Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Papers
(The median citation count of Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration is 1. The table below lists those papers that are above that threshold based on CrossRef citation counts [max. 250 papers]. The publications cover those that have been published in the past four years, i.e., from 2021-08-01 to 2025-08-01.)
ArticleCitations
Rapid classification of a novel ALS-causing I149S variant in superoxide dismutase-145
Low plasma hyaluronan is associated with faster functional decline in patients with amyotrophic lateral sclerosis35
Clinical and demographical characteristics in a cohort of MND patients treated with riluzole. Differences between tablets and oral suspension32
Correction31
Validity and reliability measures of the Swedish Karolinska version of the Edinburgh Cognitive and Behavioral ALS Screen (SK-ECAS)25
The correlation between social support, coping style, advance care planning readiness, and quality of life in patients with amyotrophic lateral sclerosis: a cross-sectional study25
Incorporating patient preferences and burden-of-disease in evaluating ALS drug candidate AMX0035: a Bayesian decision analysis perspective24
The amyotrophic lateral sclerosis-health index (ALS-HI): development and evaluation of a novel outcome measure20
Clinical epidemiology of amyotrophic lateral sclerosis in Liguria, Italy: a ten year follow up study19
Peripheral immunity relate to disease progression and prognosis in amyotrophic lateral sclerosis18
Single task-level, 2SD-based cutoffs for the Italian version of the Edinburgh Cognitive and Behavioral ALS screen (ECAS)18
Genetic ancestry and risk allele C9orf72 rs3849942 T for amyotrophic lateral sclerosis in Latin American populations18
Development, reliability, validity, and acceptability of the remote administration of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS)18
Residential exposure associations with ALS risk, survival, and phenotype: a Michigan-based case-control study15
Theme 5 Human Cell Biology and Pathology15
Amyotrophic lateral sclerosis mortality rates in Latin America and the Caribbean: a meta-analysis15
Patient engagement in research: lessons learned from CAPTURE ALS, a longitudinal observational ALS study15
Geographical clusters of amyotrophic lateral sclerosis and the Bradford Hill criteria15
Can resting lung function predict the response of a person living with motor neuron disease to a hypoxic challenge test?14
Impact of occupational categories on the incidence of amyotrophic lateral sclerosis in Sardinia Island, Italy14
Portable fixed dynamometry enables home-based, reliable assessment of muscle strength in patients with amyotrophic lateral sclerosis: a pilot study14
Theme 11 - Cognitive and Psychological Assessment and Support14
Assessing pulmonary function in ALS using electrical impedance tomography14
Incidence of amyotrophic lateral sclerosis before and during the COVID-19 pandemic: evidence from an 8-year population-based study in Central Italy based on healthcare utilization databases14
Theme 10 - Disease Stratification and Phenotyping of Patients13
Time from amyotrophic lateral sclerosis symptom onset to key disease milestones: analysis of data from a multinational cross-sectional survey13
Genetic variation in targets of lipid-lowering drugs and amyotrophic lateral sclerosis risk: a Mendelian randomization study13
Care burden and related factors among informal caregivers of patients with amyotrophic lateral sclerosis13
Amyotrophic lateral sclerosis: exploring pathophysiology in the context of treatment11
Automated speech analytics in ALS: higher sensitivity of digital articulatory precision over the ALSFRS-R11
Yield of EEG features as markers of disease severity in amyotrophic lateral sclerosis: a pilot study11
Theme 03 - IN VITRO EXPERIMENTAL MODELS11
Self-assessment of amyotrophic lateral sclerosis functional rating scale on the patient’s smartphone proves to be non-inferior to clinic data capture11
Correction11
The contralateral co-movement test in a Chinese population with amyotrophic lateral sclerosis10
