Interplay between immunity and amyotrophic lateral sclerosis: Clinical impact

Marchi, Fabiola De; Munitić, Ivana; Amedei, Amedeo; Berry, James; Feldman, Eva L.; Aronica, Eleonora; Nardo, Giovanni; Weehaeghe, Donatienne Van; Niccolai, Elena; Prtenjača, Nikolina; Sakowski, Stacey A.; Bendotti, Caterina; Mazzini, Letizia

doi:10.1016/j.neubiorev.2021.06.027

Cited by 30 publications

(28 citation statements)

References 316 publications

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“…It has been previously shown that autopsy materials of ALS patients carrying optineurin mutations have TDP-43 aggregates and CHMP2B positive autophagic vacuoles in spinal and bulbar motor neurons [ 31 , 33 ]. Although ALS is primarily a disease of motor neurons, it is now well established that microglia are critical triggers of neuropathology in ALS [ 2 , 38 , 39 ]. To elucidate the potential mechanisms for TDP-43 aggregation and neurodegeneration in ALS patients that carry optineurin mutations, we generated optineurin targeting constructs and silenced this gene using a conventional CRISPR/Cas9 approach.…”

Section: Discussionmentioning

confidence: 99%

Optineurin Deficiency and Insufficiency Lead to Higher Microglial TDP-43 Protein Levels

Prtenjača

Rob

Alam

et al. 2022

IJMS

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Section: Discussionmentioning

confidence: 99%

Optineurin Deficiency and Insufficiency Lead to Higher Microglial TDP-43 Protein Levels

Prtenjača

Rob

Alam

et al. 2022

IJMS

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“…In ALS, the infiltration of lymphocytes into the CNS has different consequences depending on the cell type. Accordingly, CD4+ CD25+ regulatory T cells (Tregs) and CD4+ T helper (Th)2 cells tend to mediate a neuroprotective effect, whereas the presence of CD4+ Th1, CD4+ Th17, cytotoxic CD8+, and Natural killer (NK) cells, and effector T lymphocytes (Teffs), is associated with a more rapid progression of ALS and an increased risk of death [ 22 , 23 ]. Few data are available on the role of B lymphocytes, plasma cells, and antibodies in the pathogenesis of ALS.…”

Section: Recent Evidence On Clonotypic Immunity In Alsmentioning

confidence: 99%

Advances on Cellular Clonotypic Immunity in Amyotrophic Lateral Sclerosis

Schirò

Stefano²,

Iacono³

et al. 2022

Brain Sciences

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Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disease, characterized by the progressive degeneration of the upper and lower motor neurons in the cortex and spinal cord. Although the pathogenesis of ALS remains unclear, evidence concerning the role of the clonotypic immune system is growing. Adaptive immunity cells often appear changed in number, or in terms of their activation profiles, both peripherally and centrally; however, their role in ALS appears conflictive. Data from human and animal model studies, which are currently reported in the literature, show that each subset of lymphocytes and their mediators may mediate a protective or toxic mechanism in ALS, affecting both its progression and risk of death. In the present review, an attempt is made to shed light on the actual role of cellular clonotypic immunity in ALS by integrating recent clinical studies and experimental observations.

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“…A multidisciplinary specialist team approach is the gold standard of ALS management for improving survival and quality of life across symptom management [ 3 ]. Nutritional status is one of the most relevant prognostic factors for ALS since malnutrition is common, and negatively affects prognosis [ 4 , 5 ] by exacerbating muscle weakness and compromising the respiratory and immune systems [ 6 , 7 ]. Weight loss is persistent during the disease [ 8 ], and is related to several factors, such as dysphagia, inappetence, depression, mobility difficulties, and changes in energy homeostasis [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

A Telehealth Intervention for Nutritional Counseling in Amyotrophic Lateral Sclerosis Patients

Marchi

Serioli

Collo

et al. 2022

JCM

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Nutritional status is one of the most relevant prognostic factors in Amyotrophic Lateral Sclerosis (ALS), and close monitoring can help avoid severe weight loss over the disease course. We describe the impact of a Chatbot webapp on improving the communications between physicians, patients, and/or caregivers for dietary monitoring. We developed a chatbot that provides patients with a tool to register their meals through an intuitive and carefully designed conversational interface. Patients recorded their dietary intake twice weekly and received an adequate nutritional recommendation monthly. We monitored their functional and nutritional parameters. The data were compared with a control group followed up by standard counseling. We enrolled 26 patients. Regarding feasibility, 96% of participants completed the three-month follow-up, and 77% ended the six months. Regarding the change in weight in the Chatbot group, we observed a weight stabilization (F = 1.874, p-value: 0.310 for changes) over the telehealth compared to the control group (F = 1.710, p-value: 0.024 for changes). A telehealth approach for nutritional support is feasible and reproducible in an ALS setting: frequent monitoring turned out to help prevent further weight loss, allowing an early nutritional strategy adjustment.

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Interplay between immunity and amyotrophic lateral sclerosis: Clinical impact

Cited by 30 publications

References 316 publications

Optineurin Deficiency and Insufficiency Lead to Higher Microglial TDP-43 Protein Levels

Optineurin Deficiency and Insufficiency Lead to Higher Microglial TDP-43 Protein Levels

Advances on Cellular Clonotypic Immunity in Amyotrophic Lateral Sclerosis

A Telehealth Intervention for Nutritional Counseling in Amyotrophic Lateral Sclerosis Patients

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