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 2020-11-01 to 2024-11-01.)

Article	Citations
The human gut microbiota in people with amyotrophic lateral sclerosis	60
Gut microbiome differences between amyotrophic lateral sclerosis patients and spouse controls	37
An old friend who has overstayed their welcome: the ALSFRS-R total score as primary endpoint for ALS clinical trials	36
Prevalence of amyotrophic lateral sclerosis in the United States using established and novel methodologies, 2017	32
Incidence of amyotrophic lateral sclerosis in the United States, 2014–2016	28
Prevalence of amyotrophic lateral sclerosis (ALS), United States, 2016	26
Defining cognitive impairment in amyotrophic lateral sclerosis: an evaluation of empirical approaches	23
An online non-meditative mindfulness intervention for people with ALS and their caregivers: a randomized controlled trial	22
Neuroimaging in primary lateral sclerosis	21
Disorders of sleep and wakefulness in amyotrophic lateral sclerosis (ALS): a systematic review	20
Prevalence of pain in amyotrophic lateral sclerosis: a systematic review and meta-analysis	19
Pulmonary function decline in amyotrophic lateral sclerosis	17
Amyotrophic lateral sclerosis caregiver burden and patients’ quality of life during COVID-19 pandemic	16
Patient reported outcomes in ALS: characteristics of the self-entry ALS Functional Rating Scale-revised and the Activities-specific Balance Confidence Scale	16
Associations between autoimmune diseases and amyotrophic lateral sclerosis: a register-based study	16
Chitotriosidase as biomarker for early stage amyotrophic lateral sclerosis: a multicenter study	15
Apathy in amyotrophic lateral sclerosis: systematic review and meta-analysis of frequency, correlates, and outcomes	15
Genetics of frontotemporal dementia in China	15
Estimation of forced vital capacity using speech acoustics in patients with ALS	15
Percutaneous gastrostomy in amyotrophic lateral sclerosis: a review	14
Increased synthesis of pro-inflammatory cytokines in C9ORF72 patients	14
Genetics of primary lateral sclerosis	14
Simultaneous ALS and SCA2 associated with an intermediate-length ATXN2 CAG-repeat expansion	14
Trauma and amyotrophic lateral sclerosis: a systematic review and meta-analysis	14
MRI biomarkers for memory-related impairment in amyotrophic lateral sclerosis: a systematic review	14

Cognitive reserve is associated with altered clinical expression in amyotrophic lateral sclerosis	14
Muscle MRI in motor neuron diseases: a systematic review	13
The clinical spectrum of primary lateral sclerosis	13
The diagnostic value of the Italian version of the Edinburgh Cognitive and Behavioral ALS Screen (ECAS)	13
Italian adaptation of the Beaumont Behavioral Inventory (BBI): psychometric properties and clinical usability	12
Neuropathology of primary lateral sclerosis	12
The path to diagnosis in ALS: delay, referrals, alternate diagnoses, and clinical progression	12
Genetic testing for amyotrophic lateral sclerosis in Canada – an assessment of current practices	12
Three different short-interval intracortical inhibition methods in early diagnosis of amyotrophic lateral sclerosis	12
Urinary p75^ECD levels in patients with amyotrophic lateral sclerosis: a meta-analysis	12
Human endogenous retrovirus K (HERV-K) env in neuronal extracellular vesicles: a new biomarker of motor neuron disease	11
Incidence of amyotrophic lateral sclerosis in Sardinia, Italy: age–sex interaction and spatial–temporal variability	11
Novel serum autoantibodies against ß-actin (ACTB) in amyotrophic lateral sclerosis	11
Cardiac troponin T is elevated and increases longitudinally in ALS patients	11
Neurophysiological features of primary lateral sclerosis	11
Remote digital assessment of amyotrophic lateral sclerosis functional rating scale – a multicenter observational study	11
Neurofilament light chain as a biological marker for amyotrophic lateral sclerosis: a meta-analysis study	11
Arterial blood gas analysis: base excess and carbonate are predictive of noninvasive ventilation adaptation and survival in amyotrophic lateral sclerosis	10
Factors associated with Noninvasive ventilation compliance in patients with ALS/MND	10
Case-control study in ALS using the National ALS Registry: lead and agricultural chemicals are potential risk factors	10
Epidemiological and genetic features of amyotrophic lateral sclerosis in Latin America and the Caribbean: a systematic review	10
Increased worsening of amyotrophic lateral sclerosis patients during Covid-19-related lockdown in France	10
Analysis of incidence of motor neuron disease in England 1998–2019: use of three linked datasets	10
Persian adaptation of Edinburgh Cognitive and Behavioural Screen (ECAS)	9
Gastrointestinal biopsies and amyotrophic lateral sclerosis – results from a cohort study of 1.1 million individuals	9
Impact of the covid-19 pandemic on amyotrophic lateral sclerosis care in the UK	9
Reliable change indices for the Italian Edinburgh Cognitive and Behavioral ALS Screen (ECAS)	9
Genotype-phenotype correlations of KIF5A stalk domain variants	9
ALSUntangled #64: butyrates	9
Clinical features and incidence trends of amyotrophic lateral sclerosis in Navarre, Spain, 2007–2018: a population-based study	9
Respiratory onset in amyotrophic lateral sclerosis: clinical features and spreading pattern	9
History of vigorous leisure-time physical activity and early onset amyotrophic lateral sclerosis (ALS), data from the national ALS registry: 2010–2018	9
Characteristics of pain and the burden it causes in patients with amyotrophic lateral sclerosis – a longitudinal study	9
Juvenile amyotrophic lateral sclerosis associated with biallelic c.757delG mutation of sorbitol dehydrogenase gene	8
Motor neuron disease beginning with frontotemporal dementia: clinical features and progression	8
Dysfunction of the left angular gyrus may be associated with writing errors in ALS	8
Dying from ALS in Sweden: clinical status, setting, and symptoms	8
Cerebellar degeneration in primary lateral sclerosis: an under-recognized facet of PLS	8
What is amyotrophic lateral sclerosis prevalence?	8
Problem-focused coping underlying lower caregiver burden in ALS-FTD: implications for caregiver intervention	8
Tracheostomy ventilation in motor neurone disease: a snapshot of UK practice	8
Comparison of the ability of the King’s and MiToS staging systems to predict disease progression and survival in amyotrophic lateral sclerosis	7
Genetic and epigenetic disease modifiers in an Italian C9orf72 family expressing ALS, FTD or PD clinical phenotypes	7
Epidemiology and economic burden of amyotrophic lateral sclerosis in the United States: a literature review	7
Clinically meaningful change: evaluation of the Rasch-built Overall Amyotrophic Lateral Sclerosis Disability Scale (ROADS) and the ALSFRS-R	7
Yield of EEG features as markers of disease severity in amyotrophic lateral sclerosis: a pilot study	7
Clinical testing panels for ALS: global distribution, consistency, and challenges	7
Therapeutic effect of a novel curcumin derivative GT863 on a mouse model of amyotrophic lateral sclerosis	7
Occupational history associates with ALS survival and onset segment	7
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	7

