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 2021-12-01 to 2025-12-01.)

Article	Citations
Correction	51
Single task-level, 2SD-based cutoffs for the Italian version of the Edinburgh Cognitive and Behavioral ALS screen (ECAS)	39
Incorporating patient preferences and burden-of-disease in evaluating ALS drug candidate AMX0035: a Bayesian decision analysis perspective	34
Clinical epidemiology of amyotrophic lateral sclerosis in Liguria, Italy: a ten year follow up study	30
Development, reliability, validity, and acceptability of the remote administration of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS)	29
Clinical and demographical characteristics in a cohort of MND patients treated with riluzole. Differences between tablets and oral suspension	26
Rapid classification of a novel ALS-causing I149S variant in superoxide dismutase-1	24
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	23
The amyotrophic lateral sclerosis-health index (ALS-HI): development and evaluation of a novel outcome measure	23
Validity and reliability measures of the Swedish Karolinska version of the Edinburgh Cognitive and Behavioral ALS Screen (SK-ECAS)	22
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	21
Theme 5 Human Cell Biology and Pathology	21
Impact of occupational categories on the incidence of amyotrophic lateral sclerosis in Sardinia Island, Italy	20
Amyotrophic lateral sclerosis mortality rates in Latin America and the Caribbean: a meta-analysis	20
Genetic ancestry and risk allele C9orf72 rs3849942 T for amyotrophic lateral sclerosis in Latin American populations	19
Patient engagement in research: lessons learned from CAPTURE ALS, a longitudinal observational ALS study	19
Epidemiology, clinical features, and management of amyotrophic lateral sclerosis in the neurology department of the Bogodogo University Hospital in Ouagadougou, Burkina Faso	18
Peripheral immunity relate to disease progression and prognosis in amyotrophic lateral sclerosis	18
Theme 7 Pre-Clinical Therapeutic Strategies	17
Residential exposure associations with ALS risk, survival, and phenotype: a Michigan-based case-control study	16
Assessing pulmonary function in ALS using electrical impedance tomography	15
Can resting lung function predict the response of a person living with motor neuron disease to a hypoxic challenge test?	15
Portable fixed dynamometry enables home-based, reliable assessment of muscle strength in patients with amyotrophic lateral sclerosis: a pilot study	14
Theme 10 - Disease Stratification and Phenotyping of Patients	13
Genetic variation in targets of lipid-lowering drugs and amyotrophic lateral sclerosis risk: a Mendelian randomization study	13

Theme 11 - Cognitive and Psychological Assessment and Support	13
Time from amyotrophic lateral sclerosis symptom onset to key disease milestones: analysis of data from a multinational cross-sectional survey	13
Yield of EEG features as markers of disease severity in amyotrophic lateral sclerosis: a pilot study	12
Automated speech analytics in ALS: higher sensitivity of digital articulatory precision over the ALSFRS-R	12
Feature selection using metaheuristics to predict annual amyotrophic lateral sclerosis progression	12
Correction	12
Self-assessment of amyotrophic lateral sclerosis functional rating scale on the patient’s smartphone proves to be non-inferior to clinic data capture	12
Slowly progressive late-onset spinal muscular atrophy Finkel-type related to p.Pro56Ser VABP mutation in Colombia	12
Care burden and related factors among informal caregivers of patients with amyotrophic lateral sclerosis	12
Amyotrophic lateral sclerosis: exploring pathophysiology in the context of treatment	12
The contralateral co-movement test in a Chinese population with amyotrophic lateral sclerosis	12
The involvement of palliative care with neurology – a comparison of UK, Switzerland and Italy	11
Drug treatments and interactions, disease progression and quality of life in ALS patients	11
Time-to-event prediction in ALS using a landmark modeling approach, using the ALS Natural History Consortium dataset	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	11
Platform Communications: Abstract Book – 33rd International Symposium on ALS/MND (Complete printable file)	10
Comparison Of King’s Clinical Staging In Multinational Amyotrophic Lateral Sclerosis Cohorts	10
The association of theory of mind with language and visuospatial abilities in amyotrophic lateral sclerosis: a pilot study	10
Quality of life and care burden of people living with amyotrophic lateral sclerosis who need home-based medical care in Korea and their family caregivers	10
Exploring the needs and preferences of people with amyotrophic lateral sclerosis (ALS) when making genomic testing decisions: an interview study	10
Response letter to: a homozygous p.Val120Leu (c.358G > C) SOD1 mutation led to slowly progressive amyotrophic lateral sclerosis in a Brazilian family	10
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
Toward genetic counseling practice standards for diagnostic testing in amyotrophic lateral sclerosis and frontotemporal dementia	9
International network for ALS research and care (INARC)	9
Exposure to electromagnetic fields does not modify neither the age of onset nor the disease progression in ALS patients	9
Expanding the phenotype of TARDBP mutation in a Tunisian family with clinical phenotype heterogeneity	9
Changes in saccadic intrusions over time as an objective biomarker to follow ALS disease progression	8
IMPACT-ALS: summary of results from a European survey of people living with ALS	8
A novel TBK1 loss-of-function variant associated with ALS and parkinsonism phenotypes	8
The natural history of ALS: Baseline characteristics from a multicenter clinical cohort	8
ALSUntangled #80: ISRIB (Integrated stress response InhiBitor)	8
Self-reported initiation apathy is related to worse quality of life in amyotrophic lateral sclerosis	8
A revision to the United States national ALS registry’s algorithm to improve Case-Ascertainment	8
Amyotrophic lateral sclerosis estimated prevalence cases from 2022 to 2030, data from the national ALS Registry	8
Increased total daily energy expenditure in mild to moderate ALS: greater contribution from physical activity energy expenditure than hyper-metabolism	8
PRECISION ALS—an integrated pan European patient data platform for ALS	8
Neuropsychological assessment practices in PRECISION-ALS: challenges and opportunities for harmonization	8
Impact of mode of training and recertification on ALSFRS-R rater performance	7
Epidemiology and economic burden of amyotrophic lateral sclerosis in the United States: a literature review	7
A speech-based prognostic model for dysarthria progression in ALS	7
Cognition in the course of ALS—a meta-analysis	7
Respiratory measurements, respiratory symptoms, and quality of life in ALS: results from the REVEALS study	7
Neurofilament light chain as a biological marker for amyotrophic lateral sclerosis: a meta-analysis study	7
Why is nutrition vital for advancing ALS care and clinical research?	7
The cholesterol depleting agent, (2-Hydroxypropyl)-ß-cyclodextrin, does not affect disease progression in SOD1 ^G93A mice	7
Dextromethorphan/quinidine (DMQ) for reducing bulbar symptoms in amyotrophic lateral sclerosis – assessment of treatment experience in a multicenter study	7
Body mass index is lower in asymptomatic C9orf72 expansion carriers but not in SOD1 pathogenic variant carriers compared to gene negatives	7
Theme 03 - In Vitro Experimental Models	7
Urine specific gravity to identify and predict hydration need in ALS	7
ALSUntangled # 69: astaxanthin	6

