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Journal articles on the topic 'Amyotrophic lateral sclerosis – Research'

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Author: Grafiati

Published: 10 December 2022

Last updated: 28 January 2023

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1

Agnello, Luisa, and Marcello Ciaccio. "Molecular Research on Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 23, no. 20 (October 11, 2022): 12069. http://dx.doi.org/10.3390/ijms232012069.

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2

Przedborski, Serge, Hiroshi Mitsumoto, and Lewis P. Rowland. "Recent advances in amyotrophic lateral sclerosis research." Current Neurology and Neuroscience Reports 3, no. 1 (January 2003): 70–77. http://dx.doi.org/10.1007/s11910-003-0041-x.

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3

Miller, Robert G., Fred Anderson, Benjamin Rix Brooks, Hiroshi Mitsumoto, Walter G. Bradley, and Steven P. Ringel. "Outcomes research in amyotrophic lateral sclerosis: Lessons learned from the amyotrophic lateral sclerosis clinical assessment, research, and education database." Annals of Neurology 65, S1 (January 2009): S24—S28. http://dx.doi.org/10.1002/ana.21556.

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4

Heo, Gi-yoon, Hee-kyung Kang, Min-hwa Kim, Irang Nam, Mariah Kim, So-yeon Kim, So-jung Park, et al. "A Case Report on a Patient with Amyotrophic Lateral Sclerosis Treated with Korean Medicine." Journal of Internal Korean Medicine 43, no. 5 (October 30, 2022): 891–900. http://dx.doi.org/10.22246/jikm.2022.43.5.891.

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Purpose: The purpose of this paper is to report the improvement of a patient with amyotrophic lateral sclerosis after long-term combined Korean medical treatment.Methods: A patient diagnosed with amyotrophic lateral sclerosis was treated with herbal medicine, acupuncture, pharmacopuncture, moxibustion, and rehabilitation for four separate hospital stays. To evaluate their respiratory discomfort and limb weakness, we used Manual Muscle Testing, the Pulmonary Function Test, and the Korean Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised.Results: The weakness of the muscles of the lower extremities and respiratory function was improved.Conclusion: We consider that combined Korean medicine treatments might be an effective treatment for muscle weakness and respiratory discomfort of amyotrophic lateral sclerosis. To verify the effectiveness of these treatments, further research is required.

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Gregory, Jenna M., Delphine Fagegaltier, Hemali Phatnani, and Matthew B. Harms. "Genetics of Amyotrophic Lateral Sclerosis." Current Genetic Medicine Reports 8, no. 4 (November 7, 2020): 121–31. http://dx.doi.org/10.1007/s40142-020-00194-8.

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Abstract Purpose of Review Amyotrophic lateral sclerosis and frontotemporal dementia (ALS-FTD) spectrum disorder is a rare fatal disease with strong genetic influences. The implementation of short-read sequencing methodologies in increasingly large patient cohorts has rapidly expanded our knowledge of the complex genetic architecture of the disease. We aim to convey the broad history of ALS gene discovery as context for a focused review of 11 ALS gene associations reported over the last 5 years. We also summarize the current level of genetic evidence for all previously reported genes. Recent Findings The history of ALS gene discovery has occurred in at least four identifiable phases, each powered by different technologies and scale of investigation. The most recent epoch, benefitting from population-scale genome data, large international consortia, and low-cost sequencing, has yielded 11 new gene associations. We summarize the current level of genetic evidence supporting these ALS genes, highlighting any genotype-phenotype or genotype-pathology correlations, and discussing preliminary understanding of molecular pathogenesis. This era has also raised uncertainty around prior ALS-associated genes and clarified the role of others. Summary Our understanding of the genetic underpinning of ALS has expanded rapidly over the last 25 years and has led directly to the clinical application of molecularly driven therapies. Ongoing sequencing efforts in ALS will identify new causative and risk factor genes while clarifying the status of genes reported in prior eras of research.

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Atsuta, Naoki, and Gen Sobue. "Japanese consortium for amyotrophic lateral sclerosis research (JaCALS)." Rinsho Shinkeigaku 50, no. 11 (2010): 928–30. http://dx.doi.org/10.5692/clinicalneurol.50.928.

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7

Andrews, Jinsy. "Amyotrophic lateral sclerosis: Clinical management and research update." Current Neurology and Neuroscience Reports 9, no. 1 (December 16, 2008): 59–68. http://dx.doi.org/10.1007/s11910-009-0010-0.

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8

de Carvalho, Mamede. "Advances in amyotrophic lateral sclerosis research in 2022." Lancet Neurology 22, no. 1 (January 2023): 21–22. http://dx.doi.org/10.1016/s1474-4422(22)00490-2.

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9

D’Antona, Salvatore, Martina Caramenti, Danilo Porro, Isabella Castiglioni, and Claudia Cava. "Amyotrophic Lateral Sclerosis: A Diet Review." Foods 10, no. 12 (December 17, 2021): 3128. http://dx.doi.org/10.3390/foods10123128.

