Jean-paul Gerard Vonsattel
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Jean-paul Gerard Vonsattel's Degrees
- Doctorate Medicine Columbia University
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(Suggest an Edit or Addition)Jean-paul Gerard Vonsattel's Published Works
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Total number of citations to an author for the works they published in a given year. This highlights publication of the most important work(s) by the author
Published Works
- Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. (1997) (2717)
- Neuropathological Classification of Huntington's Disease (1985) (2504)
- Common variants in MS4A4/MS4A6E, CD2uAP, CD33, and EPHA1 are associated with late-onset Alzheimer’s disease (2011) (1656)
- Rapid induction of Alzheimer A beta amyloid formation by zinc. (1994) (1389)
- Trinucleotide repeat length instability and age of onset in Huntington's disease (1993) (1083)
- Genetic meta-analysis of diagnosed Alzheimer’s disease identifies new risk loci and implicates Aβ, tau, immunity and lipid processing (2019) (1073)
- Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease (2000) (1073)
- Primary age-related tauopathy (PART): a common pathology associated with human aging (2014) (988)
- Cyclophilin D deficiency attenuates mitochondrial and neuronal perturbation and ameliorates learning and memory in Alzheimer's disease (2008) (820)
- Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons (1995) (782)
- Inactivation of the mouse Huntington's disease gene homolog Hdh. (1995) (746)
- Rare coding variants in PLCG2, ABI3, and TREM2 implicate microglial-mediated innate immunity in Alzheimer's disease (2017) (693)
- Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease (1999) (642)
- The first NINDS/NIBIB consensus meeting to define neuropathological criteria for the diagnosis of chronic traumatic encephalopathy (2015) (639)
- CAG repeat number governs the development rate of pathology in Huntington's disease (1997) (623)
- Office of Rare Diseases Neuropathologic Criteria for Corticobasal Degeneration (2002) (580)
- Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion (1997) (543)
- Frequency and course of mild cognitive impairment in a multiethnic community (2008) (530)
- Cerebral amyloid angiopathy without and with cerebral hemorrhages: A comparative histological study (1991) (512)
- Novel amyloid precursor protein mutation in an Iowa family with dementia and severe cerebral amyloid angiopathy (2001) (502)
- Neuropathological changes in essential tremor: 33 cases compared with 21 controls. (2007) (500)
- Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. (2000) (484)
- Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions (2010) (475)
- Early and Progressive Accumulation of Reactive Microglia in the Huntington Disease Brain (2001) (471)
- Apolipoprotein E ϵ4 and cerebral hemorrhage associated with amyloid angiopathy (1995) (464)
- Morphometric Demonstration of Atrophic Changes in the Cerebral Cortex, White Matter, and Neostriatum in Huntington's Disease (1988) (426)
- Identification of Genetic Factors that Modify Clinical Onset of Huntington’s Disease (2015) (420)
- Model‐guided microarray implicates the retromer complex in Alzheimer's disease (2005) (368)
- Direct quantification of CSF α-synuclein by ELISA and first cross-sectional study in patients with neurodegeneration (2008) (362)
- Common genetic variants in the CLDN2 and PRSS1-PRSS2 loci alter risk for alcohol-related and sporadic pancreatitis (2012) (308)
- Zinc-induced Alzheimer’s Aβ1–40 Aggregation Is Mediated by Conformational Factors* (1997) (307)
- Genome-Wide Association Meta-analysis of Neuropathologic Features of Alzheimer's Disease and Related Dementias (2014) (295)
- Differential diagnosis of parkinsonism: a metabolic imaging study using pattern analysis (2010) (289)
- Amyloid Formation by Mutant Huntingtin: Threshold, Progressivity and Recruitment of Normal Polyglutamine Proteins (1998) (281)
- Common variants at MS 4 A 4 / MS 4 A 6 E , CD 2 AP , CD 33 and EPHA 1 are associated with late-onset Alzheimer ’ s disease (2011) (274)
- CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome (2013) (262)
- De novo expansion of a (CAG)n repeat in sporadic Huntington's disease (1993) (261)
- Ablation of the Inflammatory Enzyme Myeloperoxidase Mitigates Features of Parkinson's Disease in Mice (2005) (249)
- Strain-related differences in susceptibility to transient forebrain ischemia in SV-129 and C57black/6 mice. (1997) (248)
- Five siRNAs Targeting Three SNPs May Provide Therapy for Three-Quarters of Huntington's Disease Patients (2009) (248)
- Dendrite and dendritic spine alterations in alzheimer models (2004) (240)
- Apolipoprotein E epsilon 4 and cerebral hemorrhage associated with amyloid angiopathy. (1995) (240)
