Article
Clinical Neurology
Harper S. Kim, John Son, Donghwan Lee, Joy Tsai, Danny Wang, E. Sandra Chocron, Seongwoo Jeong, Pamela Kittrell, Charles F. Murchison, Richard E. Kennedy, Alejandro Tobon, Carlayne E. Jackson, Andrew M. Pickering
Summary: In patients with ALS, different subtypes (sALS and bALS) show different dysbiosis patterns. sALS patients primarily exhibit gut dysbiosis, while bALS patients primarily exhibit oral dysbiosis. For sALS patients, gut dysbiosis is associated with microbial translocation and symptom severity, while for bALS patients, oral dysbiosis is associated with microbial translocation and disease severity. Both subtypes display oral motor deficits, but only in bALS is oral dysbiosis correlated with severity of oral motor deficits.
Review
Clinical Neurology
Qiaochu Zhu, Dandan Xu, Hai Huang, Dong Li, Dan Yang, Jing Zhou, Yan Zhao
Summary: High-calorie therapy has significant advantages in improving body weight and BMI, but the superiority is not significant in improving ALSFRS-R scores, survival time, lipid profile, and CRP indicator. Although the study quality is high and has some credibility, further validation is needed through high-quality randomized controlled trials.
JOURNAL OF NEUROLOGY
(2023)
Review
Biochemistry & Molecular Biology
Milena Jankovic, Ivana Novakovic, Phepy Gamil Anwar Dawod, Ayman Gamil Anwar Dawod, Aleksandra Drinic, Fayda I. Abdel Motaleb, Sinisa Ducic, Dejan Nikolic
Summary: ALS is a neurodegenerative motor neuron disorder with a significant genetic component, involving RNA processing, protein aggregation, oxidative stress, glutamate excitotoxicity, and inflammation. Mitochondrial dysfunction is a major contributor to disease onset and progression, highlighting the need for a broad perspective in understanding overlapping pathophysiological pathways and exploring potential therapies targeting mitochondrial dysfunction.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Nutrition & Dietetics
Kailin Xia, Yajun Wang, Linjing Zhang, Lu Tang, Gan Zhang, Tao Huang, Ninghao Huang, Dongsheng Fan
Summary: This study used Mendelian randomization analysis to investigate the potential causal relationship between essential nutrients and the risk of amyotrophic lateral sclerosis (ALS). The study found a causal association between genetically predicted linoleic acid (LA) and ALS risk, and a negative association between vitamin D/vitamin E and ALS risk. No causal effect was observed for essential amino acids and minerals on ALS risk.
Review
Medicine, General & Internal
Hee Ra Park, Eun Jin Yang
Summary: ALS, also known as motor neuron disease, is characterized by the loss of motor neurons in the spinal cord and cerebral cortex. Oxidative stress is a known cause of ALS and inhibiting it could be a potential therapeutic approach. Researching different subtypes of ALS patients is necessary for delaying disease progression and prolonging survival.
Review
Pharmacology & Pharmacy
JingSi Jiang, Yan Wang, Min Deng
Summary: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder. Currently, there are only a few drugs that can extend the survival time of patients. However, there are many new experimental drugs being tested in clinical trials, and several of them have shown promising therapeutic effects.
FRONTIERS IN PHARMACOLOGY
(2022)
Review
Neurosciences
Hui Wang, LiPing Guan, Min Deng
Summary: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the degeneration of motor neurons. The causes of ALS are not fully understood but genetic factors play a role in about 10% of cases. Recent studies have identified over 40 ALS genes, which contribute to a better understanding of the disease and the development of potential treatments.
FRONTIERS IN NEUROSCIENCE
(2023)
Review
Clinical Neurology
Michael Benatar, Joanne Wuu, Caroline McHutchison, Ronald B. Postuma, Bradley F. Boeve, Ronald Petersen, Christopher A. Ross, Howard Rosen, Jalayne J. Arias, Stephanie Fradette, Michael P. McDermott, Jeremy Shefner, Christine Stanislaw, Sharon Abrahams, Stephanie Cosentino, Peter M. Andersen, Richard S. Finkel, Volkan Granit, Anne-Laure Grignon, Jonathan D. Rohrer, Corey T. McMillan, Murray Grossman, Ammar Al-Chalabi, Martin R. Turner
Summary: Significant progress has been made in understanding the pre-symptomatic phase of amyotrophic lateral sclerosis. Advances in other neurodegenerative diseases provide valuable insights and highlight opportunities for discovery in ALS. Biomarkers play a critical role in studying pre-symptomatic ALS and are essential for early therapeutic intervention and disease prevention.
