Cortical Plasticity in Amyotrophic Lateral Sclerosis: Motor Imagery and Function

Studies describing acoustic characteristics of speech produced by individuals with dysarthria may help to explain intelligibility deficits for these speakers. One goal of the current study was to investigate the manner and extent to which nine speakers with mild to moderate dysarthria associated with amyotrophic lateral sclerosis (ALS) and nine healthy speakers acoustically distinguished /i/, / æ/, /u/, and /α/ in content and function words. A further aim was to evaluate the relationship between impaired speech in ALS and the magnitude of acoustic differences for vowels in content and function words. Speakers read the Farm Passage at a comfortable or habitual rate. F1 and F2 midpoint frequencies were measured, and vowel space areas were calculated. Vowel durations also were measured. The magnitude of F1, F2, vowel space area, and duration differences for vowels in content and function words was not statistically different for speakers with ALS and healthy controls. In addition, with the exception of /i/ produced by some speakers with ALS, vowel duration tended to be shorter in function words. Average F1 and F2 values for function words also tended to be centralized relative to content words. Although vowel space area differences for the two speaker groups were not statistically significant, there was a tendency for the difference in vowel space area for content and function words to be smaller for speakers with ALS than for controls. Regression analyses further indicated that the magnitude of temporal differences for vowels in content and function words was a better predictor of impaired speech than the magnitude of spectral differences for vowels in content and function words. One clinical implication is that individuals with ALS may benefit from therapy techniques targeting temporal properties of the acoustic signal.

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Motor Imagery EEG Classification for Patients with Amyotrophic Lateral Sclerosis Using Fractal Dimension and Fisher’s Criterion-Based Channel Selection

Sensors ◽

10.3390/s17071557 ◽

2017 ◽

Vol 17 (7) ◽

pp. 1557 ◽

Cited By ~ 9

Author(s):

Yi-Hung Liu ◽

Shiuan Huang ◽

Yi-De Huang

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Fractal Dimension ◽

Motor Imagery ◽

Channel Selection ◽

Eeg Classification ◽

Fisher’S Criterion ◽

Lateral Sclerosis

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P-glycoprotein expression and function are increased in an animal model of amyotrophic lateral sclerosis

Neuroscience Letters ◽

10.1016/j.neulet.2010.01.078 ◽

2010 ◽

Vol 472 (3) ◽

pp. 166-170 ◽

Cited By ~ 32

Author(s):

Aline Milane ◽

Christine Fernandez ◽

Luc Dupuis ◽

Marion Buyse ◽

Jean-Philippe Loeffler ◽

...

Keyword(s):

Animal Model ◽

Amyotrophic Lateral Sclerosis ◽

P Glycoprotein ◽

And Function ◽

Lateral Sclerosis

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Association Between Dietary Intake and Function in Amyotrophic Lateral Sclerosis

JAMA Neurology ◽

10.1001/jamaneurol.2016.3401 ◽

2016 ◽

Vol 73 (12) ◽

pp. 1425 ◽

Cited By ~ 30

Author(s):

Jeri W. Nieves ◽

Chris Gennings ◽

Pam Factor-Litvak ◽

Jonathan Hupf ◽

Jessica Singleton ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Dietary Intake ◽

And Function ◽

Lateral Sclerosis

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Progress in Therapy Development for Amyotrophic Lateral Sclerosis

Neurology Research International ◽

10.1155/2012/187234 ◽

2012 ◽

Vol 2012 ◽

pp. 1-9 ◽

Cited By ~ 1

Author(s):

Kalina Venkova-Hristova ◽

Alexandar Christov ◽

Zarine Kamaluddin ◽

Peter Kobalka ◽

Kenneth Hensley

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Motor Neurons ◽

Experimental Therapeutics ◽

Loss Of Function ◽

Paracrine Factors ◽

Neuronal Homeostasis ◽

Frequent Failure ◽

Clinical Tools ◽

And Function ◽

Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that cannot be slowed substantially using any currently-available clinical tools. Through decades of studying sporadic and familial ALS (SALS and FALS), researchers are coming to understand ALS as a complex syndrome with diverse genetic and environmental etiologies. It is know appreciated that motor neuron degeneration in ALS requires active (gain of function) and passive (loss of function) events to occur in non-neuronal cells, especially astrocytes and microglia. These neuroinflammatory processes produce paracrine factors that detrimentally affect motor neurons, precipitating protein aggregation and compromising cytoskeletal integrity. The result is a loss of neuronal homeostasis and progressive die-back of motor axons culminating in death of the afflicted motor neurons. This review will discuss experimental therapeutics that have been tested in murine ALS models, with an emphasis on those that have progressed to human clinical trials. Reasons will be considered for the frequent failure of preclinical successes to translate into positive clinical outcomes. Finally, this review will explore current trends in experimental therapeutics for ALS with emphasis on the emerging interest in axon guidance signaling pathways as novel targets for pharmacological support of neural cytoskeletal structure and function in order to slow ALS.

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Characterisation of mesenchymal stem cells from patients with amyotrophic lateral sclerosis

Journal of Clinical Pathology ◽

10.1136/jclinpath-2017-204681 ◽

2018 ◽

Vol 71 (8) ◽

pp. 735-742 ◽

Cited By ~ 1

Author(s):

Nicole Matejckova ◽

Alena Zajicova ◽

Barbora Hermankova ◽

Jan Kossl ◽

Pavla Bohacova ◽

...

Keyword(s):

Stem Cells ◽

Amyotrophic Lateral Sclerosis ◽

Mesenchymal Stem Cells ◽

Metabolic Activity ◽

Differentiation Potential ◽

Expression Of Genes ◽

Growth Properties ◽

And Function ◽

Hepatocyte Growth ◽

Lateral Sclerosis

AimsMesenchymal stem cells (MSCs) have recently been tested in clinical trials to treat severe diseases, including amyotrophic lateral sclerosis (ALS). Since autologous MSCs are frequently used for therapy, we aimed to evaluate the possible influence of the disease on characteristics and function of these cells.MethodsMSCs were isolated from the bone marrow of patients with ALS and compared with MSCs from healthy controls (HC). The cells were tested for phenotype, growth properties, differentiation ability, metabolic activity, secretory potential, expression of genes for immunomodulatory molecules and for the ability to regulate proliferation of mitogen-stimulated peripheral blood leucocytes. MSCs from patients with ALS and HC were either unstimulated or treated with proinflammatory cytokines for 24 hours before testing.ResultsMSCs isolated from patients with ALS have a higher differentiation potential into adipocytes, express elevated levels of mRNA for interleukin-6, but produce less hepatocyte growth factor than MSCs from HC. On the other hand, there were no significant differences between MSCs from patients with ALS and HC in the expression of phenotypic markers, growth properties, metabolic activity, osteogenic differentiation potential and immunoregulatory properties.ConclusionsThe results suggest that, in spite of some differences in cytokine production, MSCs from patients with ALS can be useful as autologous cells in therapy of ALS.

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