Power Wheelchair Use in Persons With Amyotrophic Lateral Sclerosis: Changes Over Time

AbstractBackground: Non-invasive ventilation (NIV) improves quality of life and survival in patients with amyotrophic lateral sclerosis (ALS) and respiratory symptoms. Little is known about the patterns of NIV use over time and the impact of NIV on end-of-life decision-making in ALS. Objective: This study assessed the pattern of NIV use over the course of the disease and the timing of end-of-life discussions in people living with ALS. Method: A retrospective single-center cohort study was performed at London Health Sciences Centre. Daily NIV duration of use was evaluated at 3-month intervals. The timing of diagnosis, NIV initiation, discussions relating to do-not-attempt-resuscitation (DNAR) and death were examined. Results: In total, 48 patients were included in the analysis. Duration of NIV use increased over time, and tolerance to NIV was observed to be better than expected in patients with bulbar-onset ALS. There was a high degree of variability in the timing of end-of-life discussions in patients with ALS (356±451 days from diagnosis). In this cohort, there was a strong association between the timing of discussions regarding code status and establishment of a DNAR order (r2=0.93). Conclusion: This retrospective cohort study suggests that the use of NIV in ALS increases over time and that there remains a great deal of variability in the timing of end-of-life discussions in people living with ALS. Future prospective studies exploring the use NIV over the disease trajectory and how NIV affects end-of-life decision-making in people with ALS are needed.

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Vocal Fold Diadochokinetic Function of Individuals With Amyotrophic Lateral Sclerosis

American Journal of Speech-Language Pathology ◽

10.1044/1058-0360.0401.73 ◽

1995 ◽

Vol 4 (1) ◽

pp. 73-80 ◽

Cited By ~ 5

Author(s):

Karen A. Renout ◽

Herbert A. Leeper ◽

Donna L. Bandur ◽

Arthur J. Hudson

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Vocal Fold ◽

Group Discussion ◽

Vocal Folds ◽

Laryngeal Function ◽

Analysis System ◽

Reduced Rate ◽

Opening And Closing ◽

Lateral Sclerosis ◽

Over Time

This study relates over time the changes in voluntary opening and closing of the vocal folds (vocal fold diadochokinesis, or VFDDK) of patients with amyotrophic lateral sclerosis (ALS). The rate, pattern, and periodicity of VFDDK were examined for 12 ALS subjects with bulbar and 14 subjects with nonbulbar signs of the disease. The acoustical data were analyzed with a commercially available computer-based speech analysis system. ALS patients with bulbar and nonbulbar symptoms demonstrate reduced rate and aperiodic VFDDK as the symptomatology of the neuromotor system progresses. Individuals with bulbar signs show a greater change in vocal fold activity than do the nonbulbar group. Discussion of the clinical implications for initial diagnosis and monitoring of changes over time in motor control of laryngeal function is presented.

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Correlations in health status between estimates of families of people with amyotrophic lateral sclerosis and estimates of staff

Palliative & Supportive Care ◽

10.1017/s1478951512000089 ◽

2012 ◽

Vol 11 (3) ◽

pp. 183-189 ◽

Cited By ~ 1

Author(s):

Anneli G. Ozanne ◽

Lennart I. Persson

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Analogue Scale ◽

Rating Scale ◽

Well Being ◽

Life Situation ◽

Next Of Kin ◽

Holistic View ◽

The Family ◽

Lateral Sclerosis ◽

Over Time

AbstractObjective:The aim of this study was to compare self-estimates of the physical, psychological, and general well-being of patients with amyotrophic lateral sclerosis (ALS) and their next of kin with the assessment of the nurses and physician of these participants.Method:The well-being of 35 pairs of patients and their next of kin was rated by themselves, and by a physician and nurses. The well-being was examined over time, using a visual analogue scale (VAS). Patients' physical function was estimated at the same time with the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised and the Norris scale.Results:The correlations between the staff's estimates of the well-being of patients and next of kin were similar to their own estimates, even though staff to a higher degree estimated a decrease in well-being over time among the patients. The estimates by the nurses correlated better to that of the patients and next of kin in psychological and general well-being than the physicians' estimates did.Significance of results:Even though the staff's estimates of participants were roughly equivalent to their self-estimates, there were some differences. This result calls attention to the importance of working in teams in which different professional roles are combined and integrated, making it possible to form a holistic view of the situation of each family. A concern overlooked by one member of staff might be covered by another, and different focuses on the family may give a better composite picture of their life situation, which could lead to better support to the family.