Review
Medicine, Research & Experimental
Arsh Haj Mohamad Ebrahim Ketabforoush, Rojin Chegini, Shirin Barati, Fatemeh Tahmasebi, Bardia Moghisseh, Mohammad Taghi Joghataei, Faezeh Faghihi, Fereshteh Azedi
Summary: Amyotrophic Lateral Sclerosis (ALS) is a debilitating neurodegenerative disease affecting upper and lower motor neurons. Despite the approval of a few drugs, the survival rate and quality of life for ALS patients remain low. Masitinib, a tyrosine kinase inhibitor, has shown promising results in clinical trials by targeting neuro-inflammation and inducing neuroprotection. This review discusses the pathophysiology of ALS, the mechanism of action of Masitinib, and its potential as a treatment for ALS, along with other drugs in phase 3 clinical trials.
BIOMEDICINE & PHARMACOTHERAPY
(2023)
Review
Clinical Neurology
David Brenner, Axel Freischmidt
Summary: ALS genetics research is highly dynamic and intriguing, with the discovery of numerous new ALS-associated genes and pathomechanisms. Recent findings include potential and risk genes, as well as new risk loci. Cell type and functional enrichment analyses have shed light on the genetic basis of selective motor neuron vulnerability in ALS, and major insights have been gained regarding known ALS genes and proteins. The understanding of ALS genetics and molecular basis has advanced significantly in the past years, leading to the development of novel gene-specific therapies for sporadic ALS and genetic subtypes.
CURRENT OPINION IN NEUROLOGY
(2022)
Review
Clinical Neurology
Reza Boostani, Nahid Olfati, Hosein Shamshiri, Zanireh Salimi, Farzad Fatehi, Seyed Arya Hedjazi, Atefeh Fakharian, Majid Ghasemi, Ali Asghar Okhovat, Keivan Basiri, Bahram Haghi Ashtiani, Behnaz Ansari, Gholam Reza Raissi, Seyed Ahmadreza Khatoonabadi, Payam Sarraf, Sara Movahed, Akram Panahi, Bentolhoda Ziaadini, Mohammad Yazdchi, Jalal Bakhtiyari, Shahriar Nafissi
Summary: According to Cao Yong, chief researcher of Suzhou University Medical Center, a lack of standardized clinical practice guidelines for ALS patients in China is a major obstacle in improving their quality of life and effectively utilizing medical resources.
FRONTIERS IN NEUROLOGY
(2023)
Article
Clinical Neurology
T. Dharmadasa, J. Howells, J. M. Matamala, N. G. Simon, D. Burke, S. Vucic, M. C. Kiernan
Summary: In amyotrophic lateral sclerosis, patients with inexcitability of all four limbs to transcranial magnetic stimulation have a distinct clinical profile characterized by younger age, lower-limb onset, faster disease progression, greater functional disability, and reduced survival. This measure may serve as an important prognostic marker in the disease.
EUROPEAN JOURNAL OF NEUROLOGY
(2021)
Article
Clinical Neurology
Philippe Corcia, Pascal Lejeune, Patrick Vourc'h, Stephane Beltran, Anne-Sophie Piegay, Helene Blasco, Vincent Meininger
Summary: This study characterized the prototypical phenotype of patients with amyotrophic lateral sclerosis (ALS) associated with PFN1 mutations and identified clinical indications for testing mutations in this gene. The main clinical findings for familial ALS linked to PFN1 were identified as pedigrees with over five cases, an onset age around 50 years, onset in the lower limbs, and the absence of cognitive impairment. The similarities with other ALS mutations prompt a review of ALS classifications based on both phenotype and genotype.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Review
Cell Biology
Hongmei Du, Zijun Huo, Yanchun Chen, Zhenhan Zhao, Fandi Meng, Xuemei Wang, Shiyue Liu, Haoyun Zhang, Fenghua Zhou, Jinmeng Liu, Lingyun Zhang, Shuanhu Zhou, Yingjun Guan, Xin Wang
Summary: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that leads to motor function loss in the central nervous system (CNS) and ultimately death. The mechanisms underlying ALS pathogenesis are not well understood and effective treatment is currently unavailable. Induced pluripotent stem cells (iPSCs) have the ability to self-renew and differentiate into various cells, making them a promising tool for studying ALS. iPSCs obtained from ALS patients can be differentiated into neurons and glial cells related to ALS, providing an excellent method for evaluating disease impact. Additionally, patient-derived iPSCs pose minimal risk of immune rejection and ethical concerns, offering hope for ALS treatment.
Article
Clinical Neurology
Claudia Santos Silva, Marta Gromicho, Miguel Oliveira Santos, Susana Pinto, Michael Swash, Mamede de Carvalho
Summary: In this study, the prevalence of thyroid dysfunction in amyotrophic lateral sclerosis (ALS) patients was investigated. The results showed that ALS patients had a lower prevalence of hypothyroidism, hyperthyroidism, and overall thyroid dysfunction compared to controls, but similar to the national epidemiological data for thyroid disease. This study is consistent with previous findings and suggests that thyroid dysfunction is not associated with ALS.
NEUROLOGICAL SCIENCES
(2022)
