scholarly journals Practical Measures for Dealing With the Struggles of Nurses Caring for People With Amyotrophic Lateral Sclerosis Comorbid With Cognitive Impairment in Japan

2021 ◽  
Vol 12 ◽  
Author(s):  
Mitsuko Ushikubo ◽  
Emiko Nashiki ◽  
Tadahiro Ohtani ◽  
Hiromi Kawabata
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Early Stage ◽  
Care Delivery ◽  
Care Quality ◽  
Daily Lives ◽  
Free Writing ◽  
Group Interviews ◽  
Als Patients ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease for which there is currently no cure. This study aimed to explore the situations with which nurses struggled, their implemented practical measures, and the challenges they experienced when caring for patients with ALS comorbid with cognitive impairment (hereinafter, targeted patients). In this qualitative study, we conducted a survey with nurses (n = 121) experienced in caring for ALS patients; the survey contained a free-writing section in which participants described their struggles regarding care delivery for these patients. To collect data on practical measures that nurses had already implemented or wanted to propose regarding care delivery for the targeted patients, we conducted four focus group interviews (n = 22). We used a qualitative inductive approach to extract the categories. Fifty-eight nurses (49.6%) completed the free-writing survey section. The situations in which nurses struggled in care for the targeted patients were organized into three categories: “Patients’ strong persistency on specific requirements for nursing assistance in their daily lives,” “Patients’ problematic behaviors toward nurses,” and “Struggles in communicating with and understanding patients’ wishes.” Nurses reported these situations as stressful, and they affected care quality. The practical measures implemented when caring for the targeted patients were organized into five categories: “Cognitive impairment assessment,” “Care delivery to deal with patients’ strong persistency on specific requirements for assistance in their daily lives,” “Communication,” “Supporting the decision-making process,” and “Collaboration between the hospital and the community.” Multidisciplinary collaboration in the hospital, and collaboration between the hospital and the community from an early stage is necessary to share the results of the assessment and diagnosis of cognitive impairment. Our evidence underlines that guideline and care manual establishment may lead to improved care delivery and to the unification of care deliveries to respond to patients’ strong persistency.

scholarly journals Cognitive dysfunction in amyotrophic lateral sclerosis: can we predict it?

2021 ◽  
Author(s):  
Fabiola De Marchi ◽  
◽  
Claudia Carrarini ◽  
Antonio De Martino ◽  
Luca Diamanti ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Time Course ◽  
Neurodegenerative Disorder ◽  
Early Stage ◽  
Multisystem Disorder ◽  
Behavioral Impairment ◽  
Behavioral Impairments ◽  
Als Patients ◽  
Disease Stages ◽  
Lateral Sclerosis

Abstract Background and aim Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by the degeneration of both upper and lower motoneurons in the brain and spinal cord leading to motor and extra-motor symptoms. Although traditionally considered a pure motor disease, recent evidences suggest that ALS is a multisystem disorder. Neuropsychological alterations, in fact, are observed in more than 50% of patients: while executive dysfunctions have been firstly identified, alterations in verbal fluency, behavior, and pragmatic and social cognition have also been described. Detecting and monitoring ALS cognitive and behavioral impairment even at early disease stages is likely to have staging and prognostic implications, and it may impact the enrollment in future clinical trials. During the last 10 years, humoral, radiological, neurophysiological, and genetic biomarkers have been reported in ALS, and some of them seem to potentially correlate to cognitive and behavioral impairment of patients. In this review, we sought to give an up-to-date state of the art of neuropsychological alterations in ALS: we will describe tests used to detect cognitive and behavioral impairment, and we will focus on promising non-invasive biomarkers to detect pre-clinical cognitive decline. Conclusions To date, the research on humoral, radiological, neurophysiological, and genetic correlates of neuropsychological alterations is at the early stage, and no conclusive longitudinal data have been published. Further and longitudinal studies on easily accessible and quantifiable biomarkers are needed to clarify the time course and the evolution of cognitive and behavioral impairments of ALS patients.

