OOIR: Observatory of International Research

Papers

(The median citation count of Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration is 2. 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 2020-03-01 to 2024-03-01.)

Article	Citations
Real-world evidence of riluzole effectiveness in treating amyotrophic lateral sclerosis	64
The human gut microbiota in people with amyotrophic lateral sclerosis	48
A Phase 2, Double-Blind, Randomized, Dose-Ranging Trial Of Reldesemtiv In Patients With ALS	42
Decline of cognitive and behavioral functions in amyotrophic lateral sclerosis: a longitudinal study	40
An overview of screening instruments for cognition and behavior in patients with ALS: selecting the appropriate tool for clinical practice	34
Telemedicine is a useful tool to deliver care to patients with Amyotrophic Lateral Sclerosis during COVID-19 pandemic: results from Southern Italy	31
Focus on the heterogeneity of amyotrophic lateral sclerosis	31
Gut microbiome differences between amyotrophic lateral sclerosis patients and spouse controls	30
An old friend who has overstayed their welcome: the ALSFRS-R total score as primary endpoint for ALS clinical trials	29
Increased risk and early onset of ALS in professional players from Italian Soccer Teams	28
Progression and survival of patients with motor neuron disease relative to their fecal microbiota	27
Understanding the needs of people with ALS: a national survey of patients and caregivers	27
Blended psychosocial support for partners of patients with ALS and PMA: results of a randomized controlled trial	26
Telemedicine for patients with amyotrophic lateral sclerosis during COVID-19 pandemic: an Italian ALS referral center experience	23
TDP-43 pathology in primary lateral sclerosis	20
Neuroimaging in primary lateral sclerosis	20
Prevalence of amyotrophic lateral sclerosis (ALS), United States, 2016	19
Plasma creatinine and oxidative stress biomarkers in amyotrophic lateral sclerosis	19
Incidence of amyotrophic lateral sclerosis in the United States, 2014–2016	19
Grief, depression, and anxiety in bereaved caregivers of people with motor neurone disease: a population-based national study	18
The involvement of regulatory T cells in amyotrophic lateral sclerosis and their therapeutic potential	18
TRICALS: creating a highway toward a cure	17
Prevalence of amyotrophic lateral sclerosis in the United States using established and novel methodologies, 2017	17
Disorders of sleep and wakefulness in amyotrophic lateral sclerosis (ALS): a systematic review	16
Interleukin 6 (IL6) level is a biomarker for functional disease progression within IL6R³⁵⁸Ala variant groups in amyotrophic lateral sclerosis patients	16

Differentiating needs of informal caregivers of individuals with ALS across the caregiving course: a systematic review	15
A multi-center study of neurofilament assay reliability and inter-laboratory variability	15
Amyotrophic lateral sclerosis caregiver burden and patients’ quality of life during COVID-19 pandemic	15
Who cares for the bereaved? A national survey of family caregivers of people with motor neurone disease	15
Associations between autoimmune diseases and amyotrophic lateral sclerosis: a register-based study	14
Chitotriosidase as biomarker for early stage amyotrophic lateral sclerosis: a multicenter study	14
Prevalence of pain in amyotrophic lateral sclerosis: a systematic review and meta-analysis	14
Psychological interventions for people with motor neuron disease: a scoping review	14
Genetics of primary lateral sclerosis	13
Defining cognitive impairment in amyotrophic lateral sclerosis: an evaluation of empirical approaches	13
Keratinous biomarker of mercury exposure associated with amyotrophic lateral sclerosis risk in a nationwide U.S. study	12
Trauma and amyotrophic lateral sclerosis: a systematic review and meta-analysis	12
Estimation of forced vital capacity using speech acoustics in patients with ALS	12
The use of biotelemetry to explore disease progression markers in amyotrophic lateral sclerosis	12
Increased synthesis of pro-inflammatory cytokines in C9ORF72 patients	12
An online non-meditative mindfulness intervention for people with ALS and their caregivers: a randomized controlled trial	12
A prospective study on split-hand index as a biomarker for the diagnosis of amyotrophic lateral sclerosis	12
Pre-diagnostic plasma lipid levels and the risk of amyotrophic lateral sclerosis	11
Cognitive reserve is associated with altered clinical expression in amyotrophic lateral sclerosis	11
