K A Josephs

Summary

Affiliation: Mayo Clinic
Country: USA

Publications

  1. ncbi request reprint Whole-body tremulousness: isolated generalized polymyoclonus
    Andrew McKeon
    Department of Neurology, Gonda 8 South, Mayo Clinic, 200 First St SW, Rochester, MN 55902, USA
    Arch Neurol 64:1318-22. 2007
  2. ncbi request reprint Unusual compulsive behaviors primarily related to dopamine agonist therapy in Parkinson's disease and multiple system atrophy
    Andrew McKeon
    Department of Neurology, Mayo Clinic, 200 1st St SW, Rochester, MN 55905, USA
    Parkinsonism Relat Disord 13:516-9. 2007
  3. ncbi request reprint Atypical progressive supranuclear palsy with corticospinal tract degeneration
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
    J Neuropathol Exp Neurol 65:396-405. 2006
  4. pmc Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech
    Keith A Josephs
    Department of Neurology, Division of Movement Disorders and Behavioral Neurology, Mayo Clinic, Rochester, MN 55905
    Brain 129:1385-98. 2006
  5. ncbi request reprint Benign tremulous parkinsonism
    Keith A Josephs
    Division of Movement Disorders, Department of Neurology, Mayo Clinic, Rochester, Minn, USA
    Arch Neurol 63:354-7. 2006
  6. pmc Progranulin-associated PiB-negative logopenic primary progressive aphasia
    Keith A Josephs
    Department of Neurology Division of Behavioral Neurology, Mayo Clinic, Rochester, MN, USA
    J Neurol 261:604-14. 2014
  7. pmc Staging TDP-43 pathology in Alzheimer's disease
    Keith A Josephs
    Division of Behavioral Neurology, Department of Neurology, Mayo Clinic, Rochester, MN, 55905, USA
    Acta Neuropathol 127:441-50. 2014
  8. pmc Quantitative neurofibrillary tangle density and brain volumetric MRI analyses in Alzheimer's disease presenting as logopenic progressive aphasia
    Keith A Josephs
    Department of Neurology Behavioral Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, United States Department of Neurology Movement Disorders, Mayo Clinic, 200 First St SW, Rochester, MN 55905, United States Electronic address
    Brain Lang 127:127-34. 2013
  9. doi request reprint Modeling trajectories of regional volume loss in progressive supranuclear palsy
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA
    Mov Disord 28:1117-24. 2013
  10. pmc Occupational differences between Alzheimer's and aphasic dementias: implication for teachers
    Keith A Josephs
    Department of Neurology Behavioral Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Am J Alzheimers Dis Other Demen 28:612-6. 2013

Research Grants

Detail Information

Publications136 found, 100 shown here

  1. ncbi request reprint Whole-body tremulousness: isolated generalized polymyoclonus
    Andrew McKeon
    Department of Neurology, Gonda 8 South, Mayo Clinic, 200 First St SW, Rochester, MN 55902, USA
    Arch Neurol 64:1318-22. 2007
    ..Acquired generalized repetitive myoclonus may be mistaken for tremor. Distinguishing myoclonus has etiologic and therapeutic implications...
  2. ncbi request reprint Unusual compulsive behaviors primarily related to dopamine agonist therapy in Parkinson's disease and multiple system atrophy
    Andrew McKeon
    Department of Neurology, Mayo Clinic, 200 1st St SW, Rochester, MN 55905, USA
    Parkinsonism Relat Disord 13:516-9. 2007
    ....
  3. ncbi request reprint Atypical progressive supranuclear palsy with corticospinal tract degeneration
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
    J Neuropathol Exp Neurol 65:396-405. 2006
    ..The clinicopathologic features of these 12 cases expand the spectrum of 4R tauopathies...
  4. pmc Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech
    Keith A Josephs
    Department of Neurology, Division of Movement Disorders and Behavioral Neurology, Mayo Clinic, Rochester, MN 55905
    Brain 129:1385-98. 2006
    ..Refining the classification of the degenerative aphasias and AOS may be necessary to improve our understanding of the relationships among behavioural, pathological and imaging correlations...
  5. ncbi request reprint Benign tremulous parkinsonism
    Keith A Josephs
    Division of Movement Disorders, Department of Neurology, Mayo Clinic, Rochester, Minn, USA
    Arch Neurol 63:354-7. 2006
    ..Benign tremulous parkinsonism has never been precisely defined nor has the long-term course been studied...
