Article
Clinical Neurology
Benjamin G. Trist, Sian Genoud, Stephane Roudeau, Alexander Rookyard, Amr Abdeen, Veronica Cottam, Dominic J. Hare, Melanie White, Jens Altvater, Jennifer A. Fifita, Alison Hogan, Natalie Grima, Ian P. Blair, Kai Kysenius, Peter J. Crouch, Asuncion Carmona, Yann Rufin, Stephane Claverol, Stijn Van Malderen, Gerald Falkenberg, David J. Paterson, Bradley Smith, Claire Troakes, Caroline Vance, Christopher E. Shaw, Safa Al-Sarraj, Stuart Cordwell, Glenda Halliday, Richard Ortega, Kay L. Double
Summary: This study examined the changes in SOD1 protein in post-mortem spinal cord tissues of ALS patients. The results showed mislocalization and accumulation of SOD1 protein in motor neurons of ALS patients, which was associated with instability and mismetallation of enzymatically active SOD1 dimers, as well as alterations to SOD1 post-translational modifications and molecular chaperones governing SOD1 maturation. These changes mostly occurred in regions of neurodegeneration and differentiated ALS patients from controls effectively.
Review
Geriatrics & Gerontology
Di He, Yan, Xu, Mingsheng Liu, Liying Cui
Summary: Amyotrophic lateral sclerosis (ALS) is a genetically complex neurodegenerative disease that is associated with immune dysfunction. Neuroinflammation, characterized by abnormal immune cell activation and excessive production of inflammatory cytokines, plays a significant role in the pathophysiology of ALS. This review examines recent evidence on the role of ALS-associated mutant genes in immune dysregulation, with a focus on the cGAS-STING signaling pathway and m6A-mediated immune regulation in neurodegeneration. It also discusses immune cell perturbation in both the central nervous system and peripheral tissues in ALS, as well as advancements in genetic and cell-based therapies for ALS. Understanding the complex relationship between ALS and neuroinflammation is crucial for the development of effective treatments for this debilitating disorder.
Article
Biochemistry & Molecular Biology
M. Kathryn Brewer, Pascual Torres, Victoria Ayala, Manuel Portero-Otin, Reinald Pamplona, Pol Andres-Benito, Isidro Ferrer, Joan J. Guinovart, Jordi Duran
Summary: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by motor neuron loss in the spinal cord. Glycogen accumulation in reactive astrocytes has been found to contribute to neurotoxicity and disease progression in ALS. This study demonstrates that glycogen accumulates in the spinal cord and brainstem of ALS mice and is associated with reactive astrocytes. The reduced glycogen synthesis in ALS mice leads to a longer lifespan and lower levels of pro-inflammatory cytokine Cxcl10, suggesting that glycogen accumulation is linked to inflammation and disease progression.
JOURNAL OF NEUROCHEMISTRY
(2023)
Review
Biochemistry & Molecular Biology
Raquel Garcia-Garcia, Laura Martin-Herrero, Laura Blanca-Pariente, Jesus Perez-Cabello, Cintia Roodveldt
Summary: ALS and FTD are the most common neurodegenerative diseases in adults, sharing clinical, genetic, and pathological similarities, characterized by progressive neuronal loss and chronic inflammation mediated by immune signaling kinases. The role of immune signaling kinases in determining neuroprotective or neurodegenerative states in ALS and FTD is crucial for understanding the underlying mechanisms of these disorders.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
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
Biochemistry & Molecular Biology
Teresa Pardo-Moreno, Himan Mohamed-Mohamed, Sami Suleiman-Martos, Juan Jose Ramos-Rodriguez, Antonio Rivas-Dominguez, Lucia Melguizo-Rodriguez, Jose L. Gomez-Urquiza, Beatriz Bermudez-Pulgarin, Victoria Garcia-Morales
Summary: The aim of this study was to analyze the possible relationship between the rate of disease progression and plasma lipid levels at the early stage of ALS. The results of the meta-analytic study suggest that there is no clear relationship between the symptoms observed in ALS patients and the plasma lipid levels. Further research expansion and geographic diversity would be of interest.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Genetics & Heredity
Jiahao Cai, Xiong Chen, Hongxuan Wang, Zixin Wei, Mei Li, Xiaoming Rong, Xiangpen Li, Ying Peng
Summary: The study found no causal relationship between iron status and the risk of ALS. Efforts to change iron status to reduce ALS incidence may be impractical.
