scholarly journals Serotonin depletion near end‐stage disease does not impair ventilation in a rat model of amyotrophic lateral sclerosis (ALS)

2009 ◽  
Vol 23 (S1) ◽  
Author(s):  
Nicole L. Nichols ◽  
Rebecca A. Johnson ◽  
Lisa J. Nashold ◽  
Gordon S. Mitchell
2016 ◽  
Vol 15 (1) ◽  
pp. 24-29
Author(s):  
Maria Rivara ◽  
◽  
Cornelius Barlascini ◽  
Paolo Banfi ◽  
Antonello Nicolini ◽  
...  

Objectives. The possibility of prolonging and augmenting the quality of life of respiratory patients in end-stage disease and in particular neuromuscular disease patients depends on an increased level of care. Amyotrophic lateral sclerosis (ALS) is a neurological disease characterized by a fast progressive impairment of respiratory function which leads to mechanical ventilation and high burden of care. A specifically designed questionnaire was administered to caregivers to analyze the level of care provided to these patients in our districts. Patients and methods. The study population consisted of 70 patients (44 males and 26 females). Of the 70 patients, 46 were being treated with non-invasive ventilation and 24 with invasive mechanical ventilation. The questionnaire was divided in two parts: questions 1 to 13 investigated life conditions and 14 to 22 the quality of care provided. Results. The patients studied present a heavy burden of care and this is in relation to the quantity of respiratory aids prescribed. The symptom which created statistically significant problems for care was breathlessness, as is often reported in the literature. The presence of homecare tended to create conditions of greater serenity for the caregivers. Conclusions. This study shows that the burden of care in end-stage ALS patients is very onerous, and it can help to point out problems not sufficiently contemplated in healthcare planning.


2016 ◽  
Vol 31 (2) ◽  
pp. 147-156 ◽  
Author(s):  
Delin Ma ◽  
Jeffrey M. Shuler ◽  
Aishwarya Kumar ◽  
Quincy R. Stanford ◽  
Sudheer Tungtur ◽  
...  

Background. The use of exercise in amyotrophic lateral sclerosis (ALS) is controversial. Although moderate exercise appears to be beneficial for limb muscles in ALS, the effects of exercise on bulbar muscles such as the tongue have not been studied. Objective. To determine the effects of tongue force training on bulbar motor function in the SOD1-G93A rat model of ALS. Methods. We compared the effects of tongue force training on bulbar motor function and neuromuscular junction innervation in female SOD1-G93A rats and age-matched female wild-type controls. Half of each group underwent afternoon tongue force training sessions, and all rats were tested under minimal force conditions in the mornings. Results. Tongue force did not differ between the SOD1-G93A rats and healthy controls during the morning testing sessions, nor was it affected by training. Surprisingly, decreases in tongue motility, the number of licks per session, and body weight were greater in the tongue force–trained SOD1-G93A rats. Forelimb grip force, survival, and denervation of the genioglossus (GG) muscle did not differ between the trained and untrained SOD1-G93A rats. GG innervation was correlated with changes in tongue force but not tongue motility in SOD1-G93A rats at end stage. Conclusions. The results indicate a potential deleterious effect of tongue force training on tongue motility in female SOD1-G93A rats. The lack of a relationship between GG innervation and tongue motility suggests that factors other than lower–motor neuron integrity likely accounted for this effect.


2020 ◽  
Vol 17 (3) ◽  
pp. 275-285 ◽  
Author(s):  
Si Chen ◽  
Qiao Liao ◽  
Ke Lu ◽  
Jinxia Zhou ◽  
Cao Huang ◽  
...  

Background: Amyotrophic lateral sclerosis (ALS) is a neurological disorder clinically characterized by motor system dysfunction, with intraneuronal accumulation of the TAR DNAbinding protein 43 (TDP-43) being a pathological hallmark. Riluzole is a primarily prescribed medicine for ALS patients, while its therapeutical efficacy appears limited. TDP-43 transgenic mice are existing animal models for mechanistic/translational research into ALS. Methods: We developed a transgenic rat model of ALS expressing a mutant human TDP-43 transgene (TDP-43M337V) and evaluated the therapeutic effect of Riluzole on this model. Relative to control, rats with TDP-43M337V expression promoted by the neurofilament heavy subunit (NEF) gene or specifically in motor neurons promoted by the choline acetyltransferase (ChAT) gene showed progressive worsening of mobility and grip strength, along with loss of motor neurons, microglial activation, and intraneuronal accumulation of TDP-43 and ubiquitin aggregations in the spinal cord. Results: Compared to vehicle control, intragastric administration of Riluzole (30 mg/kg/d) did not mitigate the behavioral deficits nor alter the neuropathologies in the transgenics. Conclusion: These findings indicate that transgenic rats recapitulate the basic neurological and neuropathological characteristics of human ALS, while Riluzole treatment can not halt the development of the behavioral and histopathological phenotypes in this new transgenic rodent model of ALS.


2021 ◽  
Vol 1757 ◽  
pp. 147296
Author(s):  
Hirotoshi Magota ◽  
Masanori Sasaki ◽  
Yuko Kataoka-Sasaki ◽  
Shinichi Oka ◽  
Ryo Ukai ◽  
...  

2010 ◽  
Vol 19 (5) ◽  
pp. 537-548 ◽  
Author(s):  
Dinko Mitrečić ◽  
Charles Nicaise ◽  
Srećko Gajović ◽  
Roland Pochet

2016 ◽  
Vol 5 (1) ◽  
pp. 79-96 ◽  
Author(s):  
Ke-Wei Tian ◽  
Hong Jiang ◽  
Bei-Bei Wang ◽  
Fan Zhang ◽  
Shu Han

Non-protein amino acid beta-N-methylamino-l-alanine (l-BMAA) is a neurotoxin that was associated with the high incidence of Amyotrophic Lateral Sclerosis/Parkinson–Dementia Complex (ALS/PDC) in Guam.


2012 ◽  
Vol 13 (3) ◽  
pp. 311-314 ◽  
Author(s):  
Antonio Hayes-Punzo ◽  
Patrick Mulcrone ◽  
Michael Meyer ◽  
Jacalyn Mchugh ◽  
Clive N. Svendsen ◽  
...  

Cytotherapy ◽  
2011 ◽  
Vol 13 (9) ◽  
pp. 1036-1046 ◽  
Author(s):  
Serhiy Forostyak ◽  
Pavla Jendelova ◽  
Miroslava Kapcalova ◽  
David Arboleda ◽  
Eva Sykova

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