scholarly journals Cognition, Behavior, and Respiratory Function in Amyotrophic Lateral Sclerosis

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Adriana M. Strutt ◽  
Jennette Palcic ◽  
John G. Wager ◽  
Courtney Titus ◽  
Claire MacAdam ◽  
...  
Keyword(s):  
Executive Functioning ◽  
Frontal Lobe ◽  
Cognitive Abilities ◽  
Neuropsychological Performance ◽  
Neuropsychological Assessments ◽  
Clinically Significant ◽  
Als Patients ◽  
Executive Functioning Deficits ◽  
The Relationship ◽  
Lateral Sclerosis

Objective. To examine the relationship between respiratory functioning and neuropsychological performance, mood, and frontal-lobe-mediated behaviors in ALS patients. Methods. Forty-four patients with probable or definite ALS (El Escorial criteria) completed comprehensive pulmonary and neuropsychological assessments as part of their baseline neurological evaluation. Based on their full vital respiratory capacity, 24 and 20 patients were classified as having impaired or intact respiration, respectively. Results. Comparable demographic characteristics, neuropsychological performance, and self-reported mood symptoms were found between ALS patients with intact versus impaired respiration. However, more respiratory-impaired patients were reported by their caregivers as having clinically significant impairments in frontal-lobe-mediated behaviors. Nevertheless, declines in behavior were evidenced from pre- to post-ALS symptom onset for both respiratory groups, and exploratory analyses revealed greater executive functioning deficits in patients with bulbar versus limb onset as well as respiratory-impaired patients not receiving pulmonary interventions versus those utilizing such interventions at the time of testing. Conclusions. Results suggest that the respiratory insufficiency of ALS patients may potentially produce irreversible deficits in executive functioning; yet once treated, impairments in more basic cognitive abilities may be less evident.

A-151 Cognitive Impairment in Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 36 (6) ◽  
pp. 1205-1205
Author(s):  
Etiane Navarro ◽  
Charles J Golden
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Literature Review ◽  
Cognitive Impairments ◽  
Empirical Literature ◽  
Sporadic Als ◽  
Neuropsychological Assessments ◽  
Familial Als ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Objective Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease caused by degeneration of the upper and lower motor neurons. This literature review examines the recurring etiology of cognitive impairments in ALS through empirical literature. The current study explores ALS across different subtypes and potential cognitive impairments. Two classifications are primarily examined ALS, and ALS with frontotemporal dementia (ALS-FTD). Involving three categories: familial inheritance pattern, genetic mutation, or sporadic. Neuropsychological studies affirm cognitive impairments in individuals diagnosed with ALS and ALS-FTD. Data Selection Data was culled from the American Psychological Association (PsycInfo), PubMed, Google Scholar. Terms used in this literature review include cognitive impairment in ALS and ALS-FTD, executive function deficiencies in ALS, neuropsychology in ALS, neuropsychological deficits in ALS, neuropsychological assessments for ALS, cognitive impairments in familial ALS, genetic ALS, and sporadic ALS, familial ALS, sporadic ALS, genetic mutations involved in ALS. Search dates December 20–23 of 2020 and March 3–4 of 2021. A total of 40 studies were examined. Data Synthesis ALS-patients demonstrate a significant cognitive impairment. However, influencing comorbidities accompanying the disease may be contributing to these impairments. Researchers employed neuroimaging and neuropsychological batteries to further understand influencing factors involved in ALS and cognition. Conclusions Researchers now understand ALS as a multi-symptomatic disorder and acknowledge the presence of cognitive impairments at various encased levels. There are limitations in neuropsychological batteries that accommodate for executive dysfunctions observed in ALS patients. Future studies should explore neuropsychological assessments that accommodate for motor deficits and dysarthria when assessing cognitive impairment in ALS patients.

scholarly journals Performance Differences Using a Vibro-Tactile P300 BCI in LIS-Patients Diagnosed With Stroke and ALS

2018 ◽  
Vol 12 ◽  
Author(s):  
Alexander Heilinger ◽  
Rupert Ortner ◽  
Vincenzo La Bella ◽  
Zulay R. Lugo ◽  
Camille Chatelle ◽  
...  
Keyword(s):  
Cognitive Abilities ◽  
Behavioral Assessment ◽  
Event Related Potentials ◽  
Left Hand ◽  
Related Potentials ◽  
The Right ◽  
Command Following ◽  
Als Patients ◽  
Assessment Scales ◽  
Lateral Sclerosis