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 Cognitive and Behavioral Manifestations in ALS: Beyond Motor System Involvement

Diagnostics ◽  
2021 ◽  
Vol 11 (4) ◽  
pp. 624
Author(s):  
Robert Rusina ◽  
Rik Vandenberghe ◽  
Rose Bruffaerts
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Frontotemporal Dementia ◽  
Clinical Manifestations ◽  
Executive Dysfunction ◽  
Motor Disorder ◽  
Behavioral Impairment ◽  
Caregiver Support ◽  
Als Patients ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) has long been considered to be a purely motor disorder. However, it has become apparent that many ALS patients develop cognitive and behavioral manifestations similar to frontotemporal dementia and the term amyotrophic lateral sclerosis-frontotemporal spectrum disorder (ALS-FTSD) is now used in these circumstances. This review is intended to be an overview of the cognitive and behavioral manifestations commonly encountered in ALS patients with the goal of improving case-oriented management in clinical practice. We introduce the principal ALS-FTSD subtypes and comment on their principal clinical manifestations, neuroimaging findings, neuropathological and genetic background, and summarize available therapeutic options. Diagnostic criteria for ALS-FTSD create distinct categories based on the type of neuropsychological manifestations, i.e., changes in behavior, impaired social cognition, executive dysfunction, and language or memory impairment. Cognitive impairment is found in up to 65%, while frank dementia affects about 15% of ALS patients. ALS motor and cognitive manifestations can worsen in parallel, becoming more pronounced when bulbar functions (affecting speech, swallowing, and salivation) are involved. Dementia can precede or develop after the appearance of motor symptoms. ALS-FTSD patients have a worse prognosis and shorter survival rates than patients with ALS or frontotemporal dementia alone. Important negative prognostic factors are behavioral and personality changes. From the clinician’s perspective, there are five major distinguishable ALS-FTSD subtypes: ALS with cognitive impairment, ALS with behavioral impairment, ALS with combined cognitive and behavioral impairment, fully developed frontotemporal dementia in combination with ALS, and comorbid ALS and Alzheimer’s disease. Although the most consistent ALS and ALS-FTSD pathology is a disturbance in transactive response DNA binding protein 43 kDa (TDP-43) metabolism, alterations in microtubule-associated tau protein metabolism have also been observed in ALS-FTSD. Early detection and careful monitoring of cognitive deficits in ALS are crucial for patient and caregiver support and enable personalized management of individual patient needs.

scholarly journals Extracellular Vesicles as Innovative Treatment Strategy for Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 9 ◽  
Author(s):  
Ke Wang ◽  
Yu Li ◽  
Chao Ren ◽  
Yongjing Wang ◽  
Wenshan He ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Extracellular Vesicles ◽  
Treatment Strategy ◽  
Early Stage ◽  
Membrane Vesicles ◽  
Bioactive Substances ◽  
Innovative Treatment ◽  
Application Potential ◽  
Als Patients ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive and fatal motor neuron degenerative disease, and it is hard to diagnose in the early stage, and treatment means are limited, and the treatment effect is unsatisfactory. Therefore, exploring a new effective treatment strategy is urgently needed for ALS patients. Extracellular vesicles (EVs) are a heterogeneous group of natural membrane vesicles containing many bioactive substances, and they play important roles in the paracrine pathway and exhibit neuroprotection effects. A growing body of evidence shows that EVs have great application potential in diagnosis, treatment, and drug delivery in ALS, and they represent an innovative treatment strategy for ALS. In this review, we will briefly introduce the biogenesis of EVs and focus on discussing the role of EVs in ALS treatment to further enrich and boost the development of EVs as an innovative treatment strategy for ALS.

scholarly journals The Japanese Early-Stage Trial of High-Dose Methylcobalamin for Amyotrophic Lateral Sclerosis (JETALS): Protocol for a Randomized Controlled Trial (Preprint)

2018 ◽  
Author(s):  
Ryosuke Oki ◽  
Yuishin Izumi ◽  
Hiroyuki Nodera ◽  
Yasutaka Sato ◽  
Hiroshi Nokihara ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Early Stage ◽  
Phase Iii ◽  
High Dose ◽  
Efficacy And Safety ◽  
Double Blind ◽  
One Year ◽  
Als Patients ◽  
To Receive ◽  
Lateral Sclerosis