FRONTIERS IN GENETICS
(2021)
Review
Cell Biology
Eva Sykova, Dasa Cizkova, Sarka Kubinova
Summary: MSCs, as candidates in regenerative medicine, have shown significant therapeutic potential in animal experiments and clinical studies due to their immunomodulatory, paracrine, and neural stimulation effects.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Carlota Jauregui, Idoia Blanco-Luquin, Monica Macias, Miren Roldan, Cristina Caballero, Inma Pagola, Maite Mendioroz, Ivonne Jerico
Summary: This study investigates the expression patterns of microglial-related genes in ALS spinal cord, and suggests the presence of a DAM-mediated inflammatory response and the significant role of TREM2 in the immune function of microglia in ALS.
Review
Biochemistry & Molecular Biology
Bradley Roberts, Frances Theunissen, Francis L. Mastaglia, P. Anthony Akkari, Loren L. Flynn
Summary: This review provides a comprehensive overview of the literature on the etiology and development of ALS, as well as its commonalities with synucleinopathy disorders. It discusses the involvement of alpha Syn in ALS and motor neuron disease pathology, along with current theories and strategies for therapeutics in ALS treatment, with a focus on small molecule RNA technologies.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Clinical Neurology
M. Khamaysa, M. Lefort, M. Pelegrini-Issac, A. Lackmy-Vallee, A. Preuilh, D. Devos, A. -S. Rolland, C. Desnuelle, M. Chupin, V. Marchand-Pauvert, G. Querin, Pierre-Francois Pradat
Summary: This study aimed to evaluate the predictive value of cervical spinal cord MRI parameters for motor capacity in ALS compared to clinical prognostic factors. Structural MRI measurements were significantly correlated with the ALSFRS-R score and its sub-scores. Multiple linear regression models combining spinal multimodal MRI and clinical factors could predict motor capacity in ALS.
JOURNAL OF NEUROLOGY
(2023)
Article
Neurosciences
Oumayma Aousji, Simone Feldengut, Stefano Antonucci, Michael Schoen, Tobias M. Boeckers, Jakob Matschke, Christian Mawrin, Albert C. Ludolph, Kelly Del Tredici, Francesco Roselli, Heiko Braak
Summary: This study investigated the loss of synapses associated with amyotrophic lateral sclerosis (ALS) and found that ALS patients exhibited a substantial loss of synapses in the ventral horn of lumbar and cervical spinal cord. The extent of synaptic loss was correlated with disease duration, clinical site of onset, and loss of α-motoneurons, but not with the fraction of pTDP-43-immunopositive α-motoneurons. These findings validate the synaptic pathology observed in other models and suggest the critical role of pathogenic pathways in the spinal microenvironment in the progressive disassembly of local synaptic connectivity.
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2023)
Review
Biochemistry & Molecular Biology
Courtney M. Clark, Rosemary M. Clark, Joshua A. Hoyle, Tracey C. Dickson
Summary: NPY has gained interest as a potential neuroprotective agent for neurodegenerative diseases, with evidence suggesting a possible role in ALS pathogenesis. While increased NPY levels correlate with disease progression in ALS, the functional consequences are not fully understood.
JOURNAL OF NEUROCHEMISTRY
(2021)
Review
Biochemistry & Molecular Biology
Anca Motataianu, Georgiana Serban, Laura Barcutean, Rodica Balasa
Summary: ALS is a severe neurodegenerative disease with a short survival period. Oxidative stress plays a crucial role in the pathogenesis of ALS, and genetic mutations and environmental factors can increase oxidative damage. This review explores how genetic mutations and environmental factors influence the occurrence of oxidative stress in ALS and discusses potential therapeutic molecules.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Clinical Neurology
M. Khamaysa, M. Lefort, M. Pelegrini-Issac, A. Lackmy-Vallee, M. M. El Mendili, A. Preuilh, D. Devos, G. Bruneteau, F. Salachas, T. Lenglet, Md. M. Amador, N. Le Forestier, A. Hesters, J. Gonzalez, A. -S. Rolland, C. Desnuelle, M. Chupin, G. Querin, M. Georges, C. Morelot-Panzini, V. Marchand-Pauvert, P. -F. Pradat, Pulse Study Grp
Summary: This study demonstrates that brainstem volumes and spinal cord area are promising indicators for predicting respiratory intervention needs in ALS patients, with the combined model incorporating predictors from multiple models yielding the best predictive performance.
