scholarly journals Current Difficulties, Desires and Their Meanings in Ventilator-dependent Amyotrophic Lateral Sclerosis Patients Living at Home in Japan: The Relationship with Hope as an Indicator of a Will to Live

2009 ◽  
Vol 29 (4) ◽  
pp. 41-50
Author(s):  
Yuko Hirano
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Will To Live ◽  
The Relationship ◽  
Lateral Sclerosis ◽  
At Home

scholarly journals The Relationship of Lower-Limb Muscle Force to Walking Ability in Patients With Amyotrophic Lateral Sclerosis

1999 ◽  
Vol 79 (7) ◽  
pp. 672-681 ◽  
Author(s):  
Diane U Jette ◽  
Mary D Slavin ◽  
Patricia L Andres ◽  
Theodore L Munsat
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Lower Extremity ◽  
Muscle Force ◽  
Research Unit ◽  
Walking Ability ◽  
Lower Extremity Muscle ◽  
Relationship Of ◽  
The Relationship ◽  
Lateral Sclerosis ◽  
At Home

Abstract Background and Purpose. The purpose of this study was to determine the level of muscle force associated with ability to walk in the community without assistance, in the community with assistance, or at home only in individuals with amyotrophic lateral sclerosis (ALS). Subjects and Methods. Percentage of predicted maximal muscle force (%PMF) of lower-extremity muscles was determined, and walking ability was categorized in 118 patients with ALS during periodic visits to the Neuromuscular Research Unit. Data were derived from consecutive visits in which subjects demonstrated declines in walking ability. Means for %PMF of each muscle group and a limb average were calculated at each consecutive visit. Results. The mean lower-extremity average %PMF was: (1) 54.01% (SD=12.76%) for subjects who walked independently in the community and 50.19% (SD=14.38%) during the next visit when these same subjects required assistance in the community (difference=3.82%, 95% confidence interval [CI][=] 2.45−5.19);(2) 37.52% (SD=15.17%) during the last visit that subjects walked with assistance in the community and 32.18% (SD=13.83%) during the next visit when they walked only at home (difference=5.33%, 95% CI=3.61–7.06); and (3) 19.12% (SD=9.08%) during the visit when subjects were last able to ambulate at home versus 13.70% (SD=7.36%) when they became unable to walk (difference=5.42%, 95% CI=2.97−7.96). Conclusion and Discussion. The findings suggest there are required levels of lower-extremity muscle force for various categories of walking ability. Variations in forces within and between categories of walking ability, however, indicate the complexity of this relationship.

Acoustic Differences Between Content and Function Words in Amyotrophic Lateral Sclerosis

2000 ◽  
Vol 43 (3) ◽  
pp. 769-781 ◽  
Author(s):  
Greg S. Turner ◽  
Kris Tjaden
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Vowel Duration ◽  
Function Words ◽  
Temporal Properties ◽  
Vowel Space ◽  
The Difference ◽  
And Function ◽  
Impaired Speech ◽  
The Relationship ◽  
Lateral Sclerosis

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.

The relationship between Bunina bodies, skein-like inclusions and neuronal loss in amyotrophic lateral sclerosis

2002 ◽  
Vol 103 (6) ◽  
pp. 583-589 ◽  
Author(s):  
M. van Welsem ◽  
Hogenhuis J. ◽  
Meininger V. ◽  
Metsaars W. ◽  
Hauw J.-J. ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Neuronal Loss ◽  
Bunina Bodies ◽  
The Relationship ◽  
Lateral Sclerosis

Amyotrophic Lateral Sclerosis Disease and Environmental Risk Factors

2019 ◽  
pp. 286-304
Author(s):  
Abdeljalil Elgot ◽  
Hasna Lahouaoui ◽  
Ouassil El Kherchi ◽  
Nadia Zouhairi
Keyword(s):  
Oxidative Stress ◽  
Risk Factors ◽  
Heavy Metals ◽  
Amyotrophic Lateral Sclerosis ◽  
Genetic Background ◽  
Neurodegenerative Disorder ◽  
Neurodegenerative Process ◽  
Peripheral Motor ◽  
The Relationship ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that affects central and peripheral motor neuron cells leading to a severe muscle weakness. Until now, no efficient cures exist and those existing are limited. The ALS etiology remains obscure, although the relationship between genetic background and environmental insults including pesticides and heavy metals is well documented. These latter may play a major role in the onset of the ALS neurodegenerative process. Pesticides are known to have many benefits to mankind in the agricultural and industrial areas, but their toxicities in humans have always been a debatable issue. The pathophysiological mechanisms involve, among others, inflammation processes, oxidative stress, and mitochondrial function impairments. The aim of this chapter was to examine the association between the risk of amyotrophic lateral sclerosis (ALS) and exposure to pesticides and heavy metals.

