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 2022-06-01 to 2026-06-01.)
ArticleCitations
Incorporating patient preferences and burden-of-disease in evaluating ALS drug candidate AMX0035: a Bayesian decision analysis perspective34
The correlation between social support, coping style, advance care planning readiness, and quality of life in patients with amyotrophic lateral sclerosis: a cross-sectional study32
Correction32
The amyotrophic lateral sclerosis-health index (ALS-HI): development and evaluation of a novel outcome measure30
Clinical and demographical characteristics in a cohort of MND patients treated with riluzole. Differences between tablets and oral suspension26
Clinical epidemiology of amyotrophic lateral sclerosis in Liguria, Italy: a ten year follow up study25
Validity and reliability measures of the Swedish Karolinska version of the Edinburgh Cognitive and Behavioral ALS Screen (SK-ECAS)25
Rapid classification of a novel ALS-causing I149S variant in superoxide dismutase-125
Single task-level, 2SD-based cutoffs for the Italian version of the Edinburgh Cognitive and Behavioral ALS screen (ECAS)23
Development, reliability, validity, and acceptability of the remote administration of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS)23
A novel heterozygous GRN deletion (c.302_315del) associated with behavioral variant frontotemporal dementia21
Theme 5 Human Cell Biology and Pathology20
Impact of occupational categories on the incidence of amyotrophic lateral sclerosis in Sardinia Island, Italy20
Challenges in medical care for amyotrophic lateral sclerosis: a survey of physicians from Republic of Bashkortostan (Russia), Belarus, and Kazakhstan19
Emerging strategies for interpreting variants of uncertain significance (VUS) in amyotrophic lateral sclerosis18
Residential exposure associations with ALS risk, survival, and phenotype: a Michigan-based case-control study17
Theme 7 Pre-Clinical Therapeutic Strategies17
Peripheral immunity relate to disease progression and prognosis in amyotrophic lateral sclerosis16
Patient engagement in research: lessons learned from CAPTURE ALS, a longitudinal observational ALS study16
Genetic ancestry and risk allele C9orf72 rs3849942 T for amyotrophic lateral sclerosis in Latin American populations16
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 databases15
Can resting lung function predict the response of a person living with motor neuron disease to a hypoxic challenge test?14
Theme 11 - Cognitive and Psychological Assessment and Support14
Correction14
Portable fixed dynamometry enables home-based, reliable assessment of muscle strength in patients with amyotrophic lateral sclerosis: a pilot study14
ALS motor phenotypes: a revised ‘OPM’ classification14
Assessing pulmonary function in ALS using electrical impedance tomography14
Epidemiology, clinical features, and management of amyotrophic lateral sclerosis in the neurology department of the Bogodogo University Hospital in Ouagadougou, Burkina Faso14
Self-assessment of amyotrophic lateral sclerosis functional rating scale on the patient’s smartphone proves to be non-inferior to clinic data capture13
Genetic variation in targets of lipid-lowering drugs and amyotrophic lateral sclerosis risk: a Mendelian randomization study13
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
Yield of EEG features as markers of disease severity in amyotrophic lateral sclerosis: a pilot study12
Amyotrophic lateral sclerosis: exploring pathophysiology in the context of treatment12
Care burden and related factors among informal caregivers of patients with amyotrophic lateral sclerosis12
The contralateral co-movement test in a Chinese population with amyotrophic lateral sclerosis11
Automated speech analytics in ALS: higher sensitivity of digital articulatory precision over the ALSFRS-R10
Feature selection using metaheuristics to predict annual amyotrophic lateral sclerosis progression10
The involvement of palliative care with neurology – a comparison of UK, Switzerland and Italy10
Platform Communications: Abstract Book – 33rd International Symposium on ALS/MND (Complete printable file)10
Slowly progressive late-onset spinal muscular atrophy Finkel-type related to p.Pro56Ser VABP mutation in Colombia10
