Published in Acta Neuropathol on January 01, 2012
Inflammation and white matter degeneration persist for years after a single traumatic brain injury. Brain (2013) 3.87
Chronic neuropathologies of single and repetitive TBI: substrates of dementia? Nat Rev Neurol (2013) 2.84
Stages of pTDP-43 pathology in amyotrophic lateral sclerosis. Ann Neurol (2013) 1.89
ALS-Plus syndrome: non-pyramidal features in a large ALS cohort. J Neurol Sci (2014) 1.44
Misregulated RNA processing in amyotrophic lateral sclerosis. Brain Res (2012) 1.42
Microglial activation correlates with disease progression and upper motor neuron clinical symptoms in amyotrophic lateral sclerosis. PLoS One (2012) 1.17
A platform for discovery: The University of Pennsylvania Integrated Neurodegenerative Disease Biobank. Alzheimers Dement (2013) 1.15
Sequential distribution of pTDP-43 pathology in behavioral variant frontotemporal dementia (bvFTD). Acta Neuropathol (2014) 1.14
TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord. Acta Neuropathol (2014) 0.96
Promoter DNA methylation regulates progranulin expression and is altered in FTLD. Acta Neuropathol Commun (2013) 0.90
An Assessment of Possible Neuropathology and Clinical Relationships in 46 Sporadic Amyotrophic Lateral Sclerosis Patient Autopsies. Neurodegener Dis (2015) 0.90
Phosphorylated tau as a candidate biomarker for amyotrophic lateral sclerosis. JAMA Neurol (2014) 0.90
Distinct TDP-43 pathology in ALS patients with ataxin 2 intermediate-length polyQ expansions. Acta Neuropathol (2012) 0.89
Chronic Traumatic Encephalopathy: The Neuropathological Legacy of Traumatic Brain Injury. Annu Rev Pathol (2016) 0.87
The established and emerging roles of astrocytes and microglia in amyotrophic lateral sclerosis and frontotemporal dementia. Front Cell Neurosci (2015) 0.86
Narrative discourse deficits in amyotrophic lateral sclerosis. Neurology (2014) 0.84
Widespread structural and functional connectivity changes in amyotrophic lateral sclerosis: insights from advanced neuroimaging research. Neural Plast (2012) 0.83
Topography of FUS pathology distinguishes late-onset BIBD from aFTLD-U. Acta Neuropathol Commun (2013) 0.82
Casein kinase II induced polymerization of soluble TDP-43 into filaments is inhibited by heat shock proteins. PLoS One (2014) 0.80
Examining the relationship between head trauma and neurodegenerative disease: A review of epidemiology, pathology and neuroimaging techniques. J Alzheimers Dis Parkinsonism (2014) 0.80
TDP-43 pathology in the basal forebrain and hypothalamus of patients with amyotrophic lateral sclerosis. Acta Neuropathol Commun (2014) 0.80
Amylin Treatment Reduces Neuroinflammation and Ameliorates Abnormal Patterns of Gene Expression in the Cerebral Cortex of an Alzheimer's Disease Mouse Model. J Alzheimers Dis (2016) 0.77
Inhibition of Endocannabinoid Degradation Improves Outcomes from Mild Traumatic Brain Injury: A Mechanistic Role for Synaptic Hyperexcitability. J Neurotrauma (2016) 0.77
CX3CR1 is a modifying gene of survival and progression in amyotrophic lateral sclerosis. PLoS One (2014) 0.76
Cortical neurons are a prominent source of the proinflammatory cytokine osteopontin in HIV-associated neurocognitive disorders. J Neurovirol (2015) 0.76
Glial TDP-43 regulates axon wrapping, GluRIIA clustering and fly motility by autonomous and non-autonomous mechanisms. Hum Mol Genet (2015) 0.75
"Mini-mental state". A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res (1975) 301.74
Neuropathological stageing of Alzheimer-related changes. Acta Neuropathol (1991) 49.03
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96
Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology (1998) 26.77
The Consortium to Establish a Registry for Alzheimer's Disease (CERAD). Part II. Standardization of the neuropathologic assessment of Alzheimer's disease. Neurology (1991) 23.01
El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord (2000) 15.38
Population-based norms for the Mini-Mental State Examination by age and educational level. JAMA (1993) 12.83
Consensus recommendations for the postmortem diagnosis of Alzheimer's disease. The National Institute on Aging, and Reagan Institute Working Group on Diagnostic Criteria for the Neuropathological Assessment of Alzheimer's Disease. Neurobiol Aging (1997) 10.81
The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci (1999) 9.95
Staging of Alzheimer disease-associated neurofibrillary pathology using paraffin sections and immunocytochemistry. Acta Neuropathol (2006) 8.15
Phases of A beta-deposition in the human brain and its relevance for the development of AD. Neurology (2002) 7.73
Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations. Ann Neurol (2007) 6.58
Recruitment of human muscleblind proteins to (CUG)(n) expansions associated with myotonic dystrophy. EMBO J (2000) 6.56
Consensus recommendations for the postmortem diagnosis of Alzheimer disease from the National Institute on Aging and the Reagan Institute Working Group on diagnostic criteria for the neuropathological assessment of Alzheimer disease. J Neuropathol Exp Neurol (1997) 6.12
Amyotrophic lateral sclerosis. Lancet (2011) 5.86
Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43. Acta Neuropathol (2007) 3.44
Are amyotrophic lateral sclerosis patients cognitively normal? Neurology (2003) 3.21
Cognitive impairment in amyotrophic lateral sclerosis. Lancet Neurol (2007) 3.16
Concomitant TAR-DNA-binding protein 43 pathology is present in Alzheimer disease and corticobasal degeneration but not in other tauopathies. J Neuropathol Exp Neurol (2008) 2.96
Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS. Glia (1998) 2.82
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies. Acta Neuropathol (2009) 2.76
Clinical and pathological continuum of multisystem TDP-43 proteinopathies. Arch Neurol (2009) 2.68
Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies. J Biol Chem (2009) 2.60
TDP-43 regulates retinoblastoma protein phosphorylation through the repression of cyclin-dependent kinase 6 expression. Proc Natl Acad Sci U S A (2008) 2.58
Frontal lobe degeneration: clinical, neuropsychological, and SPECT characteristics. Neurology (1991) 2.43
Evidence of widespread cerebral microglial activation in amyotrophic lateral sclerosis: an [11C](R)-PK11195 positron emission tomography study. Neurobiol Dis (2004) 2.29
Inflammatory processes in amyotrophic lateral sclerosis. Muscle Nerve (2002) 2.25
Frontal behavioral inventory: diagnostic criteria for frontal lobe dementia. Can J Neurol Sci (1997) 2.23
Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43. Hum Mol Genet (2009) 2.23
Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: a review. Brain (1981) 2.15
Immune reactivity in a mouse model of familial ALS correlates with disease progression. Neurology (2001) 2.01
Diagnostic utility of abbreviated fluency measures in Alzheimer disease and vascular dementia. Neurology (2004) 1.85
Minocycline slows disease progression in a mouse model of amyotrophic lateral sclerosis. Neurobiol Dis (2002) 1.81
Intracellular signaling in M-CSF-induced microglia activation: role of Iba1. Glia (2002) 1.80
Involvement of Iba1 in membrane ruffling and phagocytosis of macrophages/microglia. J Cell Sci (2000) 1.74
Molecular pathology and genetic advances in amyotrophic lateral sclerosis: an emerging molecular pathway and the significance of glial pathology. Acta Neuropathol (2011) 1.58
Potentiation of amyotrophic lateral sclerosis (ALS)-associated TDP-43 aggregation by the proteasome-targeting factor, ubiquilin 1. J Biol Chem (2008) 1.58
Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP-43 proteinopathies. Neuropathology (2010) 1.53
Cyclooxygenase 2 inhibition protects motor neurons and prolongs survival in a transgenic mouse model of ALS. Ann Neurol (2002) 1.52
Lymphocytic infiltrates in the spinal cord in amyotrophic lateral sclerosis. Arch Neurol (1993) 1.51
TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases. J Biol Chem (2009) 1.44
NG2 cells generate oligodendrocytes and gray matter astrocytes in the spinal cord. Neuron Glia Biol (2008) 1.42
Risk genotypes at TMEM106B are associated with cognitive impairment in amyotrophic lateral sclerosis. Acta Neuropathol (2010) 1.38
Presenile dementia combined with amyotrophy: a review of 34 Japanese cases. Arch Gerontol Geriatr (1987) 1.38
Sequence of Abeta-protein deposition in the human medial temporal lobe. J Neuropathol Exp Neurol (2000) 1.37
Pathological 43-kDa transactivation response DNA-binding protein in older adults with and without severe mental illness. Arch Neurol (2010) 1.29
Frontotemporal white matter changes in amyotrophic lateral sclerosis. J Neurol (2005) 1.27
Survival profiles of patients with frontotemporal dementia and motor neuron disease. Arch Neurol (2009) 1.26
Screening for frontotemporal dementias and Alzheimer's disease with the Philadelphia Brief Assessment of Cognition: a preliminary analysis. Dement Geriatr Cogn Disord (2007) 1.26
Classic amyotrophic lateral sclerosis with dementia. Arch Neurol (1982) 1.26
Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration. Brain (2010) 1.24
The effects of executive and behavioral dysfunction on the course of ALS. Neurology (2005) 1.15
A prospective study of cognitive impairment in ALS. Neurology (1999) 1.08
Can frontotemporal dementia and Alzheimer's disease be differentiated using a brief battery of tests? Int J Geriatr Psychiatry (1997) 1.05
Phosphorylated and cleaved TDP-43 in ALS, FTLD and other neurodegenerative disorders and in cellular models of TDP-43 proteinopathy. Neuropathology (2010) 1.04
Amyotrophic lateral sclerosis with dementia: the clinicopathological spectrum. Neuropathology (2004) 1.02
Progression of hippocampal degeneration in amyotrophic lateral sclerosis with or without memory impairment: distinction from Alzheimer disease. Acta Neuropathol (2008) 0.99
Alzheimer disease pathology in amyotrophic lateral sclerosis. Acta Neuropathol (2004) 0.94
[A clinicopathological study on 13 cases of motor neuron disease with dementia]. Rinsho Shinkeigaku (1992) 0.92
Presence of reactive microglia and neuroinflammatory mediators in a case of frontotemporal dementia with P301S mutation. Neurodegener Dis (2011) 0.84
Cognitive impairment in sporadic ALS: a pathologic continuum underlying a multisystem disorder. Neurology (2001) 0.82
Longitudinal cortical atrophy in amyotrophic lateral sclerosis with frontotemporal dementia. Arch Neurol (2009) 0.78
Medial temporal lobe metabolic impairment in dementia associated with motor neuron disease. J Neurol Sci (1999) 0.77
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96
Hypothetical model of dynamic biomarkers of the Alzheimer's pathological cascade. Lancet Neurol (2010) 19.42
Cerebrospinal fluid biomarker signature in Alzheimer's disease neuroimaging initiative subjects. Ann Neurol (2009) 14.42
A roadmap for the prevention of dementia II: Leon Thal Symposium 2008. Alzheimers Dement (2009) 10.21
Common variants at MS4A4/MS4A6E, CD2AP, CD33 and EPHA1 are associated with late-onset Alzheimer's disease. Nat Genet (2011) 10.07
Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain (2011) 9.90
Tracking pathophysiological processes in Alzheimer's disease: an updated hypothetical model of dynamic biomarkers. Lancet Neurol (2013) 9.43
Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol (2007) 8.10
Tau-mediated neurodegeneration in Alzheimer's disease and related disorders. Nat Rev Neurosci (2007) 7.96
Ways toward an early diagnosis in Alzheimer's disease: the Alzheimer's Disease Neuroimaging Initiative (ADNI). Alzheimers Dement (2005) 7.90
Exome sequencing reveals VCP mutations as a cause of familial ALS. Neuron (2010) 7.87
The Alzheimer's disease neuroimaging initiative. Neuroimaging Clin N Am (2005) 7.26
Pathological α-synuclein transmission initiates Parkinson-like neurodegeneration in nontransgenic mice. Science (2012) 6.66
Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations. Ann Neurol (2007) 6.58
Cognitive therapy for the prevention of suicide attempts: a randomized controlled trial. JAMA (2005) 6.23
Chaperone suppression of alpha-synuclein toxicity in a Drosophila model for Parkinson's disease. Science (2001) 6.15
Synapse loss and microglial activation precede tangles in a P301S tauopathy mouse model. Neuron (2007) 6.07
National Institute on Aging-Alzheimer's Association guidelines for the neuropathologic assessment of Alzheimer's disease: a practical approach. Acta Neuropathol (2011) 6.00
Diagnosis-independent Alzheimer disease biomarker signature in cognitively normal elderly people. Arch Neurol (2010) 5.53
Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions. Nat Genet (2010) 5.52
TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis. Lancet Neurol (2008) 5.33
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS. Nature (2010) 5.26
Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study. Lancet Neurol (2012) 5.18
Frontotemporal dementia: clinicopathological correlations. Ann Neurol (2006) 5.14
National Institute on Aging-Alzheimer's Association guidelines for the neuropathologic assessment of Alzheimer's disease. Alzheimers Dement (2012) 5.10
TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions. Am J Pathol (2007) 5.02
Neuronal alpha-synucleinopathy with severe movement disorder in mice expressing A53T human alpha-synuclein. Neuron (2002) 4.77
Exogenous α-synuclein fibrils induce Lewy body pathology leading to synaptic dysfunction and neuron death. Neuron (2011) 4.74
Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol (2009) 4.73
Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations. Acta Neuropathol (2008) 4.73
Brain beta-amyloid measures and magnetic resonance imaging atrophy both predict time-to-progression from mild cognitive impairment to Alzheimer's disease. Brain (2010) 4.39
Pathological heterogeneity of frontotemporal lobar degeneration with ubiquitin-positive inclusions delineated by ubiquitin immunohistochemistry and novel monoclonal antibodies. Am J Pathol (2006) 4.32
The Alzheimer's Disease Neuroimaging Initiative: a review of papers published since its inception. Alzheimers Dement (2011) 4.25
A harmonized classification system for FTLD-TDP pathology. Acta Neuropathol (2011) 4.15
Biomarkers of neurodegeneration for diagnosis and monitoring therapeutics. Nat Rev Drug Discov (2007) 4.03
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS. Nature (2013) 4.03
Intracerebral inoculation of pathological α-synuclein initiates a rapidly progressive neurodegenerative α-synucleinopathy in mice. J Exp Med (2012) 3.98
Neurodegenerative diseases: a decade of discoveries paves the way for therapeutic breakthroughs. Nat Med (2004) 3.93
Inflammation and white matter degeneration persist for years after a single traumatic brain injury. Brain (2013) 3.87
The Alzheimer's disease neuroimaging initiative: progress report and future plans. Alzheimers Dement (2010) 3.85
Correlation of Alzheimer disease neuropathologic changes with cognitive status: a review of the literature. J Neuropathol Exp Neurol (2012) 3.77
Initiation and synergistic fibrillization of tau and alpha-synuclein. Science (2003) 3.76
Primary age-related tauopathy (PART): a common pathology associated with human aging. Acta Neuropathol (2014) 3.75
Cerebrospinal fluid tau and beta-amyloid: how well do these biomarkers reflect autopsy-confirmed dementia diagnoses? Arch Neurol (2003) 3.75
Demonstrated brain insulin resistance in Alzheimer's disease patients is associated with IGF-1 resistance, IRS-1 dysregulation, and cognitive decline. J Clin Invest (2012) 3.65
Plasma multianalyte profiling in mild cognitive impairment and Alzheimer disease. Neurology (2012) 3.58
Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation. J Biol Chem (2008) 3.56
Co-morbidity of TDP-43 proteinopathy in Lewy body related diseases. Acta Neuropathol (2007) 3.55
Exogenous alpha-synuclein fibrils seed the formation of Lewy body-like intracellular inclusions in cultured cells. Proc Natl Acad Sci U S A (2009) 3.47
Imaging of tau pathology in a tauopathy mouse model and in Alzheimer patients compared to normal controls. Neuron (2013) 3.27
Distinct α-synuclein strains differentially promote tau inclusions in neurons. Cell (2013) 3.24
Prediction of MCI to AD conversion, via MRI, CSF biomarkers, and pattern classification. Neurobiol Aging (2010) 3.16
Biochemical and pathological characterization of Lrrk2. Ann Neurol (2006) 3.14
Clinical Core of the Alzheimer's Disease Neuroimaging Initiative: progress and plans. Alzheimers Dement (2010) 3.11
Cerebrospinal fluid profile in frontotemporal dementia and Alzheimer's disease. Ann Neurol (2005) 3.09
Criteria for the diagnosis of corticobasal degeneration. Neurology (2013) 3.07
Neurodegenerative diseases: new concepts of pathogenesis and their therapeutic implications. Annu Rev Pathol (2006) 3.01
Biological markers for therapeutic trials in Alzheimer's disease. Proceedings of the biological markers working group; NIA initiative on neuroimaging in Alzheimer's disease. Neurobiol Aging (2003) 2.96
Concomitant TAR-DNA-binding protein 43 pathology is present in Alzheimer disease and corticobasal degeneration but not in other tauopathies. J Neuropathol Exp Neurol (2008) 2.96
Synthetic tau fibrils mediate transmission of neurofibrillary tangles in a transgenic mouse model of Alzheimer's-like tauopathy. J Neurosci (2013) 2.90
Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration. Nat Rev Neurosci (2011) 2.84
Evidence for ordering of Alzheimer disease biomarkers. Arch Neurol (2011) 2.83
TAR DNA-binding protein 43 in neurodegenerative disease. Nat Rev Neurol (2010) 2.81
Repetitive mild brain trauma accelerates Abeta deposition, lipid peroxidation, and cognitive impairment in a transgenic mouse model of Alzheimer amyloidosis. J Neurosci (2002) 2.80
Dopamine neurons implanted into people with Parkinson's disease survive without pathology for 14 years. Nat Med (2008) 2.80
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies. Acta Neuropathol (2009) 2.76
Cytoskeletal modulators and pleiotropic strategies for Alzheimer drug discovery. Alzheimers Dement (2006) 2.73
Novel antibodies to synuclein show abundant striatal pathology in Lewy body diseases. Ann Neurol (2002) 2.70
Clinical and pathological continuum of multisystem TDP-43 proteinopathies. Arch Neurol (2009) 2.68
A yeast functional screen predicts new candidate ALS disease genes. Proc Natl Acad Sci U S A (2011) 2.68
Mechanisms of Parkinson's disease linked to pathological alpha-synuclein: new targets for drug discovery. Neuron (2006) 2.66
The natural history of multiple system atrophy: a prospective European cohort study. Lancet Neurol (2013) 2.65