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Amyotrophic lateral sclerosis (ALS) is a fatal disease related to upper and lower motor neurons degeneration. Although the environmental and genetic causes of this disease are still unclear, some factors involved in ALS onset such as oxidative stress may be influenced by diet. A higher risk of ALS has been correlated with a high fat and glutamate intake and β-methylamino-L-alanine. On the contrary, a diet based on antioxidant and anti-inflammatory compounds, such as curcumin, creatine, coenzyme Q10, vitamin E, vitamin A, vitamin C, and phytochemicals could reduce the risk of ALS. However, data are controversial as there is a discrepancy among different studies due to a limited number of samples and the many variables that are involved. In addition, an improper diet could lead to an altered microbiota and consequently to an altered metabolism that could predispose to the ALS onset. In this review we summarized some research that involve aspects related to ALS such as the epidemiology, the diet, the eating behaviour, the microbiota, and the metabolic diseases. Further research is needed to better comprehend the role of diet and the metabolic diseases in the mechanisms leading to ALS onset and progression.

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10

Lulé, Dorothée. "Neuroimaging Advances in Amyotrophic Lateral Sclerosis." European Neurological Review 5, no. 2 (2010): 54. http://dx.doi.org/10.17925/enr.2010.05.02.54.

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The development of non-invasive functional imaging techniques has allowed neuroscientists to investigate the physiological parameters of the clinical features of amyotrophic lateral sclerosis (ALS), a severe neurological disease. Modern neuroimaging techniques enable anatomy and function to be connectedin vivowith an acceptable balance between low patient load and high information capacity, making them ideal for clinical research in patients with physical restrictions, such as those with ALS. Structural imaging techniques in ALS include T1/T2-weighted structural magnetic resonance imaging, diffusion tensor imaging and voxel-based morphometry. Functional neuroimaging enables the acquisition of dynamic cortical function either in a passive (or resting) state or via active paradigms. The main technique used is functional magnetic resonance imaging. Structural and functional neuroimaging has provided evidence of alterations in motor and non-motor cortical pathways in ALS. In the future, neuroimaging may provide early diagnostic criteria to support the clinical diagnosis of ALS, help us to understand the aetiological background of the disease and pave the way for a new viewpoint on the functional capacities of these patients, which may have a major impact on our way of thinking about end-of-life decisions.

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11

Pagnini, Francesco, Zachary Simmons, Massimo Corbo, and Enrico Molinari. "Amyotrophic lateral sclerosis: Time for research on psychological intervention?" Amyotrophic Lateral Sclerosis 13, no. 5 (February 13, 2012): 416–17. http://dx.doi.org/10.3109/17482968.2011.653572.

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12

Petri, Susanne. "Major research advances in amyotrophic lateral sclerosis in 2021." Lancet Neurology 21, no. 1 (January 2022): 14–15. http://dx.doi.org/10.1016/s1474-4422(21)00420-8.

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13

Factor-Litvak, Pam, Ammar Al-Chalabi, Alberto Ascherio, Walter Bradley, Adriano Chío, Ralph Garruto, Orla Hardiman, et al. "Current pathways for epidemiological research in amyotrophic lateral sclerosis." Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration 14, sup1 (May 2013): 33–43. http://dx.doi.org/10.3109/21678421.2013.778565.

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14

Sherman, Alexander V., Amelie K. Gubitz, Ammar Al-Chalabi, Richard Bedlack, James Berry, Robin Conwit, Brent T. Harris, et al. "Infrastructure resources for clinical research in amyotrophic lateral sclerosis." Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration 14, sup1 (May 2013): 53–61. http://dx.doi.org/10.3109/21678421.2013.779058.

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15

Stefanou, Maria-Ioanna, and Iakovos Amygdalos. "Patient-led research in amyotrophic lateral sclerosis: Quo vadis?" Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration 16, no. 5-6 (March 24, 2015): 418–22. http://dx.doi.org/10.3109/21678421.2015.1013968.

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16

Rowland, Lewis P. "Motor neuron diseases and amyotrophic lateral sclerosis: research progress." Trends in Neurosciences 10, no. 10 (January 1987): 393–98. http://dx.doi.org/10.1016/0166-2236(87)90004-x.

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17

Papadeas, Sophia T., and Nicholas J. Maragakis. "Advances in stem cell research for Amyotrophic Lateral Sclerosis." Current Opinion in Biotechnology 20, no. 5 (October 2009): 545–51. http://dx.doi.org/10.1016/j.copbio.2009.09.003.

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18

Traub, Rebecca, Hiroshi Mitsumoto, and Lewis P. Rowland. "Research Advances in Amyotrophic Lateral Sclerosis, 2009 to 2010." Current Neurology and Neuroscience Reports 11, no. 1 (November 16, 2010): 67–77. http://dx.doi.org/10.1007/s11910-010-0160-0.

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19

Goutman, Stephen A., and Eva L. Feldman. "Voicing the Need for Amyotrophic Lateral Sclerosis Environmental Research." JAMA Neurology 77, no. 5 (May 1, 2020): 543. http://dx.doi.org/10.1001/jamaneurol.2020.0051.

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20

Andrew, Eisen, and Krieger Charles. "Pathogenic Mechanisms in Sporadic Amyotrophic Lateral Sclerosis." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 20, no. 4 (November 1993): 286–96. http://dx.doi.org/10.1017/s0317167100048198.

