OOIR: Observatory of International Research

Papers

(The TQCC of Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration is 5. 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 2022-08-01 to 2026-08-01.)

Article	Citations
Incorporating patient preferences and burden-of-disease in evaluating ALS drug candidate AMX0035: a Bayesian decision analysis perspective	42
Correction	34
Clinical and demographical characteristics in a cohort of MND patients treated with riluzole. Differences between tablets and oral suspension	32
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)	27
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	27
A novel heterozygous GRN deletion (c.302_315del) associated with behavioral variant frontotemporal dementia	27
The amyotrophic lateral sclerosis-health index (ALS-HI): development and evaluation of a novel outcome measure	25
Single task-level, 2SD-based cutoffs for the Italian version of the Edinburgh Cognitive and Behavioral ALS screen (ECAS)	25
Validity and reliability measures of the Swedish Karolinska version of the Edinburgh Cognitive and Behavioral ALS Screen (SK-ECAS)	24
Theme 5 Human Cell Biology and Pathology	21
Rapid classification of a novel ALS-causing I149S variant in superoxide dismutase-1	21
Challenges in medical care for amyotrophic lateral sclerosis: a survey of physicians from Republic of Bashkortostan (Russia), Belarus, and Kazakhstan	20
Emerging strategies for interpreting variants of uncertain significance (VUS) in amyotrophic lateral sclerosis	20
Theme 7 Pre-Clinical Therapeutic Strategies	19
Genetic ancestry and risk allele C9orf72 rs3849942 T for amyotrophic lateral sclerosis in Latin American populations	18
Peripheral immunity relate to disease progression and prognosis in amyotrophic lateral sclerosis	18
Epidemiology, clinical features, and management of amyotrophic lateral sclerosis in the neurology department of the Bogodogo University Hospital in Ouagadougou, Burkina Faso	17
Residential exposure associations with ALS risk, survival, and phenotype: a Michigan-based case-control study	17
Impact of occupational categories on the incidence of amyotrophic lateral sclerosis in Sardinia Island, Italy	15
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	15
Noninvasive assessment of cardiovascular autonomic reflexes in amyotrophic lateral sclerosis: a systematic review	15
Theme 11 - Cognitive and Psychological Assessment and Support	14
Assessing pulmonary function in ALS using electrical impedance tomography	14
Amyotrophic lateral sclerosis: exploring pathophysiology in the context of treatment	14

Patient engagement in research: lessons learned from CAPTURE ALS, a longitudinal observational ALS study	14
Theme 10 - Disease Stratification and Phenotyping of Patients	14
Correction	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
Portable fixed dynamometry enables home-based, reliable assessment of muscle strength in patients with amyotrophic lateral sclerosis: a pilot study	12
ALS motor phenotypes: a revised ‘OPM’ classification	12
Genetic variation in targets of lipid-lowering drugs and amyotrophic lateral sclerosis risk: a Mendelian randomization study	12
Yield of EEG features as markers of disease severity in amyotrophic lateral sclerosis: a pilot study	12
Slowly progressive late-onset spinal muscular atrophy Finkel-type related to p.Pro56Ser VABP mutation in Colombia	11
Feature selection using metaheuristics to predict annual amyotrophic lateral sclerosis progression	11
The involvement of palliative care with neurology – a comparison of UK, Switzerland and Italy	11
Time from amyotrophic lateral sclerosis symptom onset to key disease milestones: analysis of data from a multinational cross-sectional survey	11
Automated speech analytics in ALS: higher sensitivity of digital articulatory precision over the ALSFRS-R	11
Limited data capture on reproductive medicine use in amyotrophic lateral sclerosis: implications for monitoring access	11
The contralateral co-movement test in a Chinese population with amyotrophic lateral sclerosis	11
Time-to-event prediction in ALS using a landmark modeling approach, using the ALS Natural History Consortium dataset	11
Exploring the needs and preferences of people with amyotrophic lateral sclerosis (ALS) when making genomic testing decisions: an interview study	10
Pridopidine treatment in ALS: subgroup analyses from the HEALEY ALS Platform trial	10
Platform Communications: Abstract Book – 33rd International Symposium on ALS/MND (Complete printable file)	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
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	9
The natural history of ALS: Baseline characteristics from a multicenter clinical cohort	9
Self-reported initiation apathy is related to worse quality of life in amyotrophic lateral sclerosis	9
International network for ALS research and care (INARC)	9
Incidence of MND in Canterbury, New Zealand and impact of a key worker role on hospital admissions	9
IMPACT-ALS: summary of results from a European survey of people living with ALS	9
Neuropsychological assessment practices in PRECISION-ALS: challenges and opportunities for harmonization	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
Understanding psychological adjustment in patients with amyotrophic lateral sclerosis and their caregivers: a scoping review	9
Exposure to electromagnetic fields does not modify neither the age of onset nor the disease progression in ALS patients	9
A novel TBK1 loss-of-function variant associated with ALS and parkinsonism phenotypes	9
PRECISION ALS—an integrated pan European patient data platform for ALS	8
Amyotrophic lateral sclerosis estimated prevalence cases from 2022 to 2030, data from the national ALS Registry	8
ALSUntangled #80: ISRIB (Integrated stress response InhiBitor)	8
Impact of mode of training and recertification on ALSFRS-R rater performance	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
Respiratory measurements, respiratory symptoms, and quality of life in ALS: results from the REVEALS study	8
The genetics of autosomal recessive ALS: a review of the common forms and their phenotypes	8
A revision to the United States national ALS registry’s algorithm to improve Case-Ascertainment	8
Dextromethorphan/quinidine (DMQ) for reducing bulbar symptoms in amyotrophic lateral sclerosis – assessment of treatment experience in a multicenter study	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	7
Reduced penetrance in genetic ALS/FTD spectrum disorders: implications for genetic counseling, predictive testing and treatment	7
Quantitative susceptibility mapping for investigating brain iron deposits in amyotrophic lateral sclerosis: correlations with clinical phenotype and disease progression	7
Demographic changes in a large motor neuron disease cohort in Portugal: a 27 year experience	7
The cholesterol depleting agent, (2-Hydroxypropyl)-ß-cyclodextrin, does not affect disease progression in SOD1 ^G93A mice	7
Why is nutrition vital for advancing ALS care and clinical research?	7
Epidemiology and economic burden of amyotrophic lateral sclerosis in the United States: a literature review	7
How the prospect of a clinical trial impacts decision-making for predictive genetic testing in ALS	7