Demographic changes in a large motor neuron disease cohort in Portugal: a 27 year experience	6
Letter to Editor	6
Serum heat shock protein concentrations are not associated with amyotrophic lateral sclerosis risk or survival in three European populations	6
Apathy in amyotrophic lateral sclerosis: systematic review and meta-analysis of frequency, correlates, and outcomes	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
Primary lateral sclerosis natural history study – planning, designing, and early enrollment	6
Association of UNC13A with increased amyotrophic lateral sclerosis risk, bulbar onset, and lower motor neuron involvement in a Norwegian ALS cohort	6
ALSUntangled #79: alpha-lipoic acid	6
Quantitative susceptibility mapping for investigating brain iron deposits in amyotrophic lateral sclerosis: correlations with clinical phenotype and disease progression	6
Theme 10 Disease Stratification and Phenotyping of Patients	6
Theme 12 - Clinical Management and Support	6
A de novo c.113 T > C: p.L38R mutation of SPTLC1: case report of a girl with sporadic juvenile amyotrophic lateral sclerosis	6
Frequency of C9orf72 and SOD1 mutations in 302 sporadic ALS patients from three German ALS centers	5
ALSUntangled #65: glucocorticoid corticosteroids	5
The Beaumont behavioral intervention in a Chinese amyotrophic lateral sclerosis cohort	5
Study of “ALS reversals”: LifeTime environmental exposures (StARLiTE)	5
Incidence of amyotrophic lateral sclerosis in the United States, 2014–2016	5
Theme 01 - Epidemiology and Informatics	5
Longitudinal comparison of the self-administered ALSFRS-RSE and ALSFRS-R as functional outcome measures in ALS	5
Correction	5
Verbal expressive language minimally affected in non-demented people living with amyotrophic lateral sclerosis	5
A verb-naming test accurately detects cognitive changes in ALS	5
HLA and amyotrophic lateral sclerosis: a systematic review and meta-analysis	5
Theme 05 - Human Cell Biology and Pathology (including iPSC studies)	5
Theme 9 Clinical Trials and Trial Design	5
Theme 08 - Clinical Imaging and Electrophysiology	5
Serum NfL, but not GFAP, differentiates primary lateral sclerosis from adrenomyeloneuropathy and hereditary spastic paraplegia type 4	5
Acceptability and potential benefit of a self-compassion intervention for people living with amyotrophic lateral sclerosis: a mixed methods pilot study	5
Relationship between quantitative strength and functional outcomes in the phase 2 FORTITUDE-ALS trial	5
Amyotrophic lateral sclerosis in Saudi Arabia: a multicenter descriptive study	4
Thoracic electrical impedance tomography for assessing progression of pulmonary dysfunction in ALS	4
Effects of COVID-19 on motor neuron disease mortality in the United States: a population-based cross-sectional study	4
Behavioral subtypes impact prognosis and survival in amyotrophic lateral sclerosis: a clustering-based approach	4
In memoriam Ettore Beghi, 1947–2022	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
Neutrophil-to-lymphocyte ratio at diagnosis as a biomarker for survival of amyotrophic lateral sclerosis	4
Clinical trajectories of genetic variants in ALS: a European observational study within PRECISION-ALS	4
Harmonized standard operating procedures for administering the ALS functional rating scale-revised	4
Current practices in the nutrition management of people with amyotrophic lateral sclerosis (ALS): a survey of U.S. ALS care teams	4
Prevalence of depression in amyotrophic lateral sclerosis/motor neuron disease: multi-attribute ascertainment and trajectories over 30 months	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
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 3 In Vitro Experimental Models	4
Italian reference values and brain correlates of verbal fluency index – vs standard verbal fluency test – to assess executive dysfunction in ALS	4
Occupational lead exposure and amyotrophic lateral sclerosis survival in the Danish National Patient Registry	4
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 in	4