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 2022-05-01 to 2026-05-01.)

Article	Citations
Correction	34
Clinical and demographical characteristics in a cohort of MND patients treated with riluzole. Differences between tablets and oral suspension	32
Development, reliability, validity, and acceptability of the remote administration of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS)	30
Incorporating patient preferences and burden-of-disease in evaluating ALS drug candidate AMX0035: a Bayesian decision analysis perspective	30
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
Rapid classification of a novel ALS-causing I149S variant in superoxide dismutase-1	25
Clinical epidemiology of amyotrophic lateral sclerosis in Liguria, Italy: a ten year follow up study	24
The amyotrophic lateral sclerosis-health index (ALS-HI): development and evaluation of a novel outcome measure	23
Single task-level, 2SD-based cutoffs for the Italian version of the Edinburgh Cognitive and Behavioral ALS screen (ECAS)	22
Theme 5 Human Cell Biology and Pathology	21
Genetic ancestry and risk allele C9orf72 rs3849942 T for amyotrophic lateral sclerosis in Latin American populations	20
Impact of occupational categories on the incidence of amyotrophic lateral sclerosis in Sardinia Island, Italy	20
Challenges in medical care for amyotrophic lateral sclerosis: a survey of physicians from Republic of Bashkortostan (Russia), Belarus, and Kazakhstan	19
Residential exposure associations with ALS risk, survival, and phenotype: a Michigan-based case-control study	19
Theme 7 Pre-Clinical Therapeutic Strategies	18
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	17
Emerging strategies for interpreting variants of uncertain significance (VUS) in amyotrophic lateral sclerosis	17
Epidemiology, clinical features, and management of amyotrophic lateral sclerosis in the neurology department of the Bogodogo University Hospital in Ouagadougou, Burkina Faso	16
Patient engagement in research: lessons learned from CAPTURE ALS, a longitudinal observational ALS study	16
Care burden and related factors among informal caregivers of patients with amyotrophic lateral sclerosis	14
Portable fixed dynamometry enables home-based, reliable assessment of muscle strength in patients with amyotrophic lateral sclerosis: a pilot study	14
Assessing pulmonary function in ALS using electrical impedance tomography	14
Peripheral immunity relate to disease progression and prognosis in amyotrophic lateral sclerosis	14
Amyotrophic lateral sclerosis: exploring pathophysiology in the context of treatment	14

Theme 11 - Cognitive and Psychological Assessment and Support	14
Correction	13
Time from amyotrophic lateral sclerosis symptom onset to key disease milestones: analysis of data from a multinational cross-sectional survey	13
Can resting lung function predict the response of a person living with motor neuron disease to a hypoxic challenge test?	13
Self-assessment of amyotrophic lateral sclerosis functional rating scale on the patient’s smartphone proves to be non-inferior to clinic data capture	13
ALS motor phenotypes: a revised ‘OPM’ classification	13
Yield of EEG features as markers of disease severity in amyotrophic lateral sclerosis: a pilot study	12
Genetic variation in targets of lipid-lowering drugs and amyotrophic lateral sclerosis risk: a Mendelian randomization study	12
Theme 10 - Disease Stratification and Phenotyping of Patients	12
The contralateral co-movement test in a Chinese population with amyotrophic lateral sclerosis	11
Slowly progressive late-onset spinal muscular atrophy Finkel-type related to p.Pro56Ser VABP mutation in Colombia	11
Limited data capture on reproductive medicine use in amyotrophic lateral sclerosis: implications for monitoring access	10
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
Automated speech analytics in ALS: higher sensitivity of digital articulatory precision over the ALSFRS-R	10
Feature selection using metaheuristics to predict annual amyotrophic lateral sclerosis progression	10
Pridopidine treatment in ALS: subgroup analyses from the HEALEY ALS Platform trial	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