BACKGROUND Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects the upper and lower motor neurons. Currently, only riluzole and edaravone are approved as drugs to treat ALS and new agents with larger effect sizes are warranted. Exploratory analyses in our previous study (study ID #E0302-J081-761) have suggested that high-dose methylcobalamin (E0302) prolonged the overall survival of ALS patients and suppressed ALS progression in patients with a disease duration of less than 12 months. OBJECTIVE This clinical trial aims to evaluate the efficacy and safety of E0302 for treatment of ALS patients within one year of onset. METHODS The Japanese early-stage trial of high-dose methylcobalamin for ALS (JETALS) is a prospective, multicenter, placebo-controlled, double-blind, randomized phase III study conducted at 24 tertiary neurology centers and is funded by the Japan Agency for Medical Research and Development. A total of 128 ALS patients within one year of onset were randomized at a 1:1 ratio to receive intramuscular injection with E0302 50 mg or placebo twice a week for 16 weeks. The primary endpoint is changes in the ALS Functional Rating Scale-Revised (ALSFRS-R) total score at 16 weeks. If patients wish to receive E0302 50 mg after the double-blind administration period, E0302 will be provided to them until March 2020 during the continuous administration period. RESULTS This study began in October 2017 and is being conducted at 24 participating institutions in Japan. The study is in progress and the patient enrollment period is scheduled to end in August 2019, with follow-up scheduled to end in March 2020. CONCLUSIONS This study is being performed to revalidate the efficacy and safety of E0302 in patients with early-stage ALS in the first year of symptom onset. If positive results are obtained, the aim is to apply for E0302 approval as a new drug for the treatment of ALS. CLINICALTRIAL ClinicalTrials.gov NCT03548311; https://clinicaltrials.gov/ct2/show/NCT03548311 (Archived by WebCite at http://www.webcitation.org/74Fw3rDzb) INTERNATIONAL REGISTERED REPOR PRR1-10.2196/12046

Support needs of caregivers of patients with amyotrophic lateral sclerosis: A qualitative study

2018 ◽  
Vol 17 (2) ◽  
pp. 195-201 ◽  
Author(s):  
Jessica de Wit ◽  
Carin D. Schröder ◽  
Julia el Mecky ◽  
Anita Beelen ◽  
Leonard H. van den Berg ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Early Stage ◽  
Care Process ◽  
Support Needs ◽  
Structured Interviews ◽  
Personal Time ◽  
Proactive Approach ◽  
Short Period ◽  
Als Patients ◽  
Lateral Sclerosis

AbstractObjectiveThe aim of this study was to explore the support needs of Dutch informal caregivers of patients with amyotrophic lateral sclerosis (ALS).MethodIndividual semi-structured interviews were conducted with 21 caregivers of ALS patients. Audio-taped interviews were transcribed and data were analyzed thematically.ResultA total of four global support needs emerged: “more personal time”, “assistance in applying for resources”, “counseling”, and “peer contact”. Despite their needs, caregivers are reluctant to apply for and accept support. They saw their own needs as secondary to the needs of the patients.Significance of resultsALS seems to lead to an intensive caregiving situation with multiple needs emerging in a short period. This study offers targets for the development of supportive interventions. A proactive approach seems essential, acknowledging the importance of the role of the caregivers in the care process at an early stage, informing them about the risk of burden, monitoring their wellbeing, and repeatedly offering support opportunities. Using e-health may help tailor interventions to the caregivers’ support needs.

scholarly journals Plasma Uric Acid Helps Predict Cognitive Impairment in Patients With Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 12 ◽  
Author(s):  
Jiahui Tang ◽  
Yuan Yang ◽  
Zhenxiang Gong ◽  
Zehui Li ◽  
Lifang Huang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Uric Acid ◽  
Cognitive Impairment ◽  
Education Level ◽  
Older Age ◽  
Multivariate Linear Regression Analysis ◽  
Behavioral Impairment ◽  
Plasma Uric Acid ◽  
Als Patients ◽  
Lateral Sclerosis

