Breaking the news of amyotrophic lateral sclerosis (ALS) is considered as a daunting task in most cases and is not a standardizable procedure. However, proven techniques exist to reduce the trauma to the patient. Announcing ALS falls upen the neurologist who must respect the ethical principle of the patient's independence. After the diagnosis is firmly established, the patient should be informed that he or she has a progressive disease of the motor nerves, for which no curative therapy is available. The name of the disease must be stated and explained. If the family history is negative, it is reassuring for the patient and family to know that their children are unlikely to be at risk. Positive aspects (no pain, no disturbances in sensation, cognition, memory and continence) should be stressed as well as the availability of efficient palliative measures for practically all symptoms. Current research efforts, and when available, the possibility of taking part in clinical studies of new drugs should be pointed out as a means of hope. The answer to the question of prognosis should include the information that there are no sudden worsenings to be expected, that the course of ALS may vary between months and decades, that making a firm statement on prognosis all but impossible for any single patient and that respiratory function may worsen during the disease course. It is therefore mandatory to inform patients and families about the existence of ALS patients'associations. The way the patient is told the diagnosis is of great importance and is considered as a multiple-step procedure. Discussion should take place in a private and quiet room and respect some fundamental objectives such as finding out what the patient already knows or suspects and how much more the patient wants to know, observing and responding to the patient's reactions, reinforcing the information and planning the future. It is proven that communicating the diagnosis of ALS in an empathetic fashion is an important and sensitive step to disease management.
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Adv Drug Deliv Rev
January 2025
Neurodegenerative Diseases Department, Kadimastem Ltd, Pinchas Sapir 7, Weizmann Science Park, Ness-Ziona, Israel; Department of Molecular Genetics, Weizmann Institute of Science, 76100, Rehovot, Israel.
Self-renewal capacity and potential to differentiate into almost any cell type of the human body makes pluripotent stem cells a valuable starting material for manufacturing of clinical grade cell therapies. Neurodegenerative diseases are characterized by gradual loss of structure or function of neurons, often leading to neuronal death. This results in gradual decline of cognitive, motor, and physiological functions due to the degeneration of the central nervous systems.
View Article and Find Full Text PDFBrain Res Bull
January 2025
Department of Geriatrics, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China. Electronic address:
Purpose: To investigate the differences in brain spontaneous neural activity between limb-onset and bulbar-onset amyotrophic lateral sclerosis (ALS-L and ALS-B, respectively) patients using resting-state functional MRI (rs-fMRI) with amplitude of low-frequency fluctuation (ALFF) and regional homogeneity (ReHo).
Materials And Methods: The rs-fMRI data were collected from 41 ALS patients (11 ALS-B and 30 ALS-L) and 25 healthy controls (HC). ALFF and ReHo values were calculated, and group differences were assessed using one-way ANCOVA and two-sample t-tests.
Clin Nutr ESPEN
January 2025
Department of Neurology, Taiyuan Central Hospital, Taiyuan, Shanxi Province, China. Electronic address:
Background: An inconsistent yet notable relationship between dietary habits and the risk of amyotrophic lateral sclerosis (ALS) has been previously established, with the causative nature of this relationship remaining uncertain. This study aims to explore the causal connections at a genetic level.
Methods: A two-sample Mendelian Randomization (MR) based analysis was conducted utilizing a comprehensive, publicly assessable Genome-wide association study (GWAS) database.
Curr Opin Neurobiol
January 2025
Salk Institute for Biological Studies, Molecular Neurobiology Laboratory, 10010 North Torrey Pines Rd, La Jolla, CA, 92037, USA. Electronic address:
Astrocytes perform multiple functions in the nervous system, many of which are altered in neurodegenerative disorders. In this review, we explore shared astrocytic alterations across neurodegenerative disorders, including Alzheimer's disease, Huntington's disease, Parkinson's disease, amyotrophic lateral sclerosis, and frontotemporal lobe degeneration. Assessing recent datasets of single-nucleus RNA-sequencing of human brains, a theme emerges of common alterations in astrocyte state across disorders including in neuroinflammation, synaptic organization, metabolic support, and the cellular stress response.
View Article and Find Full Text PDFNeurology
February 2025
Department of Neurology, University of Michigan, Ann Arbor.
Background And Objectives: An adverse social exposome negatively affects many diseases, but its association with amyotrophic lateral sclerosis (ALS) survival is unknown. This study examined the association between the social exposome measure Area Deprivation Index (ADI) and ALS survival.
Methods: This is a retrospective analysis of patients with ALS at the University of Michigan Pranger ALS Clinic diagnosed after January 1, 2012.
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