- MHC-I expression renders catecholaminergic neurons susceptible to T-cell-mediated degeneration (2014) (240)
- CAG Repeat Not Polyglutamine Length Determines Timing of Huntington’s Disease Onset (2019) (236)
- Single cell RNA sequencing of human microglia uncovers a subset associated with Alzheimer’s disease (2020) (231)
- Clinical and neuropathologic assessment of severity in Huntington's disease (1988) (226)
- Huntington's disease gene: Regional and cellular expression in brain of normal and affected individuals (1995) (222)
- Decreased Neuronal and Increased Oligodendroglial Densities in Huntington's Disease Caudate Nucleus (1991) (219)
- A critical evaluation of the Braak staging scheme for Parkinson's disease (2008) (213)
- Evidence for a role of the rare p.A152T variant in MAPT in increasing the risk for FTD-spectrum and Alzheimer's diseases. (2012) (208)
- The Neuropathology of Huntington's Disease. (2015) (204)
- Gilles de la Tourette's syndrome A postmortem neuropathological and immunohistochemical study (1986) (198)
- Reduced Purkinje cell number in essential tremor: a postmortem study. (2008) (197)
- Huntington disease models and human neuropathology: similarities and differences (2007) (193)
- Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. (2002) (190)
- Triplet repeat mutation length gains correlate with cell-type specific vulnerability in Huntington disease brain. (2007) (182)
- CAG expansion affects the expression of mutant huntingtin in the Huntington's disease brain (1995) (177)
- Association of glucocerebrosidase mutations with dementia with lewy bodies. (2009) (175)
- Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease. (1999) (175)
- CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome (2013) (170)
- Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis (1995) (168)
- Tauopathies with parkinsonism: clinical spectrum, neuropathologic basis, biological markers, and treatment options (2009) (165)
- The emerging neuropathology of essential tremor (2008) (160)
- Quantitative neuropathological changes in presymptomatic Huntington's disease (2001) (159)
- DNA strand breaks in Alzheimer's disease (1999) (159)
- Genome-wide association study of corticobasal degeneration identifies risk variants shared with progressive supranuclear palsy (2015) (157)
- Diagnosis of cerebral amyloid angiopathy. Sensitivity and specificity of cortical biopsy. (1997) (145)
- Effects of multiple genetic loci on age at onset in late-onset Alzheimer disease: a genome-wide association study. (2014) (140)
- PART, a distinct tauopathy, different from classical sporadic Alzheimer disease (2015) (131)
- Purkinje cell axonal anatomy: quantifying morphometric changes in essential tremor versus control brains. (2013) (129)
- Essential tremor associated with focal nonnigral Lewy bodies: a clinicopathologic study. (2005) (127)
- Twenty-first century brain banking. Processing brains for research: the Columbia University methods (2007) (126)
- Late onset of Huntington's disease. (1985) (124)
- Huntington's disease (HD): the neuropathology of a multisystem neurodegenerative disorder of the human brain (2016) (123)
- Single-nucleus RNA-seq identifies Huntington disease astrocyte states (2019) (122)
- Alpha-synuclein immunoreactivity is present in axonal swellings in neuroaxonal dystrophy and acute traumatic brain injury. (1999) (121)
- Huntington's disease - neuropathology. (2011) (121)
- "Hairy Baskets" Associated With Degenerative Purkinje Cell Changes in Essential Tremor (2010) (119)
- Dysfunction of brain kynurenic acid metabolism in Huntington's disease: focus on kynurenine aminotransferases (1995) (118)
- Spinal cord ischemia. Development of a model in the mouse. (2000) (116)
- Up-regulation of the lysosomal system in experimental models of neuronal injury: implications for Alzheimer’s disease (2000) (116)
- Degeneration of the Cerebellum in Huntington's Disease (HD): Possible Relevance for the Clinical Picture and Potential Gateway to Pathological Mechanisms of the Disease Process (2013) (115)
- Polyamine pathway contributes to the pathogenesis of Parkinson disease (2010) (115)
- miR-10b-5p expression in Huntington’s disease brain relates to age of onset and the extent of striatal involvement (2015) (114)
- SORCS1 alters amyloid precursor protein processing and variants may increase Alzheimer's disease risk (2011) (112)
- Essential tremor associated with pathologic changes in the cerebellum. (2006) (111)
- Bim Is Elevated in Alzheimer's Disease Neurons and Is Required for β-Amyloid-Induced Neuronal Apoptosis (2007) (109)
- Frontal Lobe Dysfunction in Progressive Supranuclear Palsy (2000) (109)
- Association of apolipoprotein E epsilon2 and vasculopathy in cerebral amyloid angiopathy. (1998) (108)
