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)
Review
Biochemistry & Molecular Biology
Soju Seki, Yoshihiro Kitaoka, Sou Kawata, Akira Nishiura, Toshihiro Uchihashi, Shin-ichiro Hiraoka, Yusuke Yokota, Emiko Tanaka Isomura, Mikihiko Kogo, Susumu Tanaka
Summary: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the degeneration of motor neurons, with emerging evidence suggesting involvement of sensory neurons. Clinical research has shown sensory symptoms and abnormal nerve conduction in ALS patients, while preclinical research has identified genetic abnormalities in both sensory and motor neurons. This review highlights the potential of targeting sensory neuron abnormalities in ALS for new therapies.
Review
Cell Biology
Maximilian Vidovic, Lars Hendrik Mueschen, Svenja Brakemeier, Gerrit Machetanz, Marcel Naumann, Sergio Castro-Gomez
Summary: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by loss of upper and lower motor neurons, resulting in progressive weakness of voluntary muscles and eventual respiratory failure. Diagnosis is primarily based on clinical findings supported by electrophysiological and laboratory measurements, but research on disease-specific fluid biomarkers and advances in imaging techniques have improved diagnostic accuracy. Genetic testing allows for early identification of ALS-related gene mutations and access to clinical trials for disease-modifying therapies. Personalized survival prediction models provide detailed prognosis information. This review summarizes established diagnostic procedures and future directions in ALS diagnostics to improve the diagnostic pathway for this burdensome disease.
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
Clinical Neurology
Matthew J. Broadhead, Calum Bonthron, Julia Waddington, William Smith, Maite F. Lopez, Sarah Burley, Jessica Valli, Fei Zhu, Noboru H. Komiyama, Colin Smith, Seth G. N. Grant, Gareth B. Miles
Summary: Tripartite synaptopathy is a key hallmark of ALS, as revealed by extensive microscopy-based investigations in ALS model mice and post-mortem human tissue. This study demonstrates widespread synaptic changes and selective loss of tripartite synapses in ALS.
ACTA NEUROPATHOLOGICA
(2022)
Review
Neurosciences
Koy Chong Ng Kee Kwong, Pratap K. Harbham, Bhuvaneish T. Selvaraj, Jenna M. Gregory, Suvankar Pal, Giles E. Hardingham, Siddharthan Chandran, Arpan R. Mehta
Summary: This review summarizes the 40-year history of CSF toxicity studies in ALS and discusses various proposed mechanisms such as glutamate excitotoxicity, proteotoxicity, and oxidative stress. The potential implications of a toxic CSF circulatory system in the pathophysiology of ALS and its significance in current ALS research are also considered.
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2021)
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)
Review
Cell Biology
Laura Tzeplaeff, Sibylle Wilfling, Maria Viktoria Requardt, Meret Herdick
Summary: Finding effective therapy for ALS is challenging, as the exact cause and pathological pathway are unknown. Current approved drugs only have moderate effects and no curative options are available. Recent breakthroughs in genetic research bring hope for improved treatment, but more efforts are needed.
Review
Pharmacology & Pharmacy
Yvonne E. Klingl, Donya Pakravan, Ludo Van den Bosch
Summary: ALS is a devastating neurodegenerative disease with limited treatment options. Research suggests that interference with histone deacetylases may be helpful in treating ALS.
BRITISH JOURNAL OF PHARMACOLOGY
(2021)
Article
Medicine, General & Internal
Eleonora Volpato, Michele Vitacca, Luciana Ptacinsky, Agata Lax, Salvatore D'Ascenzo, Enrica Bertella, Mara Paneroni, Silvia Grilli, Paolo Banfi
Summary: In ALS patients, home-based adaptation to NIV is as effective as outpatient adaptation in terms of PaCO2, acceptance, and adherence, highlighting the need for further studies on the role of the environment in NIV adherence.
JOURNAL OF CLINICAL MEDICINE
(2022)
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
Biology
Christoph Schweingruber, Eva Hedlund
Summary: ALS is a fatal disease characterized by the loss of particular neurons called motor neurons, leading to paralysis. The reasons for the vulnerability and resilience of different motor neurons in ALS are not fully understood. Multiple cell types are involved in the disease progression and contribute to the selective vulnerability of motor neurons.
Article
Geriatrics & Gerontology
Federico Verde, Ilaria Milone, Eleonora Colombo, Alessio Maranzano, Federica Solca, Silvia Torre, Alberto Doretti, Francesco Gentile, Arianna Manini, Ruggero Bonetti, Silvia Peverelli, Stefano Messina, Luca Maderna, Claudia Morelli, Barbara Poletti, Antonia Ratti, Vincenzo Silani, Nicola Ticozzi
Summary: This study investigated the relationship between serum levels of the neuroaxonal degeneration biomarker NFL and phenotype in ALS. The results showed that sNFL levels were increased in ALS patients and could distinguish them from neurologically healthy controls. sNFL levels were higher in phenotypes with both UMN and LMN signs, especially in those with UMN predominance. sNFL was also associated with disease progression rate, King's stages, and survival.
