"https://doi.org/10.1016/j.jnrt.2022.100031","url":null,"abstract":"<div><h3>Background</h3><p>Clinicians are often faced with apparently tracheostomy mechanical ventilation dependent (TMV), patients who are ventilator unweanable due to encephalopathic/upper motor neuron conditions, that hamper return to the community.</p></div><div><h3>Objectives</h3><p>A protocol is suggested to wean these patients from mechanical ventilation and criteria offered for possible decannulation.</p></div><div><h3>Methods</h3><p>After excluding patients with severe muscle weakness due to neuromuscular diseases and high level spinal cord disorders, consecutive, apparently unweanable and unresponsive encephalopathic patients were to be weaned by a protocol that first normalized CO<sub>2</sub> levels at full ventilatory support settings. Then, supplemental oxygen was discontinued so that ambient air baseline oxyhemoglobin saturation (O<sub>2</sub> Sat) could be determined and subsequently be normalized by using mechanical insufflation<strong>–</strong>exsufflation (MIE), at 60–70 cmH<sub>2</sub>O pressures, via the tubes every 2 hours, with the tube cuffs inflated. Once ambient air O<sub>2</sub> Sat levels remained normal, ambient air spontaneous, unassisted autonomous breathing “sprints” were initiated and continued until O<sub>2</sub> Sat decreased below 95% with respiratory distress. Patients spontaneously moving over 300 mL of air into their lungs without pressure support had very good prognoses for rapid ventilator weaning. Patients were not returned to TMV irrespective of tachypnea. After the “sprint”, they were rested by using 1–3 hours of full ventilatory support before the next sprint. Sprints lengthened until being fully weaned. After weaning, potential decannulation was evaluated using MIE expiratory flows (MIE-EF) and O<sub>2</sub> Sat.</p></div><div><h3>Results</h3><p>O<sub>2</sub> Sat normalized from the initial use of MIE via the tube for 7 of 13 patients then 4 weaned from the initial ambient air sprint. Weaning occurred in 4 days or less for 11 of 13. Despite continuous TMV dependence for 31 days to 15 months before the intervention, 7 weaned patients were successfully decannulated, 6 of the 7 with MIE-EF ≥ 190 L/m. All but 2 remained unresponsive, but 6 were discharged home to their families once decannulated.</p></div><div><h3>Conclusion</h3><p>Once primary ventilatory pump failure is excluded and lung disease improved to the extent that the ambient air O<sub>2</sub> Sat can be normalized by using MIE via invasive airway tubes to clear airway secretions, encephalopathic patients can have sufficient muscle strength to wean from ventilatory support and possibly be decannulated if mechanically augmented cough flows exceed 190 L/m.</p></div>","PeriodicalId":44709,"journal":{"name":"Journal of Neurorestoratology","volume":"11 1","pages":"Article 100031"},"PeriodicalIF":3.3,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49888993","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Keqin Liu , Yi Huang , Yufei Zhu , Yan Zhao , Xiangyin Kong
{"title":"The role of maternal immune activation in immunological and neurological pathogenesis of autism","authors":"Keqin Liu , Yi Huang , Yufei Zhu , Yan Zhao , Xiangyin Kong","doi":"10.1016/j.jnrt.2022.100030","DOIUrl":"https://doi.org/10.1016/j.jnrt.2022.100030","url":null,"abstract":"<div><p>The growing body of evidence associates infections during pregnancy and other inflammatory insults with developing neurodevelopmental and neurodevelopmental disorders in children, especially autism spectrum disorder. We review the specific roles that maternal immune activation (MIA) plays in the pathogenesis of autism, the critical cytokines involved, epigenetic factors, maternal antibodies and gender bias, and how MIA affects fetal neurodevelopment in the immune and neurological pathways. A comprehensive understanding of the profound impact of MIA on fetal neurodevelopment is vital for developing diagnostic criteria and medical measures for high-risk maternity and the development of treatments for multiple offspring neurodevelopmental disorders caused by maternal inflammation.</p></div>","PeriodicalId":44709,"journal":{"name":"Journal of Neurorestoratology","volume":"11 1","pages":"Article 100030"},"PeriodicalIF":3.3,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49906192","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Zhi-Xiang Xu , Yi-Yan Zhou , Rong Wu , Ya-Jie Zhao , Xiao-Ping Wang