Patients with locked-in syndrome (LIS) are typically unable to move or communicate and can be misdiagnosed as patients with disorders of consciousness (DOC). Behavioral assessment scales are limited in their ability to detect signs of consciousness in this population. Recent research has shown that brain-computer interface (BCI) technology could supplement behavioral scales and allows to establish communication with these severely disabled patients. In this study, we compared the vibro-tactile P300 based BCI performance in two groups of patients with LIS of different etiologies: stroke (n = 6) and amyotrophic lateral sclerosis (ALS) (n = 9). Two vibro-tactile paradigms were administered to the patients to assess conscious function and command following. The first paradigm is called vibrotactile evoked potentials (EPs) with two tactors (VT2), where two stimulators were placed on the patient’s left and right wrist, respectively. The patients were asked to count the rare stimuli presented to one wrist to elicit a P300 complex to target stimuli only. In the second paradigm, namely vibrotactile EPs with three tactors (VT3), two stimulators were placed on the wrists as done in VT2, and one additional stimulator was placed on his/her back. The task was to count the rare stimuli presented to one wrist, to elicit the event-related potentials (ERPs). The VT3 paradigm could also be used for communication. For this purpose, the patient had to count the stimuli presented to the left hand to answer “yes” and to count the stimuli presented to the right hand to answer “no.” All patients except one performed above chance level in at least one run in the VT2 paradigm. In the VT3 paradigm, all 6 stroke patients and 8/9 ALS patients showed at least one run above chance. Overall, patients achieved higher accuracies in VT2 than VT3. LIS patients due to ALS exhibited higher accuracies that LIS patients due to stroke, in both the VT2 and VT3 paradigms. These initial data suggest that controlling this type of BCI requires specific cognitive abilities that may be impaired in certain sub-groups of severely motor-impaired patients. Future studies on a larger cohort of patients are needed to better identify and understand the underlying cortical mechanisms of these differences.

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 Autism Traits Predict Self-reported Executive Functioning Deficits in Everyday Life and an Aversion to Exercise

2020 ◽  
Author(s):  
Lauren A. Mason ◽  
Brandon M. Zimiga ◽  
Regina Anders-Jefferson ◽  
Kenneth R. Paap
Keyword(s):  
Physical Activity ◽  
Executive Functioning ◽  
Everyday Life ◽  
Task Performance ◽  
Negatively Associated ◽  
Autism Traits ◽  
Normal Intelligence ◽  
Autism Quotient ◽  
Executive Functioning Deficits ◽  
The Relationship

Abstract Are Autism Quotient (AQ) scores related to executive functioning (EF)? We sampled 200 students of normal intelligence and examined the relationship between AQ scores and: (a) 5 self-ratings of EF, (b) 5 performance-based measures of EF, and (c) 5 types of activities or experiences that are assumed to recruit EF and sometimes enhance EF. Our findings reveal that as AQ scores increase, self-rated EF ability decreases. AQ scores and self-reported EF measures do not correlate with objective EF task performance. Furthermore, AQ scores were shown to be negatively associated with many specific types of physical activity. As AQ scores increase, individuals report fewer positive reasons for exercise and more rationalizations for not engaging in more exercise.

The Relationship Between Emotion Regulation, Executive Functioning, and Aggressive Behaviors

2015 ◽  
Vol 32 (11) ◽  
pp. 1692-1707 ◽  
Author(s):  
Sarah R. Holley ◽  
Scott T. Ewing ◽  
Jordan T. Stiver ◽  
Lian Bloch
Keyword(s):  
Emotion Regulation ◽  
Executive Functioning ◽  
Undergraduate Students ◽  
Physical Aggression ◽  
Self Report ◽  
Aggressive Behaviors ◽  
Different Types ◽  
Executive Functioning Deficits ◽  
Main Effects ◽  
The Relationship

Emotion regulation deficits and executive functioning deficits have independently been shown to increase vulnerability toward engaging in aggressive behaviors. The effects of these risk factors, however, have not been evaluated in relation to one another. This study evaluated the degree to which each was associated with aggressive behaviors in a sample of 168 undergraduate students. Executive functioning (cognitive inhibition and mental flexibility) was assessed with a Stroop-like neuropsychological task. Emotion regulation and aggressive behaviors were assessed via self-report inventories. Results showed main effects for both emotion regulation and executive functioning, as well as a significant interaction, indicating that those who scored lowest in both domains reported engaging in aggressive behaviors the most frequently. When different types of aggression were examined, this interaction was only significant for acts of physical aggression, not for acts of verbal aggression. Therefore, for physical aggression, emotion regulation and executive functioning exerted a moderating effect on one another. The implications are that, at least for acts of physical aggression, relatively strong capabilities in either domain may buffer against tendencies to engage in aggressive behaviors. Thus, both emotion regulation skills and executive functioning abilities may be valuable targets for interventions aiming to reduce aggressive behaviors.

scholarly journals The links between diabetes mellitus and amyotrophic lateral sclerosis

2021 ◽  
Vol 42 (4) ◽  
pp. 1377-1387
Author(s):  
Rosario Vasta ◽  
Fabrizio D’Ovidio ◽  
Giancarlo Logroscino ◽  
Adriano Chiò
Keyword(s):  
Diabetes Mellitus ◽  
Metabolic Diseases ◽  
Age Of Onset ◽  
European Population ◽  
Metabolic Changes ◽  
Exact Nature ◽  
Younger Age ◽  
Als Patients ◽  
The Relationship ◽  
Lateral Sclerosis