Supporting people with Motor Neuron Disease (MND) to make decisions about gastrostomy feeding tube placement: a survey of UK healthcare professionals’ practice and beliefs10
Exploring the needs and preferences of people with amyotrophic lateral sclerosis (ALS) when making genomic testing decisions: an interview study10
Pridopidine treatment in ALS: subgroup analyses from the HEALEY ALS Platform trial10
Limited data capture on reproductive medicine use in amyotrophic lateral sclerosis: implications for monitoring access10
Time-to-event prediction in ALS using a landmark modeling approach, using the ALS Natural History Consortium dataset10
Neuropsychological assessment practices in PRECISION-ALS: challenges and opportunities for harmonization9
Exposure to electromagnetic fields does not modify neither the age of onset nor the disease progression in ALS patients9
Expanding the phenotype of TARDBP mutation in a Tunisian family with clinical phenotype heterogeneity9
Amyotrophic lateral sclerosis estimated prevalence cases from 2022 to 2030, data from the national ALS Registry9
PRECISION ALS—an integrated pan European patient data platform for ALS9
A novel TBK1 loss-of-function variant associated with ALS and parkinsonism phenotypes9
Comparison Of King’s Clinical Staging In Multinational Amyotrophic Lateral Sclerosis Cohorts9
Self-reported initiation apathy is related to worse quality of life in amyotrophic lateral sclerosis9
IMPACT-ALS: summary of results from a European survey of people living with ALS9
A revision to the United States national ALS registry’s algorithm to improve Case-Ascertainment8
Quality of life and care burden of people living with amyotrophic lateral sclerosis who need home-based medical care in Korea and their family caregivers8
International network for ALS research and care (INARC)8
Respiratory measurements, respiratory symptoms, and quality of life in ALS: results from the REVEALS study8
Changes in saccadic intrusions over time as an objective biomarker to follow ALS disease progression8
Improving the measurement properties of the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R): deriving a valid measurement total for the calculation of change8
Incidence of MND in Canterbury, New Zealand and impact of a key worker role on hospital admissions8
The natural history of ALS: Baseline characteristics from a multicenter clinical cohort8
Body mass index is lower in asymptomatic C9orf72 expansion carriers but not in SOD1 pathogenic variant car8
Response letter to: a homozygous p.Val120Leu (c.358G > C) SOD1 mutation led to slowly progressive amyotrophic lateral sclerosis in a Brazilian family8
Understanding psychological adjustment in patients with amyotrophic lateral sclerosis and their caregivers: a scoping review8
Impact of mode of training and recertification on ALSFRS-R rater performance8
Increased total daily energy expenditure in mild to moderate ALS: greater contribution from physical activity energy expenditure than hyper-metabolism8
ALSUntangled #80: ISRIB (Integrated stress response InhiBitor)7
Dextromethorphan/quinidine (DMQ) for reducing bulbar symptoms in amyotrophic lateral sclerosis – assessment of treatment experience in a multicenter study7
Cognition in the course of ALS—a meta-analysis7
Why is nutrition vital for advancing ALS care and clinical research?7
The genetics of autosomal recessive ALS: a review of the common forms and their phenotypes7
Epidemiology and economic burden of amyotrophic lateral sclerosis in the United States: a literature review6
Theme 03 - In Vitro Experimental Models6
ALSUntangled # 69: astaxanthin6
Evolving 10-year epidemiological landscape of amyotrophic lateral sclerosis (ALS) in Ceará, Brazil6
Theme 12 - Clinical Management and Support6
Letter to Editor6
Demographic changes in a large motor neuron disease cohort in Portugal: a 27 year experience6
Theme 10 Disease Stratification and Phenotyping of Patients6
ALSUntangled #79: alpha-lipoic acid6
Theme 9 Clinical Trials and Trial Design6
Association of UNC13A with increased amyotrophic lateral sclerosis risk, bulbar onset, and lower motor neuron involvement in a Norwegian ALS cohort6
