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Journal articles on the topic 'Amyotropic Lateral Sclerosis (ALS)'

Author: Grafiati

Published: 4 June 2021

Last updated: 26 July 2025

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1

Brauer, Sandra. "Amyotrophic lateral sclerosis (ALS)." Journal of Physiotherapy 59, no. 1 (2013): 61. http://dx.doi.org/10.1016/s1836-9553(13)70155-3.

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Kumar, Ravi. "Amyotrophic Lateral Sclerosis: Innovative Therapies for ALS under the Pipeline." Neurology & Neurotherapy Open Access Journal 7, no. 1 (2022): 1–9. http://dx.doi.org/10.23880/nnoaj-16000166.

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Over the last 5 decades, a multiple of experimental drugs compounds have been shown to dissuade disease progression in preclinical animal models of amyotrophic lateral sclerosis (ALS) but failed to show any efficacy in human clinical trials or are still waiting for approval under Phase I–III trials. Only 2 main drug compounds are discovered till date and approved by USA Food and Drug Administration for ALS treatment that show better efficacy, effective against ALS progression in early stages and enhances the survival rate of patients. The riluzole is a glutamatergic neurotransmission inhibitor

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Cividini, Camilla, Silvia Basaia, Edoardo G. Spinelli, et al. "Amyotrophic Lateral Sclerosis–Frontotemporal Dementia." Neurology 98, no. 4 (2021): e402-e415. http://dx.doi.org/10.1212/wnl.0000000000013123.

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Background and ObjectivesA significant overlap between amyotrophic lateral sclerosis (ALS) and behavioral variant of frontotemporal dementia (bvFTD) has been observed at clinical, genetic, and pathologic levels. Within this continuum of presentations, the presence of mild cognitive or behavioral symptoms in patients with ALS has been consistently reported, although it is unclear whether this is to be considered a distinct phenotype or rather a natural evolution of ALS. Here, we used mathematical modeling of MRI connectomic data to decipher common and divergent neural correlates across the ALS–

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Orsini, Marco, Antônio Marcos da Silva Catharino, Valéria Camargo Silveira, Carlos Henrique Melo Reis, Marcos RG de Freitas, and Acary Bulle de Oliveira. "Pseudopolyneuritic form of amyotrophic lateral sclerosis: Marie-Patrikios type." International Journal of Case Reports and Images 13, no. 2 (2022): 118–21. http://dx.doi.org/10.5348/101340z01mo2022cr.

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Introduction: Amyotrophic lateral sclerosis (ALS), also called motor neuron disease (MND), is a progressive, neurodegenerative, and inexorable disease that affects the neurons of the anterior horn of the spinal cord, as well as the lateral funiculus. A rare variant of ALS was first described in 1918 by Patrikios and Marie, called the pseudopolyneuritic form or Marie-Patrikios disease. It is characterized by an initial manifestation with melting of the feet, distal weakness of the muscles of the anterior compartment of the leg, and absence of the Achilles tendon reflex. We present an atypical c

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5

Elíasdóttir, Ólöf. "Antisense oliconucleotides-potential treatment for familial Amyotrophic lateral sclerosis (ALS)." Laeknabladid 111, no. 07/08 (2025): 311. https://doi.org/10.17992/lbl.2025.0708.846.

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Geevasinga, Nimeshan, James Howells, Parvathi Menon, et al. "Amyotrophic lateral sclerosis diagnostic index." Neurology 92, no. 6 (2019): e536-e547. http://dx.doi.org/10.1212/wnl.0000000000006876.

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ObjectiveThe aim of the study was to assess the utility of a novel amyotrophic lateral sclerosis (ALS) diagnostic index (ALSDI).MethodsA prospective multicenter study was undertaken on patients presenting with suspected ALS. The reference standard (Awaji criteria) was applied to all patients at recruitment. Patients were randomly assigned to a training (75%) and a test (25%) cohort. The ALSDI was developed in the training cohort and its diagnostic utility was subsequently assessed in the test cohort.ResultsA total of 407 patients were recruited, with 305 patients subsequently diagnosed with AL

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Zwicker, Jocelyn, Danial Qureshi, Robert Talarico, et al. "Dying of amyotrophic lateral sclerosis." Neurology 93, no. 23 (2019): e2083-e2093. http://dx.doi.org/10.1212/wnl.0000000000008582.