The future of ALS diagnosis and staging: where do we go from here?	7
Using the ALSFRS-R in multicentre clinical trials for amyotrophic lateral sclerosis: potential limitations in current standard operating procedures	7
Clinical care and therapeutic trials in PLS	6
The Latin American Epidemiology Network for ALS (Laenals)	6
Occupation and amyotrophic lateral sclerosis risk: a case-control study in the isolated island population of Malta	6
Delivery of nutritional management services to people with amyotrophic lateral sclerosis (ALS)	6
A potential upper motor neuron measure of bulbar involvement in amyotrophic lateral sclerosis using jaw muscle coherence	6
Trends in survival of ALS from a population-based registry	6
COURAGE-ALS: a randomized, double-blind phase 3 study designed to improve participant experience and increase the probability of success	6
Single breath counting is an effective screening tool for forced vital capacity in ALS	6
Progression of cognitive and behavioral disturbances in motor neuron diseases assessed using standard and computer-based batteries	6
Care burden and related factors among informal caregivers of patients with amyotrophic lateral sclerosis	6
Prevalence of amyotrophic lateral sclerosis in the United States, 2018	6
A revision to the United States national ALS registry’s algorithm to improve Case-Ascertainment	5
Preface: promoting research in PLS: current knowledge and future challenges	5
ALS patients with concurrent neuroinflammatory disorders; a nationwide clinical records study	5
An identical DCTN1 mutation in two Chinese siblings manifest as dHMN and ALS respectively: a case report	5
A survey of current practice in genetic testing in amyotrophic lateral sclerosis in the UK and Republic of Ireland: implications for future planning	5
Non-motor symptoms in amyotrophic lateral sclerosis	5
Survival and epidemiology of amyotrophic lateral sclerosis (ALS) cases in the Chicago and Detroit metropolitan cohort: incident cases 2009–2011 and survival through 2018	5
Environmental risk factors for amyotrophic lateral sclerosis: a case–control study in Canada and France	5
Monocyte CD14 and HLA-DR expression increases with disease duration and severity in amyotrophic lateral sclerosis	5
Current practices and barriers in gastrostomy indication in amyotrophic lateral sclerosis: a survey of ALS care teams in The Netherlands	5
Challenges and opportunities for Multi-National Investigator-Initiated clinical trials for ALS: European and United States collaborations	5
Clinicoradiological features in amyotrophic lateral sclerosis patients with olfactory dysfunction	5
CAPTURE ALS: the comprehensive analysis platform to understand, remedy and eliminate ALS	5
Cognition in the course of ALS—a meta-analysis	5
PRECISION ALS—an integrated pan European patient data platform for ALS	5
The patient's perspective of remote respiratory assessments during the COVID-19 pandemic	5
Assessment of risk of ALS conferred by the GGGGCC hexanucleotide repeat expansion in C9orf72 among first-degree relatives of patients with ALS carrying the repeat expans	5
Sensitivity and specificity of the ECAS in identifying executive function and social cognition deficits in MND	5
Corticospinal tract and motor cortex degeneration in pure hereditary spastic paraparesis type 4 (SPG4)	5
A speech-based prognostic model for dysarthria progression in ALS	5
Plasma neurofilament light levels show elevation two years prior to diagnosis of amyotrophic lateral sclerosis in the UK Biobank	5
Clinical trials in pediatric ALS: a TRICALS feasibility study	5
Combination of ciprofloxacin/celecoxib as a novel therapeutic strategy for ALS	5