A speech-based prognostic model for dysarthria progression in ALS6
How the prospect of a clinical trial impacts decision-making for predictive genetic testing in ALS6
The cholesterol depleting agent, (2-Hydroxypropyl)-ß-cyclodextrin, does not affect disease progression in SOD1 G93A mice6
Primary lateral sclerosis natural history study – planning, designing, and early enrollment6
Translation and validation of the Arabic version of the amyotrophic lateral sclerosis assessment questionnaire (ALSAQ40-AR)6
Quantitative susceptibility mapping for investigating brain iron deposits in amyotrophic lateral sclerosis: correlations with clinical phenotype and disease progression6
Strengthening care and research in ALS in Southeast Asia: a call for action5
Theme 01 - Epidemiology and Informatics5
Verbal expressive language minimally affected in non-demented people living with amyotrophic lateral sclerosis5
A de novo c.113 T > C: p.L38R mutation of SPTLC1: case report of a girl with sporadic juvenile amyotrophic lateral sclerosis5
Serum NfL, but not GFAP, differentiates primary lateral sclerosis from adrenomyeloneuropathy and hereditary spastic paraplegia type 45
Study of “ALS reversals”: LifeTime environmental exposures (StARLiTE)5
Amyotrophic lateral sclerosis in Saudi Arabia: a multicenter descriptive study5
Theme 05 - Human Cell Biology and Pathology (including iPSC studies)5
Relationship between quantitative strength and functional outcomes in the phase 2 FORTITUDE-ALS trial5
Correction5
Acceptability and potential benefit of a self-compassion intervention for people living with amyotrophic lateral sclerosis: a mixed methods pilot study5
The Beaumont behavioral intervention in a Chinese amyotrophic lateral sclerosis cohort5
Beyond outcomes: patients’ lived experience of exoskeleton-assisted gait training in amyotrophic lateral sclerosis5
ALSUntangled #65: glucocorticoid corticosteroids5
Amyotrophic lateral sclerosis in Colombia: a population-based study of incidence and socioeconomic determinants5
Four decades of ALS care: a retrospective study of epidemiology, clinical course and changes in management5
How effective does a new drug for Amyotrophic Lateral Sclerosis need to be – the patient perspective: a letter in response to “Estimating the minimum important difference in the ALSFRS-R-instrument in5
SOD1 mutations in Taiwanese ALS patients: Clinical characteristics, frequency, and a p.T138R founder effect5
A verb-naming test accurately detects cognitive changes in ALS5
Theme 08 - Clinical Imaging and Electrophysiology5
Serum heat shock protein concentrations are not associated with amyotrophic lateral sclerosis risk or survival in three European populations5
Longitudinal comparison of the self-administered ALSFRS-RSE and ALSFRS-R as functional outcome measures in ALS5
Frequency of C9orf72 and SOD1 mutations in 302 sporadic ALS patients from three German ALS centers5
Prevalence of depression in amyotrophic lateral sclerosis/motor neuron disease: multi-attribute ascertainment and trajectories over 30 months5
Effects of COVID-19 on motor neuron disease mortality in the United States: a population-based cross-sectional study4
Italian reference values and brain correlates of verbal fluency index – vs standard verbal fluency test – to assess executive dysfunction in ALS4
The impact of motor neurone disease on oral health: a scoping review4
Mapping the natural history of amyotrophic lateral sclerosis: time-to-event analysis of clinical milestones in the pan-European, population-based PRECISION-ALS cohort4
Current practices in the nutrition management of people with amyotrophic lateral sclerosis (ALS): a survey of U.S. ALS care teams4
Neutrophil-to-lymphocyte ratio at diagnosis as a biomarker for survival of amyotrophic lateral sclerosis4
Molecular and genetic landscape of amyotrophic lateral sclerosis in Latin America: a scoping review of pathogenic hypotheses and ancestral heterogeneity4
In memoriam Ettore Beghi, 1947–20224
Clinically meaningful change: evaluation of the Rasch-built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS) and the ALSFRS-R4
Behavioral subtypes impact prognosis and survival in amyotrophic lateral sclerosis: a clustering-based approach4