{"title":"Brain iron deposition and whole-exome sequencing of non-Wilson's disease hypoceruloplasminemia in a family","authors":"Zhi-Xiang Xu , Yi-Yan Zhou , Rong Wu , Ya-Jie Zhao , Xiao-Ping Wang","doi":"10.1016/j.jnrt.2022.100027","DOIUrl":"https://doi.org/10.1016/j.jnrt.2022.100027","url":null,"abstract":"<div><h3>Objective</h3><p>Ceruloplasmin (CP), a key human ferroxidase, can maintain the iron balance in the brain, and the familial hypoceruloplasminemia might be rare. Ceruloplasmin reduction is one of the most common features in Wilson's disease. Some patients with hypoceruloplasminemia do not fulfill the criteria for the diagnosis of Wilson's disease or other known diseases. Moreover, these patients always suffer from various degrees of abnormal brain iron deposition. We sought to investigate the genetic basis of non-Wilson's disease hypoceruloplasminemia using whole-exome sequencing.</p></div><div><h3>Methods</h3><p>We recruited four patients with non-Wilson's disease hypoceruloplasminemia, who visited the Department of Neurology, Shanghai First People's Hospital, China from December 2010 to February 2011. Three of them were from the same pedigree, and the other patient shared no blood relation with the others. Iron deposition in the brain was assessed using quantitative susceptibility mapping and peripheral blood DNA was extracted. The part of the exons' genome were sequenced using the NimbleGen Sequence Capture Array and high-throughput sequencing technologies. Intersection analysis at the single nucleotide polymorphism (SNP) loci was carried out. The selected loci were verified with Sanger sequencing.</p></div><div><h3>Results</h3><p>Magnetic resonance imaging revealed that the magnetic susceptibility in the globus pallidus, which showed with a high signal intensity, was apparently higher in three patients than that in the normal controls, indicating the presence of abnormal iron deposition in the brain. The whole-exome sequencing and Sanger sequencing primarily excluded the possibility of mutations in the <em>CP</em>, <em>ATP7B</em>, and <em>ATP7A</em> genes that may impact ceruloplasmin levels. The four patients presented the homozygous mutation c.3611A>C in the <em>C12orf51</em> gene; this was suspected to be a causative mutation after excluding SNP loci that were the same as those in the common population, as per The 1000 Genomes Project.</p></div><div><h3>Conclusion</h3><p>Non-Wilson's disease hypoceruloplasminemia showed varying degrees of abnormal iron deposition in the brain, and was possibly associated with the c.3611A>C mutation in <em>C12orf51</em>. How copper and iron regulate the levels of one another in the body is still not fully understood. The reduction of ceruloplasmin levels and brain iron deposition probably participated in the occurrence and development of neurodegenerative diseases. Following the combination of pre-existing techniques with a new generation of gene sequencing, the early diagnosis, prevention, and treatment of non-Wilson's disease hypoceruloplasminemia and other neurodegenerative diseases can be achieved.</p></div>","PeriodicalId":44709,"journal":{"name":"Journal of Neurorestoratology","volume":"11 1","pages":"Article 100027"},"PeriodicalIF":3.3,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49888992","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Zhi-Quan Yang , Meng-Fan Wei , Lin Chen , Jia-Ning Xi
{"title":"Research progress in the application of motor-cognitive dual-task training in rehabilitation of walking function in stroke patients","authors":"Zhi-Quan Yang , Meng-Fan Wei , Lin Chen , Jia-Ning Xi","doi":"10.1016/j.jnrt.2022.100028","DOIUrl":"10.1016/j.jnrt.2022.100028","url":null,"abstract":"<div><p>Restoration of walking ability in stroke patients is the basis for their return to their families and society. Motor-cognitive dual-task training (MCDTT) can effectively strengthen the functional network connection between motor and cognitive brain regions and promote brain functional network remodeling, which is more relevant to daily life than single-task training in improving walking dysfunction of stroke patients and can more effectively train patients' adaptive walking ability. This paper summarizes the application and research progress of MCDTT in post-stroke walking rehabilitation, and aims to provide a theoretical basis for further research on systematic motor-cognitive therapy techniques to promote walking rehabilitation in stroke patients.</p></div>","PeriodicalId":44709,"journal":{"name":"Journal of Neurorestoratology","volume":"11 1","pages":"Article 100028"},"PeriodicalIF":3.3,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49443717","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yutong Zhuang , Guangxiao Ni , Long Xu , Xueling Chen , Xiaoli Geng , Jianghong He
