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 2021-05-01 to 2025-05-01.)

Article	Citations
Rapid classification of a novel ALS-causing I149S variant in superoxide dismutase-1	41
Low plasma hyaluronan is associated with faster functional decline in patients with amyotrophic lateral sclerosis	31
The amyotrophic lateral sclerosis-health index (ALS-HI): development and evaluation of a novel outcome measure	30
Clinical and demographical characteristics in a cohort of MND patients treated with riluzole. Differences between tablets and oral suspension	29
Expanding the clinical and genetic spectrum of SQSTM1-related disorders in family with personality disorder and frontotemporal dementia	28
Correction	22
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	21
Clinical epidemiology of amyotrophic lateral sclerosis in Liguria, Italy: a ten year follow up study	19
Validity and reliability measures of the Swedish Karolinska version of the Edinburgh Cognitive and Behavioral ALS Screen (SK-ECAS)	19
Incorporating patient preferences and burden-of-disease in evaluating ALS drug candidate AMX0035: a Bayesian decision analysis perspective	18
Development, reliability, validity, and acceptability of the remote administration of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS)	17
Genetic ancestry and risk allele C9orf72 rs3849942 T for amyotrophic lateral sclerosis in Latin American populations	17
Impact of occupational categories on the incidence of amyotrophic lateral sclerosis in Sardinia Island, Italy	17
Residential exposure associations with ALS risk, survival, and phenotype: a Michigan-based case-control study	17
Single task-level, 2SD-based cutoffs for the Italian version of the Edinburgh Cognitive and Behavioral ALS screen (ECAS)	17
Amyotrophic lateral sclerosis mortality rates in Latin America and the Caribbean: a meta-analysis	17
Geographical clusters of amyotrophic lateral sclerosis and the Bradford Hill criteria	16
Theme 5 Human Cell Biology and Pathology	15
Patient engagement in research: lessons learned from CAPTURE ALS, a longitudinal observational ALS study	15
Peripheral immunity relate to disease progression and prognosis in amyotrophic lateral sclerosis	15
Correction	14
Theme 11 - Cognitive and Psychological Assessment and Support	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?	13
Assessing pulmonary function in ALS using electrical impedance tomography	13

Yield of EEG features as markers of disease severity in amyotrophic lateral sclerosis: a pilot study	13
Amyotrophic lateral sclerosis: exploring pathophysiology in the context of treatment	12
Time from amyotrophic lateral sclerosis symptom onset to key disease milestones: analysis of data from a multinational cross-sectional survey	12
Genetic variation in targets of lipid-lowering drugs and amyotrophic lateral sclerosis risk: a Mendelian randomization study	11
Self-assessment of amyotrophic lateral sclerosis functional rating scale on the patient’s smartphone proves to be non-inferior to clinic data capture	11
Theme 10 - Disease Stratification and Phenotyping of Patients	11
Portable fixed dynamometry enables home-based, reliable assessment of muscle strength in patients with amyotrophic lateral sclerosis: a pilot study	11
ALS patients with concurrent neuroinflammatory disorders; a nationwide clinical records study	11
The involvement of palliative care with neurology – a comparison of UK, Switzerland and Italy	11
Use of a new ALS specific respiratory questionnaire: the ARES score	11
Care burden and related factors among informal caregivers of patients with amyotrophic lateral sclerosis	11
Slowly progressive late-onset spinal muscular atrophy Finkel-type related to p.Pro56Ser VABP mutation in Colombia	10
Automated speech analytics in ALS: higher sensitivity of digital articulatory precision over the ALSFRS-R	10
Comparison Of King’s Clinical Staging In Multinational Amyotrophic Lateral Sclerosis Cohorts	10
Drug treatments and interactions, disease progression and quality of life in ALS patients	10
A case of juvenile-onset amyotrophic lateral sclerosis with a de novo frameshift FUS gene mutation presenting with bilateral abducens palsy	10
The contralateral co-movement test in a Chinese population with amyotrophic lateral sclerosis	10
PRECISION ALS—an integrated pan European patient data platform for ALS	10
Theme 03 - IN VITRO EXPERIMENTAL MODELS	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
Time-to-event prediction in ALS using a landmark modeling approach, using the ALS Natural History Consortium dataset	10
Exposure to electromagnetic fields does not modify neither the age of onset nor the disease progression in ALS patients	9
Platform Communications: Abstract Book – 33rd International Symposium on ALS/MND (Complete printable file)	9
Expanding the phenotype of TARDBP mutation in a Tunisian family with clinical phenotype heterogeneity	9
A novel TBK1 loss-of-function variant associated with ALS and parkinsonism phenotypes	9
A revision to the United States national ALS registry’s algorithm to improve Case-Ascertainment	9
Exploring the needs and preferences of people with amyotrophic lateral sclerosis (ALS) when making genomic testing decisions: an interview study	8
The association of theory of mind with language and visuospatial abilities in amyotrophic lateral sclerosis: a pilot study	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
Amyotrophic lateral sclerosis estimated prevalence cases from 2022 to 2030, data from the national ALS Registry	8
Inosine supplements only reach the CNS in molybdenum deficient humans and may cause astrocyte degeneration and bulbar–respiratory disease	8
Toward genetic counseling practice standards for diagnostic testing in amyotrophic lateral sclerosis and frontotemporal dementia	8
Monocyte CD14 and HLA-DR expression increases with disease duration and severity in amyotrophic lateral sclerosis	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
International network for ALS research and care (INARC)	8
The natural history of ALS: Baseline characteristics from a multicenter clinical cohort	8
Respiratory measurements, respiratory symptoms, and quality of life in ALS: results from the REVEALS study	7
Impact of mode of training and recertification on ALSFRS-R rater performance	7
IMPACT-ALS: summary of results from a European survey of people living with ALS	7
Neurofilament light chain as a biological marker for amyotrophic lateral sclerosis: a meta-analysis study	7
Percutaneous gastrostomy in amyotrophic lateral sclerosis: a review	7
Body mass index is lower in asymptomatic C9orf72 expansion carriers but not in SOD1 pathogenic variant carriers compared to gene negatives	6
Increased total daily energy expenditure in mild to moderate ALS: greater contribution from physical activity energy expenditure than hyper-metabolism	6
Cognition in the course of ALS—a meta-analysis	6
Changes in saccadic intrusions over time as an objective biomarker to follow ALS disease progression	6
Demographic changes in a large motor neuron disease cohort in Portugal: a 27 year experience	6
Why is nutrition vital for advancing ALS care and clinical research?	6
Epidemiology and economic burden of amyotrophic lateral sclerosis in the United States: a literature review	6
Theme 03 - In Vitro Experimental Models	6
Longitudinal comparison of the self-administered ALSFRS-RSE and ALSFRS-R as functional outcome measures in ALS	5