- Direct demonstration of transsynaptic degeneration in the human visual system: a comparison of retrograde and anterograde changes (1982) (103)
- Huntingtin's WW domain partners in Huntington's disease post-mortem brain fulfill genetic criteria for direct involvement in Huntington's disease pathogenesis. (2000) (103)
- Normal and Expanded Huntington’s Disease Gene Alleles Produce Distinguishable Proteins Due to Translation Across the CAG Repeat (1995) (102)
- Calpain activation in neurodegenerative diseases: confocal immunofluorescence study with antibodies specifically recognizing the active form of calpain 2 (2002) (98)
- Abnormal climbing fibre-Purkinje cell synaptic connections in the essential tremor cerebellum. (2014) (92)
- A disease-specific metabolic brain network associated with corticobasal degeneration. (2014) (92)
- Reduced Purkinje cell dendritic arborization and loss of dendritic spines in essential tremor. (2014) (91)
- A Comparison of Huntington Disease and Huntington Disease-Like 2 Neuropathology (2008) (90)
- Plasma p‐tau181, p‐tau217, and other blood‐based Alzheimer's disease biomarkers in a multi‐ethnic, community study (2020) (90)
- Astrogliopathy predominates the earliest stage of corticobasal degeneration pathology. (2016) (89)
- Mechanisms and Disease Associations of Haplotype-Dependent Allele-Specific DNA Methylation. (2016) (89)
- Differences between Pick disease and Alzheimer disease in clinical appearance and rate of cognitive decline. (2000) (89)
- Introduction of a foreign gene (Escherichia coli lacZ) into rat neostriatal neurons using herpes simplex virus mutants: A light and electron microscopic study (1992) (88)
- Increased number of heterotopic Purkinje cells in essential tremor (2010) (85)
- Huntington's disease CAG trinucleotide repeats in pathologically confirmed post-mortem brains (1994) (84)
- Potential genetic modifiers of disease risk and age at onset in patients with frontotemporal lobar degeneration and GRN mutations: a genome-wide association study (2018) (83)
- ASSOCIATION BETWEEN GENETIC VARIANTS IN SORL1 AND AUTOPSY-CONFIRMED ALZHEIMER DISEASE (2008) (77)
- Torpedoes in Parkinson's disease, Alzheimer's disease, essential tremor, and control brains (2009) (76)
- Quinolinic acid-induced increases in calbindin D28k immunoreactivity in rat striatal neurons in vivo and in vitro mimic the pattern seen in Huntington's disease (1995) (76)
- Biological and clinical manifestations of juvenile Huntington's disease: a retrospective analysis (2018) (75)
- α-Synuclein in human cerebrospinal fluid is principally derived from neurons of the central nervous system (2012) (72)
- The Second NINDS/NIBIB Consensus Meeting to Define Neuropathological Criteria for the Diagnosis of Chronic Traumatic Encephalopathy (2021) (72)
- N-acetylaspartylglutamate, N-acetylaspartate, and N-acetylated alpha-linked acidic dipeptidase in human brain and their alterations in Huntington and Alzheimer's diseases. (1997) (70)
- Parkinsonism, dysautonomia, and intranuclear inclusions in a fragile X carrier: A clinical–pathological study (2006) (70)
- Gene-Wise Association of Variants in Four Lysosomal Storage Disorder Genes in Neuropathologically Confirmed Lewy Body Disease (2015) (70)
- Torpedoes in the Cerebellar Vermis in Essential Tremor Cases vs. Controls (2011) (70)
- Cerebellar oscillations driven by synaptic pruning deficits of cerebellar climbing fibers contribute to tremor pathophysiology (2020) (70)
- Lingo-1 expression is increased in essential tremor cerebellum and is present in the basket cell pinceau (2013) (68)
- Heterogeneity of subcellular localization and electrophoretic mobility of survival motor neuron (SMN) protein in mammalian neural cells and tissues. (1998) (65)
- Evidence for Oxidative Stress in the Subthalamic Nucleus in Progressive Supranuclear Palsy (1999) (65)
- Genome-wide analyses as part of the international FTLD-TDP whole-genome sequencing consortium reveals novel disease risk factors and increases support for immune dysfunction in FTLD (2019) (63)
- Early neuropsychological discriminants for Lewy body disease: an autopsy series (2013) (62)
- Rest tremor in advanced essential tremor: a post-mortem study of nine cases (2010) (61)
- Soluble amyloid beta levels are elevated in the white matter of Alzheimer’s patients, independent of cortical plaque severity (2014) (61)
- Neuropathological heterogeneity in frontotemporal lobar degeneration with TDP-43 proteinopathy: a quantitative study of 94 cases using principal components analysis (2010) (59)
- Older onset essential tremor: More rapid progression and more degenerative pathology (2009) (59)
- Purkinje cell loss in essential tremor: Random sampling quantification and nearest neighbor analysis (2016) (58)