{"title":"Spinal cord stimulation improves motor function in disorders of consciousness: A case report","authors":"Yutong Zhuang , Guangxiao Ni , Long Xu , Xueling Chen , Xiaoli Geng , Jianghong He","doi":"10.1016/j.jnrt.2023.100041","DOIUrl":"10.1016/j.jnrt.2023.100041","url":null,"abstract":"<div><p>A 38-year-old male patient with disorders of consciousness (DOC) successfully recovered consciousness and get obvious motor function improvements through three stages of cervical spinal cord stimulation (SCS) treatment (6 Hz, 40 Hz, and combined frequency stimulation). We used the Coma Recovery Scale-Revised (CRS-R), Fugl–Meyer Assessment Scale, activities of daily living, and Berg Balance Scale to detect changes in consciousness and motor function during the 7-month postoperative follow-up. Electroencephalography-based individual alpha frequency (IAF) and relative power spectral density (RP) were used to assess changes in cognitive function and brain network function before and after the combined stimulation. The results revealed a gradually increased patient's CRS-R score after stimulation at 6 Hz, and the patient recovered consciousness 12 weeks postoperatively. The patient's motor function score gradually increased after adjusting the stimulation frequency to 40 Hz. However, the patient regressed in motor function when the stimulation frequency was decreased to 2 Hz. The patient's fine motor control of both hands significantly improved, accompanied by an increased RP in the beta band but the IAF decreased, after combined stimulation (40 Hz and 6 Hz). Then, motor function continued to improve with an increased IAF and RP in the alpha band after switching the stimulation order to 6 Hz and 40 Hz. Our results demonstrated that different frequency stimulations of cervical SCS may improve consciousness and motor function in patients with DOC.</p></div>","PeriodicalId":44709,"journal":{"name":"Journal of Neurorestoratology","volume":"11 1","pages":"Article 100041"},"PeriodicalIF":3.3,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41833300","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Songyang Zheng , Fangling Sun , Xin Tian , Zixin Zhu , Yufeng Wang , Wenrong Zheng , Tingting Liu , Wen Wang
{"title":"Roles of Eph/ephrin signaling pathway in repair and regeneration for ischemic cerebrovascular and cardiovascular diseases","authors":"Songyang Zheng , Fangling Sun , Xin Tian , Zixin Zhu , Yufeng Wang , Wenrong Zheng , Tingting Liu , Wen Wang","doi":"10.1016/j.jnrt.2022.100040","DOIUrl":"10.1016/j.jnrt.2022.100040","url":null,"abstract":"<div><p>Ischemic cerebrovascular and cardiovascular diseases in brain and heart tissues caused by hyperlipidemia, blood viscosity, atherosclerosis, hypertension, etc. Have been increasing in morbidity and mortality in the middle-aged and elderly population over several years. In recent years, the Eph/ephrin signaling pathway has been found to have a major role in the processes of neurogenesis, synaptic plasticity, and angiogenesis, which is likely a key pathway in the modulation of neurological and cardiac functions. Therefore, an in-depth understanding of the role of the Eph/Ephrin signaling pathway in pathophysiological processes will be the emphasis of future research. In ischemic cardiovascular and cerebrovascular diseases, in particular, the development of effective treatment to modulate the Eph/Ephrin signaling pathway to promote neural and vascular repair and regeneration is of great clinical significance and social value. In this study, the role of the Eph/ephrin signaling pathway in ischemic cerebrovascular and cardiovascular diseases is reviewed.</p></div>","PeriodicalId":44709,"journal":{"name":"Journal of Neurorestoratology","volume":"11 1","pages":"Article 100040"},"PeriodicalIF":3.3,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47831982","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}