Feature selection using metaheuristics to predict annual amyotrophic lateral sclerosis progression10
Drug treatments and interactions, disease progression and quality of life in ALS patients10
Time-to-event prediction in ALS using a landmark modeling approach, using the ALS Natural History Consortium dataset10
The involvement of palliative care with neurology – a comparison of UK, Switzerland and Italy10
Supporting people with Motor Neuron Disease (MND) to make decisions about gastrostomy feeding tube placement: a survey of UK healthcare professionals’ practice and beliefs10
Slowly progressive late-onset spinal muscular atrophy Finkel-type related to p.Pro56Ser VABP mutation in Colombia10
Platform Communications: Abstract Book – 33rd International Symposium on ALS/MND (Complete printable file)9
Response letter to: a homozygous p.Val120Leu (c.358G > C) SOD1 mutation led to slowly progressive amyotrophic lateral sclerosis in a Brazilian family9
Toward genetic counseling practice standards for diagnostic testing in amyotrophic lateral sclerosis and frontotemporal dementia9
A revision to the United States national ALS registry’s algorithm to improve Case-Ascertainment9
Exposure to electromagnetic fields does not modify neither the age of onset nor the disease progression in ALS patients9
The natural history of ALS: Baseline characteristics from a multicenter clinical cohort9
Exploring the needs and preferences of people with amyotrophic lateral sclerosis (ALS) when making genomic testing decisions: an interview study9
Improving the measurement properties of the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R): deriving a valid measurement total for the calculation of change9
A novel TBK1 loss-of-function variant associated with ALS and parkinsonism phenotypes9
IMPACT-ALS: summary of results from a European survey of people living with ALS9
Monocyte CD14 and HLA-DR expression increases with disease duration and severity in amyotrophic lateral sclerosis9
PRECISION ALS—an integrated pan European patient data platform for ALS9
Body mass index is lower in asymptomatic C9orf72 expansion carriers but not in SOD1 pathogenic variant carriers compared to gene negatives8
Amyotrophic lateral sclerosis estimated prevalence cases from 2022 to 2030, data from the national ALS Registry8
The association of theory of mind with language and visuospatial abilities in amyotrophic lateral sclerosis: a pilot study8
Comparison Of King’s Clinical Staging In Multinational Amyotrophic Lateral Sclerosis Cohorts8
Changes in saccadic intrusions over time as an objective biomarker to follow ALS disease progression8
International network for ALS research and care (INARC)8
Neuropsychological assessment practices in PRECISION-ALS: challenges and opportunities for harmonization8
Epidemiology and economic burden of amyotrophic lateral sclerosis in the United States: a literature review8
Expanding the phenotype of TARDBP mutation in a Tunisian family with clinical phenotype heterogeneity8
Cognition in the course of ALS—a meta-analysis7
Neurofilament light chain as a biological marker for amyotrophic lateral sclerosis: a meta-analysis study7
Respiratory measurements, respiratory symptoms, and quality of life in ALS: results from the REVEALS study7
Theme 03 - In Vitro Experimental Models7
Increased total daily energy expenditure in mild to moderate ALS: greater contribution from physical activity energy expenditure than hyper-metabolism7
Why is nutrition vital for advancing ALS care and clinical research?7
Impact of mode of training and recertification on ALSFRS-R rater performance7
ALSUntangled # 69: astaxanthin6
Demographic changes in a large motor neuron disease cohort in Portugal: a 27 year experience6
Urine specific gravity to identify and predict hydration need in ALS6
A speech-based prognostic model for dysarthria progression in ALS6
MiNDAUS partnership: a roadmap for the cure and management of motor Neurone disease6
Serum heat shock protein concentrations are not associated with amyotrophic lateral sclerosis risk or survival in three European populations6
