肌萎缩侧索硬化和额颞叶变性是一项令人兴奋的新举措。它代表了《肌萎缩侧索硬化》杂志的及时扩展，以应对 ALS 和额颞叶痴呆之间的临床、影像学病理和遗传重叠。扩展后的期刊对与运动神经元疾病相关的广泛问题的研究提供了出色的报道，特别是 ALS（LOU GEHRIG 病）和与额颞叶退化相关的认知衰退。该杂志还涵盖了与这些核心疾病相关的神经轴疾病。

The journal "Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration" is an exciting new initiative that represents a timely expansion of the journal "Amyotrophic Lateral Sclerosis" to address the clinical, imaging pathological, and genetic overlaps between ALS and frontotemporal dementia. The expanded journal provides excellent coverage of a wide range of issues related to motor neuron diseases, with a particular focus on ALS (Lou Gehrig's disease) and cognitive decline associated with frontotemporal degeneration. Additionally, the journal covers research related to neuroaxial diseases associated with these core conditions.

《Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration》是一个令人兴奋的新期刊，旨在扩展《肌萎缩侧索硬化》杂志，以应对肌萎缩侧索硬化（ALS）和额颞叶痴呆之间的临床、影像学病理和遗传重叠。该期刊提供了对与运动神经元疾病相关的广泛问题的深入研究，特别关注ALS（又称卢·盖里希病）及其与额颞叶退化相关的认知衰退。此外，期刊还涵盖了与这些核心疾病相关的神经轴疾病的研究。

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Our objective was to explore the value of additional MR contrasts in elucidating the decrease in fractional anisotropy (FA) as has been observed in the corticospinal tracts (CST) of patients with amyotrophic lateral sclerosis (ALS). Eleven patients and nine healthy control subjects were scanned at 3T and 7T MRI. Whole brain and tract specific comparison was performed of both diffusion weighted (3T), quantitative T1 (qT1), magnetization transfer ratio (MTR) and amide proton transfer weighted (APTw) imaging (7T). Results of whole brain comparison using histogram analyses showed no significant differences between patients and controls. Measures along the CST showed a significantly reduced FA together with a significantly increased diffusivity perpendicular to the tract direction in patients compared to controls. In addition, patients showed a small but significant increase in MTR values within the right CST. No significant changes were observed in qT1 and APTw values. In conclusion, our findings, based on a multimodal approach, revealed that the decrease in FA is most probably caused by an increased diffusivity perpendicular to the CST. This diffusivity profile, together with the increase in MTR is inconsistent with demyelination but consistent with an increase of free liquid spins in the white matter tissue.

Esther Verstraete

Daniel L. Polders

René C. W. Mandl

Martijn P. Van Den Heuvel

Jan H. Veldink

Peter Luijten

Leonard H. Van Den Berg

Johannes Hoogduin

Multimodal tract-based analysis in ALS patients at 7T: A specific white matter profile?

7T磁共振成像在ALS患者中的多模态纤维束分析：是否存在特异性白质特征？

ALSUntangled reviews alternate and off-label treatments prompted by patient interest. Here, we review psilocybin, a chemical derived from mushrooms and belonging in the category of drugs known as psychedelics. Psilocybin has plausible mechanisms for slowing ALS progression because of its ability to cross the blood brain barrier and effect neurogenesis and inflammation. Currently, there are no pre-clinical ALS models, case reports, or trials for psilocybin and ALS in the context of disease modifying therapy. Depending on dosing, there can be a high risk of psychological side effects including hallucinations and physical harm. Based on the above information, we do not currently support the use of psilocybin as a means to slow ALS progression.

Bhavya Bakshi

Sandeep Yerraguntla

Carmel Armon

Paul Barkhaus

Tulio Bertorini

Robert Bowser

Sarah Breevoort

Mark Bromberg

Andrew Brown

Gregory T. Carter

Vincent Chang

Jesse Crayle

Timothy Fullam

Maxwell Greene

Terry Heiman-Patterson

Carlayne Jackson

Sartaj Jhooty

Elise Mallon

Javier Mascias Cadavid

Christopher J. Mcdermott

Gary Pattee

Kaitlyn Pierce

Dylan Ratner

Yuyao Sun

Olivia Wang

Paul Wicks

Martina Wiedau

Richard Bedlack

ALSUntangled #77: Psilocybin

ALSUntangled 第77期：裸盖菇素

Frontotemporal dementia (FTD) is a highly heritable group of neurodegenerative disorders, characterized by varying clinical and pathological features.

Giuseppe Piga

Laura Fadda

Giuseppe Borghero

Alessandra Maccabeo

Francesca Pala

Maria Rita Murru

Sabrina Giglio

Monica Puligheddu

Gianluca Floris

Semantic behavioral variant frontotemporal dementia and semantic dementia associated with <i>TARDBP</i> mutations

与<i>TARDBP</i>突变相关的语义性行为变异型额颞叶痴呆和语义性痴呆

Objective To observe longitudinal correlations between limb anthropometry against weight, BMI and functional decline in patients with amyotrophic lateral sclerosis.