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ABSTRACT:In recognition of the 100th anniversary of Charcot’s death we have reviewed possible pathogenic mechanisms in amyotrophic lateral sclerosis (ALS). Advances in the last 5 years in molecular biology and genetics have identified mutations in the cytosolic dismutase (SODI) gene in some patients with familial ALS raising the possibility that oxidative stress may be involved in the pathogenesis. An excitotoxic pathogenesis has been implicated based on elevated plasma and CSF levels of amino acids and altered contents of amino acids in the nervous system of ALS patients and changes in the number of excitatory amino acid receptors. ALS sera containing antibodies to L-type calcium channels and the development of immune mediated lower and upper and lower motor neuron models have revitalized research efforts focusing on an immune basis for ALS. Other pathogenic mechanisms which have been the subject of recent research include elemental toxicity, apoptosis and programmed cell death and possibly a deficiency or abnormality in growth factors. Pathogenic processes for ALS must account for an increasing incidence of ALS, male preponderance, and the selective vulnerability of the corticomotoneuronal system.

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Platova, Yu A., and N. O. Zharinova. "MODERN DIAGNOSTICS OF AMYOTROPHIC LATERAL SCLEROSIS." Ulyanovsk Medico-biological Journal, no. 2 (July 15, 2020): 8–20. http://dx.doi.org/10.34014/2227-1848-2020-2-8-20.

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The purpose of this review is to systematize data on the diagnostics of amyotrophic lateral sclerosis (ALS), taking into account international practices in the application of various methods and their efficacy evaluation. For practical application research methods are divided into separate groups. Information from electronic libraries Pubmed, eLIBRARY and Elsiever was used as reference sources. Electromyography (EMG) is still the main method used in ALS diagnostics. It can be effectively combined with other tests. The combined use of ultrasound and EMG increases the number of patients with a reliably detected ALS. MRI allows the differential diagnosis of ALS with diseases that can feign the illness. Co-use of various neuroimaging methods can increase the accuracy of ALS diagnostics up to 90 %. The major part of sporadic and familial morbidity is associated with SOD1, C9orf72, TARDBP (TDP-43), and FUS gene mutations. There is still no consensus what mutations should be tested in patients during ALS diagnostics. A series of biochemical analyzes and tests for autoimmune diseases during ALS diagnostics is necessary for proper differential exclusion. Liquor test can be used to assess the neurofilament level and it is also an auxiliary method to diagnose and assess the disease development. Tissue biopsy, as an ALS diagnostic method, is rarely used due to its invasiveness; it is mainly administered in case of atypical symptoms. A promising method in ALS diagnostics is transcranial magnetic stimulation, which allows to fasten the diagnosis. However, at present this procedure is not included in the diagnostic criteria for ALS.Evaluation of respiratory and speech functions is necessary both in diagnosis and management of ALS patients. Thus, ALS patients require a multidisciplinary approach and combined diagnostic techniques for timely diagnosis. Keywords: amyotrophic lateral sclerosis, motor neuron disease, neurodegeneration, neuroimaging, electromyography, ALS diagnostics. Целью данного обзора является систематизация данных по диагностике бокового амиотрофического склероза (БАС) с учетом мирового опыта применения различных методов и оценка их эффективности. Методы исследований для удобства практического применения разбиты в статье на отдельные группы. В качестве источников информации использовались данные электронных библиотек Pubmed, eLIBRARY и Elsiever. Основным методом, применяемым в диагностике БАС, по-прежнему остается ЭМГ, которая может эффективно сочетаться с другими диагностическими процедурами. Совместное применение УЗИ и ЭМГ повышает число пациентов с достоверно установленным диагнозом БАС. Использование МРТ позволяет проводить дифференциальную диагностику БАС с заболеваниями, способными симулировать его картину. Совместное применение различных нейровизуализационных методов дает возможность увеличить точность диагностики БАС до 90 %. С мутациями генов SOD1, С9orf72, TARDBP (TDP-43) и FUS связана большая часть спорадической и семейной заболеваемости. До сих пор не достигнут консенсус по вопросу о том, на какие именно мутации необходимо обследовать пациентов при диагностике БАС. Проведение ряда биохимических анализов и исследований на наличие аутоиммунных заболеваний при постановке БАС является необходимым для надлежащей дифференциальной диагностики. Исследование ликвора может использоваться для оценки уровня нейрофиламентов и является вспомогательным методом диагностики и оценки прогрессирования заболевания. Биопсия тканей как метод диагностики БАС используется редко ввиду своей инвазивности; может применяться преимущественно при наличии нетипичных симптомов. Перспективным методом в диагностике БАС является транскраниальная магнитная стимуляция, позволяющая ускорить процесс постановки диагноза, однако на данный момент эта процедура не внесена в диагностические критерии БАС. Оценка функции дыхания и речи необходима как при постановке диагноза, так и при ведении пациентов с БАС. Таким образом, данная категория больных требует мультидисциплинарного подхода и совместного применения различных видов диагностики для своевременной постановки диагноза. Ключевые слова: боковой амиотрофический склероз, болезнь двигательного нейрона, нейродегенерация, нейровизуализация, электромиография, диагностика БАС.

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Leonov, G. A., A. S. Solomatinа, and A. O. Burshinov. "Difficulties in diagnosing amyotrophic lateral sclerosis with lumbosacral debut." NAUKA MOLODYKH (Eruditio Juvenium) 9, no. 3 (September 30, 2021): 463–70. http://dx.doi.org/10.23888/hmj202193463-470.