The diagnostic value of the Italian version of the Edinburgh Cognitive and Behavioral ALS Screen (ECAS)	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 cholesterol depleting agent, (2-Hydroxypropyl)-ß-cyclodextrin, does not affect disease progression in SOD1 ^G93A mice	5
Theme 05 - HUMAN CELL BIOLOGY AND PATHOLOGY (including iPSC studies)	5
Association of UNC13A with increased amyotrophic lateral sclerosis risk, bulbar onset, and lower motor neuron involvement in a Norwegian ALS cohort	5
Primary lateral sclerosis natural history study – planning, designing, and early enrollment	5
Serum heat shock protein concentrations are not associated with amyotrophic lateral sclerosis risk or survival in three European populations	5
Theme 12 - Clinical Management and Support	5
A speech-based prognostic model for dysarthria progression in ALS	5
Apathy in amyotrophic lateral sclerosis: systematic review and meta-analysis of frequency, correlates, and outcomes	5
ALSUntangled #65: glucocorticoid corticosteroids	5
ALSUntangled # 69: astaxanthin	5
MiNDAUS partnership: a roadmap for the cure and management of motor Neurone disease	5
Urine specific gravity to identify and predict hydration need in ALS	5
How the prospect of a clinical trial impacts decision-making for predictive genetic testing in ALS	5
Letter to Editor	5
Medical therapies for amyotrophic lateral sclerosis-related respiratory decline: an appraisal of needs, opportunities and obstacles	4
Frequency of C9orf72 and SOD1 mutations in 302 sporadic ALS patients from three German ALS centers	4
Verbal expressive language minimally affected in non-demented people living with amyotrophic lateral sclerosis	4
A verb-naming test accurately detects cognitive changes in ALS	4
Theme 01 - Epidemiology and Informatics	4
Theme 05 - Human Cell Biology and Pathology (including iPSC studies)	4
Theme 02 - Genetics and Genomics	4
Harmonized standard operating procedures for administering the ALS functional rating scale-revised	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
Study of “ALS reversals”: LifeTime environmental exposures (StARLiTE)	4
Incidence of amyotrophic lateral sclerosis in the United States, 2014–2016	4
HLA and amyotrophic lateral sclerosis: a systematic review and meta-analysis	4
Theme 10 - DISEASE STRATIFICATION AND PHENOTYPING OF PATIENTS	4
The expanding clinical and genetic spectrum of alsin-related disorders: the first cohort of Brazilian patients	4
Patients’ choices regarding ventilatory support affect opioid use in amyotrophic lateral sclerosis	4
Clinically meaningful change: evaluation of the Rasch-built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS) and the ALSFRS-R	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
ALSUntangled #71: Nuedexta	4
Theme 08 - Clinical Imaging and Electrophysiology	4
Acceptability and potential benefit of a self-compassion intervention for people living with amyotrophic lateral sclerosis: a mixed methods pilot study	4
Correction	4
The Beaumont behavioral intervention in a Chinese amyotrophic lateral sclerosis cohort	4
Relationship between quantitative strength and functional outcomes in the phase 2 FORTITUDE-ALS trial	4
ALSUntangled #61: melatonin	4
Current practices in the nutrition management of people with amyotrophic lateral sclerosis (ALS): a survey of U.S. ALS care teams	4
In memoriam Ettore Beghi, 1947–2022	4
Prevalence of depression in amyotrophic lateral sclerosis/motor neuron disease: multi-attribute ascertainment and trajectories over 30 months	4
Arterial blood gas analysis: base excess and carbonate are predictive of noninvasive ventilation adaptation and survival in amyotrophic lateral sclerosis	4