Objective: Uric acid as an antioxidant plays an important role in neurodegenerative disease. Our objective is to investigate the relationship between plasma uric acid and cognitive impairment in patients with amyotrophic lateral sclerosis (ALS).Methods: In this cross-sectional study, 124 ALS patients were screened by the Edinburgh Cognitive and Behavioral Screen (ECAS) and classified according to the revised Strong's criteria. Additionally, based on total ECAS cut-off score patients were categorized into those with cognitive impairment (ALS-cie) and those without cognitive impairment (ALS-ncie), and clinical data and uric acid level were compared between the two groups. Parameters with significant differences were further included in a multivariate linear regression analysis with ECAS score as a dependent variable. Hold-out validation was performed to evaluate the fitness of regression model.Results: Up to 60% of ALS patients showed cognitive or/and behavioral impairment. The ALS-cie group had lower education level (p < 0.001), older age at symptom onset (p = 0.001), older age at testing (p = 0.001), and lower plasma uric acid (p = 0.01). Multivariate analysis showed increased uric acid (β = 0.214, p = 0.01), lower age at testing (β = −0.378, p < 0.001), and higher education level (β = 0.424, p < 0.001) could predict higher ECAS score (F = 19.104, R2 = 0.381, p < 0.0001). Validation analysis showed that predicted ECAS score was significantly correlated with raw ECAS score in both the training set (rs = 0.621, p < 0.001) and the testing set (rs = 0.666, p < 0.001).Conclusions: Cognitive impairment was a common feature in our Chinese ALS patients. Plasma uric acid might help evaluate the risk of cognitive impairment in ALS patients when combined with education level and age at testing.

scholarly journals An Evaluation of Empirical Approaches for Defining Cognitive Impairment in Amyotrophic Lateral Sclerosis

2020 ◽  
Author(s):  
Corey T. McMillan ◽  
Joanne Wuu ◽  
Katya Rascovsky ◽  
Stephanie Cosentino ◽  
Murray Grossman ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Quantile Regression ◽  
Statistical Method ◽  
North American ◽  
Healthy Adults ◽  
Substantial Impact ◽  
The University ◽  
Als Patients ◽  
Lateral Sclerosis

AbstractImportanceAmyotrophic lateral sclerosis (ALS) is a multi-system disorder characterized primarily by motor neuron degeneration, but may be accompanied by cognitive dysfunction. Statistically appropriate criteria for establishing cognitive impairment (CI) in ALS are lacking.ObjectiveDefine thresholds for CI in ALS using quantile regression (QR) that accounts for age and education in a North American (NAmer) cohort.DesignQR of cross-sectional data from a multi-center NAmer cohort of healthy adults was used to model the 5th percentile of cognitive scores on the Edinburgh Cognitive and Behavioral ALS Screen (ECAS). The QR approach was compared to a traditional 2 standard deviation (SD) cut-off approach using the same NAmer cohort (2SD-NAmer) and to existing UK-based normative data derived using the 2SD approach (2SD-UK) to assess the impact of cohort selection and statistical model in identifying CI ALS patients.Participants269 healthy adults from NAmer, recruited by the University of Pennsylvania (PENN; N=82), the University of Miami through the CRiALS study (CRiALS; N=40), and the Canadian ALS Neuroimaging Consortium (CALSNIC; N=147) were included to establish ECAS thresholds for defining CI. We then evaluated the frequency of CI in 182 ALS patients from PENN.Main OutcomesWe defined two new sets of normative thresholds, based on NAmer heathy adult performance, for each ECAS domain score and the composite scores using QR and 2SD statistical approaches. We then applied the 2SD-NAmer and QR-NAmer, as well as the previously established and widely-used 2SD-UK, thresholds to evaluate the frequency of CI in ALS patients.ResultsQR-NAmer models revealed that increased age and reduced educational attainment negatively impact cognitive performance on the ECAS. Based on the QR-NAmer normative cutoffs, the prevalence of CI in the 182 PENN ALS patients was 15.9% for ECAS ALS-Specific and 15.4% for ECAS Total. These estimates are more conservative than estimates ranging from 15.4%-34.6% impaired based on 2SD approaches.Conclusions and RelevanceThis report establishes normative thresholds for using ECAS to identify whether ALS patients in the NAmer population have CI. The choice of statistical method and normative cohort has a substantial impact on defining CI in ALS.Key PointsQuestionHow to define cognitive impairment (CI) in amyotrophic lateral sclerosis (ALS) using the Edinburgh Cognitive and Behavioral ALS Screen (ECAS)?FindingsAge- and education-adjusted quantile regression (QR) yields thresholds for defining CI that differ meaningfully from those derived from parametric methods without age- and education-adjustment. Thresholds also differ between UK and North American cohorts. Applying our North American-based QR norms to an American ALS cohort at a single center identified CI based on ECAS performance in ∼16% patients, compared to 15.4%-34.6% patients using other approaches.MeaningThe choice of statistical method and normative cohort has a substantial impact on defining CI in ALS.