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ObjectiveTo describe health care service utilization and cost for decedents with and without amyotrophic lateral sclerosis (ALS) in the last year of life.MethodsUsing linked health administrative data, we conducted a retrospective, population-based cohort study of Ontario, Canada, decedents from 2013 to 2015. We examined demographic data, rate of utilization, and cost of health care services in the last year of life.ResultsWe identified 283,096 decedents in Ontario, of whom 1,212 (0.42%) had ALS. Decedents with ALS spent 3 times as many days in an intensive care unit (ICU) (mean 6.3 vs 2.1, p

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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 (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 F

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Leonardo, Wiranata Soesilopranoto, Sekundus Torrys Yesyurun, Martianus Randy, Istri Pramitasuri Tjokorda, Ayu Sri Wijayanti Ida, and Putu Eka Widyadharma I. "Neurofilament Light Chain Measurement with Electrochemiluminescence Method (NFL-ECL) in Cerebrospinal Fluid as Promising Diagnostic Method of Amyotrophic Lateral Sclerosis: A Literature Review." International Journal of Medical Reviews and Case Reports 2, no. 3 (2018): 53–57. https://doi.org/10.5455/IJMRCR.nfl-ecl-amyotrophic-lateral-sclerosis.

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ALS is one of the neurodegenerative diseases that have high morbidity and mortality. Beside difficulties to manage, it also has a limited diagnostic method to detect ALS in early onset. Neurofilament Light Chain (NFL) is a component of cerebrospinal fluid (CSF) which regulate axon diameter calibration and conduction speed in action potential of neurons. In this review, several literatures were evaluated and selected. The NFL measurement with ECL method has high sensitivity and specificity, that makes this method reliable to use in the diagnosis of ALS.

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10

Verhey, F. R. J., F. W. Vreeling, and J. Jolles. "Dementia and Amyotrophic Lateral Sclerosis." Acta Neuropsychiatrica 4, no. 1 (1992): 17–20. http://dx.doi.org/10.1017/s0924270800034967.

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SummaryDementia and Amyotrophic Lateral SclerosisThe case-histories of two patients are presented with Amyo-trofic Lateral Sclerosis and dementia (ALS-D), followed by a discussion of recent literature on this topic. This condition can be considered as the interface between non-Alzheimer frontal lobe dementia and amyotrophic lateral sclerosis. The nosological classification of the ALS-D complex has not been established yet.

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Alekseeva, T. M., N. V. Skripchenko, S. V. Lobzin, et al. "Difficulties in diagnosing amyotrophic lateral sclerosis in a HIV-Positive Patient." Journal Infectology 10, no. 4 (2018): 139–44. http://dx.doi.org/10.22625/2072-6732-2018-10-4-139-144.

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We described a case of amyotrophic lateral sclerosis (ALS) with comorbid HIV infection. The diagnosis was confirmed by genetic tests. The difficulty of the differential diagnosis between amyotrophic lateral sclerosis and HIV-associated ALS syndrome is discussed.

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Dilliott, Allison A., Catherine M. Andary, Meaghan Stoltz, Andrey A. Petropavlovskiy, Sali M. K. Farhan, and Martin L. Duennwald. "DnaJC7 in Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 23, no. 8 (2022): 4076. http://dx.doi.org/10.3390/ijms23084076.

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Protein misfolding is a common basis of many neurodegenerative diseases including amyotrophic lateral sclerosis (ALS). Misfolded proteins, such as TDP-43, FUS, Matrin3, and SOD1, mislocalize and form the hallmark cytoplasmic and nuclear inclusions in neurons of ALS patients. Cellular protein quality control prevents protein misfolding under normal conditions and, particularly, when cells experience protein folding stress due to the fact of increased levels of reactive oxygen species, genetic mutations, or aging. Molecular chaperones can prevent protein misfolding, refold misfolded proteins, or

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13

Chen, Anton, and C. Gaelyn Garrett. "Otolaryngologic presentations of amyotrophic lateral sclerosis." Otolaryngology–Head and Neck Surgery 132, no. 3 (2005): 500–504. http://dx.doi.org/10.1016/j.otohns.2004.09.092.