  6. pmc Progranulin-associated PiB-negative logopenic primary progressive aphasia
    Keith A Josephs
    Department of Neurology Division of Behavioral Neurology, Mayo Clinic, Rochester, MN, USA
    J Neurol 261:604-14. 2014
    ..Identification of such patients, however, should prompt testing for GRN mutations, since GRN-positive patients do not have distinctive features, yet account for 50 % of this patient population...
  7. pmc Staging TDP-43 pathology in Alzheimer's disease
    Keith A Josephs
    Division of Behavioral Neurology, Department of Neurology, Mayo Clinic, Rochester, MN, 55905, USA
    Acta Neuropathol 127:441-50. 2014
    ..Given these findings, we recommend sequential regional TDP-43 screening in AD beginning with the amygdala. ..
  8. pmc Quantitative neurofibrillary tangle density and brain volumetric MRI analyses in Alzheimer's disease presenting as logopenic progressive aphasia
    Keith A Josephs
    Department of Neurology Behavioral Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, United States Department of Neurology Movement Disorders, Mayo Clinic, 200 First St SW, Rochester, MN 55905, United States Electronic address
    Brain Lang 127:127-34. 2013
    ..This study demonstrates that lvPPA is associated with a phenomenon of enhanced temporoparietal neurodegeneration, a finding that improves our understanding of the biological basis of lvPPA. ..
  9. doi request reprint Modeling trajectories of regional volume loss in progressive supranuclear palsy
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA
    Mov Disord 28:1117-24. 2013
    ..Decline is mainly linear but can be nonlinear for some regions. The frontal lobe and midbrain seem to be playing the most significant roles in the progressive worsening of clinical signs in progressive supranuclear palsy...
  10. pmc Occupational differences between Alzheimer's and aphasic dementias: implication for teachers
    Keith A Josephs
    Department of Neurology Behavioral Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Am J Alzheimers Dis Other Demen 28:612-6. 2013
    ..We identified an association between progressive SLDs and the occupation of teaching. Since teaching is a communication demanding occupation, teachers may be more sensitive to the development of speech and language impairments. ..
  11. pmc Syndromes dominated by apraxia of speech show distinct characteristics from agrammatic PPA
    Keith A Josephs
    Department of Neurology Behavioral Neurology, Mayo Clinic, Rochester, MN, USA
    Neurology 81:337-45. 2013
    ..We assessed whether clinical and imaging features of subjects with apraxia of speech (AOS) more severe than aphasia (dominant AOS) are more similar to agrammatic primary progressive aphasia (agPPA) or to primary progressive AOS (PPAOS)...
  12. pmc TREM2 in neurodegeneration: evidence for association of the p.R47H variant with frontotemporal dementia and Parkinson's disease
    Sruti Rayaprolu
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA
    Mol Neurodegener 8:19. 2013
    ..With this in mind we set out to assess the genetic association of the Alzheimer's disease-related risk variant in TREM2 (rs75932628, p.R47H) with other related neurodegenerative disorders...
  13. doi request reprint Neuroanatomical correlates of the progressive supranuclear palsy corticobasal syndrome hybrid
    K A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Eur J Neurol 19:1440-6. 2012
    ..We refer to subjects with this presentation as Hybrids. The hybrid presentation is not rare, yet there are no studies that have assessed the neuroanatomical correlates of the hybrid syndrome to explain its occurrence...
  14. pmc Corticospinal tract degeneration associated with TDP-43 type C pathology and semantic dementia
    Keith A Josephs
    Behavioural Neurology, Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Brain 136:455-70. 2013
    ....
  15. pmc Characterizing a neurodegenerative syndrome: primary progressive apraxia of speech
    Keith A Josephs
    Behavioural Neurology and Movement Disorders, Mayo Clinic, 200 1st Street S W, Rochester, MN 55905, USA
    Brain 135:1522-36. 2012
    ..A syndrome characterized by progressive pure apraxia of speech clearly exists, with a neuroanatomic correlate of superior lateral premotor and supplementary motor atrophy, making this syndrome distinct from primary progressive aphasia...
  16. pmc Primary progressive aphasia and transient global amnesia
    Jonathan Graff-Radford
    Department of Neurology, Mayo Clinic, 200 1st St SW, Rochester, MN 55905, USA
    Arch Neurol 69:401-4. 2012
    ..To describe 3 patients with a history of transient global amnesia (TGA) who developed primary progressive aphasia (PPA)...
  17. pmc Altered microRNA expression in frontotemporal lobar degeneration with TDP-43 pathology caused by progranulin mutations
    Jannet Kocerha
    Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL 32224, USA
    BMC Genomics 12:527. 2011
    ..Here we examine the role of miRNAs in FTLD patients with TAR DNA-binding protein 43 pathology (FTLD-TDP) caused by genetic mutations in the progranulin (PGRN) gene...
