OOIR: Observatory of International Research

Papers

(The TQCC of Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration is 4. 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.)

Article	Citations
Rapid classification of a novel ALS-causing I149S variant in superoxide dismutase-1	45
Low plasma hyaluronan is associated with faster functional decline in patients with amyotrophic lateral sclerosis	35
Clinical and demographical characteristics in a cohort of MND patients treated with riluzole. Differences between tablets and oral suspension	32
Correction	31
The correlation between social support, coping style, advance care planning readiness, and quality of life in patients with amyotrophic lateral sclerosis: a cross-sectional study	25
Validity and reliability measures of the Swedish Karolinska version of the Edinburgh Cognitive and Behavioral ALS Screen (SK-ECAS)	25
Incorporating patient preferences and burden-of-disease in evaluating ALS drug candidate AMX0035: a Bayesian decision analysis perspective	24
The amyotrophic lateral sclerosis-health index (ALS-HI): development and evaluation of a novel outcome measure	20
Clinical epidemiology of amyotrophic lateral sclerosis in Liguria, Italy: a ten year follow up study	19
Development, reliability, validity, and acceptability of the remote administration of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS)	18
Peripheral immunity relate to disease progression and prognosis in amyotrophic lateral sclerosis	18
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 populations	18
Patient engagement in research: lessons learned from CAPTURE ALS, a longitudinal observational ALS study	15
Geographical clusters of amyotrophic lateral sclerosis and the Bradford Hill criteria	15
Residential exposure associations with ALS risk, survival, and phenotype: a Michigan-based case-control study	15
Theme 5 Human Cell Biology and Pathology	15
Amyotrophic lateral sclerosis mortality rates in Latin America and the Caribbean: a meta-analysis	15
Assessing pulmonary function in ALS using electrical impedance tomography	14
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 databases	14
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, Italy	14
Portable fixed dynamometry enables home-based, reliable assessment of muscle strength in patients with amyotrophic lateral sclerosis: a pilot study	14
Theme 11 - Cognitive and Psychological Assessment and Support	14
Care burden and related factors among informal caregivers of patients with amyotrophic lateral sclerosis	13

Theme 10 - Disease Stratification and Phenotyping of Patients	13
Time from amyotrophic lateral sclerosis symptom onset to key disease milestones: analysis of data from a multinational cross-sectional survey	13
Genetic variation in targets of lipid-lowering drugs and amyotrophic lateral sclerosis risk: a Mendelian randomization study	13
Self-assessment of amyotrophic lateral sclerosis functional rating scale on the patient’s smartphone proves to be non-inferior to clinic data capture	11
Correction	11
Amyotrophic lateral sclerosis: exploring pathophysiology in the context of treatment	11
Automated speech analytics in ALS: higher sensitivity of digital articulatory precision over the ALSFRS-R	11
Yield of EEG features as markers of disease severity in amyotrophic lateral sclerosis: a pilot study	11
Theme 03 - IN VITRO EXPERIMENTAL MODELS	11
Supporting people with Motor Neuron Disease (MND) to make decisions about gastrostomy feeding tube placement: a survey of UK healthcare professionals’ practice and beliefs	10
Slowly progressive late-onset spinal muscular atrophy Finkel-type related to p.Pro56Ser VABP mutation in Colombia	10
The contralateral co-movement test in a Chinese population with amyotrophic lateral sclerosis	10
Feature selection using metaheuristics to predict annual amyotrophic lateral sclerosis progression	10
Drug treatments and interactions, disease progression and quality of life in ALS patients	10
Time-to-event prediction in ALS using a landmark modeling approach, using the ALS Natural History Consortium dataset	10
The involvement of palliative care with neurology – a comparison of UK, Switzerland and Italy	10
A novel TBK1 loss-of-function variant associated with ALS and parkinsonism phenotypes	9
IMPACT-ALS: summary of results from a European survey of people living with ALS	9
Monocyte CD14 and HLA-DR expression increases with disease duration and severity in amyotrophic lateral sclerosis	9
PRECISION ALS—an integrated pan European patient data platform for ALS	9
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 family	9
Toward genetic counseling practice standards for diagnostic testing in amyotrophic lateral sclerosis and frontotemporal dementia	9
A revision to the United States national ALS registry’s algorithm to improve Case-Ascertainment	9
Exposure to electromagnetic fields does not modify neither the age of onset nor the disease progression in ALS patients	9
The natural history of ALS: Baseline characteristics from a multicenter clinical cohort	9
Exploring the needs and preferences of people with amyotrophic lateral sclerosis (ALS) when making genomic testing decisions: an interview study	9
Improving the measurement properties of the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R): deriving a valid measurement total for the calculation of change	9
Epidemiology and economic burden of amyotrophic lateral sclerosis in the United States: a literature review	8
Expanding the phenotype of TARDBP mutation in a Tunisian family with clinical phenotype heterogeneity	8
Body mass index is lower in asymptomatic C9orf72 expansion carriers but not in SOD1 pathogenic variant carriers compared to gene negatives	8
Amyotrophic lateral sclerosis estimated prevalence cases from 2022 to 2030, data from the national ALS Registry	8
The association of theory of mind with language and visuospatial abilities in amyotrophic lateral sclerosis: a pilot study	8
Comparison Of King’s Clinical Staging In Multinational Amyotrophic Lateral Sclerosis Cohorts	8
Changes in saccadic intrusions over time as an objective biomarker to follow ALS disease progression	8
International network for ALS research and care (INARC)	8
Neuropsychological assessment practices in PRECISION-ALS: challenges and opportunities for harmonization	8
Increased total daily energy expenditure in mild to moderate ALS: greater contribution from physical activity energy expenditure than hyper-metabolism	7
Why is nutrition vital for advancing ALS care and clinical research?	7
Impact of mode of training and recertification on ALSFRS-R rater performance	7
Cognition in the course of ALS—a meta-analysis	7
Neurofilament light chain as a biological marker for amyotrophic lateral sclerosis: a meta-analysis study	7
Respiratory measurements, respiratory symptoms, and quality of life in ALS: results from the REVEALS study	7
Theme 03 - In Vitro Experimental Models	7
The cholesterol depleting agent, (2-Hydroxypropyl)-ß-cyclodextrin, does not affect disease progression in SOD1 ^G93A mice	6
ALSUntangled #79: alpha-lipoic acid	6
Primary lateral sclerosis natural history study – planning, designing, and early enrollment	6
ALSUntangled #65: glucocorticoid corticosteroids	6
Theme 12 - Clinical Management and Support	6
Longitudinal comparison of the self-administered ALSFRS-RSE and ALSFRS-R as functional outcome measures in ALS	6