Platform Communications: Abstract Book – 33rd International Symposium on ALS/MND (Complete printable file)	9
Comparison Of King’s Clinical Staging In Multinational Amyotrophic Lateral Sclerosis Cohorts	9
Expanding the phenotype of TARDBP mutation in a Tunisian family with clinical phenotype heterogeneity	9
IMPACT-ALS: summary of results from a European survey of people living with ALS	9
PRECISION ALS—an integrated pan European patient data platform for ALS	9
The natural history of ALS: Baseline characteristics from a multicenter clinical cohort	9
Exposure to electromagnetic fields does not modify neither the age of onset nor the disease progression in ALS patients	9
International network for ALS research and care (INARC)	9
Neuropsychological assessment practices in PRECISION-ALS: challenges and opportunities for harmonization	9
A novel TBK1 loss-of-function variant associated with ALS and parkinsonism phenotypes	9
Exploring the needs and preferences of people with amyotrophic lateral sclerosis (ALS) when making genomic testing decisions: an interview study	9
Self-reported initiation apathy is related to worse quality of life in amyotrophic lateral sclerosis	9
Incidence of MND in Canterbury, New Zealand and impact of a key worker role on hospital admissions	8
Amyotrophic lateral sclerosis estimated prevalence cases from 2022 to 2030, data from the national ALS Registry	8
Impact of mode of training and recertification on ALSFRS-R rater performance	8
Response letter to: a homozygous p.Val120Leu (c.358G > C) SOD1 mutation led to slowly progressive amyotrophic lateral sclerosis in a Brazilian family	8
A revision to the United States national ALS registry’s algorithm to improve Case-Ascertainment	8
Changes in saccadic intrusions over time as an objective biomarker to follow ALS disease progression	8
Increased total daily energy expenditure in mild to moderate ALS: greater contribution from physical activity energy expenditure than hyper-metabolism	8
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	8
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	8
Body mass index is lower in asymptomatic C9orf72 expansion carriers but not in SOD1 pathogenic variant car	8
Respiratory measurements, respiratory symptoms, and quality of life in ALS: results from the REVEALS study	7
Cognition in the course of ALS—a meta-analysis	7
Epidemiology and economic burden of amyotrophic lateral sclerosis in the United States: a literature review	7
Dextromethorphan/quinidine (DMQ) for reducing bulbar symptoms in amyotrophic lateral sclerosis – assessment of treatment experience in a multicenter study	7
The genetics of autosomal recessive ALS: a review of the common forms and their phenotypes	7
ALSUntangled #80: ISRIB (Integrated stress response InhiBitor)	7
Why is nutrition vital for advancing ALS care and clinical research?	7
Theme 03 - In Vitro Experimental Models	7
ALSUntangled #79: alpha-lipoic acid	6
Association of UNC13A with increased amyotrophic lateral sclerosis risk, bulbar onset, and lower motor neuron involvement in a Norwegian ALS cohort	6

Longitudinal comparison of the self-administered ALSFRS-RSE and ALSFRS-R as functional outcome measures in ALS	6
Demographic changes in a large motor neuron disease cohort in Portugal: a 27 year experience	6
The cholesterol depleting agent, (2-Hydroxypropyl)-ß-cyclodextrin, does not affect disease progression in SOD1 ^G93A mice	6
Quantitative susceptibility mapping for investigating brain iron deposits in amyotrophic lateral sclerosis: correlations with clinical phenotype and disease progression	6
ALSUntangled #65: glucocorticoid corticosteroids	6
Primary lateral sclerosis natural history study – planning, designing, and early enrollment	6
How the prospect of a clinical trial impacts decision-making for predictive genetic testing in ALS	6
A speech-based prognostic model for dysarthria progression in ALS	6
Theme 12 - Clinical Management and Support	6
Serum heat shock protein concentrations are not associated with amyotrophic lateral sclerosis risk or survival in three European populations	6
ALSUntangled # 69: astaxanthin	6
Theme 10 Disease Stratification and Phenotyping of Patients	6