  18. pmc FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration
    Hazel Urwin
    UCL Institute of Neurology, London, UK
    Acta Neuropathol 120:33-41. 2010
    ..We identified three FTLD-UPS cases, which are negative for CHMP2B mutation, suggesting that the full complement of FTLD pathologies is yet to be elucidated...
  19. pmc Beta-amyloid burden is not associated with rates of brain atrophy
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Ann Neurol 63:204-12. 2008
    ..To test the hypothesis that beta-amyloid (Abeta) burden is associated with rates of brain atrophy...
  20. doi request reprint Gray matter correlates of behavioral severity in progressive supranuclear palsy
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA
    Mov Disord 26:493-8. 2011
    ..Behavioral changes occur in progressive supranuclear palsy. This study aimed to identify the anatomic correlate of behavioral severity in progressive supranuclear palsy...
  21. ncbi request reprint Capgras syndrome and its relationship to neurodegenerative disease
    Keith A Josephs
    Divisions of Behavioral Neurology and Movement Disorders, Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Arch Neurol 64:1762-6. 2007
    ..Capgras syndrome is characterized by a delusional belief that a person has been replaced by an imposter. It has been described in psychiatric and neurological (neurodegenerative and nonneurodegenerative) diseases...
  22. ncbi request reprint Clinically undetected motor neuron disease in pathologically proven frontotemporal lobar degeneration with motor neuron disease
    Keith A Josephs
    Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Arch Neurol 63:506-12. 2006
    ..The ability to detect the clinical signs of dementia and motor neuron disease in pathologically confirmed FTLD-MND has not been assessed...
  23. pmc Predicting functional decline in behavioural variant frontotemporal dementia
    Keith A Josephs
    Department of Neurology Behavioural Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA
    Brain 134:432-48. 2011
    ....
  24. pmc Frontotemporal lobar degeneration
    Keith A Josephs
    Department of Neurology, Division of Behavioral Neurology, Mayo Clinic and Mayo Foundation, Rochester, MN 55905, USA
    Neurol Clin 25:683-96, vi. 2007
    ..Imaging modalities are clinically useful in FTLD, although pathology remains the gold standard for definitive diagnosis. To date, four different genes have been identified that account for FTLD...
  25. ncbi request reprint Hippocampal sclerosis in tau-negative frontotemporal lobar degeneration
    Keith A Josephs
    Department of Neurology, Behavioral Neurology and Movement Disorders, Mayo Clinic, Rochester, MN 5590, United States
    Neurobiol Aging 28:1718-22. 2007
    ..02). The difference in frequency of HpScl in FTLD-U compared to FTLD-MND is further evidence that they are separate clinicopathologic entities...
  26. pmc Progressive aphasia secondary to Alzheimer disease vs FTLD pathology
    K A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 70:25-34. 2008
    ..The pathology causing progressive aphasia is typically a variant of frontotemporal lobar degeneration, especially with ubiquitin-positive inclusions (FTLD-U). Less commonly the underlying pathology is Alzheimer disease (AD)...
  27. pmc Abnormal TDP-43 immunoreactivity in AD modifies clinicopathologic and radiologic phenotype
    K A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 70:1850-7. 2008
    ....
  28. pmc Neuropathological background of phenotypical variability in frontotemporal dementia
    Keith A Josephs
    Behavioral Neurology and Movement Disorders, Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Acta Neuropathol 122:137-53. 2011
    ..However, there appears to be some associations between clinical phenotypes and FTLD-FUS pathologies. Clinical diagnosis is therefore promising in predicting molecular pathology...
  29. ncbi request reprint Frontotemporal lobar degeneration without lobar atrophy
    Keith A Josephs
    Department of Neurology, Division of Behavioral Neurology and Movement Disorders, Mayo Clinic, Rochester, MN 55905, USA
    Arch Neurol 63:1632-8. 2006
    ..Neuronal loss and gliosis in cornu ammonis 1 and the subiculum of the hippocampus are features of hippocampal sclerosis (HpScl), which occurs in many cases of FTLD-U...
  30. pmc Voxel-based morphometry in autopsy proven PSP and CBD
    Keith A Josephs
    Department of Neurology Movement Disorders, Mayo Clinic, Rochester, MN 55905, USA
    Neurobiol Aging 29:280-9. 2008
    ..These results show regional differences between PSP and CBD that are useful in predicting the underlying pathology, and help to shed light on the in vivo distribution of regional atrophy in PSP and CBD...