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Description of a clinical case of amyotrophic lateral sclerosis with lumbosacral debut. These data show certain difficulties for neurologists in correct diagnosing ALS or classifying it as a motor neuron disease. The differential diagnosis at admission was carried out with vertebrogenic cervical myelopathy, myasthenia gravis, and neural amyotrophy. During the collection of anamnesis, therapeutic and neurological examinations, evidence of motor neuron degeneration in the cervical-thoracic and lumbosacral parts of the spinal cord was revealed. We used auxiliary diagnostic methods (RCT of the spine and MRI of the brain, clinical and laboratory methods) to exclude other pathologies. The diagnosis of ALS is a verdict for the patient, since there is no effective treatment for the disease at the moment. CONCLUSION: The symptoms of ALS are largely similar to other potentially curable and / or benign diseases of the nervous system, so the patient's examination should be comprehensive, with the possible use of molecular genetic research methods.

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23

Chieia, Marco A., Acary S. B. Oliveira, Helga C. A. Silva, and Alberto Alain Gabbai. "Amyotrophic lateral sclerosis: considerations on diagnostic criteria." Arquivos de Neuro-Psiquiatria 68, no. 6 (December 2010): 837–42. http://dx.doi.org/10.1590/s0004-282x2010000600002.

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder, compromising the motor neuron, characterized by progressive muscle weakness, with reserved prognosis. The diagnosis is based on inclusion and exclusion clinical criteria, since there is no specific confirmation test. The objective of this research is to critically examine the main diagnosis instrument - El Escorial revisited, from the World Federation of Neurology (1998). Of the 540 patients with initial ALS diagnosis, either probable or definite, seen at UNIFESP-EPM, 190 underwent thorough investigation, following regular clinical and therapeutic treatment for over two years. Thirty patients (15.78%) had their diagnosis completely changed. The false-positive diagnoses were related to: early age, clinical presentation of symmetry, weakness greater than atrophy, symptomatic exacerbation. In addition, three patients with myasthenia gravis developed framework for ALS, suggesting the post-synaptic disability as a sign of early disease.

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Liguori, Francesco, Susanna Amadio, and Cinzia Volonté. "Where and Why Modeling Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 22, no. 8 (April 12, 2021): 3977. http://dx.doi.org/10.3390/ijms22083977.

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Over the years, researchers have leveraged a host of different in vivo models in order to dissect amyotrophic lateral sclerosis (ALS), a neurodegenerative/neuroinflammatory disease that is heterogeneous in its clinical presentation and is multigenic, multifactorial and non-cell autonomous. These models include both vertebrates and invertebrates such as yeast, worms, flies, zebrafish, mice, rats, guinea pigs, dogs and, more recently, non-human primates. Despite their obvious differences and peculiarities, only the concurrent and comparative analysis of these various systems will allow the untangling of the causes and mechanisms of ALS for finally obtaining new efficacious therapeutics. However, harnessing these powerful organisms poses numerous challenges. In this context, we present here an updated and comprehensive review of how eukaryotic unicellular and multicellular organisms that reproduce a few of the main clinical features of the disease have helped in ALS research to dissect the pathological pathways of the disease insurgence and progression. We describe common features as well as discrepancies among these models, highlighting new insights and emerging roles for experimental organisms in ALS.

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Layalle, Sophie, Laetitia They, Sarah Ourghani, Cédric Raoul, and Laurent Soustelle. "Amyotrophic Lateral Sclerosis Genes in Drosophila melanogaster." International Journal of Molecular Sciences 22, no. 2 (January 18, 2021): 904. http://dx.doi.org/10.3390/ijms22020904.

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Amyotrophic lateral sclerosis (ALS) is a devastating adult-onset neurodegenerative disease characterized by the progressive degeneration of upper and lower motoneurons. Most ALS cases are sporadic but approximately 10% of ALS cases are due to inherited mutations in identified genes. ALS-causing mutations were identified in over 30 genes with superoxide dismutase-1 (SOD1), chromosome 9 open reading frame 72 (C9orf72), fused in sarcoma (FUS), and TAR DNA-binding protein (TARDBP, encoding TDP-43) being the most frequent. In the last few decades, Drosophila melanogaster emerged as a versatile model for studying neurodegenerative diseases, including ALS. In this review, we describe the different Drosophila ALS models that have been successfully used to decipher the cellular and molecular pathways associated with SOD1, C9orf72, FUS, and TDP-43. The study of the known fruit fly orthologs of these ALS-related genes yielded significant insights into cellular mechanisms and physiological functions. Moreover, genetic screening in tissue-specific gain-of-function mutants that mimic ALS-associated phenotypes identified disease-modifying genes. Here, we propose a comprehensive review on the Drosophila research focused on four ALS-linked genes that has revealed novel pathogenic mechanisms and identified potential therapeutic targets for future therapy.

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Furtado Bastos, Aline, Marco Orsini, Dionis Machado, Mariana Pimentel Mello, Sergio Nader, Júlio Guilherme Silva, Antonio Marcos Da Silva Catharino, et al. "Amyotrophic lateral sclerosis: one or multiple causes?" Neurology International 3, no. 1 (April 29, 2011): 4. http://dx.doi.org/10.4081/ni.2011.e4.