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OBJECTIVES/HYPOTHESIS: To determine the incidence of voice disturbance as a presenting symptom of amyotrophic lateral sclerosis (ALS) and describe laryngologic features of ALS. STUDY DESIGN: Retrospective review. METHODS: Records of patients with voice disturbance at a voice center and ALS patients at a neurology clinic were reviewed from January 1998 to March 2003. RESULTS: 15 of 1759 patients with voice disturbance were later diagnosed with ALS. Of 220 ALS patients presenting to neurology clinic, 44 had bulbar symptoms and 19 had initially presented to an otolaryngologist. Dysarthria, dyspha

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Carmine, Ungaro, and Sprovieri Teresa. "Mercury toxicity and amyotrophic lateral sclerosis." Journal of Neuroscience and Neurological Disorders 7, no. 1 (2023): 011–13. http://dx.doi.org/10.29328/journal.jnnd.1001074.

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Recent clinical, experimental and epidemiological studies report that ALS is thought possibly due to a multi-stage process, arising from a combination of genetic susceptibility and environmental factors, which alone or superimposed, perhaps on genetic polymorphism yet to be identified, may contribute to the incidence rate of sporadic ALS. In particular, a large amount of evidence suggests that mercury is toxic to motor neurons and may be a risk factor for ALS, playing a part in its pathogenesis. In fact, there have been case reports of ALS or ALS-like symptoms associated with mercury exposure,

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Sauter, W. F., G. Bush, and J. Sommerville. "A single case study: myoelectrically controlled exoskeletal mobilizer for amyotrophic lateral sclerosis (ALS) patients." Prosthetics and Orthotics International 13, no. 3 (1989): 145–48. http://dx.doi.org/10.3109/03093648909079423.

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Introduction and clinical pathology of Amyotrophic Lateral Sclerosis (ALS) Amyotrophic Lateral Sclerosis (ALS) also known as Lou Gehrig's disease is the generic name for progressive muscular atrophy and bulbar palsy. It refers to all disorders of the cortico spinal pathways which are characterized by progressive muscle weakness. After multiple sclerosis, ALS is the most common purely neurological disorder (Janiszewski et al. 1983). Information available from the ALS Society indicates that the most common age of onset is 55 years.

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Oprisan, Alexandra L., and Bogdan Ovidiu Popescu. "Dysautonomia in Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 24, no. 19 (2023): 14927. http://dx.doi.org/10.3390/ijms241914927.

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, characterized in its typical presentation by a combination of lower and upper motor neuron symptoms, with a progressive course and fatal outcome. Due to increased recognition of the non-motor symptoms, it is currently considered a multisystem disorder with great heterogeneity, regarding genetical, clinical, and neuropathological features. Often underestimated, autonomic signs and symptoms have been described in patients with ALS, and various method analyses have been used to assess autonomic nervous system involvement. The aim

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Vishwas, Sukriti, Bushra Bashir, Nikhil B. Khandale, Sachin Kumar Singh, and Uma Devi. "Persea Americana’s Extract Could Offer Effective Treatment for Amyotrophic Lateral Sclerosis: A Hypothesis." Journal of Pharmaceutical Technology, Research and Management 11, no. 2 (2023): 125–31. http://dx.doi.org/10.15415/jprtm.2023.112005.

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Background: Amyotrophic lateral sclerosis (ALS) is a rare neuro-degenerative disease characterized by the degeneration of upper and lower motor neurons in the central and peripheral nervous systems. Several pathological conditions, including neuroinflammation, oxidative stress, and mitochondrial dysfunction, contribute to the degeneration of motor neurons. Despite extensive research, no current drug therapies have proven effective in treating ALS. Purpose: This study explores the therapeutic potential of Persea americana (PA) Mill, commonly known as avocado, in the treatment of ALS. Methods: T

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Vishwas, Sukriti, Bushra Bashir, Nikhil B. Khandale, Sachin Kumar Singh, and Uma Devi. "Persea Americana’s Extract Could Offer Effective Treatment for Amyotrophic Lateral Sclerosis: A Hypothesis." Journal of Pharmaceutical Technology, Research and Management 11, no. 2 (2023): 125–31. http://dx.doi.org/10.15415/jptrm.2023.112005.