  31. pmc Visual hallucinations in posterior cortical atrophy
    Keith A Josephs
    Divisions of Movement Disorders and Behavioral Neurology, Department of Neurology, Mayo Clinic, Rochester, Minn, USA
    Arch Neurol 63:1427-32. 2006
    ..It is not known, however, whether patients who meet the criteria for PCA and have hallucinations are different from those who meet the criteria and do not have hallucinations...
  32. ncbi request reprint Fluorodeoxyglucose F18 positron emission tomography in progressive apraxia of speech and primary progressive aphasia variants
    Keith A Josephs
    Department of Neurology, Division of Behavioral Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA
    Arch Neurol 67:596-605. 2010
    ....
  33. pmc Anatomic correlates of stereotypies in frontotemporal lobar degeneration
    Keith A Josephs
    Department of Neurology, Behavioral Neurology and Movement Disorders, Mayo Clinic, Rochester, MN 55905, United States
    Neurobiol Aging 29:1859-63. 2008
    ..Stereotypies in FTLD are therefore associated with a greater proportion of striatal to cortical volume loss than those without stereotypies...
  34. pmc Anatomical differences between CBS-corticobasal degeneration and CBS-Alzheimer's disease
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA
    Mov Disord 25:1246-52. 2010
    ..In subjects presenting with CBS, prominent temporoparietal, especially posterior temporal and inferior parietal, atrophy may be a clue to the presence of underlying AD pathology...
  35. doi request reprint Apraxia of speech and nonfluent aphasia: a new clinical marker for corticobasal degeneration and progressive supranuclear palsy
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA
    Curr Opin Neurol 21:688-92. 2008
    ..To highlight the fact that patients with corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) can sometimes present with a progressive apraxia of speech, nonfluent aphasia, or a combination of the two disorders...
  36. ncbi request reprint Frontotemporal dementia and related disorders: deciphering the enigma
    Keith A Josephs
    Department of Neurology, Behavioral Neurology and Movement Disorders, Mayo Clinic, Rochester, MN 55905, USA
    Ann Neurol 64:4-14. 2008
    ..These associations could ultimately allow the identification of appropriate patient phenotypes for future targeted treatments...
  37. ncbi request reprint Survival in two variants of tau-negative frontotemporal lobar degeneration: FTLD-U vs FTLD-MND
    K A Josephs
    Department of Neurology, Mayo Clinic, 200 First St S W, Rochester, MN 55905, USA
    Neurology 65:645-7. 2005
    ..An analysis of patient outcomes in these cases reveals that FTLD-MND has significantly shorter survival than FTLD-U, suggesting that FTLD-MND is a more aggressive disease process...
  38. ncbi request reprint Neurophysiologic studies in Morvan syndrome
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    J Clin Neurophysiol 21:440-5. 2004
    ..The absence of abnormalities on imaging studies suggests that central nervous system symptoms are related to functional rather than structural disruption of neural networks...
  39. ncbi request reprint Heterozygous Niemann-Pick disease type C presenting with tremor
    Keith A Josephs
    Department of Neurology, Mayo Clinic and Mayo Foundation, Rochester, MN 55905, USA
    Neurology 63:2189-90. 2004
  40. ncbi request reprint Correlation between antemortem magnetic resonance imaging findings and pathologically confirmed corticobasal degeneration
    Keith A Josephs
    Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Arch Neurol 61:1881-4. 2004
    ..Various pathologic findings, including corticobasal degeneration (CBD), progressive supranuclear palsy, and frontotemporal degenerations, underlie CBS...
  41. ncbi request reprint Apolipoprotein E epsilon 4 is a determinant for Alzheimer-type pathologic features in tauopathies, synucleinopathies, and frontotemporal degeneration
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Arch Neurol 61:1579-84. 2004
    ....
  42. pmc The anatomic correlate of prosopagnosia in semantic dementia
    K A Josephs
    Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Neurology 71:1628-33. 2008
    ..To determine the anatomic correlate of prosopagnosia in subjects with semantic dementia...
  43. pmc Rapidly progressive neurodegenerative dementias
    Keith A Josephs
    Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA
    Arch Neurol 66:201-7. 2009
    ..Neurodegenerative dementias are typically characterized by an insidious onset and a relatively slowly progressive course. Less common are patients with a rapidly progressive course to death...
  44. pmc Adult onset Niemann-Pick disease type C presenting with psychosis
    K A Josephs
    Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    J Neurol Neurosurg Psychiatry 74:528-9. 2003
    ..NPC may initially present in adulthood with psychosis, and when psychosis is associated with VSGP, various dyskinesias, and seizures, NPC should be suspected...
