Review
Clinical Neurology
Georgiana Soares Leandro, Mario Emilio Teixeira Dourado Junior, Glauciane Costa Santana, Luan Samy Xavier Dantas
Summary: The main coping strategy used by ALS patients is seeking social support, while Confrontive coping and Distancing are less commonly mentioned. The coping strategies of ALS patients do not seem to focus on emotions or stress-triggering problems, and age and gender do not modify the chosen strategy.
JOURNAL OF NEUROLOGY
(2022)
Article
Cell Biology
Seung-Hye Choi, Ali Yousefian-Jazi, Seung Jae Hyeon, Phuong Thi Thanh Nguyen, Jiyeon Chu, Sojung Kim, Suhyun Kim, Hannah L. Ryu, Neil W. Kowall, Hoon Ryu, Junghee Lee
Summary: In this study, researchers found that modulating the activity of LSD1 can improve the neuropathology of ALS mice, delay disease onset, and extend lifespan, potentially serving as an effective therapeutic strategy for treating ALS.
JOURNAL OF BIOMEDICAL SCIENCE
(2022)
Article
Medicine, General & Internal
Wen-Chao Liu, Na Liu, Yan Wang, Chen Huang, Yan-Fang Li, Hao Wang, Xiao-Gang Li, Min Deng
Summary: Research shows that motor neurons (MNs) derived from ALS patient-specific iPSC lines can replicate key aspects of ALS pathogenesis, providing insights into the disease's pathophysiological processes. Incremental mutant expressions of SOD1 in MNs may disrupt cellular function, leading to intracellular calcium disturbances and contributing to the onset of the disease.
CHINESE MEDICAL JOURNAL
(2021)
Article
Biochemistry & Molecular Biology
Oluwamolakun Bankole, Ilaria Scambi, Edoardo Parrella, Matilde Muccilli, Roberta Bonafede, Ermanna Turano, Marina Pizzi, Raffaella Mariotti
Summary: This study demonstrates the beneficial effects of combined treatment with valproate and resveratrol in ALS mice, with a sexually dimorphic response. The combination of drugs restores NF-kB to its neuroprotective state, reducing pathology and symptoms, and prolonging survival.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Medicine, General & Internal
Christine Zizzi, Jamison Seabury, Spencer Rosero, Danae Alexandrou, Ellen Wagner, Jennifer S. Weinstein, Anika Varma, Nuran Dilek, John Heatwole, Joanne Wuu, James Caress, Richard Bedlack, Volkan Granit, Jeffrey M. Statland, Paul Mehta, Michael Benatar, Chad Heatwole
Summary: This study used data from two multi-center randomized controlled trials and found that depression symptoms in patients can be categorized into four clusters, which have differential responses to repetitive transcranial magnetic stimulation (rTMS) treatment. Further research aims to personalize rTMS protocols based on individual clinical presentations.
Article
Food Science & Technology
Salvatore D'Antona, Martina Caramenti, Danilo Porro, Isabella Castiglioni, Claudia Cava
Summary: Amyotrophic lateral sclerosis (ALS) is a fatal disease linked to motor neurons degeneration, with diet components like oxidative stress potentially influencing its onset. Some diets with antioxidant and anti-inflammatory properties may reduce the risk of ALS, but current data remains controversial.
Article
Clinical Neurology
Melina Pazian Martins, Carelis Gonzalez-Salazar, Fabricio Diniz de Lima, Tauana Bernardes Leoni, Alberto R. M. Martinez, Joao Pedro Nunes Goncalves, Anamarli Nucci, Marcondes Cavalcante Franca Jr
Summary: This study characterized and compared the autonomic function of patients with sporadic ALS and familial ALS type 8. The results showed that both types of ALS patients had dysautonomia involving both sympathetic and parasympathetic divisions, but the symptoms were different between the two groups.
CLINICAL NEUROPHYSIOLOGY
(2023)
Article
Pharmacology & Pharmacy
J. Daniel Bireley, John A. Morren
Summary: This article provides an overview of current amyotrophic lateral sclerosis (ALS) treatments, including riluzole, edaravone, AMX0035, and tofersen. It also discusses the potential of CNM-Au8, an energy metabolism catalyst, as a disease-modifying treatment for ALS. While the phase 2/3 clinical trials of CNM-Au8 did not meet primary endpoints, exploratory endpoints suggest its potential in slowing clinical worsening, improving quality of life, and prolonging survival in ALS. Phase 3 clinical trials of CNM-Au8 are currently underway.