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The Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease in the adulthood, and it is characterized by rapid and progressive compromise of the upper and lower motor neurons. The majority of the cases of ALS are classified as sporadic and, until now, a specific cause for these cases still is unknown. To present the different hypotheses on the etiology of ALS. It was carried out a search in the databases: Bireme, Scielo and Pubmed, in the period of 1987 to 2011, using the following keywords: Amyotrophic lateral sclerosis, motor neuron disease, etiology, causes and epidemiology and its similar in Portuguese and Spanish. It did not have consensus as regards the etiology of ALS. Researches demonstrates evidences as regards intoxication by heavy metals, environmental and occupational causes, genetic mutations (superoxide dismutase 1), certain viral infections and the accomplishment of vigorous physical activity for the development of the disease. There is still no consensus regarding the involved factors in the etiology of ALS. In this way, new research about these etiologies are necessary, for a better approach of the patients, promoting preventive programs for the disease and improving the quality of life of the patients.

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Ricci, Claudia, Carlotta Marzocchi, and Stefania Battistini. "MicroRNAs as Biomarkers in Amyotrophic Lateral Sclerosis." Cells 7, no. 11 (November 20, 2018): 219. http://dx.doi.org/10.3390/cells7110219.

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Amyotrophic lateral sclerosis (ALS) is an incurable and fatal disorder characterized by the progressive loss of motor neurons in the cerebral cortex, brain stem, and spinal cord. Sporadic ALS form accounts for the majority of patients, but in 1–13.5% of cases the disease is inherited. The diagnosis of ALS is mainly based on clinical assessment and electrophysiological examinations with a history of symptom progression and is then made with a significant delay from symptom onset. Thus, the identification of biomarkers specific for ALS could be of a fundamental importance in the clinical practice. An ideal biomarker should display high specificity and sensitivity for discriminating ALS from control subjects and from ALS-mimics and other neurological diseases, and should then monitor disease progression within individual patients. microRNAs (miRNAs) are considered promising biomarkers for neurodegenerative diseases, since they are remarkably stable in human body fluids and can reflect physiological and pathological processes relevant for ALS. Here, we review the state of the art of miRNA biomarker identification for ALS in cerebrospinal fluid (CSF), blood and muscle tissue; we discuss advantages and disadvantages of different approaches, and underline the limits but also the great potential of this research for future practical applications.

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Gunton, Adrianna, Gregory Hansen, and Kerri Lynn Schellenberg. "Photovoice as a Participatory Research Tool in Amyotrophic Lateral Sclerosis." Journal of Neuromuscular Diseases 8, no. 1 (January 1, 2021): 91–99. http://dx.doi.org/10.3233/jnd-200537.

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Background: Photovoice is a qualitative research tool increasingly utilized in the healthcare field to understand the illness experience from the patient and caregiver perspective. This is the first study to evaluate photovoice in the context of amyotrophic lateral sclerosis (ALS). Objective: A patient and caregiver centered research tool was utilized to gain a greater understanding of challenges faced when living with ALS. Methods: Eight patients and three corresponding caregivers participating by taking photographs, writing descriptive text, and participating in individual and group interviews. Inductive thematic analysis was employed to uncover recurring themes. Results: Five main themes were identified; 1) facing the diagnosis, 2) loss of function, 3) isolation, 4) health system challenges, and 5) hope. Despite the devasting impact of ALS, the majority of participants reported a surprising amount of positivity in the face of receiving this difficult diagnosis, and demonstrated incredible creativity and adaptability to meet the ensuing loss of function. However, patients and caregivers discussed feelings of isolation and health care system challenges. The importance of hope was a strong and recurring theme. Conclusions: The photovoice research tool demonstrates the profound resilience of these participants, and challenges the medical community to find ways of fostering positivity and hope throughout the ALS disease course. Further clinic and community resources, education, and supports are needed to combat the sense of isolation and health care system challenges experienced by patients and their caregivers.

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Hegde, Krupa N., and Ajay Srivastava. "Drosophila melanogaster as a Tool for Amyotrophic Lateral Sclerosis Research." Journal of Developmental Biology 10, no. 3 (August 30, 2022): 36. http://dx.doi.org/10.3390/jdb10030036.

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Reliable animal model systems are an integral part of biological research. Ever since Thomas Hunt Morgan won a Nobel Prize for genetic work done using the fruit fly (Drosophila melanogaster) as a model organism, it has played a larger and more important role in genetic research. Drosophila models have long been used to study neurodegenerative diseases and have aided in identifying key disease progression biological pathways. Due to the availability of a vast array of genetic manipulation tools, its relatively short lifespan, and its ability to produce many progenies, D. melanogaster has provided the ability to conduct large-scale genetic screens to elucidate possible genetic and molecular interactions in neurodegenerative diseases such as Alzheimer’s disease, Parkinson’s disease, Huntington’s Disease, and Amyotrophic Lateral Sclerosis (ALS). With regards to ALS, many of the gene mutations that have been discovered to be linked to the disease have been modeled in Drosophila to provide a look into a detailed model of pathogenesis. The aim of this review is to summarize key and newer developments in ALS research that have utilized Drosophila and to provide insight into the profound use of Drosophila as a tool for modeling this disease.

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PARTON, M. "Amyotrophic Lateral Sclerosis: A Synthesis of Research and Clinical Practice." Journal of Neurology, Neurosurgery & Psychiatry 66, no. 4 (April 1, 1999): 554a. http://dx.doi.org/10.1136/jnnp.66.4.554a.