Quantitative susceptibility mapping for investigating brain iron deposits in amyotrophic lateral sclerosis: correlations with clinical phenotype and disease progression6
How the prospect of a clinical trial impacts decision-making for predictive genetic testing in ALS6
The diagnostic value of the Italian version of the Edinburgh Cognitive and Behavioral ALS Screen (ECAS)6
Theme 05 - HUMAN CELL BIOLOGY AND PATHOLOGY (including iPSC studies)6
Letter to Editor6
Apathy in amyotrophic lateral sclerosis: systematic review and meta-analysis of frequency, correlates, and outcomes6
The cholesterol depleting agent, (2-Hydroxypropyl)-ß-cyclodextrin, does not affect disease progression in SOD1 G93A mice6
ALSUntangled #79: alpha-lipoic acid6
Primary lateral sclerosis natural history study – planning, designing, and early enrollment6
ALSUntangled #65: glucocorticoid corticosteroids6
Theme 12 - Clinical Management and Support6
Longitudinal comparison of the self-administered ALSFRS-RSE and ALSFRS-R as functional outcome measures in ALS6
A de novo c.113 T > C: p.L38R mutation of SPTLC1: case report of a girl with sporadic juvenile amyotrophic lateral sclerosis5
The Beaumont behavioral intervention in a Chinese amyotrophic lateral sclerosis cohort5
Frequency of C9orf72 and SOD1 mutations in 302 sporadic ALS patients from three German ALS centers5
HLA and amyotrophic lateral sclerosis: a systematic review and meta-analysis5
Theme 10 - DISEASE STRATIFICATION AND PHENOTYPING OF PATIENTS5
Relationship between quantitative strength and functional outcomes in the phase 2 FORTITUDE-ALS trial5
Theme 08 - Clinical Imaging and Electrophysiology5
Verbal expressive language minimally affected in non-demented people living with amyotrophic lateral sclerosis5
Acceptability and potential benefit of a self-compassion intervention for people living with amyotrophic lateral sclerosis: a mixed methods pilot study5
Association of UNC13A with increased amyotrophic lateral sclerosis risk, bulbar onset, and lower motor neuron involvement in a Norwegian ALS cohort5
Correction5
Study of “ALS reversals”: LifeTime environmental exposures (StARLiTE)5
A verb-naming test accurately detects cognitive changes in ALS5
Italian reference values and brain correlates of verbal fluency index – vs standard verbal fluency test – to assess executive dysfunction in ALS4
ALSUntangled #71: Nuedexta4
Characterization, epidemiology, and factors associated with evolution and survival in patients with amyotrophic lateral sclerosis in southeastern Spain, 2008–2021: a population-based study4
The expanding clinical and genetic spectrum of alsin-related disorders: the first cohort of Brazilian patients4
Current practices in the nutrition management of people with amyotrophic lateral sclerosis (ALS): a survey of U.S. ALS care teams4
Clinical trajectories of genetic variants in ALS: a European observational study within PRECISION-ALS4
Effects of COVID-19 on motor neuron disease mortality in the United States: a population-based cross-sectional study4
Neutrophil-to-lymphocyte ratio at diagnosis as a biomarker for survival of amyotrophic lateral sclerosis4
Medical therapies for amyotrophic lateral sclerosis-related respiratory decline: an appraisal of needs, opportunities and obstacles4
Theme 05 - Human Cell Biology and Pathology (including iPSC studies)4
Incidence of amyotrophic lateral sclerosis in the United States, 2014–20164
Theme 02 - Genetics and Genomics4
Clinically meaningful change: evaluation of the Rasch-built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS) and the ALSFRS-R4
Theme 06 - TISSUE BIOMARKERS4
In memoriam Ettore Beghi, 1947–20224
Occupational lead exposure and amyotrophic lateral sclerosis survival in the Danish National Patient Registry4
Prevalence of depression in amyotrophic lateral sclerosis/motor neuron disease: multi-attribute ascertainment and trajectories over 30 months4
Theme 01 - Epidemiology and Informatics4
Patients’ choices regarding ventilatory support affect opioid use in amyotrophic lateral sclerosis4
Mapping the natural history of amyotrophic lateral sclerosis: time-to-event analysis of clinical milestones in the pan-European, population-based PRECISION-ALS cohort4
