ArticleActa neuropathologica2023
Histopathology of the cerebellar cortex in essential tremor and other neurodegenerative motor disorders: comparative analysis of 320 brains.
Article in Acta neuropathologica, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 46 papers.
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Who cites it
46 citing papers in PubMed, 55 citations in OpenAlex.
- Objective response to ethanol in essential tremor: results from a standardized ethanol challenge study.Annals of clinical and translational neurology · 2024Trial
- Impaired Glur2 palmitoylation in cerebellar Purkinje cells of a Friedreich ataxia mouse model.Experimental neurology · 2026Article
- Neuropathology of Friedreich ataxia and its links to metabolic pathways.Neurodegenerative disease management · 2026Review
- Impact of Losartan Nanoparticles on Carbon Tetrachloride-induced Cerebellar Injury in Rats: Association with Alterations in RIPK1/RIPK3/MLKL Necroptosis Markers.Molecular neurobiology · 2026Article
- Advances in Gene and Cellular Therapy in Friedreich Ataxia.Molecular diagnosis & therapy · 2026Review
- Purkinje Cell Loss and Changes in Basket Cell Morphology in Essential Tremor and Other Cerebellar Degenerative Diseases: A Postmortem Study of 332 Brains.Cerebellum (London, England) · 2026Article
- Autopsy-Confirmed Non-Paraneoplastic Lambert-Eaton Myasthenic Syndrome with Cerebellar Degeneration: A Case Report.Diagnostics (Basel, Switzerland) · 2026Article
- High Evans Index in progressive supranuclear palsy may mimic normal pressure hydrocephalus: a retrospective cross-sectional study.Acta neurologica Belgica · 2026Article
- An Investigation of Heterogeneity in Cerebellar Cortical Pathology Across Essential Tremor Cases: A Cluster Analysis.Cerebellum (London, England) · 2026Article
- Integrity of Brainstem Connections in Essential Tremor Using Auditory and Vestibular Evoked Potentials.Movement disorders clinical practice · 2026Article
- Essential Tremor Cases with Subtle Cerebellar Pathology: A Systematic Study of Postmortem Cerebellar Changes.Cerebellum (London, England) · 2026Article
- Degenerative Changes in the Essential Tremor Cerebellum.Movement disorders : official journal of the Movement Disorder Society · 2026Article
- Comparative study of the pathology in anterior versus posterior hemispheric regions of cerebellum in essential tremor and controls.Acta neuropathologica · 2026Article
- Continued Dialogue about the Clinical Heterogeneity and Degenerative Pathology That Characterizes the Essential Tremors.Tremor and other hyperkinetic movements (New York, N.Y.) · 2026Article
- Purkinje Cell Loss in Essential Tremor: Collective Data From 215 Brains Over a 21-Year Period.Annals of clinical and translational neurology · 2026Article
- The cerebellum shapes motions by encoding motor frequencies with precision and cross-individual uniformity.Nature biomedical engineering · 2025Article
- The Relationship between Rest Tremor and Underlying Lewy Pathology in Essential Tremor: A Clinical-Pathological Study of 201 Cases.Annals of neurology · 2025Article
- The cerebellar involvement in essential tremor: the connecting roads.Arquivos de neuro-psiquiatria · 2025Review
- Serum glial fibrillary acidic protein is elevated in early-stage late-onset essential tremor and associated with tremor progression.Journal of neural transmission (Vienna, Austria : 1996) · 2025Observational
- Axonal pathology differentially affects human Purkinje cell subpopulations in the essential tremor cerebellum.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
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Authors and funding
10 authors at 3 institutions in 1 country.
Funding
Abstract
In recent years, numerous morphologic changes have been identified in the essential tremor (ET) cerebellar cortex, distinguishing ET from control brains. These findings have not been fully contextualized within a broader degenerative disease spectrum, thus limiting their interpretability. Building off our prior study and now doubling the sample size, we conducted comparative analyses in a postmortem series of 320 brains on the severity and patterning of cerebellar cortex degenerative changes in ET (n = 100), other neurodegenerative disorders of the cerebellum [spinocerebellar ataxias (SCAs, n = 47, including 13 SCA3 and 34 SCA1, 2, 6, 7, 8, 14); Friedreich's ataxia (FA, n = 13); multiple system atrophy (MSA), n = 29], and other disorders that may involve the cerebellum [Parkinson's disease (PD), n = 62; dystonia, n = 19] versus controls (n = 50). We generated data on 37 quantitative morphologic metrics, grouped into 8 broad categories: Purkinje cell (PC) loss, heterotopic PCs, PC dendritic changes, PC axonal changes (torpedoes), PC axonal changes (other than torpedoes), PC axonal changes (torpedo-associated), basket cell axonal hypertrophy, and climbing fiber-PC synaptic changes. Principal component analysis of z scored raw data across all diagnoses (11,651 data items) revealed that diagnostic groups were not uniform with respect to pathology. Dystonia and PD each differed from controls in only 4/37 and 5/37 metrics, respectively, whereas ET differed in 21, FA in 10, SCA3 in 10, MSA in 21, and SCA1/2/6/7/8/14 in 27. Pathological changes were generally on the milder end of the degenerative spectrum in ET, FA and SCA3, and on the more severe end of that spectrum in SCA1/2/6/7/8/14. Comparative analyses across morphologic categories demonstrated differences in relative expression, defining distinctive patterns of changes in these groups. In summary, we present a robust and reproducible method that identifies somewhat distinctive signatures of degenerative changes in the cerebellar cortex that mark each of these disorders.
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