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England, John D. "Amyotrophic Lateral Sclerosis. A Synthesis of Research and Clinical Practice." Journal of Clinical Neurophysiology 16, no. 3 (May 1999): 296. http://dx.doi.org/10.1097/00004691-199905000-00007.

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Rowland, Lewis P. "Six important themes in amyotrophic lateral sclerosis (ALS) research, 1999." Journal of the Neurological Sciences 180, no. 1-2 (November 2000): 2–6. http://dx.doi.org/10.1016/s0022-510x(00)00428-7.

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33

Kelly, J. J. "Amyotrophic Lateral Sclerosis: A Synthesis of Research and Clinical Practice." Archives of Neurology 56, no. 11 (November 1, 1999): 1418. http://dx.doi.org/10.1001/archneur.56.11.1418.

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Denys, Eric H. "Amyotrophic lateral sclerosis. A synthesis of research and clinical practice." Muscle & Nerve 22, no. 6 (June 1999): 783. http://dx.doi.org/10.1002/(sici)1097-4598(199906)22:6<783::aid-mus22>3.0.co;2-l.

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Munsat, Theodore L. "Amyotrophic lateral sclerosis: A synthesis of research and clinical practice." Annals of Neurology 46, no. 1 (July 1999): 140. http://dx.doi.org/10.1002/1531-8249(199907)46:1<140::aid-ana30>3.0.co;2-2.

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Pagani, Mario Rafael, Laura Elisabeth Gonzalez, and Osvaldo Daniel Uchitel. "Autoimmunity in Amyotrophic Lateral Sclerosis: Past and Present." Neurology Research International 2011 (2011): 1–11. http://dx.doi.org/10.1155/2011/497080.

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Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting particularly motor neurons for which no cure or effective treatment is available. Although the cause of ALS remains unknown, accumulative evidence suggests an autoimmune mechanism of pathogenesis. In this paper, we will summarize the current research related to autoimmunity in the sporadic form of ALS and discuss the potential underlying pathogenic mechanisms and perspectives. Presented data supports the view that humoral immune responses against motor nerve terminals can initiate a series of physiological changes leading to alteration of calcium homeostasis. In turn, loss of calcium homeostasis may induce neuronal death through apoptotic signaling pathways. Additional approaches identifying specific molecular features of this hypothesis are required, which will hopefully allow us to develop techniques of early diagnosis and effective therapies.

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Goldberg, Michael E. "The neurology clinic needs monkey research." Proceedings of the National Academy of Sciences 116, no. 52 (December 23, 2019): 26255–58. http://dx.doi.org/10.1073/pnas.1907759116.

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This report discusses how a number of currently incurable diseases might be treated by advances developed as the result of current ongoing research on monkeys. The diseases discussed include Parkinson's disease, amyotrophic lateral sclerosis, spinal cord injury, peripheral neuropathy, and stroke. Finally, the report discusses the devastating effect the animal rights movement and adverse publicity can have on basic neurobiological research on monkeys.

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Parekh, Bhavin. "A(a)LS: Ammonia-induced amyotrophic lateral sclerosis." F1000Research 4 (May 14, 2015): 119. http://dx.doi.org/10.12688/f1000research.6364.1.

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Amyotrophic lateral sclerosis (ALS) is a dreadful, devastating and incurable motor neuron disease. Aetiologically, it is a multigenic, multifactorial and multiorgan disease. Despite intense research, ALS pathology remains unexplained. Following extensive literature review, this paper posits a new integrative explanation. This framework proposes that ammonia neurotoxicity is a main player in ALS pathogenesis. According to this explanation, a combination of impaired ammonia removal— mainly because of impaired hepatic urea cycle dysfunction—and increased ammoniagenesis— mainly because of impaired glycolytic metabolism in fast twitch skeletal muscle—causes chronic hyperammonia in ALS. In the absence of neuroprotective calcium binding proteins (calbindin, calreticulin and parvalbumin), elevated ammonia—a neurotoxin—damages motor neurons. Ammonia-induced motor neuron damage occurs through multiple mechanisms such as macroautophagy-endolysosomal impairment, endoplasmic reticulum (ER) stress, CDK5 activation, oxidative/nitrosative stress, neuronal hyperexcitability and neuroinflammation. Furthermore, the regional pattern of calcium binding proteins’ loss, owing to either ER stress and/or impaired oxidative metabolism, determines clinical variability of ALS. Most importantly, this new framework can be generalised to explain other neurodegenerative disorders such as Huntington’s disease and Parkinsonism.

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Skaper, Stephen D. "Commentary((Research Highlights)(Amyotrophic Lateral Sclerosis: Targeting the Body’s Energy Engine))." CNS & Neurological Disorders - Drug Targets 12, no. 2 (April 1, 2013): 294. http://dx.doi.org/10.2174/1871527311312020015.

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Barnes, Stephanie L., and Neil G. Simon. "Clinical and research applications of neuromuscular ultrasound in amyotrophic lateral sclerosis." Degenerative Neurological and Neuromuscular Disease Volume 9 (July 2019): 89–102. http://dx.doi.org/10.2147/dnnd.s215318.

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Contestabile, A. "Amyotrophic Lateral Sclerosis: From Research to Therapeutic Attempts and Therapeutic Perspectives." Current Medicinal Chemistry 18, no. 36 (December 1, 2011): 5655–65. http://dx.doi.org/10.2174/092986711798347289.