Increased worsening of amyotrophic lateral sclerosis patients during Covid-19-related lockdown in France	11
The clinical spectrum of primary lateral sclerosis	11
Pulmonary function decline in amyotrophic lateral sclerosis	11
Genetics of frontotemporal dementia in China	11
Simultaneous ALS and SCA2 associated with an intermediate-length ATXN2 CAG-repeat expansion	11
Muscle MRI in motor neuron diseases: a systematic review	11
UK case control study of smoking and risk of amyotrophic lateral sclerosis	10
Motor Neuron Disease Register for England, Wales and Northern Ireland—an analysis of incidence in England	10
Apathy in amyotrophic lateral sclerosis: systematic review and meta-analysis of frequency, correlates, and outcomes	10
Italian adaptation of the Beaumont Behavioral Inventory (BBI): psychometric properties and clinical usability	10
Urinary p75^ECD levels in patients with amyotrophic lateral sclerosis: a meta-analysis	10
Neurophysiological features of primary lateral sclerosis	10
The reading the mind in the eyes test short form (A & B): validation and outcomes in an amyotrophic lateral sclerosis cohort	10
The diagnostic value of the Italian version of the Edinburgh Cognitive and Behavioral ALS Screen (ECAS)	10
Resting state functional MRI brain signatures of fast disease progression in amyotrophic lateral sclerosis: a retrospective study	9
Epidemiological and genetic features of amyotrophic lateral sclerosis in Latin America and the Caribbean: a systematic review	9
Genetic testing for amyotrophic lateral sclerosis in Canada – an assessment of current practices	9
Natural history and clinical features of ALS in Malaysia	9
Neuropathology of primary lateral sclerosis	9
Novel serum autoantibodies against ß-actin (ACTB) in amyotrophic lateral sclerosis	9
Case-control study in ALS using the National ALS Registry: lead and agricultural chemicals are potential risk factors	9
Novel FUS mutation Y526F causing rapidly progressive familial amyotrophic lateral sclerosis	9
Patient reported outcomes in ALS: characteristics of the self-entry ALS Functional Rating Scale-revised and the Activities-specific Balance Confidence Scale	9
Neurofilament light chain as a biological marker for amyotrophic lateral sclerosis: a meta-analysis study	8
A de novo c.1509dupA:p.R503fs mutation of FUS: report of a girl with sporadic juvenile amyotrophic lateral sclerosis	8
Human endogenous retrovirus K (HERV-K) env in neuronal extracellular vesicles: a new biomarker of motor neuron disease	8
Mutations of DNAJC7 are rare in Chinese amyotrophic lateral sclerosis patients	8
MRI biomarkers for memory-related impairment in amyotrophic lateral sclerosis: a systematic review	8
Dyslipidemia in patients with amyotrophic lateral sclerosis – a case control retrospective study	8
Clinical characteristics in young-adult ALS – results from a Portuguese cohort study	8
Arterial blood gas analysis: base excess and carbonate are predictive of noninvasive ventilation adaptation and survival in amyotrophic lateral sclerosis	8

Chorea is a pleiotropic clinical feature of mutated fused-in-sarcoma in amyotrophic lateral sclerosis	8
Levels of clusterin, CD5L, ficolin-3, and gelsolin in ALS patients and controls	8
Reliable change indices for the Italian Edinburgh Cognitive and Behavioral ALS Screen (ECAS)	7
The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) in frontotemporal dementia	7
The epidemiology of amyotrophic lateral sclerosis in Moscow (Russia)	7
Cardiac troponin T is elevated and increases longitudinally in ALS patients	7
The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) for Japanese ALS and FTD patients	7
Percutaneous gastrostomy in amyotrophic lateral sclerosis: a review	7
Genotype-phenotype correlations of KIF5A stalk domain variants	7
History of vigorous leisure-time physical activity and early onset amyotrophic lateral sclerosis (ALS), data from the national ALS registry: 2010–2018	7
Cerebellar degeneration in primary lateral sclerosis: an under-recognized facet of PLS	7
Analysis of incidence of motor neuron disease in England 1998–2019: use of three linked datasets	7
Dysfunction of the left angular gyrus may be associated with writing errors in ALS	7
Arabic adaptation and validation of the revised amyotrophic lateral sclerosis functional rating scale (ALSFRS-R): Egyptian study	7
Motor neuron disease beginning with frontotemporal dementia: clinical features and progression	7
Validation of the Italian version of self-administered ALSFRS-R scale	7
Gastrointestinal biopsies and amyotrophic lateral sclerosis – results from a cohort study of 1.1 million individuals	7
