Published in Neurology on October 26, 2004
Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol (2007) 8.10
TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions. Am J Pathol (2007) 5.02
HDDD2 is a familial frontotemporal lobar degeneration with ubiquitin-positive, tau-negative inclusions caused by a missense mutation in the signal peptide of progranulin. Ann Neurol (2006) 3.00
Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease. Acta Neuropathol (2009) 2.68
FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathol (2010) 2.30
Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol (2011) 2.17
Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes. Acta Neuropathol (2009) 1.89
Frontotemporal lobar degeneration: epidemiology, pathophysiology, diagnosis and management. CNS Drugs (2010) 1.72
Neuropsychological decline in frontotemporal lobar degeneration: a longitudinal analysis. Neuropsychology (2009) 1.35
Neuropathology of non-Alzheimer degenerative disorders. Int J Clin Exp Pathol (2009) 1.27
Frequency of ubiquitin and FUS-positive, TDP-43-negative frontotemporal lobar degeneration. J Neurol (2009) 1.22
The role of FUS gene variants in neurodegenerative diseases. Nat Rev Neurol (2014) 1.04
Imaging signatures of molecular pathology in behavioral variant frontotemporal dementia. J Mol Neurosci (2011) 1.03
Neuroimaging in frontotemporal lobar degeneration--predicting molecular pathology. Nat Rev Neurol (2012) 0.99
PRKAR1B mutations are a rare cause of FUS negative neuronal intermediate filament inclusion disease. Brain (2014) 0.97
Caudate atrophy on MRI is a characteristic feature of FTLD-FUS. Eur J Neurol (2010) 0.97
Clinicopathological and genetic correlates of frontotemporal lobar degeneration and corticobasal degeneration. J Neurol (2008) 0.91
Neuronal intranuclear inclusions are ultrastructurally and immunologically distinct from cytoplasmic inclusions of neuronal intermediate filament inclusion disease. Acta Neuropathol (2005) 0.90
Early limited nitrosamine exposures exacerbate high fat diet-mediated type 2 diabetes and neurodegeneration. BMC Endocr Disord (2010) 0.89
FUS immunogold labeling TEM analysis of the neuronal cytoplasmic inclusions of neuronal intermediate filament inclusion disease: a frontotemporal lobar degeneration with FUS proteinopathy. J Mol Neurosci (2011) 0.88
The spectrum and severity of FUS-immunoreactive inclusions in the frontal and temporal lobes of ten cases of neuronal intermediate filament inclusion disease. Acta Neuropathol (2010) 0.87
Different molecular pathologies result in similar spatial patterns of cellular inclusions in neurodegenerative disease: a comparative study of eight disorders. J Neural Transm (Vienna) (2012) 0.83
Impact of very old age on the expression of cervical spinal cord cell markers in rats. J Chem Neuroanat (2008) 0.83
Corticospinal tract degeneration associated with TDP-43 type C pathology and semantic dementia. Brain (2013) 0.82
Nuclear carrier and RNA-binding proteins in frontotemporal lobar degeneration associated with fused in sarcoma (FUS) pathological changes. Neuropathol Appl Neurobiol (2013) 0.82
Neuropathological changes in ten cases of neuronal intermediate filament inclusion disease (NIFID): a study using alpha-internexin immunohistochemistry and principal components analysis (PCA). J Neural Transm (Vienna) (2005) 0.81
Composition of Rosenthal Fibers, the Protein Aggregate Hallmark of Alexander Disease. J Proteome Res (2016) 0.80
Parkinsonism, movement disorders and genetics in frontotemporal dementia. Nat Rev Neurol (2016) 0.79
Anti-alpha-internexin autoantibody from neuropsychiatric lupus induce cognitive damage via inhibiting axonal elongation and promote neuron apoptosis. PLoS One (2010) 0.79
Comparative quantitative study of 'signature' pathological lesions in the hippocampus and adjacent gyri of 12 neurodegenerative disorders. J Neural Transm (Vienna) (2015) 0.78