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Mancuso, Renzo, and Xavier Navarro. "Amyotrophic lateral sclerosis: Current perspectives from basic research to the clinic." Progress in Neurobiology 133 (October 2015): 1–26. http://dx.doi.org/10.1016/j.pneurobio.2015.07.004.

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Kabashi, Edor, Paul N. Valdmanis, Hussein Daoud, Véronique V. Belzil, Patrick A. Dion, and Guy A. Rouleau. "TDP–43, Protein Aggregation, and Amyotrophic Lateral Sclerosis." US Neurology 05, no. 02 (2010): 35. http://dx.doi.org/10.17925/usn.2010.05.02.35.

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Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disorder and the third most common neurodegenerative cause of adult death after Alzheimers and Parkinsons diseases. TAR DNA binding protein (TDP-43) has been found to be a major component of inclusion bodies in motor neurons of ALS patients. Inclusion bodies are protein aggregates considered a pathological hallmark of neurodegeneration. Our group and eight independent research groups screened TDP-43 for mutations. Overall, 19 missense mutations and one truncating mutation were identified only in ALS patients. These mutations were not found in a considerable number of controls sequenced in several independent studies. Ten of these mutations were found in patients with a family history of ALS. To further support a pathogenic role, the mutations segregated with the disease in three families, including one family with a significant logarithm of odds (LOD) score. Nineteen of these 20 mutations are located in the C-terminus of the TDP-43 protein. These mutations could disrupt several of the already known functions of TDP-43, including nuclear localization, exon splicing, and RNA and heterogenous ribonucleoprotein particle (hnRNP) binding, or could introduce a novel gain of function that is toxic to motor neurons. In the future, animal and cellular models using these mutations should elucidate the role of TDP-43 mutations in ALS pathogenesis and could provide new means to test pharmaceutical compounds for this neurological disease.

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Zhao, Jiantao, Xuemei Wang, Zijun Huo, Yanchun Chen, Jinmeng Liu, Zhenhan Zhao, Fandi Meng, et al. "The Impact of Mitochondrial Dysfunction in Amyotrophic Lateral Sclerosis." Cells 11, no. 13 (June 28, 2022): 2049. http://dx.doi.org/10.3390/cells11132049.

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Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and highly fatal neurodegenerative disease. Although the pathogenesis of ALS remains unclear, increasing evidence suggests that a key contributing factor is mitochondrial dysfunction. Mitochondria are organelles in eukaryotic cells responsible for bioenergy production, cellular metabolism, signal transduction, calcium homeostasis, and immune responses and the stability of their function plays a crucial role in neurons. A single disorder or defect in mitochondrial function can lead to pathological changes in cells, such as an impaired calcium buffer period, excessive generation of free radicals, increased mitochondrial membrane permeability, and oxidative stress (OS). Recent research has also shown that these mitochondrial dysfunctions are also associated with pathological changes in ALS and are believed to be commonly involved in the pathogenesis of the disease. This article reviews the latest research on mitochondrial dysfunction and its impact on the progression of ALS, with specific attention to the potential of novel therapeutic strategies targeting mitochondrial dysfunction.

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Štětkářová, Ivana, and Edvard Ehler. "Diagnostics of Amyotrophic Lateral Sclerosis: Up to Date." Diagnostics 11, no. 2 (February 3, 2021): 231. http://dx.doi.org/10.3390/diagnostics11020231.

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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by gradual loss of upper and lower motor neurons and their pathways, usually without affecting the extraocular and sphincter muscles. The cause of the disease is not yet known. It is a chain of subsequent events, ending in programmed cell death in selective neuronal subpopulations. The prognosis for survival is rather short with a median of 2 to 4 years. Survival may be prolonged based on prompt diagnosis, ALS subtype and proper management with supportive treatment (tracheostomy, gastrostomy, etc.). According to the clinical picture, the typical form of ALS with upper and lower motoneuron involvement and progressive bulbar paralysis with bulbar muscle involvement is observed. The ALS form with progressive muscle atrophy, where only the lower motoneuron is affected, and primary lateral sclerosis with only upper motoneuron damage are rare. Familiar forms of ALS (FALS) associated with specific genes (the most common is C9orf72) have been discovered. FALS is usually associated with dementia (frontotemporal lobar dementia, FTLD), behavioral disorders, cognitive dysfunction and impairment of executive functions. The diagnosis of ALS is determined by excluding other conditions and utilizing clinical examinations, laboratory and genetic tests and nerve conduction/needle electromyography studies (EMG). Needle EMG records abnormal activities at rest and looks for neurogenic patterns during muscle contraction. Motor evoked potentials after transcranial magnetic stimulation remain the test of choice to identify impairment of upper motor neurons. New biochemical, neurophysiological and morphological biomarkers are extensively studied as early diagnostic and prognostic factors and have implications for clinical trials, research and drug development.

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Areprintseva, Daria K., and Mansur A. Kutlubaev. "Cognitive and behavioral changes in amyotrophic lateral sclerosis." Neurology Bulletin LIV, no. 3 (November 4, 2022): 27–32. http://dx.doi.org/10.17816/nb109965.