Persian adaptation of Edinburgh Cognitive and Behavioural Screen (ECAS)	7
Improvements in the multidisciplinary care are beneficial for survival in amyotrophic lateral sclerosis (ALS): experience from a tertiary ALS center	7
Mouth occlusion pressure at 100ms (P0.1) as a respiratory biomarker in amyotrophic lateral sclerosis	7
Incidence of amyotrophic lateral sclerosis in Sardinia, Italy: age–sex interaction and spatial–temporal variability	7
The path to diagnosis in ALS: delay, referrals, alternate diagnoses, and clinical progression	7
Problem-focused coping underlying lower caregiver burden in ALS-FTD: implications for caregiver intervention	7
Elevated plasma levels of D-serine in some patients with amyotrophic lateral sclerosis	6
Using the ALSFRS-R in multicentre clinical trials for amyotrophic lateral sclerosis: potential limitations in current standard operating procedures	6
Respiratory onset in amyotrophic lateral sclerosis: clinical features and spreading pattern	6
A novel p.N66T mutation in exon 3 of the SOD1 gene: report of two families of ALS patients with early cognitive impairment	6
VRK1 variants in two Portuguese unrelated patients with childhood-onset motor neuron disease	6
Factors associated with Noninvasive ventilation compliance in patients with ALS/MND	6
Impact of the covid-19 pandemic on amyotrophic lateral sclerosis care in the UK	6
Clinical care and therapeutic trials in PLS	6
Juvenile amyotrophic lateral sclerosis associated with biallelic c.757delG mutation of sorbitol dehydrogenase gene	6
Three different short-interval intracortical inhibition methods in early diagnosis of amyotrophic lateral sclerosis	6
ALSUntangled #64: butyrates	6
Tracheostomy ventilation in motor neurone disease: a snapshot of UK practice	6
Development and validation of a machine-learning ALS survival model lacking vital capacity (VC-Free) for use in clinical trials during the COVID-19 pandemic	6
The future of ALS diagnosis and staging: where do we go from here?	6
What is amyotrophic lateral sclerosis prevalence?	6
Juvenile amyotrophic lateral sclerosis with complex phenotypes associated with novel SYNE1 mutations	6
Comparison of the ability of the King’s and MiToS staging systems to predict disease progression and survival in amyotrophic lateral sclerosis	6
Characterization of the amyotrophic lateral sclerosis-linked P56S mutation of the VAPB gene in Southern Brazil	6
Clinical features and incidence trends of amyotrophic lateral sclerosis in Navarre, Spain, 2007–2018: a population-based study	6
ALS patients with concurrent neuroinflammatory disorders; a nationwide clinical records study	5
Delivery of nutritional management services to people with amyotrophic lateral sclerosis (ALS)	5
Therapeutic effect of a novel curcumin derivative GT863 on a mouse model of amyotrophic lateral sclerosis	5
Amyotrophic lateral sclerosis in the Faroe Islands – a genealogical study	5
Preface: promoting research in PLS: current knowledge and future challenges	5
Occupation and amyotrophic lateral sclerosis risk: a case-control study in the isolated island population of Malta	5
The NEALS primary lateral sclerosis registry	5
A potential upper motor neuron measure of bulbar involvement in amyotrophic lateral sclerosis using jaw muscle coherence	5
Environmental risk factors for amyotrophic lateral sclerosis: a case–control study in Canada and France	5
Measuring quality of life in ALS/MND: validation of the WHOQOL-BREF	5
Validity of death certificates in the identification of cases of amyotrophic lateral sclerosis (ALS) in the Limousin region, France. A population-based study	5
Putative founder effect in the Polish, Iranian and United States populations for the L144S SOD1 mutation associated with slowly uniform phenotype of amyotrophic lateral sclerosis	5
Concurrent sodium channelopathies and amyotrophic lateral sclerosis supports shared pathogenesis	5
The distinct manifestation of young-onset amyotrophic lateral sclerosis in China	5
ALSUntangled 55: vitamin E (α-tocopherol)	5
Healthcare provision in amyotrophic lateral sclerosis: procedures, queries and pitfalls in Germany and Poland	4
Monocyte CD14 and HLA-DR expression increases with disease duration and severity 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
Diagnostic delay among ALS patients: Egyptian study	4
Heterogeneity of predictors of nocturnal hypoventilation in amyotrophic lateral sclerosis	4
Challenges and opportunities for Multi-National Investigator-Initiated clinical trials for ALS: European and United States collaborations	4