Theme 05 - Human Cell Biology and Pathology (including iPSC studies)	5
Translation and validation of the Arabic version of the amyotrophic lateral sclerosis assessment questionnaire (ALSAQ40-AR)	5
The Beaumont behavioral intervention in a Chinese amyotrophic lateral sclerosis cohort	5
Acceptability and potential benefit of a self-compassion intervention for people living with amyotrophic lateral sclerosis: a mixed methods pilot study	5
Correction	5
Frequency of C9orf72 and SOD1 mutations in 302 sporadic ALS patients from three German ALS centers	5
Patients’ choices regarding ventilatory support affect opioid use in amyotrophic lateral sclerosis	5
Theme 01 - Epidemiology and Informatics	5
Amyotrophic lateral sclerosis in Colombia: a population-based study of incidence and socioeconomic determinants	5
Verbal expressive language minimally affected in non-demented people living with amyotrophic lateral sclerosis	5
Serum NfL, but not GFAP, differentiates primary lateral sclerosis from adrenomyeloneuropathy and hereditary spastic paraplegia type 4	5
Letter to Editor	5
HLA and amyotrophic lateral sclerosis: a systematic review and meta-analysis	5
Characterization, epidemiology, and factors associated with evolution and survival in patients with amyotrophic lateral sclerosis in southeastern Spain, 2008–2021: a population-based study	5
Prevalence of depression in amyotrophic lateral sclerosis/motor neuron disease: multi-attribute ascertainment and trajectories over 30 months	5
SOD1 mutations in Taiwanese ALS patients: Clinical characteristics, frequency, and a p.T138R founder effect	5
Theme 9 Clinical Trials and Trial Design	5
Theme 08 - Clinical Imaging and Electrophysiology	5
A verb-naming test accurately detects cognitive changes in ALS	5
Study of “ALS reversals”: LifeTime environmental exposures (StARLiTE)	5
Relationship between quantitative strength and functional outcomes in the phase 2 FORTITUDE-ALS trial	5
ALSUntangled #71: Nuedexta	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
Theme 02 - Genetics and Genomics	4
Challenges and solutions to complex data governance issues in cross-national, cross-sectoral, multidisciplinary real world health research: a descriptive overview	4
Current practices in the nutrition management of people with amyotrophic lateral sclerosis (ALS): a survey of U.S. ALS care teams	4
Behavioral subtypes impact prognosis and survival in amyotrophic lateral sclerosis: a clustering-based approach	4
Clinical trajectories of genetic variants in ALS: a European observational study within PRECISION-ALS	4
Occupational lead exposure and amyotrophic lateral sclerosis survival in the Danish National Patient Registry	4
Theme 3 In Vitro Experimental Models	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
Italian reference values and brain correlates of verbal fluency index – vs standard verbal fluency test – to assess executive dysfunction in ALS	4
Spanish adaptation of the Primary Lateral Sclerosis Functional Rating Scale (PLSFRS)	4
The impact of motor neurone disease on oral health: a scoping review	4
Neutrophil-to-lymphocyte ratio at diagnosis as a biomarker for survival of amyotrophic lateral sclerosis	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
Amyotrophic lateral sclerosis in Saudi Arabia: a multicenter descriptive study	4
Effects of COVID-19 on motor neuron disease mortality in the United States: a population-based cross-sectional study	4
Clinically meaningful change: evaluation of the Rasch-built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS) and the ALSFRS-R	4
Prevalence of cognitive impairment in motor neuron diseases: alternative norming methods lead to considerably diverse estimates	4
Prodromal symptoms in amyotrophic lateral sclerosis from the perspective of the patient and of the caregiver	4
Thoracic electrical impedance tomography for assessing progression of pulmonary dysfunction in ALS	4
Four decades of ALS care: a retrospective study of epidemiology, clinical course and changes in management	4
In memoriam Ettore Beghi, 1947–2022	4
Harmonized standard operating procedures for administering the ALS functional rating scale-revised	4