EXPERT OPINION ON INVESTIGATIONAL DRUGS
(2023)
Article
Clinical Neurology
Megan Yerton, Allison Winter, Dario Gelevski, Grace Addy, Anthony Kostov, Cassandra Lieberman, Harli Weber, Michael Doyle, Geli Kane, Caroline Cohen, Neil Parikh, Katherine M. Burke, Margot Rohrer, Taylor Stirrat, Margaret Bruno, Alison Hochman, Sarah Luppino, Jennifer Scalia, Derek D'Agostino, Ervin Sinani, Hong Yu, Kristin Drake, Jennifer Hagar, Alexander V. Sherman, Suma Babu, James D. Berry, Merit E. Cudkowicz, Sabrina Paganoni
Summary: This report shares the design, operational features, and costs of an Expanded Access Program (EAP) for ALS, which provides investigational products (IPs) to individuals with serious diseases who are ineligible for clinical trials. From July 2018 to February 2022, 17 EAPs were submitted and approved by the FDA and IRB. A total of 164 participants enrolled in the program, with 77 participants still receiving IP treatment. The average duration of participation was 12.6 months, and no drug-related serious adverse events were reported.
Article
Clinical Neurology
Robert David Henderson, Kasper Planeta Kepp, Andrew Eisen
Summary: Amyotrophic lateral sclerosis and frontotemporal dementia are neurodegenerative diseases with complex cellular pathology. The paper emphasizes the importance of evolution in the development of higher cortical function and the impact of factors related to increased lifespan on neuronal function.
FRONTIERS IN NEUROLOGY
(2022)
Article
Clinical Neurology
Giammarco Milella, Stefano Zoccolella, Alessia Giugno, Marco Filardi, Daniele Urso, Salvatore Nigro, Benedetta Tafuri, Ludovica Tamburrino, Valentina Gnoni, Giancarlo Logroscino
Summary: This study found that spinal-onset ALS can be categorized into three groups based on the extent of upper motor neuron (UMN) and lower motor neuron (LMN) involvement. UMN burden is associated with higher diagnostic certainty and broader disease spread, while LMN involvement is associated with more severe disease and shorter survival.
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Edoardo Nicolo Aiello, Debora Pain, Alice Radici, Kalliopi Marinou Aktipi, Riccardo Sideri, Ildebrando Appollonio, Gabriele Mora
Summary: This study found cognitive deficits in ALS patients, with classical and predominant-upper motor neuron phenotypes performing the worst. Poorer functional scores correlated with poorer cognitive scores. These findings are important for understanding the cognitive impact of ALS.
NEUROLOGICAL SCIENCES
(2022)
Article
Biology
Sang Hwa Kim, Kye D. Nichols, Eric N. Anderson, Yining Liu, Nandini Ramesh, Weiyan Jia, Connor J. Kuerbis, Mark Scalf, Lloyd M. Smith, Udai Bhan Pandey, Randal S. Tibbetts, Jeannie Chin
Summary: Mutations in UBQLN2 cause X-linked forms of ALS and FTD, but the underlying mechanisms are unknown. Studying ALS-associated mutants of UBQLN2 in Drosophila, it was found that they trigger heat stress-dependent neurodegeneration. A genetic modifier screen identified endolysosomal and axon guidance genes, including the netrin receptor Unc-5, as key regulators of UBQLN2 toxicity. Reduced dosage of Unc-5 or its coreceptor Dcc/frazzled alleviated neurodegenerative phenotypes in flies expressing UBQLN2(ALS) alleles. Further experiments using iPSCs and iMNs demonstrated lysosomal defects and cytosolic UBQLN2 inclusions, neurite complexity reduction, and growth cone defects, which were partially reversed by silencing of UNC5B and DCC. These findings suggest altered growth cone dynamics as a conserved pathomechanism in UBQLN2-associated ALS/FTD.
Article
Clinical Neurology
Eleonora Colombo, Alberto Doretti, Francesco Scheveger, Alessio Maranzano, Giulia Pata, Delia Gagliardi, Megi Meneri, Stefano Messina, Federico Verde, Claudia Morelli, Stefania Corti, Luca Maderna, Vincenzo Silani, Nicola Ticozzi
Summary: This study aimed to analyze the correlation between electrophysiological parameters and clinical characteristics of ALS. The results showed a significant association between AD and CD scores and disease progression, survival, and functional disability in ALS patients.
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Patrizia M. Maier, Deetje Iggena, Thomas Meyer, Carsten Finke, Christoph J. Ploner
Summary: This study found no evidence of hippocampal dysfunction in non-demented ALS patients, suggesting that the cognitive phenotype of ALS may relate to distinct disease subtypes rather than being a variable expression of the same underlying condition.
JOURNAL OF NEUROLOGY
(2023)
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)