Spatial patterns of the pathological changes in neuronal intermediate filament inclusion disease (NIFID): an alpha-internexin immunohistochemical study. J Neural Transm (Vienna) (2006) 0.78
Spatial patterns of FUS-immunoreactive neuronal cytoplasmic inclusions (NCI) in neuronal intermediate filament inclusion disease (NIFID). J Neural Transm (Vienna) (2011) 0.77
Neuronal degeneration in autonomic nervous system of Dystonia musculorum mice. J Biomed Sci (2011) 0.76
[Molecular neuropathology of Non-Alzheimer dementia]. Pathologe (2008) 0.75
NIFID: a new molecular pathology with a frontotemporal dementia phenotype. Neurology (2004) 0.75
Fronto-temporal lobar degeneration: neuropathology in 60 cases. J Neural Transm (Vienna) (2011) 0.75
Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology (1998) 26.77
Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol (2003) 3.22
Frontal lobe dementia and motor neuron disease. J Neurol Neurosurg Psychiatry (1990) 2.91
Novel antibodies to synuclein show abundant striatal pathology in Lewy body diseases. Ann Neurol (2002) 2.70
Two-stage expression of neurofilament polypeptides during rat neurogenesis with early establishment of adult phosphorylation patterns. J Neurosci (1987) 2.15
Epitope map of neurofilament protein domains in cortical and peripheral nervous system Lewy bodies. Am J Pathol (1991) 1.86
Neuronal intermediate filaments. Annu Rev Neurosci (1996) 1.78
Familial frontotemporal dementia with ubiquitin-positive, tau-negative inclusions. Neurology (2000) 1.76
Phosphate dependent and independent neurofilament epitopes in the axonal swellings of patients with motor neuron disease and controls. Lab Invest (1987) 1.73
New ubiquitin-positive intraneuronal inclusions in the extra-motor cortices in patients with amyotrophic lateral sclerosis. Neurosci Lett (1991) 1.60
Neurofilament inclusion body disease: a new proteinopathy? Brain (2003) 1.59
alpha-internexin is present in the pathological inclusions of neuronal intermediate filament inclusion disease. Am J Pathol (2004) 1.53
Neurofilaments and neurological disease. Bioessays (2003) 1.50
alpha-Internexin aggregates are abundant in neuronal intermediate filament inclusion disease (NIFID) but rare in other neurodegenerative diseases. Acta Neuropathol (2004) 1.43
Phosphorylation of neurofilaments is altered in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol (1988) 1.40
Alzheimer disease tangles share immunological similarities with multiphosphorylation repeats in the two large neurofilament proteins. Proc Natl Acad Sci U S A (1988) 1.35
Frontotemporal and motor neurone degeneration with neurofilament inclusion bodies: additional evidence for overlap between FTD and ALS. Neuropathol Appl Neurobiol (2003) 1.29
Phosphorylation protects neurofilaments against proteolysis. J Neuroimmunol (1987) 1.27
Patients with a novel neurofilamentopathy: dementia with neurofilament inclusions. Neurosci Lett (2003) 1.27
Comparative epitope analysis of neuronal cytoskeletal proteins in Alzheimer's disease senile plaque neurites and neuropil threads. Lab Invest (1991) 1.27
Overexpression of alpha-internexin causes abnormal neurofilamentous accumulations and motor coordination deficits in transgenic mice. J Neurosci (1999) 1.19
Cytoskeletal abnormalities in motor neuron disease. An immunocytochemical study. Brain (1989) 1.10
Expression of the gene for the neuronal intermediate filament protein alpha-internexin coincides with the onset of neuronal differentiation in the developing rat nervous system. J Comp Neurol (1994) 1.09
Roles of head and tail domains in alpha-internexin's self-assembly and coassembly with the neurofilament triplet proteins. J Cell Sci (1998) 1.02
Relative abundance of tau and neurofilament epitopes in hippocampal neurofibrillary tangles. Am J Pathol (1990) 0.99