Clinicoradiological features in amyotrophic lateral sclerosis patients with olfactory dysfunction	4
Epidemiology and economic burden of amyotrophic lateral sclerosis in the United States: a literature review	4
The Latin American Epidemiology Network for ALS (Laenals)	4
Trends in survival of ALS from a population-based registry	4
A placebo-controlled trial to investigate the safety and efficacy of Penicillin G/Hydrocortisone in patients with ALS (PHALS trial)	4
Characteristics of pain and the burden it causes in patients with amyotrophic lateral sclerosis – a longitudinal study	4
Fatigue and anxiety mediate the effect of dyspnea on quality of life in amyotrophic lateral sclerosis	4
Longitudinal evaluation of upper motor neuron burden scales in primary lateral sclerosis	4
Genetic and epigenetic disease modifiers in an Italian C9orf72 family expressing ALS, FTD or PD clinical phenotypes	4
Progression of cognitive and behavioral disturbances in motor neuron diseases assessed using standard and computer-based batteries	4
A revision to the United States national ALS registry’s algorithm to improve Case-Ascertainment	4
Survival and epidemiology of amyotrophic lateral sclerosis (ALS) cases in the Chicago and Detroit metropolitan cohort: incident cases 2009–2011 and survival through 2018	4
Degeneration of gray and white matter differs between hypometabolic and hypermetabolic brain regions in a patient with ALS-FTD: a longitudinal MRI − PET multimodal study	4
Yield of EEG features as markers of disease severity in amyotrophic lateral sclerosis: a pilot study	4
Remote digital assessment of amyotrophic lateral sclerosis functional rating scale – a multicenter observational study	4
Serum creatinine levels in patients with amyotrophic lateral sclerosis: a systematic review and meta-analysis	4
Single breath counting is an effective screening tool for forced vital capacity in ALS	3

Clinical trials in pediatric ALS: a TRICALS feasibility study	3
A case of juvenile-onset amyotrophic lateral sclerosis with a de novo frameshift FUS gene mutation presenting with bilateral abducens palsy	3
Care burden and related factors among informal caregivers of patients with amyotrophic lateral sclerosis	3
The quality of life and depression in primary caregivers of patients with amyotrophic lateral sclerosis is affected by patient-related and culture-specific conditions	3
Association of apolipoprotein E ε4 allele and amyotrophic lateral sclerosis in Chinese population	3
Amyotrophic lateral sclerosis (ALS) health charities are central to ALS care: perspectives of Canadians affected by ALS	3
MiNDAUS partnership: a roadmap for the cure and management of motor Neurone disease	3
Does limited EMG denervation in early primary lateral sclerosis predict amyotrophic lateral sclerosis?	3
NEK1 and GRN mutations coexist in a sporadic Chinese Hui descent ALS patient	3
The patient's perspective of remote respiratory assessments during the COVID-19 pandemic	3
Measuring disability in amyotrophic lateral sclerosis/motor neuron disease: the WHODAS 2.0-36, WHODAS 2.0-32, and WHODAS 2.0-12	3
Prevalence of depression in amyotrophic lateral sclerosis/motor neuron disease: multi-attribute ascertainment and trajectories over 30 months	3
Occupational history associates with ALS survival and onset segment	3
The importance of maintaining the same order of performance of lung function and SNIP tests in patients with amyotrophic lateral sclerosis	3
Facial onset amyotrophic lateral sclerosis with K3E variant in the Cu/Zn superoxide dismutase gene	3
Geographical clusters of amyotrophic lateral sclerosis and the Bradford Hill criteria	3
Dying from ALS in Sweden: clinical status, setting, and symptoms	3
Predicting dysphagia onset in patients with ALS: the ALS dysphagia risk score	3
Combination of ciprofloxacin/celecoxib as a novel therapeutic strategy for ALS	3
Evidence for generalizability of edaravone efficacy using a novel machine learning risk-based subgroup analysis tool	3
Phrenic nerve study as outcome in clinical trials for amyotrophic lateral sclerosis	3
Clinical testing panels for ALS: global distribution, consistency, and challenges	3
The association of theory of mind with language and visuospatial abilities in amyotrophic lateral sclerosis: a pilot study	3
Electrical impedance myography (EIM) in a natural history study of C9ORF72 mutation carriers	3
A Brazilian multicentre study on the clinical and epidemiological profiles of 1116 patients with amyotrophic lateral sclerosis and its phenotypic variants	3
