US neurology最新文献_第8页

New Data Reveal More Americans Than Ever Have Epilepsy 新的数据显示，越来越多的美国人患有癫痫

US neurology Pub Date : 2018-01-01 DOI: 10.17925/USN.2018.14.1.23

M. Privitera

引用次数: 0

Effect of Positive Airway Pressure Therapy on Seizure Control in Patients with Epilepsy and Obstructive Sleep Apnea 气道正压治疗对癫痫合并阻塞性睡眠呼吸暂停患者癫痫发作控制的影响

US neurology Pub Date : 2018-01-01 DOI: 10.17925/USN.2018.14.1.25

Thapanee Somboon

引用次数: 2

High-definition Fiber Tractography as Surgical Adjunct for Management of Hyper-eloquent Brain Lesions 高清晰度纤维束造影在高口才脑病变治疗中的辅助作用

US neurology Pub Date : 2018-01-01 DOI: 10.17925/USN.2018.14.2.78

R. Friedlander

引用次数: 0

The Challenge of Distinguishing POEMS from Chronic Inflammatory Demyelinating Polyneuropathy—Importance of Early Recognition and Diagnosis of POEMS 诗歌与慢性炎症性脱髓鞘性多发性神经病鉴别的挑战——早期识别和诊断诗歌的重要性

US neurology Pub Date : 2018-01-01 DOI: 10.17925/USN.2018.14.2.94

F. Chow, L. Darki, S. Beydoun

引用次数: 2

The Challenges of Diagnosis in Alzheimer’s Disease 阿尔茨海默病诊断的挑战

US neurology Pub Date : 2018-01-01 DOI: 10.17925/USN.2018.14.1.15

N. Bogdanovic

引用次数: 6

Homocysteine Lowering with B Vitamins for Stroke Prevention—A History 降低同型半胱氨酸与B族维生素预防中风的历史

US neurology Pub Date : 2018-01-01 DOI: 10.17925/USN.2018.14.1.35

J. Spence

{"title":"Homocysteine Lowering with B Vitamins for Stroke Prevention—A History","authors":"J. Spence","doi":"10.17925/USN.2018.14.1.35","DOIUrl":"https://doi.org/10.17925/USN.2018.14.1.35","url":null,"abstract":"Early trials of B vitamin therapy to lower plasma total homocysteine (tHcy) reported no reduction of stroke with high doses of folate/B6 and cyanocobalamin 400–1,000 μg daily. In patients with diabetic nephropathy, folate/B6 and cyanocobalamin 1,000 μg daily accelerated the decline of renal function and doubled cardiovascular events. Patients with renal failure have high cyanide levels. The French SUpplementation with FOlate, vitamin B6 and B12 and/or OMega-3 fatty acids (Su.Fol.OM3) trial—with the best renal function of the early trials and the lowest dose of cyanocobalamin (20 μg daily)—reported a 43% reduction of stroke. Then the China Stroke Primary Prevention Trial (CSPPT) reported that folic acid alone reduced stroke and was beneficial even in patients with impaired renal function. Patient-level data from the Vitamin Intervention to Prevent Stroke (VISP) and VITAmins TO Prevent Stroke (VITATOPS) trials and meta-analyses stratified by renal function and dose of cyanocobalamin confirmed that harm from cyanocobalamin among participants with renal impairment obscured the benefit of B vitamins in the early trials. It does seem that B vitamins reduce the risk of stroke. In the era of folate fortification, B12 is the main nutritional determinant of tHcy, and metabolic B12 deficiency is very common and usually missed. Therefore, folate alone is not the optimal way to lower tHcy: the use of folate (and possibly B6) with methylcobalamin or oxocobalamin should be considered.","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"14 1","pages":"35"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67612160","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 6

Edaravone in Amyotrophic Lateral Sclerosis’Lessons from the Clinical Development Program and the Importance of a Strategic Clinical Trial Design 依达拉奉治疗肌萎缩性侧索硬化症的临床开发项目和战略性临床试验设计的重要性