Aggregation of intermediate filaments by 2,5-hexanedione: comparison of effects on neurofilaments, GFAP-filaments and vimentin-filaments in dissociated cultures of mouse spinal cord-dorsal root ganglia. J Neuropathol Exp Neurol (1988) 0.98
Phosphorylation state of the native high-molecular-weight neurofilament subunit protein from cervical spinal cord in sporadic amyotrophic lateral sclerosis. J Neurochem (2001) 0.98
Structure of the gene for the neuronal intermediate filament protein alpha-internexin and functional analysis of its promoter. J Biol Chem (1991) 0.96
Neurofilament inclusion body disease with early onset frontotemporal dementia and primary lateral sclerosis. Clin Neuropathol (2004) 0.93
A case of Pick's disease with unusual neuronal inclusions. Acta Neuropathol (1994) 0.93
Neurofilament-rich intraneuronal inclusions exacerbate neurodegenerative sequelae of brain trauma in NFH/LacZ transgenic mice. Exp Neurol (2000) 0.89
Utilizing the peptidyl-prolyl cis-trans isomerase pin1 as a probe of its phosphorylated target proteins. Examples of binding to nuclear proteins in a human kidney cell line and to tau in Alzheimer's diseased brain. J Histochem Cytochem (2001) 0.89
Neuropathology of amyotrophic lateral sclerosis with extra-motor system degeneration: characteristics and differences in the molecular pathology between ALS with dementia and Guamanian ALS. Amyotroph Lateral Scler Other Motor Neuron Disord (2000) 0.83
Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology (2005) 21.67
Alpha-synuclein in Lewy bodies. Nature (1997) 20.83
Symmetric diffeomorphic image registration with cross-correlation: evaluating automated labeling of elderly and neurodegenerative brain. Med Image Anal (2007) 13.54
Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. Neurology (1996) 12.33
Classification of primary progressive aphasia and its variants. Neurology (2011) 11.82
Clinical and pathological diagnosis of frontotemporal dementia: report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol (2001) 9.96
Neurodegenerative tauopathies. Annu Rev Neurosci (2001) 9.53
Transplantation of embryonic dopamine neurons for severe Parkinson's disease. N Engl J Med (2001) 9.51
The FAB: a Frontal Assessment Battery at bedside. Neurology (2000) 8.93
Second consensus statement on the diagnosis of multiple system atrophy. Neurology (2008) 7.27
Alzheimer's Disease Neuroimaging Initiative (ADNI): clinical characterization. Neurology (2009) 7.04
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
Clinicopathologic studies in cognitively healthy aging and Alzheimer's disease: relation of histologic markers to dementia severity, age, sex, and apolipoprotein E genotype. Arch Neurol (1998) 5.93
Comparing predictors of conversion and decline in mild cognitive impairment. Neurology (2010) 5.69
Oxidative damage linked to neurodegeneration by selective alpha-synuclein nitration in synucleinopathy lesions. Science (2000) 5.62
A68: a major subunit of paired helical filaments and derivatized forms of normal Tau. Science (1991) 5.60
Aggregation of alpha-synuclein in Lewy bodies of sporadic Parkinson's disease and dementia with Lewy bodies. Am J Pathol (1998) 5.47
MRI of hippocampal volume loss in early Alzheimer's disease in relation to ApoE genotype and biomarkers. Brain (2009) 4.51
Relationships between biomarkers in aging and dementia. Neurology (2009) 4.16
Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol (1996) 4.10
Mutation-specific functional impairments in distinct tau isoforms of hereditary FTDP-17. Science (1998) 4.07
The neural basis of the central executive system of working memory. Nature (1995) 4.03
A hydrophobic stretch of 12 amino acid residues in the middle of alpha-synuclein is essential for filament assembly. J Biol Chem (2000) 3.97
Complications of gamma knife surgery for Parkinson disease. Arch Neurol (2001) 3.97
