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Amyotrophic Lateral Sclerosis - Springer Nature

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Last Updated: 27 June 2022

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Psychoeducational groups for people with Amyotrophic Lateral Sclerosis and their caregiver: a qualitative study

Objective The current research sought to describe the implementation of a coordinated psychoeducational intervention in Amyotrophic Lateral Sclerosis (AKA) in Amyotrophic Lateral Sclerosis, revealing the needs of both patients and caregivers. After the group sessions ended, five patients and thirteen caregivers attended eight psychoeducational group meetings at the Respiratory Rehabilitation Unit of Fondazione Don Carlo Gnocchi, Milan. These findings indicated the usefulness of psychoeducational groups in regular clinical practice by encouraging patients and caregivers to freely share information, emotions, and experiences with others in the same situation. This report demonstrates the effectiveness of psychoeducational group interventions in helping people with ALS and their caregivers for two main reasons: first for the psychoeducation provided by professionals; second for the possibility of sharing experiences and emotions with people in the same situation.

Source link: https://doi.org/10.1007/s10072-022-05930-2


Overcoming therapeutic nihilism. Breaking bad news of amyotrophic lateral sclerosis—a patient-centred perspective in rare diseases

Breaking bad news about ALS diagnosis is a challenging challenge for doctors and a life-changing event for patients. Our aim was to determine to what extent these recommendations are followed in Polish ALS patientsu2019 experience, as well as any other patients'u2019 preferences that have not been addressed by the guidelines. Nine patients with confirmed ALS diagnosis were interviewed through an in-depth interview and a self-constructed questionnaire, ranging from 30 years old to 60 years old, ranging from 30 to 60 years old. As a result, we recommend using extended breaking bad news protocols, giving the patient hope and a sense of purpose, providing psychological assistance and counseling directed to patients and caregivers, and referring to appropriate health care centers.

Source link: https://doi.org/10.1007/s10072-022-05931-1


Predicting functional impairment trajectories in amyotrophic lateral sclerosis: a probabilistic, multifactorial model of disease progression

The aim of this article is to model, predict, and predict the amyotrophic lateral sclerosis progression over time in terms of diverse influences, cognitive impairments, and survival. Methods We used socioeconomic and clinical data, as well as support services, of 3940 ALS patients from four Italian and two Israeli registers, to suggest a new strategy based on Dynamic Bayesian Networks that reflects the change of ALS in two distinct scenarios of variable availability. The procedure helps to simulate patients'U2019 disease trajectories and predict the likelihood of functional impairment and survival at different time points. Results DBNs explicitly state the links between the variables and the pathways through which they influence disease progression.

Source link: https://doi.org/10.1007/s00415-022-11022-0


Alteration of interoceptive sensitivity: expanding the spectrum of behavioural disorders in amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis is a neurodegenerative disorder with progressive loss of upper and lower motor neurons. Interoception refers to the ability to specifically assess the inner body's physical appearance, including one's heartbeat — according to one'u2019's heartbeat. Objects The heartbeat perception task is used to determine IS in ALS patients. ALS patients had lower IS than CG, according to the results. On questionnaires for depression and anxiety among patients with ALS and their caregivers, there were no significant differences. ALS patients' sensitivity has reduced interoceptive sensitivity, decreased ability to express emotions, and less focus on pain, regardless of cognitive and motor impairment. Alternation of interoception may be a specific behavioral characteristic present in ALS patients with a variety of emotion processing deficits.

Source link: https://doi.org/10.1007/s10072-022-06231-4


Granulocyte-colony stimulating factor (G-CSF): an emerging therapeutic approach for amyotrophic lateral sclerosis (ALS)

Amyotrophic lateral sclerosis is a neurodegenerative disease characterized by neuronal degeneration and inflammation in the nerves. In mSOD1 G93A mice models, pre-clinical research has shown significant safety of G-CSF therapy. However, pre-clinical studies on ALS patients failed to clone pre-clinical findings. This study sought to comprehensively review the research and pre-clinical studies regarding G-CSF in ALS therapy, considering the potential role of G-CSF in nervous system regeneration.