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Longitudinal Study of Cognitive and Emotional Alterations in Amyotrophic Lateral Sclerosis: Clinical and Imaging Data

Frontiers in Neurology ◽

10.3389/fneur.2021.620198 ◽

2021 ◽

Vol 12 ◽

Author(s):

Soumia Benbrika ◽

Franck Doidy ◽

Laurence Carluer ◽

Audrey Mondou ◽

Alice Pélerin ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Executive Functions ◽

Inferior Frontal Gyrus ◽

Disease Onset ◽

Psychological State ◽

Imaging Data ◽

Cortical Thinning ◽

Lateral Sclerosis ◽

Over Time

Objectives: Extra-motor manifestations occur in 50% of patients with amyotrophic lateral sclerosis (ALS). These mainly concern cognition, emotional processing and behavior. Depression and anxiety are less frequent. Little is known about how these manifestations change as the disease progresses. Similarly, although cortical thinning has been well-documented at disease onset, there are scant data about cortical thinning over time and how this correlates with extra-motor manifestations. The present study therefore assessed cognitive, emotional and psychological state and cortical thinning in a group of patients with ALS at baseline and after a follow-up period.Methods: We assessed executive functions, facial emotion recognition, depressive and anxious symptoms, and cortical thinning in 43 patients with ALS at baseline, comparing them with 28 healthy controls, and 21 of them 9 months later. We looked for links among the extra-motor manifestations and correlations with cortical thickness.Results: At baseline, patients had poor executive function and recognition of complex emotions from the eyes, and more anxious and depressive symptoms than controls. At follow-up, only inhibition abilities had worsened. Cortical thinning was observed in bilateral pre-central regions and other parts of the cerebral cortex at baseline. Over time, it worsened in motor and extra-motor areas. Executive functions correlated with thinning in the middle and inferior frontal gyrus and orbitofrontal cortex.Conclusions: During follow-up, there was little deterioration in extra-motor manifestations and psychological state, despite continuing cortical thinning. Patients with affective Theory of Mind (ToM) changes seemed less depressed than the others. Impaired mental flexibility was subtended by prefrontal regions with cortical thinning.

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Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis

Frontiers in Neurology ◽

10.3389/fneur.2021.749736 ◽

2021 ◽

Vol 12 ◽

Author(s):

Matteo Paoletti ◽

Luca Diamanti ◽

Shaun I. Muzic ◽

Elena Ballante ◽

Francesca Solazzo ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Disease Progression ◽

Rating Scale ◽

Quantitative Mri ◽

Triceps Surae ◽

Muscle Mri ◽

Muscle Involvement ◽

Leg Muscles ◽

Lateral Sclerosis ◽

Over Time

Background: Biomarkers of disease progression and outcome measures are still lacking for patients with amyotrophic lateral sclerosis (ALS). Muscle MRI can be a promising candidate to track longitudinal changes and to predict response to the therapy in clinical trials.Objective: Our aim is to apply quantitative muscle MRI in the evaluation of disease progression, focusing on thigh and leg muscles of patients with ALS, and to explore the correlation between radiological and clinical scores.Methods: We enrolled newly diagnosed patients with ALS, longitudinally scored using the ALS Functional Rating Scale-Revised (ALSFRS-R), who underwent a 3T muscle MRI protocol including a 6-point Dixon gradient-echo sequence and multi-echo turbo spin echo (TSE) T2-weighted sequence for quantification of fat fraction (FF), cross-sectional area (CSA), and water T2 (wT2). A total of 12 muscles of the thigh and six muscles of the leg were assessed by the manual drawing of 18 regions of interest (ROIs), for each side. A group of 11 age-matched healthy controls (HCs) was enrolled for comparison.Results: 15 patients (M/F 8/7; mean age 62.2 years old, range 29–79) diagnosed with possible (n = 2), probable (n = 12), or definite (n = 1) ALS were enrolled. Eleven patients presented spinal onset, whereas four of them had initial bulbar involvement. All patients performed MRI at T0, nine of them at T1, and seven of them at T2. At baseline, wT2 was significantly elevated in ALS subjects compared to HCs for several muscles of the thigh and mainly for leg muscles. By contrast, FF was elevated in few muscles, and mainly at the level of the thigh. The applied mixed effects model showed that FF increased significantly in the leg muscles over time (mainly in the triceps surae) and that wT2 decreased significantly in line with worsening in the leg subscore of ALSFRS-R, mainly at the leg level and in the anterior and medial compartment of the thigh.Conclusions: Quantitative MRI represents a non-invasive tool that is able to outline the trajectory of pathogenic modifications at the muscle level in ALS. In particular, wT2 was found to be increased early in the clinical history of ALS and also tended to decrease over time, also showing a positive correlation with leg subscore of ALSFRS-R.