- Decreased Coenzyme Q10 Levels in Multiple System Atrophy Cerebellum (2016) (57)
- The Neuropathology of Huntington’s Disease: Classical Findings, Recent Developments and Correlation to Functional Neuroanatomy (2015) (57)
- Quantification of cerebellar hemispheric purkinje cell linear density: 32 ET cases versus 16 controls (2013) (57)
- Structural study of Purkinje cell axonal torpedoes in essential tremor (2009) (56)
- Huntington's Disease (HD): Degeneration of Select Nuclei, Widespread Occurrence of Neuronal Nuclear and Axonal Inclusions in the Brainstem (2014) (55)
- The inferior olivary nucleus: A postmortem study of essential tremor cases versus controls (2013) (55)
- Selective reduction of soluble Tau proteins in sporadic and familial frontotemporal dementias: an international follow-up study (2003) (52)
- Network correlates of disease severity in multiple system atrophy (2012) (50)
- Assessment of cortical and striatal involvement in 523 Huntington disease brains (2012) (49)
- PARK10 is a major locus for sporadic neuropathologically confirmed Parkinson disease (2015) (48)
- Twenty-first century brain banking: practical prerequisites and lessons from the past: the experience of New York Brain Bank, Taub Institute, Columbia University (2008) (47)
- An MRI measure of degenerative and cerebrovascular pathology in Alzheimer disease (2018) (46)
- Distribution of N-acetylaspartylglutamate immunoreactivity in human brain and its alteration in neurodegenerative disease (1997) (46)
- Huntington's disease like‐2 neuropathology (2007) (45)
- Genome-Wide Methylation Analyses in Glioblastoma Multiforme (2014) (45)
- Purkinje cell loss is a characteristic of essential tremor. (2011) (45)
- Patterns of CAG repeat instability in the central nervous system and periphery in Huntington’s disease and in spinocerebellar ataxia type 1 (2020) (45)
- An in vitro 1H nuclear magnetic resonance study of the temporoparietal cortex of Alzheimer brains (2004) (45)
- TP73 allelic expression in human brain and allele frequencies in Alzheimer's disease (2004) (43)
- Frequency of GBA Variants in Autopsy‐proven Multiple System Atrophy (2017) (43)
- Clinical and Pathological Characteristics of LRRK2 G2019S Patients with PD (2012) (42)
- Essential Tremor Followed by Progressive Supranuclear Palsy: Postmortem Reports of 11 Patients (2013) (41)
- Torpedo Formation and Purkinje Cell Loss: Modeling their Relationship in Cerebellar Disease (2014) (41)
- Minocycline is protective in a mouse model of Huntington's disease (2003) (40)
- Pathogenic Huntingtin Repeat Expansions in Patients with Frontotemporal Dementia and Amyotrophic Lateral Sclerosis (2020) (40)
- Purkinje cell loss is a characteristic of essential tremor: towards a more mature understanding of pathogenesis. (2012) (40)
- Rarity of the Alzheimer disease-protective APP A673T variant in the United States. (2015) (39)
- Clinicopathological characteristics of freezing of gait in autopsy‐confirmed Parkinson's disease (2015) (39)
- LATE to the PART-y (2019) (38)
- C9orf72 intermediate repeats are associated with corticobasal degeneration, increased C9orf72 expression and disruption of autophagy (2019) (38)
- Contextualizing the pathology in the essential tremor cerebellar cortex: a patholog-omics approach (2019) (37)
- Memory performance is related to amyloid and tau pathology in the hippocampus (2009) (37)
- Expression and activity of antioxidants in the brain in progressive supranuclear palsy (2002) (37)
- Elevated brain harmane (1-methyl-9H-pyrido[3,4-b]indole) in essential tremor cases vs. controls. (2013) (36)
- Regional metabolic alterations in Alzheimer's disease: an in vitro 1H NMR study of the hippocampus and cerebellum. (1997) (35)
- Multiple system atrophy in a patient with the spinocerebellar ataxia 3 gene mutation (2007) (35)
- Reduced LRRK2 in association with retromer dysfunction in post-mortem brain tissue from LRRK2 mutation carriers (2017) (35)
- Abnormalities in the Tricarboxylic Acid Cycle in Huntington Disease and in a Huntington Disease Mouse Model (2015) (34)
- Neuropathology of Huntington's disease. (2008) (33)
- Cerebellar Pathology in Early Onset and Late Onset Essential Tremor (2017) (33)
- Patterns of protein nitration in dementia with Lewy bodies and striatonigral degeneration (2002) (32)
- Generation of iPSC lines from archived non-cryoprotected biobanked dura mater (2014) (28)
- Heterotopic Purkinje Cells: a Comparative Postmortem Study of Essential Tremor and Spinocerebellar Ataxias 1, 2, 3, and 6 (2018) (28)
- Native Mutant Huntingtin in Human Brain (2012) (27)
- Effect of Fluosol on oxygen availability, regional cerebral blood flow, and infarct size in a model of temporary focal cerebral ischemia. (1986) (27)