Harmonized standard operating procedures for administering the ALS functional rating scale-revised4
Thoracic electrical impedance tomography for assessing progression of pulmonary dysfunction in ALS4
Comparing Amyotrophic lateral sclerosis (ALS) patient characteristics from the National ALS Registry and the Massachusetts ALS Registry, data through 20153
REVEALS—a longitudinal cohort study of multifaceted respiratory assessment in ALS3
Previous psychiatric disorders in the multistep hypothesis of amyotrophic lateral sclerosis: a South Korean population study3
A nurse coaching intervention to improve support to individuals living with ALS3
Prevalence of cognitive impairment in motor neuron diseases: alternative norming methods lead to considerably diverse estimates3
Theme 08 - CLINICAL IMAGING AND ELECTROPHYSIOLOGY3
Physical activity in amyotrophic lateral sclerosis: a systematic review of the methodologies used to assess a possible association3
The quality of life and depression in primary caregivers of patients with amyotrophic lateral sclerosis is affected by patient-related and culture-specific conditions3
A geographical study on amyotrophic lateral sclerosis in Rio Grande Do Norte, Brazil, from 2005 to 20183
Genetic and epigenetic disease modifiers in an Italian C9orf72 family expressing ALS, FTD or PD clinical phenotypes3
ALS incidence and population aging in Northern Italy3
Prevalence of amyotrophic lateral sclerosis in the United States, 20183
Platform Communications: Abstract Book – 34th International Symposium on ALS/MND (Complete printable file)3
Specialized multidisciplinary care improves ALS survival in Belgium: a population-based retrospective study3
ECAS correlation with metabolic alterations on FDG-PET imaging in ALS3
Neurologists’ understanding of reproductive medicine options for genetic forms of motor neuron disease3
Research access barriers in amyotrophic lateral sclerosis3
Challenges and solutions to complex data governance issues in cross-national, cross-sectoral, multidisciplinary real world health research: a descriptive overview3
Multistep modeling applied to a Malaysian ALS registry3
Genetically predicted coffee consumption and amyotrophic lateral sclerosis3
Correction3
Theme 04 - IN VIVO EXPERIMETAL MODELS3
Stigma experienced by ALS/PMA patients and their caregivers: a mixed-methods study3
Percutaneous gastrostomy, mechanical ventilation and survival in amyotrophic lateral sclerosis: an observational study in an incident cohort3
PROSA—a multicenter prospective observational study to develop low-burden digital speech biomarkers in ALS and FTD3
Perils of predictive testing for unaffected people from motor neuron disease families with no identified causal gene3
Self-care mobile application for people with Amyotrophic Lateral Sclerosis: a development and usability test3
Validation of the Italian version of the Rasch-Built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS) administered to patients and their caregivers3
Psychometric properties of the Russian version of the Edinburgh cognitive and behavioral amyotrophic lateral sclerosis screen3
Platform Communications: Abstract Book - 32nd International Symposium on ALS/MND (Complete printable file)3
A novel homozygous loss-of-function variant in SOD1 causing progressive spastic tetraplegia and axial hypotonia3
Theme 11 - Cognitive and Psychological Assessment and Support3
Focal epilepsy followed by rapidly progressive frontotemporal dementia: a rare manifestation of VCP mutation2
Theme 01 - EPIDEMIOLOGY AND INFORMATICS2
Platform Communications: Abstract Book 35th International Symposium on ALS/MND (Complete printable file)2
Occupational lead exposure and survival with amyotrophic lateral sclerosis2
SOD1 gene screening in ALS – frequency of mutations, patients’ attitudes to genetic information and transition to tofersen treatment in a multi-center program2
Living with a parent with ALS - adolescents’ need for professional support from the adolescents’ and the parents’ perspectives2
Unraveling the contribution of executive functions to language impairment in ALS2