Chlamydial infections. Annu Rev Med (1981) 3.94
Synucleins are developmentally expressed, and alpha-synuclein regulates the size of the presynaptic vesicular pool in primary hippocampal neurons. J Neurosci (2000) 3.90
Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron (1997) 3.73
MRI and CSF biomarkers in normal, MCI, and AD subjects: predicting future clinical change. Neurology (2009) 3.72
Correlation of cholinergic abnormalities with senile plaques and mental test scores in senile dementia. Br Med J (1978) 3.59
In vivo confocal scanning laser microscopy of human skin: melanin provides strong contrast. J Invest Dermatol (1995) 3.53
Age-dependent emergence and progression of a tauopathy in transgenic mice overexpressing the shortest human tau isoform. Neuron (1999) 3.49
Abnormal tau phosphorylation at Ser396 in Alzheimer's disease recapitulates development and contributes to reduced microtubule binding. Neuron (1993) 3.45
Behavioural disorders, Parkinson's disease and subthalamic stimulation. J Neurol Neurosurg Psychiatry (2002) 3.41
Depressive symptoms, cognitive decline, and risk of AD in older persons. Neurology (2002) 3.40
Education modifies the relation of AD pathology to level of cognitive function in older persons. Neurology (2003) 3.35
Epitopes that span the tau molecule are shared with paired helical filaments. Neuron (1988) 3.28
Induction of myelination in the central nervous system by electrical activity. Proc Natl Acad Sci U S A (1996) 3.06
Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol (2002) 3.02
Increased lipid peroxidation precedes amyloid plaque formation in an animal model of Alzheimer amyloidosis. J Neurosci (2001) 2.89
DLB and PDD boundary issues: diagnosis, treatment, molecular pathology, and biomarkers. Neurology (2007) 2.86
MRI and CSF biomarkers in normal, MCI, and AD subjects: diagnostic discrimination and cognitive correlations. Neurology (2009) 2.83
Prospective study of chlamydial infection in neonates. Lancet (1979) 2.80
Positron emission tomography studies on the dopaminergic system and striatal opioid binding in the olivopontocerebellar atrophy variant of multiple system atrophy. Ann Neurol (1995) 2.76
Histopathologic findings of duodenal biopsy specimens in HIV-infected patients with and without diarrhea and malabsorption. Am J Clin Pathol (1992) 2.74
The levels of soluble versus insoluble brain Abeta distinguish Alzheimer's disease from normal and pathologic aging. Exp Neurol (1999) 2.73
CSF amyloid {beta} 1-42 predicts cognitive decline in Parkinson disease. Neurology (2010) 2.67
Glucocerebrosidase mutations are an important risk factor for Lewy body disorders. Neurology (2006) 2.65
Staging and natural history of cerebrovascular pathology in dementia. Neurology (2012) 2.61
Amyloid vs FDG-PET in the differential diagnosis of AD and FTLD. Neurology (2011) 2.61
Epstein-Barr virus in AIDS-related primary central nervous system lymphoma. Lancet (1991) 2.60
Relation of cerebral infarctions to dementia and cognitive function in older persons. Neurology (2003) 2.60
Genetic evidence for the involvement of tau in progressive supranuclear palsy. Ann Neurol (1997) 2.60
Cytokine expression in the brain during the acquired immunodeficiency syndrome. Ann Neurol (1992) 2.52
Cognitive deficits in Parkinson's disease. J Neurol (1997) 2.49
Adenovirus-mediated transfer of the CFTR gene to lung of nonhuman primates: biological efficacy study. Hum Gene Ther (1993) 2.48
Diabetes is related to cerebral infarction but not to AD pathology in older persons. Neurology (2006) 2.48
Glial cytoplasmic inclusions in white matter oligodendrocytes of multiple system atrophy brains contain insoluble alpha-synuclein. Ann Neurol (1998) 2.47
Cholinergic correlates of cognitive impairment in Parkinson's disease: comparisons with Alzheimer's disease. J Neurol Neurosurg Psychiatry (1985) 2.47
Chromosome 9p-linked families with frontotemporal dementia associated with motor neuron disease. Neurology (2009) 2.46
Cerebral thrombotic microangiopathy and antiphospholipid antibodies in Aicardi-Goutieres syndrome--report of two sisters. Neuropediatrics (2005) 2.44
CSF biomarkers in frontotemporal lobar degeneration with known pathology. Neurology (2008) 2.43
Most cases of dementia with hippocampal sclerosis may represent frontotemporal dementia. Neurology (2004) 2.42
Large amounts of neocortical beta A4 deposits without neuritic plaques nor tangles in a psychometrically assessed, non-demented person. Neurosci Lett (1990) 2.40
Mitochondrial DNA variants observed in Alzheimer disease and Parkinson disease patients. Genomics (1993) 2.39
Synucleinopathies: clinical and pathological implications. Arch Neurol (2001) 2.36
Assessment of cerebral blood flow in Alzheimer's disease by spin-labeled magnetic resonance imaging. Ann Neurol (2000) 2.35
Interlaboratory comparison of neuropathology assessments in Alzheimer's disease: a study of the Consortium to Establish a Registry for Alzheimer's Disease (CERAD) J Neuropathol Exp Neurol (1994) 2.34
Chronic distress and incidence of mild cognitive impairment. Neurology (2007) 2.33
Apolipoprotein E epsilon4 allele, AD pathology, and the clinical expression of Alzheimer's disease. Neurology (2003) 2.32
Nestin expression in embryonic human neuroepithelium and in human neuroepithelial tumor cells. Lab Invest (1992) 2.30
Axon pathology in Parkinson's disease and Lewy body dementia hippocampus contains alpha-, beta-, and gamma-synuclein. Proc Natl Acad Sci U S A (1999) 2.30
The mental representation of hand movements after parietal cortex damage. Science (1996) 2.28
D2 dopamine receptor gene (DRD2) Taq1 A polymorphism: reduced dopamine D2 receptor binding in the human striatum associated with the A1 allele. Pharmacogenetics (1997) 2.28
Alpha-synuclein cortical Lewy bodies correlate with dementia in Parkinson's disease. Neurology (2000) 2.27
The neural system that bridges reward and cognition in humans: an fMRI study. Proc Natl Acad Sci U S A (2002) 2.26
Localization of HIV-1 in human brain using polymerase chain reaction/in situ hybridization and immunocytochemistry. Ann Neurol (1996) 2.22
Single-cell expression profiling of dopaminergic neurons combined with association analysis identifies pyridoxal kinase as Parkinson's disease gene. Ann Neurol (2009) 2.21
Demented and nondemented patients with AIDS differ in brain-derived human immunodeficiency virus type 1 envelope sequences. J Virol (1994) 2.21
Serial MRI and CSF biomarkers in normal aging, MCI, and AD. Neurology (2010) 2.20
Endothelin stimulates phospholipase C, Na+/H+ exchange, c-fos expression, and mitogenesis in rat mesangial cells. J Clin Invest (1989) 2.17
Induction of alpha-synuclein aggregation by intracellular nitrative insult. J Neurosci (2001) 2.16
Haplotypes and gene expression implicate the MAPT region for Parkinson disease: the GenePD Study. Neurology (2008) 2.16
Two-stage expression of neurofilament polypeptides during rat neurogenesis with early establishment of adult phosphorylation patterns. J Neurosci (1987) 2.15
Mutant and wild type human alpha-synucleins assemble into elongated filaments with distinct morphologies in vitro. J Biol Chem (1999) 2.13
Pathogenic implications of mutations in the tau gene in pallido-ponto-nigral degeneration and related neurodegenerative disorders linked to chromosome 17. Proc Natl Acad Sci U S A (1998) 2.13
Biopsy-derived adult human brain tau is phosphorylated at many of the same sites as Alzheimer's disease paired helical filament tau. Neuron (1994) 2.13
Targeted neuronal death affects neuronal replacement and vocal behavior in adult songbirds. Neuron (2000) 2.12
Long-term improvement of hypercholesterolemia after ex vivo gene therapy in LDLR-deficient rabbits. Science (1991) 2.11