- Huntington's Disease (HD): Neurodegeneration of Brodmann's Primary Visual Area 17 (BA17) (2015) (27)
- Author Correction: Genetic meta-analysis of diagnosed Alzheimer’s disease identifies new risk loci and implicates Aβ, tau, immunity and lipid processing (2019) (26)
- Alzheimer's-related changes in non-demented essential tremor patients vs. controls: links between tau and tremor? (2014) (26)
- Early‐Onset Parkinsonism Is a Manifestation of the PPP2R5D p.E200K Mutation (2020) (26)
- Early Selective Vulnerability of the CA2 Hippocampal Subfield in Primary Age-Related Tauopathy. (2020) (25)
- Polyglutamine expansion affects huntingtin conformation in multiple Huntington’s disease models (2017) (25)
- Neuropathologic Features and Grading of Alzheimer-Related and Sporadic CAA (2000) (24)
- Selective loss of striatal preprotachykinin neurons in a phenocopy of Huntington's disease (2002) (23)
- Relationship of clinical efficacy to postmortem-determined anatomic subthalamic stimulation in Parkinson syndrome. (2007) (23)
- IT15 gene expression in fetal human brain (1994) (23)
- Tau protein expression in frontotemporal dementias (2001) (22)
- Regional vulnerability in Huntington's disease: fMRI-guided molecular analysis in patients and a mouse model of disease (2013) (22)
- SorCS2-mediated NR2A trafficking regulates motor deficits in Huntington's disease. (2017) (22)
- Update on Hippocampal Sclerosis (2015) (22)
- Parkinson's disease with Lewy bodies associated with a heterozygous PARKIN dosage mutation (2014) (21)
- Changes of peripheral TGF-β1 depend on monocytes-derived macrophages in Huntington disease (2013) (21)
- Marked tissue eosinophilia within organizing chronic subdural hematoma membranes. (1994) (20)
- Cerebellar Pathology of a Dual Clinical Diagnosis: Patients with Essential Tremor and Dystonia (2012) (20)
- P301L tauopathy confocal immunofluorescence study of perinuclear aggregation of the mutated protein (2002) (20)
- Genetic Risk Underlying Psychiatric and Cognitive Symptoms in Huntington’s Disease (2019) (20)
- Clinical and neuropathological features of progressive supranuclear palsy in Leucine rich repeat kinase (LRRK2) G2019S mutation carriers (2018) (18)
- Neurofilament protein levels: Quantitative analysis in essential tremor cerebellar cortex (2012) (18)
- A soluble truncated tau species related to cognitive dysfunction and caspase-2 is elevated in the brain of Huntington’s disease patients (2019) (18)
- Neuropathologic Changes of Multiple System Atrophy and Diffuse Lewy Body Disease (2014) (17)
- Autopsy-proven Alzheimer disease in a patient with dementia who retained musical skill in life. (1997) (17)
- Essential tremor with ubiquitinated Purkinje cell intranuclear inclusions (2010) (15)
- Macroautophagy Abnormality in Essential Tremor (2012) (15)
- Cerebellar Pathology in Familial vs. Sporadic Essential Tremor (2017) (15)
- Upregulation of the lysosomal system in experimental models of neuronal injury: Implications for Alzheimer's disease (2000) (14)
- Matching Asymmetry of Tremor with Asymmetry of Postmortem Cerebellar Hemispheric Changes in Essential Tremor (2014) (14)
- Essential tremor with ubiquitinated intranuclear inclusions and cerebellar degeneration (2012) (14)
- Developmental malformations in Huntington disease: neuropathologic evidence of focal neuronal migration defects in a subset of adult brains (2021) (14)
- The development of a brain bank. (1993) (14)
- Purkinje cell axonal torpedoes are unrelated to advanced aging and likely reflect cerebellar injury (2009) (13)
- Huntington's disease. (1982) (13)
- Comparison of the effect of fluosol DA and dextran 40 on regional cerebral blood flow, infarction size, and mortality in cats with temporary occlusion of the middle cerebral artery. (1986) (13)
- Targeted inactivation of the mouse Huntington's disease gene homolog Hdh. (1996) (13)
- Expansile, enhancing cervical cord lesion with an associated syrinx secondary to demyelination. Case report and review of the literature. (2007) (13)
- Inferior Olivary nucleus degeneration does not lessen tremor in essential tremor (2018) (13)
- Infrastructure resources for clinical research in amyotrophic lateral sclerosis (2013) (13)
- The effect of MAPT haplotype on neocortical Lewy body pathology in Parkinson disease (2016) (13)
- Fulminant corticobasal degeneration: a distinct variant with predominant neuronal tau aggregates (2020) (13)
- Apolipoprotein E ϵ4/4 in a Neuropathologically Normal Very Elderly Individual (1996) (13)
- Wolframin is a novel regulator of tau pathology and neurodegeneration (2022) (12)
- 122 NEUROPATHOLOGIC CLASSIFICATION OF HUNTINGTONʼS DISEASE (1983) (12)
- Amyotrophic lateral sclerosis and neurosarcoidosis: A case report (2009) (11)
- Eosinophilic vasculitis in an isolated central nervous system distribution (2009) (11)