Harmonized standard operating procedures for administering the ALS functional rating scale-revised4
Prodromal symptoms in amyotrophic lateral sclerosis from the perspective of the patient and of the caregiver4
Characterization, epidemiology, and factors associated with evolution and survival in patients with amyotrophic lateral sclerosis in southeastern Spain, 2008–2021: a population-based study4
Occupational lead exposure and amyotrophic lateral sclerosis survival in the Danish National Patient Registry4
Theme 3 In Vitro Experimental Models4
Theme 02 - Genetics and Genomics4
Thoracic electrical impedance tomography for assessing progression of pulmonary dysfunction in ALS4
Clinical trajectories of genetic variants in ALS: a European observational study within PRECISION-ALS4
Spanish adaptation of the Primary Lateral Sclerosis Functional Rating Scale (PLSFRS)4
ALSUntangled #71: Nuedexta4
Patients’ choices regarding ventilatory support affect opioid use in amyotrophic lateral sclerosis4
Multistep modeling applied to a Malaysian ALS registry3
Theme 2 Genetics and Genomics3
Psychometric properties of the Russian version of the Edinburgh cognitive and behavioral amyotrophic lateral sclerosis screen3
Research access barriers in amyotrophic lateral sclerosis3
REVEALS—a longitudinal cohort study of multifaceted respiratory assessment in ALS3
Specialized multidisciplinary care improves ALS survival in Belgium: a population-based retrospective study3
Comparing Amyotrophic lateral sclerosis (ALS) patient characteristics from the National ALS Registry and the Massachusetts ALS Registry, data through 20153
Correction3
Neurologists’ understanding of reproductive medicine options for genetic forms of motor neuron disease3
Physical activity in amyotrophic lateral sclerosis: a systematic review of the methodologies used to assess a possible association3
Neurofilament light chain dynamics in the pre-symptomatic phase of amyotrophic lateral sclerosis: a case report3
Prevalence of cognitive impairment in motor neuron diseases: alternative norming methods lead to considerably diverse estimates3
PROSA—a multicenter prospective observational study to develop low-burden digital speech biomarkers in ALS and FTD3
Percutaneous gastrostomy, mechanical ventilation and survival in amyotrophic lateral sclerosis: an observational study in an incident cohort3
Self-care mobile application for people with Amyotrophic Lateral Sclerosis: a development and usability test3
Serum small non-coding RNA define molecular subtypes in amyotrophic lateral sclerosis3
ECAS correlation with metabolic alterations on FDG-PET imaging in ALS3
Challenges and solutions to complex data governance issues in cross-national, cross-sectoral, multidisciplinary real world health research: a descriptive overview3
The quality of life and depression in primary caregivers of patients with amyotrophic lateral sclerosis is affected by patient-related and culture-specific conditions3
Assessment of language and executive functions in ALS: the brief executive Language screen3
Perils of predictive testing for unaffected people from motor neuron disease families with no identified causal gene3
A novel homozygous loss-of-function variant in SOD1 causing progressive spastic tetraplegia and axial hypotonia3
A geographical study on amyotrophic lateral sclerosis in Rio Grande Do Norte, Brazil, from 2005 to 20183
A nurse coaching intervention to improve support to individuals living with ALS3
Stigma experienced by ALS/PMA patients and their caregivers: a mixed-methods study3
Prevalence of amyotrophic lateral sclerosis in the United States, 20183
Theme 06 - Tissue Biomarkers2
Describing and characterising variability in ALS disease progression2
Remote digital assessment of amyotrophic lateral sclerosis functional rating scale – a multicenter observational study2
‘Outcomes of genetic testing in the London MND Center: the importance of achieving timely results and correlations to family history’2
Platform Communications: Abstract Book – 34th International Symposium on ALS/MND (Complete printable file)2
Interleukin-6 in ALS: metabolic mediator or marker of ventilatory decline?2