scholarly journals Selective and Inverse U-Shaped Curve Alteration of the Retinal Nerve in Amyotrophic Lateral Sclerosis: A Potential Mirror of the Disease

2022 ◽  
Vol 13 ◽  
Author(s):  
Yixuan Zhang ◽  
Xiangyi Liu ◽  
Jiayu Fu ◽  
Yuanjin Zhang ◽  
Xue Yang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Early Stage ◽  
Compound Muscle Action Potential ◽  
Progression Rate ◽  
Fiber Layer ◽  
Abductor Pollicis Brevis ◽  
Thickness Change ◽  
Als Patients ◽  
Lateral Sclerosis

Introduction: Alterations in the visual pathway involving the retina have been reported in amyotrophic lateral sclerosis (ALS) but they lack consistency and subgroup analysis. We aimed to assess the retinal nerve fiber layer (RNFL) and retinal ganglion cells (RGCs) alterations in different stages of ALS patients and their association with ALS progression parameters.Methods: The study population consisted of 70 clinically diagnosed ALS patients and 55 age, sex matched controls. All of them underwent ophthalmic assessments and optical coherence tomography imaging. Four quadrants of the peripapillary RNFL and ganglion cell/inner plexiform complex (GCIP) were observed and automatically measured. Early-stage distal motor neuron axon dysfunction in ALS was detected by compound muscle action potential (CMAP) of the distal limbs within 12 months. The ALS disease parameters included the ALSFRS-R score and the disease progression rate (ΔFS).Results: Generally compared with controls, the nasal (p = 0.016) quadrant of the RNFL was thicker in ALS patients. When controlling for age and ΔFS, the RNFL(r = 0.37, p = 0.034) and GCIP(r = 0.40, p = 0.021) were significantly thickened as disease progressed within 12 months, while the RNFL declined with time after one year (r = −0.41, p = 0.037). ALS patients was subclassified into thickened RNFL (T-RNFL, >95th percentile of normal), impaired RNFL (I-RNFL, <5th percentile of normal) and normal RNFL. There were significant differences in the GCIP among the three groups (p < 0.001). In the T-RNFL group (n = 18), the RNFL was negatively correlated with the abductor pollicis brevis-CMAP amplitude within 12 months (r = −0.56, p = 0.01). Patients within 12 months in this group progressed faster than others (p = 0.039). In the normal RNFL group (n = 22), 13 patients were diagnosed beyond 12 months, whose ΔFS was remarkably lower (p = 0.007). In I-RNFL group (n = 30), the early stage patients (<12 months) had significant higher ΔFS (p = 0.006). One patient was with SOD1 pathogenic variant (p.A5V).Conclusion: Alterations of retinal nerve were not consistent in ALS patients with diverse phenotypes and progression rates. Generally speaking, the RNFL thickened during the first year and then gradually declined, which is related to but preceding the thickness change of the RGCs. Patients with a significant RNFL thinning in the early stage may have a faster progression rate. The inverse U-shaped curve transformation might be in accordance with early-stage motor neuron axonopathy.

Riluzole Exhibits No Therapeutic Efficacy on a Transgenic Rat model of Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 17 (3) ◽  
pp. 275-285 ◽  
Author(s):  
Si Chen ◽  
Qiao Liao ◽  
Ke Lu ◽  
Jinxia Zhou ◽  
Cao Huang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Rat Model ◽  
Microglial Activation ◽  
Transgenic Rat ◽  
Intragastric Administration ◽  
Behavioral Deficits ◽  
Als Patients ◽  
Lateral Sclerosis ◽  
Transgenic Rat Model

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.

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