- Increased Steady-State Mutant Huntingtin mRNA in Huntington's Disease Brain. (2013) (11)
- Altered Brain Expression of Insulin and Insulin-Like Growth Factors in Frontotemporal Lobar Degeneration: Another Degenerative Disease Linked to Dysregulation of Insulin Metabolic Pathways (2019) (11)
- Genome-wide association study and functional validation implicates JADE1 in tauopathy (2021) (10)
- Movement disorders rounds: A case of missing pathology in a patient with LRRK2 Parkinson's disease. (2019) (10)
- Hippocampal laminar distribution of tau relates to Alzheimer's disease and age of onset. (2014) (10)
- [Cerebral amyloid angiopathy. A clinicopathological study (author's transl)]. (1981) (10)
- Cerebral Microbleeds, Cerebral Amyloid Angiopathy, and Their Relationships to Quantitative Markers of Neurodegeneration (2022) (9)
- Banking brains: a pre-mortem “how to” guide to successful donation (2018) (9)
- Genetic load determines atrophy in hand cortico‐striatal pathways in presymptomatic Huntington's disease (2018) (9)
- Amyotrophic lateral sclerosis is over-represented in two Huntington’s disease brain bank cohorts: further evidence to support genetic pleiotropy of pathogenic HTT gene expansion (2021) (9)
- Evidence for the spread of human-derived mutant huntingtin protein in mice and non-human primates (2020) (9)
- Apolipoprotein E epsilon4/4 in a neuropathologically normal very elderly individual. (1996) (8)
- Disease-related Huntingtin seeding activities in cerebrospinal fluids of Huntington’s disease patients (2020) (8)
- CLASSIC ESSENTIAL TREMOR CHANGES FOLLOWING CEREBELLAR HEMORRHAGE (2009) (8)
- Parallel fiber counts and parallel fiber integrated density are similar in essential tremor cases and controls (2011) (8)
- Cellular density in the cerebellar molecular layer in essential tremor, spinocerebellar ataxia, and controls. (2014) (8)
- Familial frontotemporal dementia: a report of three cases of severe cerebral atrophy with rare inclusions that are negative for tau and synuclein, but positive for ubiquitin (2004) (8)
- Dementia with cerebrovascular disease. (2006) (8)
- The Complexity of Clinical Huntington's Disease: Developments in Molecular Genetics, Neuropathology and Neuroimaging Biomarkers. (2017) (7)
- Late‐life action tremor in a southern sea otter (enhydris lutris nereis) (2004) (7)
- Electronic tracking of human brain samples for research (2008) (7)
- Neuropathological correlation supports automated image-based differential diagnosis in parkinsonism (2021) (7)
- Common Themes in the Pathogenesis of Neurodegeneration (2011) (7)
- Pathological findings of anti-Yo cerebellar degeneration with Holmes tremor (2014) (7)
- The New York Brain Bank of Columbia University: practical highlights of 35 years of experience. (2018) (7)
- Eosinophilic vasculitis in an isolated central nervous system distribution (2006) (6)
- Failure of fluosol to influence the incidence of cerebral infarction or mortality in gerbils subjected to temporary carotid occlusion (1986) (6)
- Huntington’s Disease: Neuropathological Grading (1987) (6)
- Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 20-1990. A 16-year-old boy with a lesion of the left frontal lobe. (1990) (6)
- Interpretable deep learning of myelin histopathology in age-related cognitive impairment (2022) (6)
- Infantile progressive striato-thalamic degeneration in two siblings: a new syndrome. (1995) (5)
- Sorcs1 Alters APP Processing and Variants may Increase Alzheimer's Disease Risk (2010) (5)
- Presenting Neuropsychological Testing Profile of Autopsy-Confirmed Frontotemporal Lobar Degeneration (2013) (5)
- 70 COINCIDENCE OF FIBRINOID NECROSIS WITH AMYLOID ANGIOPATHY AS THE CAUSE OF CEREBRAL HEMORRHAGE (1984) (5)
- SorCS 2-mediated NR 2 A trafficking regulates motor deficits in Huntington ’ s disease (2017) (5)
- Manifestations of Alzheimer’s disease genetic risk in the blood are evident in a multiomic analysis in healthy adults aged 18 to 90 (2022) (4)
- Juvenile Huntington’s disease: neuropathology (2009) (4)
- A Familial Form of Pallidoluysionigral Degeneration and Amyotrophic Lateral Sclerosis With Divergent Clinical Presentations (2007) (4)
- The distribution and density of Huntingtin inclusions across the Huntington disease neocortex: regional correlations with Huntingtin repeat expansion independent of pathologic grade (2022) (4)
- The Neuropathology of Dementia: Huntington's disease (2004) (4)
- Huntington's disease. (2003) (4)
- Characterizing Lewy Pathology in 231 Essential Tremor Brains From the Essential Tremor Centralized Brain Repository. (2022) (4)
- Tau Isoform Profile in Essential Tremor Diverges From Other Tauopathies. (2021) (3)
- Correlation of plasma and neuroimaging biomarkers in Alzheimer's disease (2022) (3)