Primary lateral sclerosis natural history study – planning, designing, and early enrollment	7
Theme 10 Disease Stratification and Phenotyping of Patients	7
Theme 03 - In Vitro Experimental Models	7
ALSUntangled # 69: astaxanthin	7
Body mass index is lower in asymptomatic C9orf72 expansion carriers but not in SOD1 pathogenic variant car	7
Evolving 10-year epidemiological landscape of amyotrophic lateral sclerosis (ALS) in Ceará, Brazil	7
ALSUntangled #79: alpha-lipoic acid	7
Association of UNC13A with increased amyotrophic lateral sclerosis risk, bulbar onset, and lower motor neuron involvement in a Norwegian ALS cohort	6
Serum heat shock protein concentrations are not associated with amyotrophic lateral sclerosis risk or survival in three European populations	6
SOD1 mutations in Taiwanese ALS patients: Clinical characteristics, frequency, and a p.T138R founder effect	6
Acceptability and potential benefit of a self-compassion intervention for people living with amyotrophic lateral sclerosis: a mixed methods pilot study	6
Frequency of C9orf72 and SOD1 mutations in 302 sporadic ALS patients from three German ALS centers	6
Amyotrophic lateral sclerosis in Colombia: a population-based study of incidence and socioeconomic determinants	6
Letter to Editor	6
ALSUntangled #65: glucocorticoid corticosteroids	6
Translation and validation of the Arabic version of the amyotrophic lateral sclerosis assessment questionnaire (ALSAQ40-AR)	6
Theme 08 - Clinical Imaging and Electrophysiology	6
Correction	6
Longitudinal comparison of the self-administered ALSFRS-RSE and ALSFRS-R as functional outcome measures in ALS	6
A speech-based prognostic model for dysarthria progression in ALS	6
Beyond outcomes: patients’ lived experience of exoskeleton-assisted gait training in amyotrophic lateral sclerosis	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
Theme 9 Clinical Trials and Trial Design	6
The Beaumont behavioral intervention in a Chinese amyotrophic lateral sclerosis cohort	6
Verbal expressive language minimally affected in non-demented people living with amyotrophic lateral sclerosis	6
Strengthening care and research in ALS in Southeast Asia: a call for action	6
Theme 12 - Clinical Management and Support	6
Patients’ choices regarding ventilatory support affect opioid use in amyotrophic lateral sclerosis	5
Theme 01 - Epidemiology and Informatics	5
Serum NfL, but not GFAP, differentiates primary lateral sclerosis from adrenomyeloneuropathy and hereditary spastic paraplegia type 4	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
Behavioral subtypes impact prognosis and survival in amyotrophic lateral sclerosis: a clustering-based approach	5
Harmonized standard operating procedures for administering the ALS functional rating scale-revised	5
Theme 3 In Vitro Experimental Models	5
Prevalence of depression in amyotrophic lateral sclerosis/motor neuron disease: multi-attribute ascertainment and trajectories over 30 months	5
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	5
Theme 05 - Human Cell Biology and Pathology (including iPSC studies)	5
ALSUntangled #71: Nuedexta	5
Italian reference values and brain correlates of verbal fluency index – vs standard verbal fluency test – to assess executive dysfunction in ALS	5
Current practices in the nutrition management of people with amyotrophic lateral sclerosis (ALS): a survey of U.S. ALS care teams	5
Occupational lead exposure and amyotrophic lateral sclerosis survival in the Danish National Patient Registry	5
In memoriam Ettore Beghi, 1947–2022	5
Molecular and genetic landscape of amyotrophic lateral sclerosis in Latin America: a scoping review of pathogenic hypotheses and ancestral heterogeneity	5
Amyotrophic lateral sclerosis in Saudi Arabia: a multicenter descriptive study	5
Relationship between quantitative strength and functional outcomes in the phase 2 FORTITUDE-ALS trial	5
Four decades of ALS care: a retrospective study of epidemiology, clinical course and changes in management	5
Theme 02 - Genetics and Genomics	5
Clinical trajectories of genetic variants in ALS: a European observational study within PRECISION-ALS	5
Neutrophil-to-lymphocyte ratio at diagnosis as a biomarker for survival of amyotrophic lateral sclerosis	5
Mapping the natural history of amyotrophic lateral sclerosis: time-to-event analysis of clinical milestones in the pan-European, population-based PRECISION-ALS cohort	5