Remote digital assessment of amyotrophic lateral sclerosis functional rating scale – a multicenter observational study2
Rapid progression of amyotrophic lateral sclerosis after initiation of GLP-1 agonist: a case report2
Incidence of amyotrophic lateral sclerosis in Chile2
A novel mutation in the ALS2 gene in an iranian kurdish family with juvenile amyotrophic lateral sclerosis2
CAPTURE ALS: the comprehensive analysis platform to understand, remedy and eliminate ALS2
‘Outcomes of genetic testing in the London MND Center: the importance of achieving timely results and correlations to family history’2
The neuroradiology of upper motor neuron degeneration: PLS, HSP, ALS2
ALSUntangled #78: Zinc2
ALSUntangled #75: Portable neuromodulation stimulator therapy2
Natural history of the revised ALS functional rating scale and its association with survival: the PRECISION-ALS Extant Study2
MRI biomarkers for memory-related impairment in amyotrophic lateral sclerosis: a systematic review2
What is the extent of reliability and validity evidence for screening tools for cognitive and behavioral change in people with ALS? A systematic review2
Cerebrospinal fluid levels of NfM in relation to NfL and pNfH as prognostic markers in amyotrophic lateral sclerosis2
Can amyotrophic lateral sclerosis progression really pause? A cohort study using the medical research council scale2
A phase 2, proof-of-concept, placebo-controlled, randomized, multicenter, double-blind study to evaluate the efficacy, safety, tolerability, pharmacokinetics, and pharmacodynamics of Usnoflast (ZYIL1)2
Profiles of disease progression and predictors of mortality in Colombian patients with amyotrophic lateral sclerosis: a comprehensive longitudinal study2
Theme 06 - Tissue Biomarkers2
An electronic health record cohort of Veterans with amyotrophic lateral sclerosis2
Environmental risk factors for amyotrophic lateral sclerosis: a case–control study in Canada and France2
SOD1-ALS mimicking an inflammatory neuropathy: a case report2
ALSUntangled #76: Wahls protocol2
Psychological resilience is protective against cognitive deterioration in motor neuron diseases2
Combination of ciprofloxacin/celecoxib as a novel therapeutic strategy for ALS2
Cognitive reserve in ALS: the role of occupational skills and requirements2
Refining items for a preference-based, amyotrophic lateral sclerosis specific, health-related quality of life scale2
Trends in survival of ALS from a population-based registry2
Development of ‘gastrostomy tube – is it for me?’, a web-based patient decision aid for people living with motor neurone disease considering having a gastrostomy tube placed2
Analysis of incidence of motor neuron disease in England 1998–2019: use of three linked datasets2
Table of Contents - Poster Communications2
Determinants and progression of stigma in amyotrophic lateral sclerosis/motor neuron disease2
Racial disparities in ALS diagnostic delay: a single center’s experience and review of potential contributing factors2
Describing and characterising variability in ALS disease progression2
Short tandem repeat expansions in LRP12 are absent in cohorts of familial and sporadic amyotrophic lateral sclerosis patients of European ancestry2
ALSUntangled #63: ketogenic diets2
Persistent high levels of perceived fatigue are not associated with hypermetabolism in patients with amyotrophic lateral sclerosis2
Lack of habituation in somatosensory cortex but not in visual cortex of ALS patients2
Assessment of risk of ALS conferred by the GGGGCC hexanucleotide repeat expansion in C9orf72 among first-degree relatives of patients with ALS carrying the repeat expans2
Amyotrophic lateral sclerosis caused by the C9orf72 expansion in Norway – prevalence, ancestry, clinical characteristics and sociodemographic status2
Incidence of amyotrophic lateral sclerosis in Sardinia, Italy: age–sex interaction and spatial–temporal variability1
Treatment continuity of amyotrophic lateral sclerosis with available riluzole formulations: state of the art and current challenges in a ‘real-world’ setting1