- GLOBAL LOSS OF CEREBRAL CORTEX AND WHITE MATTER VOLUME IN HUNTINGTON'S DISEASE (HD).: 31 (1987) (3)
- Intraventricular Administration of a Caspase Inhibitor Delays Disease Progression and Mortality in a Mouse Model of Huntington's Disease (1999) (3)
- Neuropathological Findings in a Case of Parkinsonism and Developmental Delay Associated with a Monoallelic Variant in PLXNA1 (2021) (3)
- [Huntington disease: 7 cases with relatively preserved neostriatal islets]. (1992) (3)
- Basal Ganglia Gliosis in a Case of Rapid‐Onset Dystonia‐Parkinsonism (DYT12) with a Novel Mutation in ATPase 1A3 (ATP1A3) (2016) (3)
- Huntington disease oligodendrocyte maturation deficits revealed by single-nucleus RNAseq are rescued by thiamine-biotin supplementation (2022) (3)
- What is It? Difficult to Pigeon Hole Tremor: a Clinical–Pathological Study of a Man with Jaw Tremor (2013) (3)
- Clinicopathological correlates of pyramidal signs in multiple system atrophy (2022) (2)
- Genetic overlap between psychiatric disorders and neuropsychiatric symptoms in HD (2019) (2)
- Preliminary Results of the NINDS/NIBIB Consensus Meeting to Evaluate Pathological Criteria for the Diagnosis of CTE (P2.178) (2015) (2)
- Degeneration of the cerebellum and brainstem in Huntington's disease (HD) (2013) (2)
- The Neuropathological Grading of Huntington's Disease (HD). (2015) (2)
- Multiple System Atrophy With Predominant Striatonigral Degeneration and TAR DNA‐Binding Protein of 43 kDa Pathology: An Unusual Variant of Multiple System Atrophy (2019) (2)
- Matching Asymmetry of Tremor with Asymmetry of Postmortem Cerebellar Hemispheric Changes in Essential Tremor (2014) (1)
- The Neuropathology of Huntington’s Disease in the Human Brain (2014) (1)
- ALZHEIMER DISEASE DOES NOT PROTECT THE NEOSTRIATUM IN COMBINED CASES OF HUNTINGTON (HD) AND ALZHEIMER DISEASE (AD) (1997) (1)
- CLASSIC ESSENTIAL TREMOR CHANGES FOLLOWING CEREBELLAR HEMORRHAGE. Authors' reply (2009) (1)
- Brain imaging and neuropathologic mechanisms in Alzheimer's disease: vascular versus neurodegenerative and amyloid-beta versus tau. (2009) (1)
- NONAGENARIANS WITH HUNTINGTON DISEASE (HD) HAVE LOW CAG REPEATS (1999) (1)
- Banking brains: a pre-mortem “how to” guide to successful donation (2018) (1)
- Case records of the Massachusetts General Hospital. Weekly clinicopathological excises. Case 10-1991. A 30-year-old man with polydipsia, hypopituitarism, and a mediastinal mass. (1991) (1)
- Reply to “PPP2R5D Genetic Mutations and Early Onset Parkinsonism” (2020) (1)
- Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 6-1990. Rapidly developing confusion, impaired memory, and unsteady gait in an elderly man. (1990) (1)
- DNA DAMAGE IN ALZHEIMER??S DISEASE: (1999) (1)
- Progressive supranuclear palsy. (2004) (1)
- Single nuclei RNAseq analysis of HD mouse models and human brain reveals impaired oligodendrocyte maturation and potential role for thiamine metabolism (2022) (1)
- miR-10b-5p expression in Huntington’s disease brain relates to age of onset and the extent of striatal involvement (2015) (1)
- ARGYROPHILIC GLIAL CYTOPLASMIC INCLUSIONS IN PROGRESSIVE SUPRANUCLEAR PALSY - SENSITIVE AND SPECIFIC DETECTION BY BIELSCHOWSKY STAIN (1996) (1)
- Erratum: LATE to the PART-y (Brain (2019) 142 (e47) DOI: 10.1093/brain/awz224) (2019) (1)
- SELECTIVE LOSS OF PREPROTACHYKININ NEURONS IN A PHENOCOPY OF HUNTINGTONʼS DISEASE (1997) (1)
- Tangle-only dementia is associated with variation in the MAPT 3' UTR (2012) (0)
- Increased Number of Purkinje Cell Dendritic Swellings: Evidence of Another Structural Abnormality in Essential Tremor (P04.043) (2012) (0)
- PHENOTYPIC CHARACTERIZATION OF EARLY ONSET FAMILIAL AD ASSOCIATED WITH A PSEN1 L418F MUTATION (2014) (0)
- Parkinson's Progression Markers Initiative brain autopsy program. (2022) (0)
- Contents Vol. 36, 2013 (2013) (0)
- Abstract WP127: Cerebral Microbleeds, Cerebral Amyloid Angiopathy, And Their Relationships To Quantitative Markers Of Neurodegeneration (2022) (0)
- Torpedo Formation and Purkinje Cell Loss: Modeling their Relationship in Cerebellar Disease (2014) (0)
- Somatic mitochondrial DNA mutations and parkinsonism (2012) (0)
- Progressive degeneration of corticospinal tract in ALS: a DTI single case longitudinal study (2001) (0)
- Edward Peirson Richardson, Jr. 1918–1998 (1999) (0)
- The distribution and density of Huntingtin inclusions across the Huntington disease neocortex: regional correlations with Huntingtin repeat expansion independent of pathologic grade (2022) (0)
- Inhibitionof caspase-1 slowsdiseaseprogression inamousemodel ofHuntington ’ sdisease (0)
- Heterotopic Purkinje Cells: a Comparative Postmortem Study of Essential Tremor and Spinocerebellar Ataxias 1, 2, 3, and 6 (2017) (0)