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Background: Amyotrophic lateral sclerosis (ALS) is a rare neuro-degenerative disease characterized by the degeneration of upper and lower motor neurons in the central and peripheral nervous systems. Several pathological conditions, including neuroinflammation, oxidative stress, and mitochondrial dysfunction, contribute to the degeneration of motor neurons. Despite extensive research, no current drug therapies have proven effective in treating ALS. Purpose: This study explores the therapeutic potential of Persea americana (PA) Mill, commonly known as avocado, in the treatment of ALS. Methods: T

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19

Nascimento, Osvaldo JM, Marco Orsini, Camila Pupe, et al. "Amyotrophic lateral sclerosis with sensitive findings." Revista Neurociências 18, no. 3 (2001): 320–23. http://dx.doi.org/10.34024/rnc.2010.v18.8469.

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Introduction. Classical amyotrophic lateral sclerosis (ALS) is not hard to diagnose, but when it comes to atypical forms of motor neuron disease (MND) which account for about 20% in clinical setting, we may face some difficulties in differentiating clearly between atypical forms of ALS/MND and other non-ALS diseases, such as multifocal motor neuropathy, chronic inflammatory demyelinating polyneuropathy and cervical spondylosis. Association between neuropathy and ALS has been reported rarely. Method. We report a patient who presented with clinical/electrophysiological features and investigation

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20

Roggenbuck, Jennifer, Kelly A. Rich, Leah Vicini, et al. "Amyotrophic Lateral Sclerosis Genetic Access Program." Neurology Genetics 7, no. 5 (2021): e615. http://dx.doi.org/10.1212/nxg.0000000000000615.

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ObjectiveTo report the frequency of amyotrophic lateral sclerosis (ALS) genetic variants in a nationwide cohort of clinic-based patients with ALS with a family history of ALS (fALS), dementia (dALS), or both ALS and dementia (fALS/dALS).MethodsA multicenter, prospective cohort of 573 patients with fALS, dALS, or fALS/dALS, underwent genetic testing in the ALS Genetic Access Program (ALS GAP), a clinical program for clinics of the Northeast ALS Consortium. Patients with dALS underwent C9orf72 hexanucleotide repeat expansion (HRE) testing; those with fALS or fALS/dALS underwent C9orf72 HRE testi

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Kutlubaev, M. A., D. K. Areprintceva, E. V. Pervushina, and L. V. Brylev. "Cognitive disorders in amyotrophic lateral sclerosis." Neurology, Neuropsychiatry, Psychosomatics 15, no. 2 (2023): 68–74. http://dx.doi.org/10.14412/2074-2711-2023-2-68-74.

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Cognitive disorders (CD) are observed in more than half of patients with amyotrophic lateral sclerosis (ALS), but rarely reach the degree of dementia. Currently, a classification of ALS has been proposed depending on the presence of cognitive and/or behavioral disorders. CD in ALS can be represented by speech fluency disorders, various aphasic disorders, disorders of regulatory functions, social cognitive functions, and verbal memory. The most diagnostically sensitive are special scales, such as the Edinburgh Cognitive and Behavioral Impairment Screening Scale for ALS. CD in ALS have a negativ

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Eriksson, Ann-Sofie, and Kerstin Johansson. "Dysphagia in Amyotrophic Lateral Sclerosis (ALS)." Acta Logopaedica 2 (February 25, 2025): 1–19. https://doi.org/10.58986/al.2025.23611.

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Around 80 % of ALS patients suffer from dysphagia, which increases the risk of malnutrition and pneumonia, and correlates with death. To diminish that risk, it is important to involve patients in their dysphagia care to be able to introduce interventions in time. However, Swedish ALS teams lack national guidelines for how to involve patients. As a basis for a future checklist, a 27-item survey was distributed to professionals in ALS teams in Sweden. Less than half of the respondents (46 %) reported a routine of regular dysphagia screening. Dysphagia screening, early information, and training o

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D’Antona, Salvatore, Martina Caramenti, Danilo Porro, Isabella Castiglioni, and Claudia Cava. "Amyotrophic Lateral Sclerosis: A Diet Review." Foods 10, no. 12 (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

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Goncharova, P. S., T. K. Davidova, N. A. Shnayder, M. A. Novitsky, and R. F. Nasyrova. "Epidemiology of Amyotrophic Lateral Sclerosis." Personalized Psychiatry and Neurology 2, no. 1 (2022): 57–66. http://dx.doi.org/10.52667/2712-9179-2022-2-1-57-66.