Source link: https://doi.org/10.1007/s13760-022-01996-z


Clonally expanded CD8 T cells characterize amyotrophic lateral sclerosis-4

In the brain and blood of mice with amyotrophic lateral sclerosis-4, an immune signature characterized by activated antigen-specific CD8 T cells has been found, indicating that the immune system is involved in ALS4 neurodegeneration. Although several ALS-related genes have been shown to influence immune functions, 2, it is unclear if particular immune systems account for ALS heterogeneity. Here, we describe an immunological signature in the central nervous system and mouse blood of knock-in mice that carry the ALS4-causative L389S mutation. In knock-in mice, the growth of motor neuron disease and antigen-specific CD8 T cells mimic the progression of motor neuron disease progression and are associated with anti-glioma immunity. T_EMRA CD8 T cells that have clonically increased in patients with ALS4 are present in the peripheral blood.

Source link: https://doi.org/10.1038/s41586-022-04844-5


A strong association between VEGF-A rs28357093 and amyotrophic lateral sclerosis: a brazilian genetic study

Amyotrophic Lateral Sclerosis (Sclerosis) is a rare neurodegenerative disorder that affects motor neurons and promotes progressive muscle atrophy. Vascular Endothelial Growth Factor A of the central nervous system A appears in several ways. Methods and findings This case-control study was carried out in 101 ALS patients and 119 healthy volunteers. Our results showed a strong correlation between VEGF-A rs28357093 and ALS in all genetic inheritance models, with a 9-fold risk of A/C genotypes in A/C. Conclusions The mutant allele was more prevalent in ALS patients, and this finding could be related to ALS risk.

Source link: https://doi.org/10.1007/s11033-022-07647-z


Progressive medial temporal degeneration with TDP-43 pathology is associated with upper limb and bulbar onset types of amyotrophic lateral sclerosis

Objective This study sought to clarify the connection between progressive medial temporal atrophy and onset subtype in patients with amyotrophic lateral sclerosis patients. Methods in 119 patients were categorized into three ALS subtypes: bulbar, upper limb, and lower leg onset. In addition, TDP-43 pathology in 20 autopsied patients was investigated to determine the associations between medial temporal atrophy, onset subtypes, and the severity of the hippocampal TDP-43 pathology. Conclusions Multiple regression analysis revealed that the Z-score at baseline was directly related to the time of onset and duration of illness. TDP-43 pathology score for bulbar and upper limb subtypes was found in a pathological examination. Also, the Z-core before death correlated with the hippocampal TDP-43 pathology score, which is also linked to the hippocampal TDP-43 pathology score. ALS's medial temporal atrophy is attributed to bulbar and upper limb subtypes, according to a morphological study.

Source link: https://doi.org/10.1007/s00415-022-11217-5


Modulation of spontaneous motor unit potentials by a new motor cortical magnetic stimulation method in amyotrophic lateral sclerosis

Patients with amyotrophic lateral sclerosis with sclerosis have elevated cortical excitability, according to the amyotrophic lateral sclerosis. With a newly invented wearable transcranial rotating permanent magnet stimulator, we can determine modulation of spontaneous motor unit potentials in hand muscles by multifocal cortical stimulation in this study. Methods We conducted cross-sectional and longitudinal electromyographic studies in 40 and 20 ALS patients, respectively, of the stimulation-induced rise in the number of sMUPs in two hand muscles modulated by unilateral TRPMS stimulation of the primary motor cortex. During several short sessions consisting of ten stimuli over 60 s and 30 seconds post-stimulation times, we monitored peak sMUP values. In cross-sectional results, the ratios of ALS subjects with peak sMUP count increase among early and intermediate stages and early and late stages were significantly different. Conclusions These findings are consistent with a delayed onset of upper motor neuronal dysfunction with respect to the onset of clinical conditions.

Source link: https://doi.org/10.1007/s00415-022-11214-8

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions