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
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)
Review
Clinical Neurology
Christian Wilson R. Turalde, Kevin Michael C. Moalong, Adrian I. Espiritu, Mario B. Prado
Summary: This study aimed to evaluate the efficacy and safety of perampanel in ALS patients in terms of functional status improvement. After reviewing relevant studies, the results showed that perampanel did not have sufficient evidence to support its role in improving functional status in ALS patients. However, it was found to improve cortical motor hyperexcitability. Adverse events such as aggression, somnolence, anger, and dysarthria were associated with perampanel use among ALS patients. Further studies are needed to investigate the potential benefits of perampanel in ALS, considering factors such as disease stage, specific patient characteristics, and titration schedule.
NEUROLOGICAL SCIENCES
(2022)
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)
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
Radiology, Nuclear Medicine & Medical Imaging
Giorgio Conte, Valeria Elisa Contarino, Silvia Casale, Claudia Morelli, Sara Sbaraini, Elisa Scola, Francesca Trogu, Silvia Siggillino, Claudia Maria Cinnante, Luca Caschera, Francesco Maria Lo Russo, Fabio Maria Triulzi, Vincenzo Silani
Summary: The study aimed to investigate whether magnetic susceptibility varies according to ALS phenotypes based on UMN/LMN sign predominance. Results showed significant differences in susceptibility properties of the precentral cortex among different clinical ALS phenotypes. Combined MRI-histopathology investigations are needed to confirm the evidence of iron overload in UMN-ALS unlike in LMN-ALS.
EUROPEAN RADIOLOGY
(2021)
Review
Biology
Gavin McCluskey, Karen E. Morrison, Colette Donaghy, Frederique Rene, William Duddy, Stephanie Duguez
Summary: Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease with complex pathogenesis involving disruption of multiple pathways. Extracellular vesicles (EVs), nanoscopic membrane-bound particles, play a crucial role in intercellular communication, and their involvement in ALS pathogenesis is increasingly recognized.
Article
Ophthalmology
Arvin Behzadi, Anton Erik Tjust, Jing-Xia Liu, Peter Munch Andersen, Thomas Braennstroem, Fatima Pedrosa Domelloef
Summary: This study investigates changes in myofiber composition in the global and orbital layers of extraocular muscles (EOMs) from terminal amyotrophic lateral sclerosis (ALS) donors. The results show that ALS patients have a smaller proportion of MyHCIIa-containing myofibers and a larger proportion of MyHCeom-containing myofibers in the global layer of EOMs. The changes are more prominent in bulbar-onset ALS patients. The myofiber composition in the orbital layer shows no significant differences.
INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE
(2023)
Article
Clinical Neurology
Nga Yan Tse, Sicong Tu, Yu Chen, Jashelle Caga, Carol Dobson-Stone, John B. Kwok, Glenda M. Halliday, Rebekah M. Ahmed, John R. Hodges, Olivier Piguet, Matthew C. Kiernan, Emma M. Devenney
Summary: This study provides the first exploration of schizotypal traits and its neural underpinnings in the ALS-FTD spectrum, revealing the presence of a wide spectrum of subclinical schizotypal symptoms and suggesting a potential overlap with schizophrenia in terms of underlying mechanisms involving disruptions to frontal-striatal-limbic networks.
JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
Justin Kwan, Mithila Vullaganti
Summary: Amyotrophic lateral sclerosis (ALS) is a common motor neuron disorder in adults, and it is important to differentiate it from similar diseases for accurate diagnosis.
Article
Genetics & Heredity
Tanya Lehky, Christopher Grunseich
Summary: Juvenile amyotrophic lateral sclerosis (JALS) is a rare group of motor neuron disorders with gene association in 40% of cases, defined by onset before age 25. The most common gene mutations associated with JALS are FUS, SETX, and ALS2, with familial cases mostly inherited in an autosomal recessive pattern.
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.
Article
Biochemistry & Molecular Biology
Niccolo Candelise, Illari Salvatori, Silvia Scaricamazza, Valentina Nesci, Henri Zenuni, Alberto Ferri, Cristiana Valle
Summary: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the loss of upper and lower motor neurons. Despite efforts to understand its pathogenesis, ALS remains obscure and lacks effective therapies. Recent studies have highlighted the central role of mitochondrial dysfunction in ALS, leading to impaired energy metabolism.
Review
Clinical Neurology
Emily Beswick, Emily Park, Charis Wong, Arpan R. Mehta, Rachel Dakin, Siddharthan Chandran, Judith Newton, Alan Carson, Sharon Abrahams, Suvankar Pal
Summary: Up to 50% of ALS patients experience cognitive dysfunction, with high rates of depression and anxiety reported. However, past 25 years of clinical trials have largely neglected the importance of neuropsychiatric symptoms and cognitive impairment in ALS. Evaluation of these non-motor features is crucial for understanding the impact of candidate drugs on all symptoms of ALS.
JOURNAL OF NEUROLOGY
(2021)
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.