AbstractALS etiology and prognostic factors are mostly unknown. Metabolic diseases and especially diabetes mellitus (DM) have been variously related to ALS. However, pieces of evidence have been variegated and often conflicting so far. This review aims to give an overview of recent contributions focusing on the relationship between DM and ALS. DM seems to reduce the risk of developing ALS if diagnosed at a younger age; conversely, when diagnosed at an older age, DM seems protective against ALS. Such a relationship was not confirmed in Asian countries where DM increases the risk of ALS independently of the age of onset. Interestingly, DM does not affect ALS prognosis, possibly weakening the potential causal relationship between the two diseases. However, since most studies are observational, it is difficult to state the exact nature of such a relationship and several hypotheses have been made. A recent study using Mendelian randomization suggested that DM is indeed protective against ALS in the European population. However, these analyses are not without limits and further evidence is needed. DM is usually the core of a larger metabolic syndrome. Thus, other metabolic changes such as dyslipidemia, body mass index, and cardiovascular diseases should be collectively considered. Finally, hypermetabolism usually found in ALS patients should be considered too since all these metabolic changes could be compensation (or the cause) of the higher energy expenditure.

Neuropsychological Correlates of Hazard Perception in Older Adults

2015 ◽  
Vol 22 (3) ◽  
pp. 332-340 ◽  
Author(s):  
Katalina McInerney ◽  
Julie Suhr
Keyword(s):  
Executive Functioning ◽  
Block Design ◽  
Driving Ability ◽  
Neuropsychological Performance ◽  
Trail Making Test ◽  
Hazard Perception ◽  
Neuropsychological Correlates ◽  
Trail Making ◽  
Relationship Of ◽  
The Relationship

AbstractObjectives: Hazard perception, the ability to identify and react to hazards while driving, is of growing importance in driving research, given its strong relationship to real word driving variables. Furthermore, although poor hazard perception is associated with novice drivers, recent research suggests that it declines with advanced age. In the present study, we examined the neuropsychological correlates of hazard perception in a healthy older adult sample. Methods: A total of 68 adults age 60 and older who showed no signs of dementia and were active drivers completed a battery of neuropsychological tests as well as a hazard perception task. Tests included the Repeatable Battery for the Assessment of Neuropsychological Status, Wechsler Test of Adult Reading, Trail Making Test, Block Design, Useful Field of View, and the Delis-Kaplan Executive Function System Color Word Interference Test. Results: Hazard perception errors were related to visuospatial/constructional skills, processing speed, memory, and executive functioning skills, with a battery of tests across these domains accounting for 36.7% of the variance in hazard perception errors. Executive functioning, particularly Trail Making Test part B, emerged as a strong predictor of hazard perception ability. Conclusions: Consistent with prior work showing the relationship of neuropsychological performance to other measures of driving ability, neuropsychological performance was associated with hazard perception skill. Future studies should examine the relationship of neuropsychological changes in adults who are showing driving impairment and/or cognitive changes associated with Mild Cognitive Impairment or dementia. (JINS, 2015, 21, 332–340)

scholarly journals Shortened TDP43 isoforms upregulated by neuronal hyperactivity drive TDP43 pathology in ALS

2019 ◽  
Author(s):  
Kaitlin Weskamp ◽  
Elizabeth M. Tank ◽  
Roberto Miguez ◽  
Jonathon P. McBride ◽  
Nicolás B. Gómez ◽  
...  
Keyword(s):  
Motor Neurons ◽  
Rna Binding ◽  
Loss Of Function ◽  
Pathogenic Role ◽  
Splice Isoforms ◽  
Cortical Hyperexcitability ◽  
Als Patients ◽  
Truncated Isoforms ◽  
The Relationship ◽  
Lateral Sclerosis

AbstractCortical hyperexcitability and mislocalization of the RNA-binding protein TDP43 are highly-conserved features in amyotrophic lateral sclerosis (ALS). Nevertheless, the relationship between these phenomena remains poorly defined. Here, we showed that hyperexcitability recapitulates TDP43 pathology by upregulating shortened (s) TDP43 splice isoforms. These truncated isoforms accumulated in the cytoplasm and formed insoluble inclusions that sequestered full-length TDP43 via preserved N-terminal interactions. Consistent with these findings, sTDP43 overexpression was toxic to mammalian neurons, suggesting neurodegeneration arising from complementary gain- and loss-of-function mechanisms. In humans and mice, sTDP43 transcripts were enriched in vulnerable motor neurons, and we observed a striking accumulation of sTDP43 within neurons and glia of ALS patients. Collectively, these studies uncover a pathogenic role for alternative TDP43 isoforms in ALS, and implicate sTDP43 as a key contributor to the susceptibility of motor neurons in this disorder.

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