- Molecular abnormalities in sporadic amyotrophic lateral sclerosis (ALS): 41.11 (2007) (0)
- Generation of iPSC lines from archived non-cryoprotected biobanked dura mater (2014) (0)
- IMMUNOCYTOCHEMICAL AND GENOTYPIC DISTINCTIONS AMONG 3 VARIANTS OF PICKʼS LOBAR ATROPHY (1996) (0)
- Soluble amyloid beta levels are elevated in the white matter of Alzheimer’s patients, independent of cortical plaque severity (2014) (0)
- Cerebellar Pathology in Early Onset and Late Onset Essential Tremor (2016) (0)
- Inferior Olivary nucleus degeneration does not lessen tremor in essential tremor (2018) (0)
- Abnormal Climbing Fiber-Purkinje Cell Synaptic Connections in the Essential Tremor Cerebellum (P3.011) (2015) (0)
- High‐Frequency Palatal Tremor and Stimulus‐Sensitive Leg Myoclonus with Degeneration of Inferior Olivary Nuclei in Celiac Disease (2020) (0)
- O1-01-05: Memory performance is specifically related to amyloid and Tau pathology in the entorhinal cortex and hippocampus (2008) (0)
- Early tau pathology in corticobasal degeneration: a morphometric study of 3 preclinical cases (2016) (0)
- Increased Regional Expression of Lingo-1 in the Essential Tremor Cerebellum: A Postmortem, Case-Control Study (S32.003) (2012) (0)
- La leucoencéphalopathie multifocal progressive. (1979) (0)
- Polyglutamine expansion affects huntingtin conformation in multiple Huntington’s disease models (2017) (0)
- Reply (1998) (0)
- Corrigendum to “Regional vulnerability in Huntington's disease: fMRI-guided molecular analysis in patients and a mouse model of disease” [Neurobiol Dis 52 (2013) 84–93] (2013) (0)
- Histopathology of the cerebellar cortex in essential tremor and other neurodegenerative motor disorders: comparative analysis of 320 brains (2023) (0)
- The most significant canonical pathways represented in our validated gene list. (2014) (0)
- 714 Alzheimer's Disease: Variables in clinico-pathological correlations (1996) (0)
- HUNTINGTONʼS DISEASE AND PROGRESSIVE SUPRANUCLEAR PALSY IN AN 80 YEAR-OLD MAN (1996) (0)
- S 22-3 Chronic Traumatic Encephalopathy in Athletes : The Aftermath of Repetitive Head Injuries (2018) (0)
- THE EFFECTS OF POST-MORTEM INTERVAL AND DURATION OF STORAGE AT −80° C OF HUMAN BRAIN TISSUE ON mRNA LEVELS DETECTED BY 'IN SITU' HYBRIDIZATION (1993) (0)
- Cerebellar Pathology in Familial vs. Sporadic Essential Tremor (2017) (0)
- The Neuropathology of Neurodegenerative Dementias (2014) (0)
- Authors' replies to the comments of Koga et al. on "Movement disorders rounds: A case of missing pathology in a patient with LRRK2 Parkinson's disease". (2020) (0)
- Spinal Cord Arterial Blood Supply Determination / Carbon Black Perfusion Study (1999) (0)
- Hierarchical pathological progression of corticobasal degeneration (2017) (0)
- Increased Rate of GBA Variants in MSA Brains at a Brain Bank in New York City (P1.006) (2016) (0)
- A Case of "Prodromal" Huntington's Disease with Neuropathology (P5.296) (2015) (0)
- Unique Astrocyte Pathology in a Case of Rapid Onset Dystonia-Parkinsonism (DYT12) with Novel Mutation in ATP1A3 (P2.137) (2015) (0)
- Clinical and Pathological Characteristics of LRRK2 G2019S Patients with PD (2011) (0)
- Molecular Abnormalities in Sporadic Amyotrophic Lateral Sclerosis (ALS) (2007) (0)
- Reply: More than a co-incidence? Exploring the increased frequency of amyotrophic lateral sclerosis in Huntington disease (2022) (0)
- The integrated neurofunctional MRI exam in ALS (2000) (0)
- Raymond D. Adams (1911–2008) (2010) (0)
- Genetic meta-analysis of diagnosed Alzheimer’s disease identifies new risk loci and implicates Aβ, tau, immunity and lipid processing (2019) (0)
- Case Series: Eosinophilic Vasculitis Isolated to the Central Nervous System (P02.053) (2013) (0)
- P2-223: Lewy body and Alzheimer pathological findings in the same brains: Coincidence or a common diathesis? (2008) (0)
- Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 10-1988. A 73-year-old hypertensive man with intracranial hemorrhages 15 months apart. (1988) (0)
- [Progressive multifocal leukoencephalopathy]. (1979) (0)
- Association Studies of Neuropathologically-Confirmed Cases and Controls Reveals that the PARK10 Locus is a Major Contributor to Sporadic Parkinson disease. (S7.005) (2015) (0)
- α-SYNUCLEIN IMMUNOREACTIVE NEURONAL CYTOPLASMIC INCLUSIONS AND AXONAL SWELLINGS IN NEUROAXONAL DYSTROPHY (1999) (0)
- Classic Parkinson's Disease and Lewy Body Pathology Associated with a Heterozygous PARKIN Dosage Mutation (P05.055) (2013) (0)
- ARCHITECTONIC APPROACHES TO NEUROPIL THREADS IN DEMENTIA (1996) (0)
- Clinical and Pathological Characterization of VPS16 Dystonia (P11-11.005) (2023) (0)
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What Schools Are Affiliated With Jean-paul Gerard Vonsattel?
Jean-paul Gerard Vonsattel is affiliated with the following schools:
