Nutritional and Metabolic Support in Adults With Amyotrophic Lateral Sclerosis

2013 ◽  
Vol 22 (1) ◽  
pp. 12-16
Author(s):  
Michelle McDonagh
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
High Standard ◽  
Nutrition Support ◽  
Nutrition Status ◽  
Team Approach ◽  
Registered Dietitian ◽  
Loss Of Appetite ◽  
Evidence Based Guidelines ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is an incurable, degenerative, neuromuscular condition. Nutritional and metabolic support is an integral component of care for patients with ALS because those capable of maintaining a normal nutrition status exhibit a slower rate of disease progression. Although there are multiple barriers to maintaining a normal nutrition status, the multidisciplinary team approach can facilitate early identification of symptoms, early intervention, and better outcomes for these patients. Barriers that patients often encounter include dysphagia, hypermetabolism, hypometabolism, upper extremity weakness, early satiety, constipation, depression, and loss of appetite. The role of the registered dietitian in the multidisciplinary ALS clinic is to intervene by providing a high standard of nutrition support. Further research into other facets of nutrition for ALS could lead to new interventions to slow weight loss and provide evidence-based guidelines for nutrition professionals. Areas for further research include the use of appetite stimulants, dietary supplements, delayed gastric motility, diet composition, metabolic rate, and the development of a standard equation for estimation of caloric needs in ALS.

The Role of Autophagy: What can be Learned from the Genetic Forms of Amyotrophic Lateral Sclerosis

2010 ◽  
Vol 9 (3) ◽  
pp. 268-278 ◽  
Author(s):  
Livia Pasquali ◽  
Riccardo Ruffoli ◽  
Federica Fulceri ◽  
Sara Pietracupa ◽  
Gabriele Siciliano ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Lateral Sclerosis

scholarly journals Cortical and Striatal Electroencephalograms and Apomorphine Effects in the FUS Mouse Model of Amyotrophic Lateral Sclerosis

2021 ◽  
pp. 1-15
Author(s):  
Vasily Vorobyov ◽  
Alexander Deev ◽  
Frank Sengpiel ◽  
Vladimir Nebogatikov ◽  
Aleksey A. Ustyugov
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Cortical Neurons ◽  
Primary Motor Cortex ◽  
Beta Activity ◽  
Systemic Injection ◽  
Eeg Spectra ◽  
Electroencephalogram Eeg ◽  
Lateral Sclerosis

Background: Amyotrophic lateral sclerosis (ALS) is characterized by degeneration of motor neurons resulting in muscle atrophy. In contrast to the lower motor neurons, the role of upper (cortical) neurons in ALS is yet unclear. Maturation of locomotor networks is supported by dopaminergic (DA) projections from substantia nigra to the spinal cord and striatum. Objective: To examine the contribution of DA mediation in the striatum-cortex networks in ALS progression. Methods: We studied electroencephalogram (EEG) from striatal putamen (Pt) and primary motor cortex (M1) in ΔFUS(1–359)-transgenic (Tg) mice, a model of ALS. EEG from M1 and Pt were recorded in freely moving young (2-month-old) and older (5-month-old) Tg and non-transgenic (nTg) mice. EEG spectra were analyzed for 30 min before and for 60 min after systemic injection of a DA mimetic, apomorphine (APO), and saline. Results: In young Tg versus nTg mice, baseline EEG spectra in M1 were comparable, whereas in Pt, beta activity in Tg mice was enhanced. In older Tg versus nTg mice, beta dominated in EEG from both M1 and Pt, whereas theta and delta 2 activities were reduced. In younger Tg versus nTg mice, APO increased theta and decreased beta 2 predominantly in M1. In older mice, APO effects in these frequency bands were inversed and accompanied by enhanced delta 2 and attenuated alpha in Tg versus nTg mice. Conclusion: We suggest that revealed EEG modifications in ΔFUS(1–359)-transgenic mice are associated with early alterations in the striatum-cortex interrelations and DA transmission followed by adaptive intracerebral transformations.

scholarly journals A modular tool to query and inducibly disrupt biomolecular condensates

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Carmen N. Hernández-Candia ◽  
Sarah Pearce ◽  
Chandra L. Tucker
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cellular Function ◽  
Biological Role ◽  
Cellular Biology ◽  
Condensate Formation ◽  
Health And Disease ◽  
Lateral Sclerosis

AbstractDynamic membraneless compartments formed by protein condensates have multifunctional roles in cellular biology. Tools that inducibly trigger condensate formation have been useful for exploring their cellular function, however, there are few tools that provide inducible control over condensate disruption. To address this need we developed DisCo (Disassembly of Condensates), which relies on the use of chemical dimerizers to inducibly recruit a ligand to the condensate-forming protein, triggering condensate dissociation. We demonstrate use of DisCo to disrupt condensates of FUS, associated with amyotrophic lateral sclerosis, and to prevent formation of polyglutamine-containing huntingtin condensates, associated with Huntington’s disease. In addition, we combined DisCo with a tool to induce condensates with light, CRY2olig, achieving bidirectional control of condensate formation and disassembly using orthogonal inputs of light and rapamycin. Our results demonstrate a method to manipulate condensate states that will have broad utility, enabling better understanding of the biological role of condensates in health and disease.

The epidemiology of amyotrophic lateral sclerosis in the Mount Etna region: a possible pathogenic role of volcanogenic metals

2016 ◽  
Vol 23 (5) ◽  
pp. 964-972 ◽  
Author(s):  
A. Nicoletti ◽  
R. Vasta ◽  
V. Venti ◽  
G. Mostile ◽  
S. Lo Fermo ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mount Etna ◽  
Pathogenic Role ◽  
Lateral Sclerosis

Neurodegeneration in amyotrophic lateral sclerosis: the role of oxidative stress and altered homeostasis of metals

2003 ◽  
Vol 61 (4) ◽  
pp. 365-374 ◽  
Author(s):  
Maria Teresa Carrı̀ ◽  
Alberto Ferri ◽  
Mauro Cozzolino ◽  
Lilia Calabrese ◽  
Giuseppe Rotilio
Keyword(s):  
Oxidative Stress ◽  
Amyotrophic Lateral Sclerosis ◽  
Lateral Sclerosis

scholarly journals Questioning on the role of D amino acid oxidase in familial amyotrophic lateral sclerosis

2010 ◽  
Vol 107 (26) ◽  
pp. E107-E107 ◽  
Author(s):  
S. Millecamps ◽  
S. Da Barroca ◽  
C. Cazeneuve ◽  
F. Salachas ◽  
P.-F. Pradat ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Amino Acid ◽  
Familial Amyotrophic Lateral Sclerosis ◽  
Acid Oxidase ◽  
Amino Acid Oxidase ◽  
Lateral Sclerosis

Blenderized Tube Feeding and Enterostomy Tube Occlusions Among Adults with Amyotrophic Lateral Sclerosis and Primary Lateral Sclerosis

2021 ◽  
pp. 1-4
Author(s):  
Claire Kariya ◽  
Lisa Vardi
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Perceived Risk ◽  
Tube Feeding ◽  
Feeding Tube ◽  
Nutrition Support ◽  
Primary Lateral Sclerosis ◽  
Swallowing Difficulties ◽  
Enterostomy Tube ◽  
Primary Lateral ◽  
Lateral Sclerosis

Adults with amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS) may develop swallowing difficulties and elect to receive an enterostomy feeding tube for nutrition support. Blenderized tube feeding (BTF) appeals to those interested in a homemade enteral nutrition option, but there are concerns of feeding tube occlusion and limited research on this potential risk. Therefore, our purpose was to determine the frequency of, and risk factors for, feeding tube occlusions among adults with ALS or PLS who use BTF. For this retrospective study, the electronic medical records of tube-fed adults with ALS or PLS who received outpatient care at a provincial ALS clinic during a two-year period were reviewed (n = 651). There were 97 tube-fed patients identified, of which 20 (21%) used BTF. Average duration of BTF use was 11.25 ± 7.5 months. Seven subjects (35%) used BTF exclusively, while 13 (65%) used a combination of BTF and commercial enteral formula. All received BTF by gastrostomy tube, sized 14 to 24 French. BTF administration methods and compliance with water flush recommendations varied. Despite the perceived risk of feeding tube occlusions with blenderized tube feeding, no occlusions were found to have occurred in this study.

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