FDG-PET shows weak correlation between focal motor weakness and brain metabolic alterations in ALS	3
Amyotrophic lateral sclerosis in Antalya, Turkey. A prospective study, 2016–2018	3
Cognition in the course of ALS—a meta-analysis	3
An identical DCTN1 mutation in two Chinese siblings manifest as dHMN and ALS respectively: a case report	3
CAPTURE ALS: the comprehensive analysis platform to understand, remedy and eliminate ALS	3
Corticospinal tract and motor cortex degeneration in pure hereditary spastic paraparesis type 4 (SPG4)	3
Total serum immunoglobulin A in ALS	3
Better understanding the neurobiology of primary lateral sclerosis	3
Theme 12 - CLINICAL MANAGEMENT, SUPPORT AND INFORMATION	2
Compound heterozygous P67S/D91A SOD1 mutations in an ALS family with apparently sporadic case	2
Study of “ALS reversals”: LifeTime environmental exposures (StARLiTE)	2
Caregivers’ divergent perspectives on patients’ well-being and attitudes towards hastened death in Germany, Poland and Sweden	2
Transcranial magnetic stimulation to monitor disease progression in ALS: a review	2
Variability of clinical syndromes and cerebral glucose metabolism in symptomatic frontotemporal lobar degeneration associated with progranulin mutations	2
ALSUntangled #63: ketogenic diets	2
Recruitment of population-based controls for ALS cases from the National ALS Registry	2
Compensatory metabolic and central respiratory drive mechanisms in ALS	2
Prescription and acceptance of durable medical equipment in FORTITUDE-ALS, a study of reldesemtiv in ALS: post hoc analyses of a randomized, double-blind, placebo-controlled clinical trial	2
Adaptation and norming of the Edinburgh Cognitive and behavioural amyotrophic lateral sclerosis screen (ECAS) for three language groups in South Africa	2
Mixed neuropathology in frontotemporal lobar degeneration	2
ALSUntangled #67: rituximab	2
Frequency of C9orf72 and SOD1 mutations in 302 sporadic ALS patients from three German ALS centers	2
Measuring disease progression in primary lateral sclerosis	2
The utility of ALS staging systems in a multi-ethnic patient cohort	2
Association between vascular risk factors and cognitive impairment in amyotrophic lateral sclerosis: a case-control study	2
Early juvenile reading epilepsy and later frontotemporal dementia (FTD): expanding the clinical phenotype of C9ORF72 mutation?	2
A survey of current practice in genetic testing in amyotrophic lateral sclerosis in the UK and Republic of Ireland: implications for future planning	2
Fit for purpose? A cross-sectional study to evaluate the acceptability and usability of HeadUp, a novel neck support collar for neurological neck weakness	2
Previous psychiatric disorders in the multistep hypothesis of amyotrophic lateral sclerosis: a South Korean population study	2
Italian reference values and brain correlates of verbal fluency index – vs standard verbal fluency test – to assess executive dysfunction in ALS	2
Investigating TBP CAG/CAA trinucleotide repeat expansions in a Taiwanese cohort with ALS	2
Theme 09 - CLINICAL TRIALS AND TRIAL DESIGN	2
The expanding clinical and genetic spectrum of alsin-related disorders: the first cohort of Brazilian patients	2
Correlation between oculometric measures and clinical assessment in ALS patients participating in a phase IIb clinical drug trial	2
MiToS and King’s staging as clinical outcome measures in ALS: a retrospective analysis of the FORTITUDE-ALS trial	2
The relationship between ventilatory function and cognitive and behavioral impairment in ALS	2
The incidence and prevalence of motor neurone disease in South Australia	2
Intuitive Staging Correlates With King’s Clinical Stage	2
An exon 5 mutation (c.425G>C, p.Gly141Ala) in the SOD1 gene in a Chinese family associated with incomplete penetrance	2
Validation of the Italian version of the Rasch-Built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS) administered to patients and their caregivers	2
Effects of MSC-NTF cells on T and B regulatory cell function in ALS	2
Prevalence of C9orf72 hexanucleotide repeat expansion in Greek patients with sporadic ALS	2
Correlations between measures of ALS respiratory function: is there an alternative to FVC?	2
Platform Communications: Abstract Book – 33rd International Symposium on ALS/MND (Complete printable file)	2
Occupational lead exposure and survival with amyotrophic lateral sclerosis	2
Current practices and barriers in gastrostomy indication in amyotrophic lateral sclerosis: a survey of ALS care teams in The Netherlands	2