Sarah Roscoe

Scott P. Allen

Christopher McDermott

Theocharis Stavroulakis

Exploring the role of anthropometric measurements to assess nutritional status in amyotrophic lateral sclerosis: a longitudinal prospective cohort study

探索人体测量指标在评估肌萎缩侧索硬化症营养状况中的作用：一项纵向前瞻性队列研究

Objective: This study aimed to evaluate the safety and tolerability of a fixed-dose co-formulation of ciprofloxacin and celecoxib (PrimeC) in patients with amyotrophic lateral sclerosis (ALS), and to examine its effects on disease progression and ALS-related biomarkers. Methods: In this proof of concept, open-label, phase IIa study of PrimeC in 15 patients with ALS, participants were administered PrimeC thrice daily for 12 months. The primary endpoints were safety and tolerability. Exploratory endpoints included disease progression outcomes such as forced vital capacity, revised ALS functional rating scale, and effect on algorithm-predicted survival. In addition, indications of a biological effect were assessed by selected biomarker analyses, including TDP-43 and LC3 levels in neuron-derived exosomes (NDEs), and serum neurofilaments. Results: Four participants experienced adverse events (AEs) related to the study drug. None of these AEs were unexpected, and most were mild or moderate (69%). Additionally, no serious AEs were related to the study drug. One participant tested positive for COVID-19 and recovered without complications, and no other abnormal laboratory investigations were found. Participants' survival compared to their predictions showed no safety concerns. Biomarker analyses demonstrated significant changes associated with PrimeC in neural-derived exosomal TDP-43 levels and levels of LC3, a key autophagy marker. Interpretation: This study supports the safety and tolerability of PrimeC in ALS. Biomarker analyses suggest early evidence of a biological effect. A placebo-controlled trial is required to disentangle the biomarker results from natural progression and to evaluate the efficacy of PrimeC for the treatment of ALS. Summary for social media if publishedTwitter handles: @NeurosenseT, @ShiranZimri•What is the current knowledge on the topic? ALS is a severe neurodegenerative disease, causing death within 2–5 years from diagnosis. To date there is no effective treatment to halt or significantly delay disease progression.•What question did this study address? This study assessed the safety, tolerability and exploratory efficacy of PrimeC, a fixed dose co-formulation of ciprofloxacin and celecoxib in the ALS population.•What does this study add to our knowledge? This study supports the safety and tolerability of PrimeC in ALS, and exploratory biomarker analyses suggest early insight for disease related-alteration.•How might this potentially impact the practice of neurology? These results set the stage for a larger, placebo-controlled study to examine the efficacy of PrimeC, with the potential to become a new drug candidate for ALS.

Shiran Salomon-Zimri

Avital Pushett

Niva Russek-Blum

Ruben P. A. Van Eijk

Nurit Birman

Beatrice Abramovich

Erez Eitan

Katya Elgrart

Danielle Beaulieu

David L. Ennist

James D. Berry

Sabrina Paganoni

Jeremy M. Shefner

Vivian E. Drory

Combination of ciprofloxacin/celecoxib as a novel therapeutic strategy for ALS

环丙沙星/塞来昔布联合治疗作为ALS新型治疗策略

To evaluate neurologists and other clinicians' contributions to U.S. ALS diagnostic timelines.

Kuldip D. Dave

Björn Oskarsson

Jill Yersak

Ramona Krauss

Catherine Lomen-Hoerth

Wendy K. D. Selig

Ilisa Halpern Paul

Melody Schaeffer

Brittany Garcia-Trujillo

Daniel Waldo

Neil Thakur

Suma Babu

Contributions of neurologists to diagnostic timelines of ALS and thinkALS as an early referral instrument for clinicians

神经科医生对ALS诊断时间线的影响及thinkALS作为临床医生早期转诊工具的作用

Alisdair Mcneill

Andrew G L Douglas

Rhona Macleod

Nayana Lahiri

Amyotroph Lateral Scler Frontotemporal Degener

Perils of predictive testing for unaffected people from motor neuron disease families with no identified causal gene

未识别致病基因的运动神经元疾病家族中未患病个体的预测性检测风险

Abbreviation	Amyotroph. Lateral. Scler. Frontotemporal. Degener.
Journal Impact	2.57
Quartiles(Global)	CLINICAL NEUROLOGY(Q2)

ISSN	2167-8421, 2167-9223
h-index	85

Publication Information	Publisher: Informa Healthcare，Publishing cycle: ，Journal Type: journal，Open Access Journals: No
Basic data	Year of publication: 2013，Proportion of original research papers: 85.71%，Self Citation Rate:12.00%， Gold OA Rate: 41.47%

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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Multimodal tract-based analysis in ALS patients at 7T: A specific white matter profile?

ALSUntangled #77: Psilocybin

Semantic behavioral variant frontotemporal dementia and semantic dementia associated with TARDBP mutations

Exploring the role of anthropometric measurements to assess nutritional status in amyotrophic lateral sclerosis: a longitudinal prospective cohort study

Combination of ciprofloxacin/celecoxib as a novel therapeutic strategy for ALS

ALSUntangled #77: Psilocybin

Exploring the role of anthropometric measurements to assess nutritional status in amyotrophic lateral sclerosis: a longitudinal prospective cohort study

Semantic behavioral variant frontotemporal dementia and semantic dementia associated with TARDBP mutations

Contributions of neurologists to diagnostic timelines of ALS and thinkALS as an early referral instrument for clinicians

Perils of predictive testing for unaffected people from motor neuron disease families with no identified causal gene