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Stem cell-derived cranial and spinal motor neurons reveal proteostatic differences between ALS resistant and sensitive motor neurons

eLife ◽

10.7554/elife.44423 ◽

2019 ◽

Vol 8 ◽

Cited By ~ 4

Author(s):

Disi An ◽

Ryosuke Fujiki ◽

Dylan E Iannitelli ◽

John W Smerdon ◽

Shuvadeep Maity ◽

...

Keyword(s):

Stem Cells ◽

Amyotrophic Lateral Sclerosis ◽

Motor Neurons ◽

Embryonic Stem ◽

Spinal Motor Neurons ◽

Spinal Motor ◽

Chemically Induced ◽

Late Stages ◽

Lateral Sclerosis ◽

Over Time

In amyotrophic lateral sclerosis (ALS) spinal motor neurons (SpMN) progressively degenerate while a subset of cranial motor neurons (CrMN) are spared until late stages of the disease. Using a rapid and efficient protocol to differentiate mouse embryonic stem cells (ESC) to SpMNs and CrMNs, we now report that ESC-derived CrMNs accumulate less human (h)SOD1 and insoluble p62 than SpMNs over time. ESC-derived CrMNs have higher proteasome activity to degrade misfolded proteins and are intrinsically more resistant to chemically-induced proteostatic stress than SpMNs. Chemical and genetic activation of the proteasome rescues SpMN sensitivity to proteostatic stress. In agreement, the hSOD1 G93A mouse model reveals that ALS-resistant CrMNs accumulate less insoluble hSOD1 and p62-containing inclusions than SpMNs. Primary-derived ALS-resistant CrMNs are also more resistant than SpMNs to proteostatic stress. Thus, an ESC-based platform has identified a superior capacity to maintain a healthy proteome as a possible mechanism to resist ALS-induced neurodegeneration.

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Progressive Neurochemical Abnormalities in Cognitive and Motor Subgroups of Amyotrophic Lateral Sclerosis: A Prospective Multicenter Study

Neurology ◽

10.1212/wnl.0000000000012367 ◽

2021 ◽

pp. 10.1212/WNL.0000000000012367

Author(s):

Daniel Ta ◽

Abdullah Ishaque ◽

Ojas Srivastava ◽

Chris Hanstock ◽

Peter Seres ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Prefrontal Cortex ◽

Motor Cortex ◽

Disease Progression ◽

Multicenter Study ◽

Rapid Disease Progression ◽

Prospective Longitudinal ◽

Lateral Sclerosis ◽

Over Time ◽

Cerebral Degeneration

OBJECTIVE:To evaluate progressive cerebral degeneration in amyotrophic lateral sclerosis (ALS) by assessing alterations in N-acetylaspartate (NAA) ratios in the motor and prefrontal cortex within clinical subgroups of ALS.METHODS:Seventy-six ALS patients and 59 healthy controls were enrolled in a prospective, longitudinal, multicenter study in the Canadian ALS Neuroimaging Consortium (CALSNIC). Participants underwent serial clinical evaluations and MR spectroscopy at baseline, 4 and 8 months using a harmonized protocol across 5 centers. NAA ratios were quantified in the motor cortex and prefrontal cortex. Patients were stratified into subgroups based on disease progression rate, upper motor neuron (UMN) signs, and cognitive status. Linear mixed models were used for baseline and longitudinal comparisons of NAA metabolite ratios.RESULTS:Patients with ALS had reduced NAA ratios in the motor cortex at baseline (P < 0.001). Ratios were lower in those with more rapid disease progression and greater UMN signs (P < 0.05). A longitudinal decline in NAA ratios was observed in the motor cortex in the rapid progressing (P < 0.01) and high UMN burden (P < 0.01) cohorts. The severity of UMN signs did not change significantly over time. NAA ratios were reduced in the prefrontal cortex only in cognitively impaired patients (P < 0.05); prefrontal cortex metabolites did not change over time.CONCLUSIONS:Progressive degeneration of the motor cortex in ALS is associated with more aggressive clinical presentations These findings provide biological evidence of variable spatial and temporal cerebral degeneration linked to the disease heterogeneity of ALS. The use of standardized imaging protocols may have a role to play in clinical trials for patient selection or subgrouping.CLASSIFICATION OF EVIDENCE:This study provides Class II evidence that MRS NAA metabolite ratios of the motor cortex are associated with more rapid disease progression and greater UMN signs in patients with ALS.

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