ArticleNeuro-degenerative diseases2015
An Assessment of Possible Neuropathology and Clinical Relationships in 46 Sporadic Amyotrophic Lateral Sclerosis Patient Autopsies.
Article in Neuro-degenerative diseases, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers, 1 of them a synthesis that pooled it.
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Who cites it
31 citing papers in PubMed, 1 synthesis or guideline pooled it, 48 citations in OpenAlex.
- Cognitive Decline in Preclinical Alzheimer's Disease: Amyloid-Beta versus Tauopathy.Journal of Alzheimer's disease : JAD · 2018Pooled it
- Axonal dying back of upper motor neurons in human ALS.Scientific reports · 2026Article
- Axonal dying back of upper motor neurons in human ALS.Research square · 2026Article
- Sex-related differences in amyotrophic lateral sclerosis: A 2-[European journal of neurology · 2025Article
- Clinicopathological correlates in the frontotemporal lobar degeneration-motor neuron disease spectrum.Brain : a journal of neurology · 2024Observational
- Review
- Betz cells of the primary motor cortex.The Journal of comparative neurology · 2024Review
- Dysautonomia in Amyotrophic Lateral Sclerosis.International journal of molecular sciences · 2023Review
- Upper motor neurons are a target for gene therapy and UCHL1 is necessary and sufficient to improve cellular integrity of diseased upper motor neurons.Gene therapy · 2022Article
- Cognitive decline in amyotrophic lateral sclerosis: Neuropathological substrate and genetic determinants.Brain pathology (Zurich, Switzerland) · 2021Article
- Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP-43 pathology.Clinical and translational medicine · 2021Article
- Associations of Patient Mood, Modulators of Quality of Life, and Pharmaceuticals with Amyotrophic Lateral Sclerosis Survival Duration.Behavioral sciences (Basel, Switzerland) · 2020Article
- Antioxidant Alternatives in the Treatment of Amyotrophic Lateral Sclerosis: A Comprehensive Review.Frontiers in physiology · 2020Review
- A Comprehensive Examination of Percutaneous Endoscopic Gastrostomy and Its Association with Amyotrophic Lateral Sclerosis Patient Outcomes.Brain sciences · 2019Article
- Increased Tau Phosphorylation in Motor Neurons From Clinically Pure Sporadic Amyotrophic Lateral Sclerosis Patients.Journal of neuropathology and experimental neurology · 2019Article
- A critical appraisal of amyloid-β-targeting therapies for Alzheimer disease.Nature reviews. Neurology · 2019Review
- Mitoautophagy: A Unique Self-Destructive Path Mitochondria of Upper Motor Neurons With TDP-43 Pathology Take, Very Early in ALS.Frontiers in cellular neuroscience · 2019Article
- Notch Signalling in the Hippocampus of Patients With Motor Neuron Disease.Frontiers in neuroscience · 2019Article
- Neuroanatomical Quantitative Proteomics Reveals Common Pathogenic Biological Routes between Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD).International journal of molecular sciences · 2018Review
- Associative Increases in Amyotrophic Lateral Sclerosis Survival Duration With Non-invasive Ventilation Initiation and Usage Protocols.Frontiers in neurology · 2018Article
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
backgroundRecent studies have suggested overlapping pathological features among motor neuron, cognitive and neurodegenerative diseases. AIMS/
methodsSecondary analysis of 46 amyotrophic lateral sclerosis (ALS) patient autopsies was performed to independently assess pathological feature prevalence (e.g. percent of patients with any positive finding), degree of severity (e.g. mild, moderate, severe), and 2,200+ potential clinical/neuropathological correlations. The possible impact of gender, onset age, onset type (limb vs. bulbar), riluzole treatment, and severe TDP-43 pathology was assessed within patient subgroups.
resultsAssessed features (prevalence, severity) include: lateral corticospinal tract degeneration (89%, moderate); Purkinje cell loss (85%, mild); localized neuronal loss (83%, mild to moderate); TDP-43 inclusions (80%, moderate); Betz cell loss (76%, mild); neurofibrillary tangles (78%, severe); anterior corticospinal tract degeneration (72%, moderate); spinal ventral root atrophy (65%, moderate); atherosclerosis (35%, mild); β-amyloid (35%, mild); tauopathy/tau inclusions (17%, mild); ventricular dilation (13%, mild); Lewy body formation (11%, mild); microinfarcts (7%, mild); and α-synuclein (4%, mild). Twenty-two percent of patients met criteria for Alzheimer's disease (AD) and 26% for frontotemporal lobar degeneration. Substantial differences were identified in the AD group and in the different onset age groups.
conclusionOur findings support the hypothesis that ALS and its variants could comprise a larger neuropathological continuum.
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