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We searched for full-text publications in Russian and English in the E-Library, PubMed, Springer, Clinical keys, Google Scholar databases, using keywords and combined word searches (amyotrophic lateral sclerosis - ALS, motor neuron disease, epidemiology, incidence, prevalence), for 2015 – 2020. In addition, the review included earlier publications of historical interest. Despite our comprehensive searches of these commonly used databases and search terms, it cannot be excluded that some publications may have been missed. A total of 74 publications were analyzed, reflecting epidemiological stud

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Lasiene, Jurate, and Koji Yamanaka. "Glial Cells in Amyotrophic Lateral Sclerosis." Neurology Research International 2011 (2011): 1–7. http://dx.doi.org/10.1155/2011/718987.

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Amyotrophic lateral sclerosis (ALS) is an adult motor neuron disease characterized by premature death of upper and lower motor neurons. Two percent of ALS cases are caused by the dominant mutations in the gene for superoxide dismutase 1 (SOD1) through a gain of toxic property of mutant protein. Genetic and chimeric mice studies using SOD1 models indicate that non-neuronal cells play important roles in neurodegeneration through non-cell autonomous mechanism. We review the contribution of each glial cell type in ALS pathology from studies of the rodent models and ALS patients. Astrogliosis and m

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Saudagar, Ravindranath, and Lajri Garge. "Amyotrophic Lateral Sclerosis: An Overview." Journal of Drug Delivery and Therapeutics 9, no. 3 (2019): 613–16. http://dx.doi.org/10.22270/jddt.v9i3.2874.

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Amyotrophic Lateral Sclerosis is an adult-onset neurodegenerative disease that causes paralysis. Presently it is incurable and rapidly progressive with a survival of 4-5 years from onset. It is degeneration of upper and lower motor neurons which results in weakness and wasting of muscles in arms, legs, trunk and bulbar region. There are two types sporadic and familial amyotrophic lateral sclerosis. The age of onset of people with familial is widely reported as being about 10 years younger than for those with apparently sporadic amyotrophic lateral sclerosis. Epidemiological studies show a worl

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Godeiro-Junior, Clecio, Acary S. B. Oliveira, Andre C. Felicio, Marco A. Chieia, and Alberto Alain Gabbai. "Conjugal amyotrophic lateral sclerosis in Brazil." Arquivos de Neuro-Psiquiatria 67, no. 4 (2009): 1045–48. http://dx.doi.org/10.1590/s0004-282x2009000600015.

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The origin of amyotrophic lateral sclerosis (ALS) remains unknown, although it seems to be multifactorial. The role of environmental factors has been frequently investigated and suspicion of its influence can be obtained when clusters of a rare disease are described. OBJECTIVE: To describe conjugal cases of ALS in Brazil. METHOD: We describe 2 couples in which both spouses were affected by ALS. Both couples had lived in southeast Brazil and were married for at least 20 years. RESULTS: There was a great variability in clinical presentation of ALS in our patients. In both couples the interval be

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Fang, Ton, Goun Je, Peter Pacut, Kiandokht Keyhanian, Jeff Gao, and Mehdi Ghasemi. "Gene Therapy in Amyotrophic Lateral Sclerosis." Cells 11, no. 13 (2022): 2066. http://dx.doi.org/10.3390/cells11132066.

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Since the discovery of Cu/Zn superoxide dismutase (SOD1) gene mutation, in 1993, as the first genetic abnormality in amyotrophic lateral sclerosis (ALS), over 50 genes have been identified as either cause or modifier in ALS and ALS/frontotemporal dementia (FTD) spectrum disease. Mutations in C9orf72, SOD1, TAR DNA binding protein 43 (TARDBP), and fused in sarcoma (FUS) genes are the four most common ones. During the last three decades, tremendous effort has been made worldwide to reveal biological pathways underlying the pathogenesis of these gene mutations in ALS/FTD. Accordingly, targeting e

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K, Jayavasavi, and Sathesh Kumar Sukumaran. "Amyotrophic Lateral Sclerosis – A Comprehensive Review." International Journal of Membrane Science and Technology 10, no. 3 (2023): 3711–20. http://dx.doi.org/10.15379/ijmst.v10i5.3569.

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Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, generally said to affect people with an age between 50 years and 70 years. Signs of upper motor neuron and lower motor neuron damage, which are not explained by any other disease process, and are the reasons behind ALS. It attacks the neurons in the brain and spinal cord. These neurons transmit messages from brain and spinal cord to the voluntary muscles. At first, it causes mild muscle problems. Some people show symptoms like difficulty in walking, running, writing and speech. Eventually, the patient may lose the strengt

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McCluskey, Gavin, Karen E. Morrison, Colette Donaghy, Frederique Rene, William Duddy, and Stephanie Duguez. "Extracellular Vesicles in Amyotrophic Lateral Sclerosis." Life 13, no. 1 (2022): 121. http://dx.doi.org/10.3390/life13010121.

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Amyotrophic Lateral Sclerosis is a progressive neurodegenerative disease and is the most common adult motor neuron disease. The disease pathogenesis is complex with the perturbation of multiple pathways proposed, including mitochondrial dysfunction, RNA processing, glutamate excitotoxicity, endoplasmic reticulum stress, protein homeostasis and endosomal transport/extracellular vesicle (EV) secretion. EVs are nanoscopic membrane-bound particles that are released from cells, involved in the intercellular communication of proteins, lipids and genetic material, and there is increasing evidence of

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Srivastava, Ojas, Chris Hanstock, Sneha Chenji, et al. "Cerebral degeneration in amyotrophic lateral sclerosis." Neurology: Clinical Practice 9, no. 5 (2019): 400–407. http://dx.doi.org/10.1212/cpj.0000000000000674.

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BackgroundWe investigated cerebral degeneration and neurochemistry in patients with amyotrophic lateral sclerosis (ALS) using magnetic resonance spectroscopy (MRS).MethodsWe prospectively studied 65 patients and 43 age-matched healthy controls. Participants were recruited from 4 centers as part of a study in the Canadian ALS Neuroimaging Consortium. All participants underwent single-voxel proton MRS using a protocol standardized across all sites. Metabolites reflecting neuronal integrity (total N-acetyl aspartyl moieties [tNAA]) and gliosis (myo-inositol [Ino]), as well as creatine (Cr) and ch

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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, dif

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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 r

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Kimura, Fumiharu. "Guideline for Amyotrophic Lateral Sclerosis (ALS)." Nihon Naika Gakkai Zasshi 98, no. 5 (2009): 1148–54. http://dx.doi.org/10.2169/naika.98.1148.

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Irwin, D., Carol F. Lippa, and J. M. Swearer. "Cognition and Amyotrophic Lateral Sclerosis (ALS)." American Journal of Alzheimer's Disease & Other Dementiasr 22, no. 4 (2007): 300–312. http://dx.doi.org/10.1177/1533317507301613.

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36

Fawad, Laiba, and Mehrab Tahir. "Emerging Therapies in Amyotrophic Lateral Sclerosis." Molecular Medicine Communications 2, no. 01 (2022): 31–42. http://dx.doi.org/10.55627/mmc.002.001.0041.

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Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the loss of cortical and spinal motor neurons, leading to weakness, muscle atrophy, and, in a substantial number of patients, cognitive impairment. Most patients die within 2 to 5 years of diagnosis. The disease initiates from the death of upper and lower motor neurons leading to a degeneration of motor pathways and the paralytic effects of the disease. The disease has huge economic costs as well. FDA has approved two drugs, riluzole, and edaravone for the treatment of ALS. However, these drugs provide m

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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 (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 mode

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Martins, Melina Pazian, Fabrício Diniz de Lima, Tauana Bernardes Leoni, et al. "Laryngeal electromyography in amyotrophic lateral sclerosis." Journal of Neurology, Neurosurgery & Psychiatry 91, no. 7 (2020): 730–32. http://dx.doi.org/10.1136/jnnp-2020-322910.

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BackgroundBulbar involvement is a hallmark of amyotrophic lateral sclerosis (ALS), but surprisingly very few studies have addressed the frequency, pattern and clinical relevance of laryngeal involvement in the disease.MethodsTwenty-six patients with spinal-onset ALS underwent nasofibroscopy (NF), followed by laryngeal electromyography (LEMG). We also studied resting activity and motor unit potentials of the genioglossus and masseter muscles.ResultsTwenty-four patients presented neurogenic changes in at least one laryngeal muscle. There were fibrillation and/or fasciculation potentials associat

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Etemadifar, Masoud, Seyed-Hossein Abtahi, Mojtaba Akbari, and Amir-Hadi Maghzi. "Multiple sclerosis and amyotrophic lateral sclerosis: is there a link?" Multiple Sclerosis Journal 18, no. 6 (2011): 902–4. http://dx.doi.org/10.1177/1352458511427719.

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To date, there are no reports studying the rate of amyotrophic lateral sclerosis (ALS) in relatives of multiple sclerosis (MS) patients and vice versa. This study was designed to look into this issue using two population-based databases of MS and ALS in Isfahan province of Iran. We have searched for any first, second or third degree familial kinship between the Isfahan MS Society database and Isfahan ALS population. We compared the rate of ALS among the population of first degree relatives of MS patients, with the crude prevalence of ALS in the general population of Isfahan. On the other hand,

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Navarro, Etiane, and Charles J. Golden. "A-151 Cognitive Impairment in Amyotrophic Lateral Sclerosis." Archives of Clinical Neuropsychology 36, no. 6 (2021): 1205. http://dx.doi.org/10.1093/arclin/acab062.169.

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Abstract Objective Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease caused by degeneration of the upper and lower motor neurons. This literature review examines the recurring etiology of cognitive impairments in ALS through empirical literature. The current study explores ALS across different subtypes and potential cognitive impairments. Two classifications are primarily examined ALS, and ALS with frontotemporal dementia (ALS-FTD). Involving three categories: familial inheritance pattern, genetic mutation, or sporadic. Neuropsychological studies affirm cognitive i

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Ricci, Claudia, Carlotta Marzocchi, and Stefania Battistini. "MicroRNAs as Biomarkers in Amyotrophic Lateral Sclerosis." Cells 7, no. 11 (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 practic

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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 (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 nu

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Oliveira, Guilherme Camargo, Quoc Cuong Ngo, Leandro Aparecido Passos, et al. "Video Assessment to Detect Amyotrophic Lateral Sclerosis." Digital Biomarkers 8, no. 1 (2024): 171–80. http://dx.doi.org/10.1159/000540547.

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Introduction: Weakened facial movements are early-stage symptoms of amyotrophic lateral sclerosis (ALS). ALS is generally detected based on changes in facial expressions, but large differences between individuals can lead to subjectivity in the diagnosis. We have proposed a computerized analysis of facial expression videos to detect ALS. Methods: This study investigated the action units obtained from facial expression videos to differentiate between ALS patients and healthy individuals, identifying the specific action units and facial expressions that give the best results. We utilized the Tor

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Thompson, Alexander G., Elizabeth Gray, Alexander Bampton, Dominika Raciborska, Kevin Talbot, and Martin R. Turner. "CSF chitinase proteins in amyotrophic lateral sclerosis." Journal of Neurology, Neurosurgery & Psychiatry 90, no. 11 (2019): 1215–20. http://dx.doi.org/10.1136/jnnp-2019-320442.

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ObjectiveTo evaluate the classifier performance, clinical and biochemical correlations of cerebrospinal fluid (CSF) levels of the chitinase proteins Chitotriosidase-1 (CHIT1), Chitinase-3-like protein 1 (CHI3L1) and Chitinase-3-like protein 2 (CHI3L2) in amyotrophic lateral sclerosis (ALS).MethodsCSF levels of CHIT1, CHI3L1, CHI3L2, phosphorylated neurofilament heavy chain (pNFH) and C-reactive protein were measured by ELISA in a longitudinal cohort of patients with ALS (n=82), primary lateral sclerosis (PLS, n=10), ALS-mimic conditions (n=12), healthy controls (n=25) and asymptomatic carriers

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Smukowski, Samuel N., Heather Maioli, Caitlin S. Latimer, Thomas D. Bird, Suman Jayadev, and Paul N. Valdmanis. "Progress in Amyotrophic Lateral Sclerosis Gene Discovery." Neurology Genetics 8, no. 3 (2022): e669. http://dx.doi.org/10.1212/nxg.0000000000000669.