US neurology Pub Date : 2018-01-01 DOI: 10.17925/USN.2018.14.1.47

S. Beydoun, J. Rosenfeld

{"title":"Edaravone in Amyotrophic Lateral Sclerosis’Lessons from the Clinical Development Program and the Importance of a Strategic Clinical Trial Design","authors":"S. Beydoun, J. Rosenfeld","doi":"10.17925/USN.2018.14.1.47","DOIUrl":"https://doi.org/10.17925/USN.2018.14.1.47","url":null,"abstract":"Edaravone significantly slows progression of amyotrophic lateral sclerosis (ALS), and is the first therapy to receive approval by the Food and Drug Administration (FDA) for the disease in 22 years. Approval of edaravone has marked a new chapter in pharmaceutical development since the key trial included a novel strategic clinical design involving cohort enrichment. In addition, approval was based on clinical trials that had a relatively small patient number and were performed outside of the US. Edaravone was developed through a series of clinical trials in Japan where it was determined that a well-defined subgroup of patients was required to reveal a treatment effect within the study period. Amyotrophic lateral sclerosis is associated with wide-ranging disease heterogeneity (both within the spectrum of ALS phenotypes as well as in the rate of progression). The patient cohort enrichment strategy aimed to address this heterogeneity and should now be considered as a viable, and perhaps preferred, trial design for future studies. Future research incorporating relevant biomarkers may help to better elucidate edaravone’s mechanism of action, pharmacodynamics, and subsequently ALS phenotypes that may preferentially benefit from treatment. In this review, we discuss the edaravone clinical development program, outline the strategic clinical trial design, and highlight important lessons for future trials.","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"14 1","pages":"47"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67612178","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 6

Sporadic Hemiplegic Migraine with SCN1A Gene Mutation—A Case Report 散发偏瘫偏头痛伴SCN1A基因突变1例报告

US neurology Pub Date : 2018-01-01 DOI: 10.17925/USN.2018.14.2.108

Mukesh Dube, A. Lakhotia, Vaibhav Yadav, R. Jain

引用次数: 1

Updated American Academy Guidelines on the Use of Disease-modifying Therapies in Multiple Sclerosis—What’s New? 美国学会关于多发性硬化症疾病改善治疗的最新指南——有什么新进展?

US neurology Pub Date : 2018-01-01 DOI: 10.17925/USN.2018.14.2.76

A. Rae-Grant

引用次数: 0

Late-onset Hereditary Transthyretin Amyloidosis in Two Patients with Acquired Demyelinating Features 2例获得性脱髓鞘特征的迟发性遗传性甲状腺转蛋白淀粉样变患者

US neurology Pub Date : 2018-01-01 DOI: 10.17925/USN.2018.14.2.98

N. Rad, S. Beydoun

{"title":"Late-onset Hereditary Transthyretin Amyloidosis in Two Patients with Acquired Demyelinating Features","authors":"N. Rad, S. Beydoun","doi":"10.17925/USN.2018.14.2.98","DOIUrl":"https://doi.org/10.17925/USN.2018.14.2.98","url":null,"abstract":"Autosomal-dominant transthyretin (TTR)-related amyloidosis usually manifests in the third to fifth decade with a length-dependent axonal neuropathy and prominent involvement of the small diameter nerve fibers. Objectives: To describe the clinical and para-clinical findings in patients with hereditary transthyretin amyloidosis (hATTR), formerly known as transthyretin-related familial amyloid polyneuropathy (TTR-FAP). Methods: Electrodiagnostic, cerebrospinal fluid (CSF), and TTR gene findings in two patients misdiagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP). Results: A 78-year-old, right-handed, Caucasian male (patient 1) and a 69-year-old, right-handed, Japanese male (patient 2) were referred for late-onset sensory symptoms of the hands and feet as initial manifestations. The first patient, after several years, developed progressive leg weakness affecting his gait and balance, as well as dysautonomic complaints. The second patient had relatively rapid progression with bilateral foot drop and ambulation difficulty after a few months. In both patients, CSF findings were unremarkable. Lumbar spine magnetic resonance imaging did not reveal abnormal thickening or enhancement of the lumbar plexus and exiting nerve roots. Both patients were initially diagnosed with CIDP before being referred to our institution. Patient 2 was started on intravenous immunoglobulin by his primary neurologist, which was maintained for a year without a meaningful response. Repeat electrodiagnostic study at our institution revealed non-length-dependent axonal sensory loss and features of acquired demyelinating neuropathy. TTR gene testing identified pathogenic variants p.Val30Met or V30M, and p.Ala 117Ser or A117S, in the first and the second patient, respectively. Conclusion: hATTR can mimic CIDP clinically and electrodiagnostically. The presence of significant sensory axonal loss, rapid course, and lack of response to immunomodulation therapy should prompt consideration of this diagnosis and TTR gene testing.","PeriodicalId":90076,"journal":{"name":"US neurology","volume":"14 1","pages":"98"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67612495","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0