Theme 10 Disease Stratification and Phenotyping of Patients1
Blood glycated hemoglobin level is not associated with disease progression in amyotrophic lateral sclerosis1
How to break the news in amyotrophic lateral sclerosis/motor neuron disease: practical guidelines from experts1
Subcortical grey matter involvement in ALS and PLS – vulnerable hubs of cortico-cortical and cortico-basal circuits: extrapyramidal, cognitive, bulbar and respiratory correlates1
Rurality modifies the association between symptoms and the diagnosis of amyotrophic lateral sclerosis1
Transcranial magnetic stimulation to monitor disease progression in ALS: a review1
Analysis of STMN2 CA repeats in italian ALS patients shows no association1
Measuring disability in amyotrophic lateral sclerosis/motor neuron disease: the WHODAS 2.0-36, WHODAS 2.0-32, and WHODAS 2.0-121
Genetic overlap between ALS and other neurodegenerative or neuromuscular disorders1
Autophagy dysregulation plays a crucial role in regulatory T-cell loss and neuroinflammation in amyotrophic lateral sclerosis (ALS)1
Examining cognitive decline over time in Iranian ALS patients: Adapting successive versions B and C of the Edinburgh cognitive and behavioral screen to Persian1
Expanding the spectrum of KIF5A mutations—case report of a large kindred with familial ALS and overlapping syndrome1
Geographical distribution of clinical trials in amyotrophic lateral sclerosis: a scoping review1
Neurodegenerative biomarkers outperform neuroinflammatory biomarkers in amyotrophic lateral sclerosis1
Association of the practice of contact sports with the development of amyotrophic lateral sclerosis1
Predicting amyotrophic lateral sclerosis (ALS) progression with machine learning1
Premorbid lipid levels and long-term risk of ALS—a population-based cohort study1
Theme 01 - Epidemiology and Informatics1
Clinical factors and vascular endothelial growth factor as determinants of disease progression in Indian patients with amyotrophic lateral sclerosis1
Plasma neurofilament light levels show elevation two years prior to diagnosis of amyotrophic lateral sclerosis in the UK Biobank1
ALSUntangled #74: Withania Somnifera (Ashwagandha)1
A novel DHTKD1 gene mutation with ALS like presentation: a case report1
Urinary p75ECD levels in patients with amyotrophic lateral sclerosis: a meta-analysis1
Analysis of non-motor symptoms in amyotrophic lateral sclerosis1
Theme 2 Genetics and Genomics1
How the coronavirus pandemic affected the lives of people with ALS and their spouses in the UK from spouses’ perspectives: a qualitative study1
Cross-cultural adaptation and psychometric evaluation of a Turkish version of the Amyotrophic Lateral Sclerosis-Specific Quality of Life-Short Form1
Theme 08 - Clinical Imaging and Electrophysiology1
Prognosis of amyotrophic lateral sclerosis patients after tracheostomy invasive ventilation in Korea1
FUS mutations in Asian amyotrophic lateral sclerosis patients: a case report and literature review of genotype-phenotype correlations1
A survey of current practice in genetic testing in amyotrophic lateral sclerosis in the UK and Republic of Ireland: implications for future planning1
Integrated analysis of transcriptomic and proteomic alterations in mouse models of ALS/FTD identify early metabolic adaptions with similarities to mitochondrial dysfunction disorders1
ALSUntangled #66: antimycobacterial antibiotics.1
Prevalence and predictors of prolonged grief disorder, anxiety and depression in bereaved ALS family caregivers: a national survey of distress and support needs after bereavement1
SOD1-ALS-Browser: a web-utility for investigating the clinical phenotype in SOD1 amyotrophic lateral sclerosis1
ALS due to c.1189 + 1G > T (splice donor) TBK1 mutation1
Theme 07 - PRE-CLINICAL THERAPEUTIC STRATEGIES1
LAENALS: epidemiological and clinical features of amyotrophic lateral sclerosis in Latin America1
ALS due to a novel TBK1 mutation in Brazil1
Participant, site personnel and sponsor perspectives on decentralized trial features in COURAGE-ALS: a randomized clinical trial1
The incidence and prevalence of motor neurone disease in South Australia1
0.04552698135376