Intelligibility and the Acoustic Characteristics of Speech in Amyotrophic Lateral Sclerosis (ALS)

1994 ◽  
Vol 37 (3) ◽  
pp. 496-503 ◽  
Author(s):  
Moira Mulligan ◽  
Joseph Carpenter ◽  
Joanne Riddel ◽  
Maureen Kenny Delaney ◽  
Gary Badger ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Transition Rate ◽  
Transition Rates ◽  
Single Word ◽  
Acoustic Parameters ◽  
Acoustic Characteristics ◽  
Als Patients ◽  
The Relationship ◽  
Lateral Sclerosis

The purpose of this study was to analyze the changes in specific speech parameters in 14 patients, 7 dysarthric and 7 non-dysarthric, with amyotrophic lateral sclerosis (ALS), over a 6-month period. Measurements of single word intelligibility, F2 formant trajectories (extent, duration and rate) and diadochokinetic rate showed decreased performance in dysarthric patients as compared to non-dysarthric patients at baseline. F2 transition rates of less than 4 Hz/msec were seen only in dysarthric ALS patients. A relationship between the F2 transition rate and single word intelligibility was noted for patients with moderate to high intelligibility, but at lower levels of intelligibility the F2 rate reached a plateau despite continued decline in intelligibility. Our results support the need for frequent evaluation of dysarthric ALS patients to better understand the relationship between intelligibility and the acoustic parameters of speech.

scholarly journals Cognition and behaviour in frontotemporal dementia with and without amyotrophic lateral sclerosis

2020 ◽  
Vol 91 (12) ◽  
pp. 1304-1311 ◽  
Author(s):  
Jennifer A Saxon ◽  
Jennifer C Thompson ◽  
Jennifer M Harris ◽  
Anna M Richardson ◽  
Tobias Langheinrich ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Frontotemporal Dementia ◽  
Neuropsychological Testing ◽  
Behavioural Change ◽  
Repeat Expansion ◽  
Phenotypic Differences ◽  
Prospective Comparative Study ◽  
Precise Relationship ◽  
The Relationship ◽  
Lateral Sclerosis

ObjectiveThe precise relationship between frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) is incompletely understood. The association has been described as a continuum, yet data suggest that this may be an oversimplification. Direct comparisons between patients who have behavioural variant FTD (bvFTD) with and without ALS are rare. This prospective comparative study aimed to determine whether there are phenotypic differences in cognition and behaviour between patients with FTD-ALS and bvFTD alone.MethodsPatients with bvFTD or FTD-ALS and healthy controls underwent neuropsychological testing, focusing on language, executive functions and social cognition. Behavioural change was measured through caregiver interview. Blood samples were screened for known FTD genes.Results23 bvFTD, 20 FTD-ALS and 30 controls participated. On cognitive tests, highly significant differences were elicited between patients and controls, confirming the tests’ sensitivities to FTD. bvFTD and FTD-ALS groups performed similarly, although with slightly greater difficulty in patients with ALS-FTD on category fluency and a sentence-ordering task that assesses grammar production. Patients with bvFTD demonstrated more widespread behavioural change, with more frequent disinhibition, impulsivity, loss of empathy and repetitive behaviours. Behaviour in FTD-ALS was dominated by apathy. The C9ORF72 repeat expansion was associated with poorer performance on language-related tasks.ConclusionsDifferences were elicited in cognition and behaviour between bvFTD and FTD-ALS, and patients carrying the C9ORF72 repeat expansion. The findings, which raise the possibility of phenotypic variation between bvFTD and FTD-ALS, have clinical implications for early detection of FTD-ALS and theoretical implications for the nature of the relationship between FTD and ALS.

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