Cognitive and behavioral involvement in ALS has been known for more than a century

Zago, Stefano; Lorusso, Lorenzo; Aiello, Edoardo Nicolò; Ugolini, Martino; Poletti, Barbara; Ticozzi, Nicola; Silani, Vincenzo

doi:10.1007/s10072-022-06340-0

Cited by 13 publications

(6 citation statements)

References 73 publications

(79 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…It occurs after damage to the upper and lower motor neurons, resulting in atrophy and cognitive/behavioral dysfunctions. 97,98 Currently, the mechanism of ALS is unclear, but several studies suggest that impaired RNA metabolism, mitochondrial dysfunction, neuroinflammation, and neuronal death are involved in disease pathogenesis. 99 For example, in ALS, the misfolded SOD1 protein causes mitochondrial damage.…”

Section: Amyotrophic Lateral Sclerosismentioning

confidence: 99%

The cytosolic DNA‐sensing cGAS–STING pathway in neurodegenerative diseases

Guo,

Yang,

Wang

et al. 2024

CNS Neurosci Ther

View full text Add to dashboard Cite

BackgroundWith the widespread prevalence of neurodegenerative diseases (NDs) and high rates of mortality and disability, it is imminent to find accurate targets for intervention. There is growing evidence that neuroimmunity is pivotal in the pathology of NDs and that interventions targeting neuroimmunity hold great promise. Exogenous or dislocated nucleic acids activate the cytosolic DNA sensor cyclic GMP‐AMP synthase (cGAS), activating the stimulator of interferon genes (STING). The activated STING triggers innate immune responses and then the cGAS‐STING signaling pathway links abnormal nucleic acid sensing to the immune response. Recently, numerous studies have shown that neuroinflammation regulated by cGAS‐STING signaling plays an essential role in NDs.AimsIn this review, we summarized the mechanism of cGAS‐STING signaling in NDs and focused on inhibitors targeting cGAS‐STING.ConclusionThe cGAS‐STING signaling plays an important role in the pathogenesis of NDs. Inhibiting the cGAS‐STING signaling may provide new measures in the treatment of NDs.

show abstract

Section: Amyotrophic Lateral Sclerosismentioning

confidence: 99%

The cytosolic DNA‐sensing cGAS–STING pathway in neurodegenerative diseases

Guo,

Yang,

Wang

et al. 2024

CNS Neurosci Ther

View full text Add to dashboard Cite

show abstract

“…Cognitive and behavioural abnormalities have long been known to be concomitant with ALS [222][223][224], especially relating to behavioural and verbal variants of the frontotemporal dementia (FTD) spectrum [223]. However, in ALS patients, cognitive dysfunction is not confined solely to the FTD phenotype.…”

Section: Cognitive and Behavioural Dysfunction In Alsmentioning

confidence: 99%

Non-motor symptoms in patients with amyotrophic lateral sclerosis: current state and future directions

Bjelica,

Bartels,

Hesebeck-Brinckmann

et al. 2024

J Neurol

View full text Add to dashboard Cite

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive degeneration of both upper and lower motor neurons. A defining histopathological feature in approximately 97% of all ALS cases is the accumulation of phosphorylated trans-activation response (TAR) DNA-binding protein 43 protein (pTDP-43) aggregates in the cytoplasm of neurons and glial cells within the central nervous system. Traditionally, it was believed that the accumulation of TDP-43 aggregates and subsequent neurodegeneration primarily occurs in motor neurons. However, contemporary evidence suggests that as the disease progresses, other systems and brain regions are also affected. Despite this, there has been a limited number of clinical studies assessing the non-motor symptoms in ALS patients. These studies often employ various outcome measures, resulting in a wide range of reported frequencies of non-motor symptoms in ALS patients. The importance of assessing the non-motor symptoms reflects in a fact that they have a significant impact on patients’ quality of life, yet they frequently go underdiagnosed and unreported during clinical evaluations. This review aims to provide an up-to-date overview of the current knowledge concerning non-motor symptoms in ALS. Furthermore, we address their diagnosis and treatment in everyday clinical practice.

show abstract

“…In contrast, FTD is characterized by changes in behavior, personality, and language, with patients often exhibiting apathy, disinhibition, and loss of empathy. However, there is significant overlap between the two diseases, with some ALS patients exhibiting cognitive and behavioral deficits, and some FTD patients exhibiting motor symptoms (3,4).…”

Section: Clinical Featuresmentioning

confidence: 99%

Current understanding of the association between Amyotrophic lateral sclerosis and frontotemporal dementia: Letter to Editor

Zhang

Wang

2023

View full text Add to dashboard Cite

This open-access article distributed under the terms of the Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0) Dear Editor, Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two devastating neurodegenerative diseases that have a significant overlap in clinical and pathological features (1). ALS is a progressive motor neuron disease that leads to muscle weakness, atrophy, and eventually paralysis, while FTD is a form of dementia that primarily affects the frontal and temporal lobes of the brain, leading to changes in behavior, personality, and language. In recent years, there has been growing evidence of an association between ALS and FTD, with many cases exhibiting features of both diseases. This paper will discuss the recent advances in our understanding of the link between ALS and FTD, including their clinical and pathological features, genetic factors, and potential treatments (2). Genetic FactorsThe link between ALS and FTD is further supported by the identification of specific genetic mutations that are associated

show abstract

Cognitive and behavioral involvement in ALS has been known for more than a century

Cited by 13 publications

References 73 publications

The cytosolic DNA‐sensing cGAS–STING pathway in neurodegenerative diseases

The cytosolic DNA‐sensing cGAS–STING pathway in neurodegenerative diseases

Non-motor symptoms in patients with amyotrophic lateral sclerosis: current state and future directions

Current understanding of the association between Amyotrophic lateral sclerosis and frontotemporal dementia: Letter to Editor

Contact Info

Product

Resources

About