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Amyotrophic lateral sclerosis (ALS) is the most prominent motor neuron disease in humans. Its etiology consists of progressive motor neuron degeneration resulting in a rapid decline in motor function starting in the limbs or bulbar muscles and eventually fatally impairing central organs most typically resulting in loss of respiration. Pathogenic variants in 4 main genes, SOD1, TARDBP, FUS, and C9orf72, have been well characterized as causative for more than a decade now. However, these only account for a small fraction of all ALS cases. In this review, we highlight many additional variants tha

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Sinanović, Osman, Muhamed Lepuzanović, and Edin Bašagić. "PHYSICAL ACTIVITY AND AMYOTROPHIC LATERAL SCLEROSIS." Medicina Academica Integrativa 1, no. 1 (2024): 34–41. http://dx.doi.org/10.47960/3029-3316.2024.1.1.34.

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Motor neuron diseases (MNDs) are a group of relatively rare, progressive neurodegenerative conditions (with amyotrophic lateral sclerosis/ALS being the most common) characterized by degeneration of upper and lower motor neurons leading to motor and extra motor symptoms. The etiology of MND is believed to involve complex interactions of environmental, lifestyle, and genetic factors, but so far only a few convincing risk factors have been established. Several putative risk factors associated with sporadic cases have been suggested, including repetitive blows to the head and traumatic brain injur

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Koberskaya, N. N., D. A. Grishina, and N. N. Yakhno. "Syndrome amyotrophic lateral sclerosis — Alz heimer's dementia." Russian neurological journal 26, no. 2 (2021): 17–24. http://dx.doi.org/10.30629/2658-7947-2021-26-2-17-24.

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Recently, there is more and more evidence of the presence of a cognitive defect of varying severity in the clinical picture of ALS. A rare form of the disease is the amyotrophic lateral sclerosis (ALS) — dementia complex, characterized by a combination of dementia (usually frontotemporal) with ALS symptoms. The profile of cognitive deficit in ALS includes impairment of executive functions, memory, speech and visual-spatial disorders. A literature review on this problem is presented with a description of the clinical observation of ALS–dementia syndrome (frontal variant of possible Alzheimer’s

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Calvo, Andrea, Paolo Ghiglione, and Adriano Chiò. "Management of patients with amyotrophic lateral sclerosis." Clinical Management Issues 2, no. 3 (2008): 103–11. http://dx.doi.org/10.7175/cmi.v2i3.567.

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Amyotrophic lateral sclerosis (ALS) is a progressive, fatal, neurodegenerative disease caused by the degeneration of motor neurons. We report a case of a 45-years-old patient with ALS to underline difficulties and challenges in ALS management. Even though ALS remains fatal, several advances have been made in improving the consequences of this disease: symptomatic treatments have an important role in controlling sialorrhea, bronchial secretions, pseudobulbar emotional lability, cramps, spasticity, depression and anxiety, insomnia and pain. An adequate management of ALS should be multidisciplina

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Shimizu, Toshio, and Kota Bokuda. "Complex Fasciculation Potentials in Amyotrophic Lateral Sclerosis." US Neurology 11, no. 01 (2015): 53. http://dx.doi.org/10.17925/usn.2015.11.01.53.

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Fasciculation and fasciculation potentials (FPs) are most frequently observed in progressive motor neuron disorders, including amyotrophic lateral sclerosis (ALS). Recent diagnostic criteria, the so-called Awaji criteria, emphasize the importance of FPs in the electrodiagnosis of ALS. Although FPs are found in other benign syndromes, FPs in ALS often show a complex morphology (complex fasciculation potential [CFP]) with instability in the form of increased jitters with blocking of some components of the FPs. The criteria have raised the diagnostic significance of FPs as FPs have clinical impor

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Pasqualucci, Elena, Diletta Angeletti, Pamela Rosso, et al. "Management of Dysarthria in Amyotrophic Lateral Sclerosis." Cells 14, no. 14 (2025): 1048. https://doi.org/10.3390/cells14141048.

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Amyotrophic lateral sclerosis (ALS) stands as the leading neurodegenerative disorder affecting the motor system. One of the hallmarks of ALS, especially its bulbar form, is dysarthria, which significantly impairs the quality of life of ALS patients. This review provides a comprehensive overview of the current knowledge on the clinical manifestations, diagnostic differentiation, underlying mechanisms, diagnostic tools, and therapeutic strategies for the treatment of dysarthria in ALS. We update on the most promising digital speech biomarkers of ALS that are critical for early and differential d

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