ALSUntangled # 69: astaxanthin	6
Demographic changes in a large motor neuron disease cohort in Portugal: a 27 year experience	6
Urine specific gravity to identify and predict hydration need in ALS	6
A speech-based prognostic model for dysarthria progression in ALS	6
MiNDAUS partnership: a roadmap for the cure and management of motor Neurone disease	6
Serum heat shock protein concentrations are not associated with amyotrophic lateral sclerosis risk or survival in three European populations	6
Quantitative susceptibility mapping for investigating brain iron deposits in amyotrophic lateral sclerosis: correlations with clinical phenotype and disease progression	6
How the prospect of a clinical trial impacts decision-making for predictive genetic testing in ALS	6
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 Editor	6
Apathy in amyotrophic lateral sclerosis: systematic review and meta-analysis of frequency, correlates, and outcomes	6
Study of “ALS reversals”: LifeTime environmental exposures (StARLiTE)	5
A verb-naming test accurately detects cognitive changes in ALS	5
A de novo c.113 T > C: p.L38R mutation of SPTLC1: case report of a girl with sporadic juvenile amyotrophic lateral sclerosis	5
The Beaumont behavioral intervention in a Chinese amyotrophic lateral sclerosis cohort	5
Frequency of C9orf72 and SOD1 mutations in 302 sporadic ALS patients from three German ALS centers	5
HLA and amyotrophic lateral sclerosis: a systematic review and meta-analysis	5
Theme 10 - DISEASE STRATIFICATION AND PHENOTYPING OF PATIENTS	5
Theme 08 - Clinical Imaging and Electrophysiology	5
Verbal expressive language minimally affected in non-demented people living with amyotrophic lateral sclerosis	5
Relationship between quantitative strength and functional outcomes in the phase 2 FORTITUDE-ALS trial	5
Acceptability and potential benefit of a self-compassion intervention for people living with amyotrophic lateral sclerosis: a mixed methods pilot study	5
Association of UNC13A with increased amyotrophic lateral sclerosis risk, bulbar onset, and lower motor neuron involvement in a Norwegian ALS cohort	5
Correction	5
Occupational lead exposure and amyotrophic lateral sclerosis survival in the Danish National Patient Registry	4
Prevalence of depression in amyotrophic lateral sclerosis/motor neuron disease: multi-attribute ascertainment and trajectories over 30 months	4
Theme 01 - Epidemiology and Informatics	4
Patients’ choices regarding ventilatory support affect opioid use in amyotrophic lateral sclerosis	4
Mapping the natural history of amyotrophic lateral sclerosis: time-to-event analysis of clinical milestones in the pan-European, population-based PRECISION-ALS cohort	4
Harmonized standard operating procedures for administering the ALS functional rating scale-revised	4
Thoracic electrical impedance tomography for assessing progression of pulmonary dysfunction in ALS	4
Italian reference values and brain correlates of verbal fluency index – vs standard verbal fluency test – to assess executive dysfunction in ALS	4
ALSUntangled #71: Nuedexta	4
Characterization, epidemiology, and factors associated with evolution and survival in patients with amyotrophic lateral sclerosis in southeastern Spain, 2008–2021: a population-based study	4
The expanding clinical and genetic spectrum of alsin-related disorders: the first cohort of Brazilian patients	4
Current practices in the nutrition management of people with amyotrophic lateral sclerosis (ALS): a survey of U.S. ALS care teams	4
Clinical trajectories of genetic variants in ALS: a European observational study within PRECISION-ALS	4
Effects of COVID-19 on motor neuron disease mortality in the United States: a population-based cross-sectional study	4
Neutrophil-to-lymphocyte ratio at diagnosis as a biomarker for survival of amyotrophic lateral sclerosis	4
Medical therapies for amyotrophic lateral sclerosis-related respiratory decline: an appraisal of needs, opportunities and obstacles	4
Theme 05 - Human Cell Biology and Pathology (including iPSC studies)	4
Incidence of amyotrophic lateral sclerosis in the United States, 2014–2016	4
Theme 02 - Genetics and Genomics	4
Clinically meaningful change: evaluation of the Rasch-built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS) and the ALSFRS-R	4
Theme 06 - TISSUE BIOMARKERS	4
In memoriam Ettore Beghi, 1947–2022	4