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BACKGROUND. Amyotrophic lateral sclerosis (ALS) is not limited only to motor impairment, and can also manifest with cognitive and behavioral disorders. Currently these presentations of ALS are unrecognized. Hence the research of ALS variants with cognitive and behavioral impairments is necessary. AIM. To study the frequency and clinical characteristics of ALS variants with cognitive and behavioral impairments. MATERIAL AND METHODS. The patients were recruited in 20202021. All patients with definite and possible ALS according to El Escorial criteria were included. The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) was used to assess cognitive and behavioral changes. 44 patients, 26 males and 18 females, aged 39 to 77 years (median of age 61 years, interquartile range 53.574.5). Statistical analysis was performed using IBM SPSS Statistics 21. Continuous data were analyzed using the MannWhitney test, categorical data was analyzed by exact Fisher criteria. A p-value less than 0.05 was statistically significant. RESULTS. In 43.2% of patients cognitive and/or behavioral changes were observed. The majority of patients (approximately 20%) suffered from a variant of ALS with cognitive impairment, which was mostly presented with executive and social cognitive dysfunction. ALS with behavioral impairment was observed at 9% of patients, which mostly presented with apathy and disinhibition. A combination of cognitive and behavioral impairment was registered in 7% of patients. In another 7% of patients severity of cognitive and behavioral impairment reached a degree of dementia and corresponded to frontotemporal degeneration. CONCLUSION. This cognitive and behavioral impairment is observed in a substantial number of patients with ALS and mostly presented with moderate executive and social cognitive dysfunction, as well as apathy and less often disinhibition.

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Ravnik-Glavač, Metka, and Damjan Glavač. "Circulating RNAs as Potential Biomarkers in Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 21, no. 5 (March 3, 2020): 1714. http://dx.doi.org/10.3390/ijms21051714.

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Amyotrophic lateral sclerosis (ALS) is a complex multi-system neurodegenerative disorder with currently limited diagnostic and no therapeutic options. Despite the intense efforts no clinically applicable biomarkers for ALS are yet established. Most current research is thus focused, in particular, in identifying potential non-invasive circulating biomarkers for more rapid and accurate diagnosis and monitoring of the disease. In this review, we have focused on messenger RNA (mRNA), non-coding RNAs (lncRNAs), micro RNAs (miRNAs) and circular RNA (circRNAs) as potential biomarkers for ALS in peripheral blood serum, plasma and cells. The most promising miRNAs include miR-206, miR-133b, miR-27a, mi-338-3p, miR-183, miR-451, let-7 and miR-125b. To test clinical potential of this miRNA panel, a useful approach may be to perform such analysis on larger multi-center scale using similar experimental design. However, other types of RNAs (lncRNAs, circRNAs and mRNAs) that, together with miRNAs, represent RNA networks, have not been yet extensively studied in blood samples of patients with ALS. Additional research has to be done in order to find robust circulating biomarkers and therapeutic targets that will distinguish key RNA interactions in specific ALS-types to facilitate diagnosis, predict progression and design therapy.

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Hardiman, Orla. "Taking Genetics of Amyotrophic Lateral Sclerosis to Next Steps." US Neurology 11, no. 02 (2015): 108. http://dx.doi.org/10.17925/usn.2015.11.02.108.

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Great advances have been made in understanding the genetics of amyotrophic lateral sclerosis, but we are really merely at the end of the beginning. Many new genes have been discovered, but these need to be contextualized within populations and across different phenotypes. Increased international collaboration through whole-genome sequencing initiatives, such as Project MinE (www.projectMinE.com), combined with detailed clinical phenotyping is most likely to drive future research in disease pathogenesis and new drug development

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Menounos, Spiro, Philip M. Hansbro, Ashish D. Diwan, and Abhirup Das. "Pathophysiological Correlation between Cigarette Smoking and Amyotrophic Lateral Sclerosis." NeuroSci 2, no. 2 (April 20, 2021): 120–34. http://dx.doi.org/10.3390/neurosci2020008.

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Cigarette smoke (CS) has been consistently demonstrated to be an environmental risk factor for amyotrophic lateral sclerosis (ALS), although the molecular pathogenic mechanisms involved are yet to be elucidated. Here, we propose different mechanisms by which CS exposure can cause sporadic ALS pathogenesis. Oxidative stress and neuroinflammation are widely implicated in ALS pathogenesis, with blood–spinal cord barrier disruption also recognised to be involved in the disease process. In addition, immunometabolic, epigenetic and microbiome alterations have been implicated in ALS recently. Identification of the underlying pathophysiological mechanisms that underpin CS-associated ALS will drive future research to be conducted into new targets for treatment.

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Verleger, Rolf. "Malfunctions of Central Control of Movement Studied with Slow Brain Potentials in Neurological Patients." Journal of Psychophysiology 18, no. 2/3 (January 2004): 105–20. http://dx.doi.org/10.1027/0269-8803.18.23.105.

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Abstract Studies are reviewed that used movement-related EEG potentials to investigate impairments of movement control in neurological patients. The EEG potentials reviewed are the Bereitschaftspotential (BP), Contingent Negative Variation (CNV), and components of the lateralized readiness potential (LRP). Patient groups included in this review are patients with infarction of the middle cerebral artery, Parkinson's disease, cerebellar disease, and amyotrophic lateral sclerosis. A rich body of evidence has been collected on Parkinson's disease, and somewhat less on cerebellar atrophy, contributing to an understanding of the impairments caused by these diseases. In contrast, not much research has been done in amyotrophic lateral sclerosis and in infarction patients. The latter is particularly striking since utility of this method for assessing residual capacities of affected motor areas seems rather obvious.

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