The terms amyotrophic lateral sclerosis (ALS) or motor neuron disease (MND) refer to a condition characterized by motor system degeneration with relative preservation of other pathways. Although there have been advances in symptomatic treatment, ALS remains an incurable condition. Advances in ALS management prolong survival but simultaneously raise challenging ethical dilemmas for physicians, patients and their families. Here, we review current practice in the management of ALS including pharmacological treatment, nutritional management, respiratory care, and evolving strategies in the management of cognitive impairment.
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F waves in amyotrophic lateral sclerosis: A systematic review and meta-analysis.
Neurophysiol Clin
March 2025
Department of Neurology, University Hospital of Patras, School of Medicine, University of Patras, Patras, Rio 26504, Greece. Electronic address:
Objective: This systematic review and meta-analysis aimed to determine the pattern of F-wave abnormalities and their potential utility in the early diagnosis of amyotrophic lateral sclerosis (ALS).
Methods: Medline and Embase were thoroughly searched. We primarily emphasized F-wave recordings from the abductor digiti minimi, following stimulation of the ulnar nerve at the wrist.
The phenotyping dilemma in VRK1-related motor neuron disease: a Turkish family with young-onset amyotrophic lateral sclerosis caused by a novel mutation.
Amyotroph Lateral Scler Frontotemporal Degener
March 2025
Department of Neurology, Bakirkoy Dr. Sadi Konuk Training and Research Hospital, Istanbul, Turkey and.
: Vaccinia-related kinase 1 (VRK1)-related disease is an extremely rare autosomal recessive disorder primarily affecting the peripheral and/or central nervous system. In this report, we describe the genetic and clinical features of two siblings from a Turkish family presenting with an amyotrophic lateral sclerosis (ALS) phenotype due to a novel homozygous VRK1 mutation, and discuss the broad phenotypic spectrum associated with pathogenic variants in this gene. : We analyzed the demographic data, clinical histories, neurological examinations, laboratory findings, and genetic results of 53 patients, including our cases, derived from 27 different reports.
View Article and Find Full Text PDFALSUntangled #78: Zinc.
Amyotroph Lateral Scler Frontotemporal Degener
March 2025
Department of Neurology, Duke University, Durham, NC, USA.
ALSUntangled reviews alternative and off-label treatments for people living with amyotrophic lateral sclerosis (PALS). In this review, we assess the utilization of dietary zinc supplements for modulating ALS pathology and progression. Studies in mouse models of ALS have demonstrated that high-dose zinc supplementation may be harmful, but moderate doses could potentially be beneficial.
View Article and Find Full Text PDFThe Potential of cfDNA as Biomarker: Opportunities and Challenges for Neurodegenerative Diseases.
J Mol Neurosci
March 2025
Department of Molecular Biology and Genetics, Faculty of Science, Atatürk University, Erzurum, Turkey.
Neurodegenerative disorders, including Alzheimer's disease (AD), Parkinson's disease (PD), multiple sclerosis (MS), and amyotrophic lateral sclerosis (ALS), are characterized by the progressive and gradual degeneration of neurons. The prevalence and rates of these disorders rise significantly with age. As life spans continue to increase in many countries, the number of cases is expected to grow in the foreseeable future.
View Article and Find Full Text PDFEvidence-Based Nutritional Recommendations for Maintaining or Restoring Nutritional Status in Patients with Amyotrophic Lateral Sclerosis: A Systematic Review.
Nutrients
February 2025
Laboratory of Technological Innovation in Health (LAIS), Federal University of Rio Grande do Norte, Natal 59012-300, Brazil.
: This study is a systematic review of guidelines that aims to synthesize evidence-based recommendations to support appropriate nutritional management for patients with amyotrophic lateral sclerosis (ALS). : PubMed/MEDLINE, Embase, Scopus, SciELO, Web of Science, LILACS, ScienceDirect, and Google Scholar were searched for records published up to July 2024. Clinical practice guidelines addressing any aspect of nutritional intervention in ALS were included.
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