MRI biomarkers for memory-related impairment in amyotrophic lateral sclerosis: a systematic review2
Unraveling the contribution of executive functions to language impairment in ALS2
What is the extent of reliability and validity evidence for screening tools for cognitive and behavioral change in people with ALS? A systematic review2
Types and frequencies of adverse events across clinical trials for patients with amyotrophic lateral sclerosis: an analysis of the Pooled Resource Open-Access ALS Clinical Trials (PRO-ACT) database2
ALSUntangled #78: Zinc2
Profiles of disease progression and predictors of mortality in Colombian patients with amyotrophic lateral sclerosis: a comprehensive longitudinal study2
Amyotrophic lateral sclerosis caused by the C9orf72 expansion in Norway – prevalence, ancestry, clinical characteristics and sociodemographic status2
Four families with slowly progressive ALS due to p.Val120Leu SOD1 variant in Northeast Brazil2
ALSUntangled #75: Portable neuromodulation stimulator therapy2
Theme 11 Cognitive and Psychological Assessment and Support2
Deciphering ALS-linked genetic variants in indian patients using targeted and exome sequencing approaches2
Short tandem repeat expansions in LRP12 are absent in cohorts of familial and sporadic amyotrophic lateral sclerosis patients of European ancestry2
A novel mutation in the ALS2 gene in an iranian kurdish family with juvenile amyotrophic lateral sclerosis2
Theme 11 - Cognitive and Psychological Assessment and Support2
Living with a parent with ALS - adolescents’ need for professional support from the adolescents’ and the parents’ perspectives2
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
Reliable change indices for the cognitive section of Portuguese version of the Edinburgh Cognitive and Behavioural ALS screen (ECAS)2
Plasma neurofilament light levels show elevation two years prior to diagnosis of amyotrophic lateral sclerosis in the UK Biobank2
Cognitive reserve in ALS: the role of occupational skills and requirements2
Clinical characterization of the proximal lower-limb ALS phenotype: a retrospective cohort study2
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
Homozygosity for the C allele at UNC13A rs12608932 seems to compromise cognition in ALS independently of the cognitive domains2
Psychological resilience is protective against cognitive deterioration in motor neuron diseases2
Incidence of amyotrophic lateral sclerosis in Chile2
Racial disparities in ALS diagnostic delay: a single center’s experience and review of potential contributing factors2
Persistent high levels of perceived fatigue are not associated with hypermetabolism in patients with amyotrophic lateral sclerosis2
Table of Contents - Poster Communications2
An electronic health record cohort of Veterans with amyotrophic lateral sclerosis2
Rapid progression of amyotrophic lateral sclerosis after initiation of GLP-1 agonist: a case report2
SOD1-ALS mimicking an inflammatory neuropathy: a case report2
How to measure person-centred care in people living with ALS: development and validation of the patient experience PEMALS questionnaire2
Subjective sleep quality in amyotrophic lateral sclerosis: a systematic review and meta-analysis2
Platform Communications: Abstract Book 35th International Symposium on ALS/MND (Complete printable file)2
Natural history of the revised ALS functional rating scale and its association with survival: the PRECISION-ALS Extant Study2
Combination of ciprofloxacin/celecoxib as a novel therapeutic strategy for ALS2
Cerebrospinal fluid levels of NfM in relation to NfL and pNfH as prognostic markers in amyotrophic lateral sclerosis2
Diagnostic differences between military veterans and non-veterans: data from the United States National ALS Registry2
A study of patient recall and comprehension of genetic testing results in amyotrophic lateral sclerosis (ALS)2
SOD1 gene screening in ALS – frequency of mutations, patients’ attitudes to genetic information and transition to tofersen treatment in a multi-center program2
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
Determinants and progression of stigma in amyotrophic lateral sclerosis/motor neuron disease2
Targeting metabolic dysfunction in amyotrophic lateral sclerosis: therapeutic potential of GLP-1 receptor agonists1