JOURNAL OF NEUROLOGY
(2023)
Editorial Material
Clinical Neurology
John R. Bach, Giulio Chiarello, William Weiss, Nizar Souayah
EXPERT REVIEW OF NEUROTHERAPEUTICS
(2021)
Review
Biochemistry & Molecular Biology
Parisorn Thepmankorn, John Bach, Ahmed Lasfar, Xilin Zhao, Sami Souayah, Zhao Zhong Chong, Nizar Souayah
Summary: COVID-19 caused by SARS-CoV-2 can lead to a hyperinflammatory state known as cytokine storm, especially in severe patients. In addition to respiratory complications, neurological issues have also been reported in COVID-19 patients. Understanding and targeting aberrant cytokine levels, particularly in those with neurological symptoms, may be crucial in effective treatment and management of the disease.
Article
Mathematics, Applied
Nabil Mlaiki, Nizar Souayah, Thabet Abdeljawad, Hassen Aydi
Summary: In this paper, a new extension of b-metric spaces called controlled metric-like spaces is introduced by changing the condition to allow for a non-zero self-distance. Several fixed point theorems are proven, which generalize existing results, along with an application involving controlled metric-like spaces endowed with a graph.
ADVANCES IN DIFFERENCE EQUATIONS
(2021)
Meeting Abstract
Clinical Neurology
Parisorn Thepmankorn, Rishita Patlolla, Keyvan Heshmati, Stefano Malerba, Gabriel Arismendi, Sami Souayah, Nabil Adam, Nizar Souayah
Meeting Abstract
Clinical Neurology
Parisorn Thepmankorn, Nour Jedidi, Sami Souayah, Abu Nasar, Nizar Souayah
Meeting Abstract
Clinical Neurology
Colin Anderson-Smits, J. Bradley Layton, Mary E. Ritchey, Vanessa Hayden, Shailesh Chavan, Nizar Souayah
Meeting Abstract
Clinical Neurology
Gabriel Arismendi, Parisorn Thepmankorn, Stefano Malerba, Keyvan Heshmati, Rishita Patlolla, Sami Souayah, Nabil Adam, Nizar Souayah
Meeting Abstract
Clinical Neurology
Keyvan Heshmati, Gabriel Arismendi, Parisorn Thepmankorn, Abu Nasar, Nizar Souayah
Meeting Abstract
Clinical Neurology
Parisorn Thepmankorn, Keyvan Heshmati, Stefano Malerba, Rishita Patlolla, Gabriel Arismendi, Claire Ruane, Sami Souayah, Nabil Adam, Nizar Souayah
Meeting Abstract
Clinical Neurology
Colin Anderson-Smits, J. Bradley Layton, Mary E. Ritchey, Vanessa Hayden, Shailesh Chavan, Nizar Souayah
JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM
(2021)
Meeting Abstract
Clinical Neurology
Colin Anderson-Smits, J. Bradley Layton, Mary E. Ritchey, Shailesh Chavan, Nizar Souayah
JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM
(2021)
Meeting Abstract
Clinical Neurology
Rishita Patlolla, Stefano Malerba, Nizar Souayah
Meeting Abstract
Clinical Neurology
Keyvan Heshmati, Abu Nasar, Rishita Patlolla, Stefano Malerba, Nizar Souayah
Meeting Abstract
Clinical Neurology
Keyvan Heshmati, Abu Nasar, Stefano Malerba, Rishita Patlolla, Nizar Souayah
Meeting Abstract
Clinical Neurology
Nirav Sanghani, Leila Maybodi, Nizar Souayah
Article
Neurosciences
Wenzhu Wang, Zihan Li, Yitong Yan, Shuo Wu, Xinyu Yao, Chen Gao, Lanxiang Liu, Yan Yu
Summary: This study investigated the reparative mechanisms of low-intensity pulsed ultrasound (LIPUS) in traumatic brain injury (TBI) and found that LIPUS promotes hippocampal neurogenesis, enhances neural electrical activity and neural plasticity, ultimately restoring neuronal function and cognitive capabilities in TBI mice.