FRONTIERS IN AGING NEUROSCIENCE
(2023)
Review
Biochemistry & Molecular Biology
Joni Vanneste, Ludo van den Bosch
Summary: There is evidence suggesting that nucleocytoplasmic-transport deficits may be important in the pathogenesis of ALS, but current data are limited in clarifying the exact causal and temporal role. Understanding this will be significant for therapeutically targeting nucleocytoplasmic transport and associated proteins.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Neurosciences
Alexander Schmitz, Joao Pinheiro Marques, Irina Oertig, Niran Maharjan, Smita Saxena
Summary: The most common genetic cause of ALS and FTD is a hexanucleotide expansion in the C9ORF72 gene, which leads to various disease pathologies. Different forms of DPRs have different contributions to disease pathology, and recent advances in neuropathology and cellular studies have provided clues to understand their effects better.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2021)
Article
Behavioral Sciences
Andrea Gajardo-Vidal, Maxime Montembeault, Diego L. Lorca-Puls, Abigail E. Licata, Rian Bogley, Sabrina Erlhoff, Buddhika Ratnasiri, Zoe Ezzes, Giovanni Battistella, Elena Tsoy, Christa Watson Pereira, Jessica Deleon, Boon Lead Tee, Maya L. Henry, Zachary A. Miller, Katherine P. Rankin, Maria Luisa Mandelli, Katherine L. Possin, Maria Luisa Gorno-Tempini
Summary: This study investigates the potential differences in processing speed and neural correlates among the three variants of Primary Progressive Aphasia (PPA). The findings reveal that non-verbal cognitive abilities, such as processing speed, are significantly impacted in nfvPPA and lvPPA patients compared to healthy controls and svPPA patients. Neuroimaging results confirm the importance of fronto-parietal regions associated with processing speed and executive control.
Article
Behavioral Sciences
Holger Wiese, Tsvetomila Popova, Maya Schipper, Deni Zakriev, Mike Burton, Andrew W. Young
Summary: Previous experiments have shown that brief exposure to unfamiliar individuals leads to the formation of new facial representations, which undergo changes and consolidation within the first day after learning.
Article
Behavioral Sciences
Astrid Prochnow, Xianzhen Zhou, Foroogh Ghorbani, Paul Wendiggensen, Veit Roessner, Bernhard Hommel, Christian Beste
Summary: Individuals organize events in their environment by partitioning them into discrete units. This study reveals that the neural activity in the brain plays a critical role in this process, reflecting the key elements of event segmentation.
Article
Behavioral Sciences
Zhenzhen Huo, Zhiyi Chen, Rong Zhang, Junye Xu, Tingyong Feng
Summary: Procrastination has adverse effects on personal growth and social development. Reward sensitivity is positively correlated with procrastination. This study used VBM and RSFC analyses to investigate the neural substrates underlying the association between reward sensitivity and procrastination. The results showed that the functional connectivity of the right parahippocampal gyrus-precuneus mediated the relationship between reward sensitivity and procrastination.
Article
Behavioral Sciences
Stefano Lasaponara, Gabriele Scozia, Silvana Lozito, Mario Pinto, David Conversi, Marco Costanzi, Tim Vriens, Massimo Silvetti, Fabrizio Doricchi
Summary: Cholinergic (Ach), Noradrenergic (NE), and Dopaminergic (DA) pathways are crucial in regulating spatial attention and determining inter-individual differences in temperamental traits. This study found that temperamental traits predict individual differences in the ability to orient spatial attention based on the probabilistic association between cues and targets. These findings highlight the importance of considering temperamental and personality traits in social and professional environments where attention control is essential.
Article
Behavioral Sciences
Darren J. Yeo, Courtney Pollack, Benjamin N. Conrad, Gavin R. Price
Summary: The processing of numerals as visual objects is supported by an Inferior Temporal Numeral Area (ITNA) in the bilateral inferior temporal gyri (ITG). Extant findings suggest some degree of hemispheric asymmetry in how the bilateral ITNAs process numerals. The study found that digit sensitivity did not differ between ITNAs, and digit sensitivity in both left and right ITNAs was associated with calculation skills. The study also revealed a right lateralization in engagement in alphanumeric categorization, and that the right ITNA showed greater discriminability between digits and letters.
Review
Behavioral Sciences
Beste Gulsuna, Abuzer Gungor, Alp O. Borcer, Ugur Ture
Summary: The fiber dissection technique has been used to study the internal structures of the brain, with less focus on white matter. The sagittal stratum, a white matter structure, has not received enough attention and has been a subject of controversy. Recent studies suggest potential functions of the sagittal stratum, emphasizing the importance of understanding this structure accurately.
Article
Behavioral Sciences
Nora Geiser, Brigitte Charlotte Kaufmann, Samuel Elia Johannes Knobel, Dario Cazzoli, Tobias Nef, Thomas Nyffeler
Summary: This study compared the effects of auditory and visual motion stimulation on spatial neglect and found that both interventions were equally effective in improving neglect. Multimodal motion stimulation also improved neglect, but did not show greater improvement than unimodal auditory or visual motion stimulation alone.
Article
Behavioral Sciences
Anna E. Hughes, Anna Nowakowska, Alasdair D. F. Clarke
Summary: This study examines the relationship between search slopes and search efficiency in visual search tasks, introduces the Target Contrast Signal (TCS) Theory, and extends it to a Bayesian multi-level framework. The findings demonstrate that TCS can predict data well, but distinguishing between contrast combination models proves to be difficult.