Neck flexion weakness predicts respiratory dysfunction in amyotrophic lateral sclerosis1
Cognitive and behavioral involvement in spinal and bulbar muscular atrophy (SBMA): a systematic review1
ALSUntangled #82: N-acetylcysteine1
What can vowel acoustics reveal about the communicative participation of people living with ALS?1
ALSUntangled #76: Wahls protocol1
Association of the practice of contact sports with the development of amyotrophic lateral sclerosis1
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
Subcortical grey matter involvement in ALS and PLS – vulnerable hubs of cortico-cortical and cortico-basal circuits: extrapyramidal, cognitive, bulbar and respiratory correlates1
Focal epilepsy followed by rapidly progressive frontotemporal dementia: a rare manifestation of VCP mutation1
How the coronavirus pandemic affected the lives of people with ALS and their spouses in the UK from spouses’ perspectives: a qualitative study1
Measuring disability in amyotrophic lateral sclerosis/motor neuron disease: the WHODAS 2.0-36, WHODAS 2.0-32, and WHODAS 2.0-121
Geographical distribution of clinical trials in amyotrophic lateral sclerosis: a scoping review1
Clinical parameters affecting the course of amyotrophic lateral sclerosis: a longitudinal analysis of the Greek registry for amyotrophic lateral sclerosis (ALS-GR)1
The incidence and prevalence of motor neurone disease in South Australia1
Analysis of non-motor symptoms in amyotrophic lateral sclerosis1
Novel in-frame duplication variant of SOD1 in a Japanese family with familial amyotrophic lateral sclerosis1
Gadolinium enhancement of the cauda equina in a case of familial ALS with p.S135G SOD1 mutation1
Genetic overlap between ALS and other neurodegenerative or neuromuscular disorders1
Integrated analysis of transcriptomic and proteomic alterations in mouse models of ALS/FTD identify early metabolic adaptions with similarities to mitochondrial dysfunction disorders1
SOD1-ALS-Browser: a web-utility for investigating the clinical phenotype in SOD1 amyotrophic lateral sclerosis1
Treatment continuity of amyotrophic lateral sclerosis with available riluzole formulations: state of the art and current challenges in a ‘real-world’ setting1
Lack of habituation in somatosensory cortex but not in visual cortex of ALS patients1
Theme 08 - Clinical Imaging and Electrophysiology1
Theme 8 Clinical Imaging and Electrophysiology1
Clinical factors and vascular endothelial growth factor as determinants of disease progression in Indian patients with amyotrophic lateral sclerosis1
Patient experience and clinical outcome assessment validity in amyotrophic lateral sclerosis: a targeted literature review1
Theme 12 - Clinical Management and Support1
Clinical drug development in amyotrophic lateral sclerosis: industry-reported challenges and opportunities1
Development of a machine learning-based survival prediction model for ALS inclusive of the advanced-stage population1
MAPT p.V363I mutation in a patient with presenile dementia and late amyotrophic lateral sclerosis1
Examining cognitive decline over time in Iranian ALS patients: Adapting successive versions B and C of the Edinburgh cognitive and behavioral screen to Persian1
Autophagy dysregulation plays a crucial role in regulatory T-cell loss and neuroinflammation in amyotrophic lateral sclerosis (ALS)1
Premorbid lipid levels and long-term risk of ALS—a population-based cohort study1
Transcranial magnetic stimulation to monitor disease progression in ALS: a review1
Analysis of STMN2 CA repeats in italian ALS patients shows no association1
ALSUntangled #74: Withania Somnifera (Ashwagandha)1
LAENALS: epidemiological and clinical features of amyotrophic lateral sclerosis in Latin America1
ALS due to c.1189 + 1G > T (splice donor) TBK1 mutation1
Air pollutants and amyotrophic lateral sclerosis in a population-based registry: investigating disease susceptibility, progression and survival1
Participant, site personnel and sponsor perspectives on decentralized trial features in COURAGE-ALS: a randomized clinical trial1
Theme 07 - Pre-Clinical Therapeutic Strategies1
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