EXPERIMENTAL NEUROLOGY
(2024)
Article
Neurosciences
Wenmin Yi, Fei Chen, Minghao Yuan, Chuanling Wang, Shengyuan Wang, Jie Wen, Qian Zou, Yinshuang Pu, Zhiyou Cai
Summary: The study suggests that a high-fat diet may lead to tau hyperphosphorylation and synaptic dysfunction by inhibiting the SIRT1/AMPK pathway and disrupting autophagy flux, ultimately resulting in cognitive decline.
EXPERIMENTAL NEUROLOGY
(2024)
Article
Neurosciences
Kim M. Hemsley, Helen Beard, Glyn Chidlow, Teresa Mammone, Leanne K. Winner, Daniel Neumann, Barbara King, Marten F. Snel, Paul J. Trim, Robert J. Casson
Summary: Optical coherence tomography (OCT) is a non-invasive method that can be used to rapidly and quantitatively examine the integrity of the neuroretina. It has been shown that OCT can be used to observe retinal thinning in patients with childhood dementia, and to assess the improvement of retinal structure after treatment. Furthermore, OCT can provide insights into other childhood dementias based on the correlation between retinal and brain degeneration in Sanfilippo syndrome.
EXPERIMENTAL NEUROLOGY
(2024)
Article
Neurosciences
Qianling Jiang, Xin Ma, Gaochen Zhu, Wen Si, Lingyu He, Guan Yang
Summary: This study investigated the effects of EAE induction on thymopoiesis and T cell development, revealing changes such as increased apoptosis, decreased proliferation, and a blockade in the transition from double-negative thymocytes to double-positive cells. It was also found that positive selection was disrupted in the thymus of EAE mice, along with an increased production of regulatory T cells.
EXPERIMENTAL NEUROLOGY
(2024)
Article
Neurosciences
Alice McDouall, Guido Wassink, Sumudu Ranasinghe, Kelly Q. Zhou, Rashika N. Karunasinghe, Justin M. Dean, Joanne O. Davidson
Summary: This study found that blocking connexin 43 hemichannels can attenuate brain injury and promote neurodevelopment in infants with mild hypoxic-ischemic encephalopathy, without causing hypothermia.
EXPERIMENTAL NEUROLOGY
(2024)
Article
Neurosciences
Hannah Sweetman, Mahmudur Rahman, Aditya Vedantam, Kajana Satkunendrarajah
Summary: Degenerative cervical myelopathy (DCM) is a neurological condition characterized by chronic compression of the cervical spinal cord, leading to impaired limb function. While respiratory dysfunction is not a common symptom of DCM, it can affect the ventilatory response to respiratory challenges. Surgical decompression improves sensorimotor function in DCM, but its impact on respiratory function is unclear. This study evaluates respiratory function and adaptive ventilation in a DCM model, showing that DCM impairs acute adaptive ventilatory ability and surgical decompression does not fully restore it.
EXPERIMENTAL NEUROLOGY
(2024)
Article
Neurosciences
Chengmei Sun, Muhammad Saif Ur Rahman, Budbazar Enkhjargal, Jianhua Peng, Keren Zhou, Zhiyi Xie, Lingyun Wu, Tongyu Zhang, Qiquan Zhu, Jiping Tang, Yujia Zeng, John H. Zhang, Shanshan Xu
Summary: This study found that Osteopontin (OPN) can attenuate inflammatory responses after subarachnoid hemorrhage (SAH) by promoting an anti-inflammatory microglial state. This effect may be mediated through the integrin-FAK-STAT3 and NF-kappa B signaling pathways.
EXPERIMENTAL NEUROLOGY
(2024)
Article
Neurosciences
Yang Yang, Xuezhu Chen, Chuanyan Yang, Mei Liu, Qianying Huang, Likun Yang, Yuhai Wang, Hua Feng, Zhongyang Gao, Tunan Chen
Summary: The study explores the effects of specific chemogenetic stimulation of intact corticospinal tract on functional recovery after stroke in mice. The findings demonstrate that combining chemogenetic activation with rehabilitation training leads to significant motor functional recovery by promoting axon sprouting and rewiring new functional circuits.
EXPERIMENTAL NEUROLOGY
(2024)