  45. ncbi request reprint Neurologic manifestations in welders with pallidal MRI T1 hyperintensity
    K A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 64:2033-9. 2005
    ..Increased T1 MRI signal in the basal ganglia is a biologic marker of manganese accumulation. Recent studies have associated welding and parkinsonism, but generally without MRI corroboration...
  46. pmc Two distinct subtypes of right temporal variant frontotemporal dementia
    K A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 73:1443-50. 2009
    ..We aimed to determine whether right temporal FTD is a homogeneous clinical, imaging, and pathologic/genetic entity...
  47. ncbi request reprint Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP
    K A Josephs
    Division of Movement Disorders, Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 66:41-8. 2006
    ..To examine the relationship between early clinical features, pathologies, and biochemistry of the frontotemporal lobar degenerations (FTLDs), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD)...
  48. pmc Caudate atrophy on MRI is a characteristic feature of FTLD-FUS
    K A Josephs
    Department of Neurology Behavioral Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Eur J Neurol 17:969-75. 2010
    ..We have observed severe caudate atrophy at autopsy in FTLD-FUS, and hence, we aimed to determine whether caudate atrophy on MRI is a feature that can distinguish FTLD-FUS from FTLD-TDP and FTLD-TAU...
  49. ncbi request reprint Rates of cerebral atrophy in autopsy-confirmed progressive supranuclear palsy
    Keith A Josephs
    Department of Neurology, Mayo Clinic, 200 First Street NW, Rochester, MN 55905, USA
    Ann Neurol 59:200-3. 2006
    ..To determine the rates of cerebral atrophy and ventricular expansion in six patients with autopsy confirmed progressive supranuclear palsy (PSP) and multiple antemortem volumetric head MRI scans...
  50. ncbi request reprint Atypical progressive supranuclear palsy underlying progressive apraxia of speech and nonfluent aphasia
    K A Josephs
    Department of Neurology, Division of Behavioural Neurology, Mayo Clinic, Mayo Foundation, Rochester, MN 55905, USA
    Neurocase 11:283-96. 2005
    ..These cases demonstrate that atypical PSP can present as AOS and PNFA without the classic features of PSP...
  51. pmc Atrophy patterns in IVS10+16, IVS10+3, N279K, S305N, P301L, and V337M MAPT mutations
    J L Whitwell
    Department of Radiology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 73:1058-65. 2009
    ..To use a case-control study to assess and compare patterns of gray matter loss across groups of subjects with different mutations in the microtubule-associated protein tau (MAPT) gene...
  52. ncbi request reprint Late-onset frontotemporal dementia associated with progressive supranuclear palsy/argyrophilic grain disease/Alzheimer's disease pathology
    G A Rippon
    Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA
    Neurocase 11:204-11. 2005
    ..We suggest that PSP with or without coexisting AD and AGD be included in the differential diagnosis of patients presenting with FTD...
  53. pmc Imaging correlates of pathology in corticobasal syndrome
    J L Whitwell
    Department of Radiology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USA
    Neurology 75:1879-87. 2010
    ..The aim of this study was to determine whether patterns of atrophy on imaging could be useful to help predict underlying pathology in CBS...
  54. pmc Gray and white matter water diffusion in the syndromic variants of frontotemporal dementia
    J L Whitwell
    Department of Radiology, Mayo Clinic, Rochester MN 55905, USA
    Neurology 74:1279-87. 2010
    ..To use diffusion tensor imaging (DTI) to assess gray matter and white matter tract diffusion in behavioral variant frontotemporal dementia (bvFTD), semantic dementia (SMD), and progressive nonfluent aphasia (PNFA)...
  55. pmc Trajectories of brain and hippocampal atrophy in FTD with mutations in MAPT or GRN
    J L Whitwell
    Department of Radiology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA
    Neurology 77:393-8. 2011
    ..To use multiple serial MRI to assess rates and trajectories of brain and hippocampal atrophy in subjects with frontotemporal dementia (FTD) with progranulin (GRN) or microtubule-associated protein tau (MAPT) gene mutations...
  56. pmc Does TDP-43 type confer a distinct pattern of atrophy in frontotemporal lobar degeneration?
    J L Whitwell
    Department of Radiology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 75:2212-20. 2010
    ..To determine whether TDP-43 type is associated with distinct patterns of brain atrophy on MRI in subjects with pathologically confirmed frontotemporal lobar degeneration (FTLD)...
  57. pmc Voxel-based morphometry patterns of atrophy in FTLD with mutations in MAPT or PGRN
    J L Whitwell
    Department of Radiology Research, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 72:813-20. 2009
    ..To compare patterns of gray matter loss in subjects with mutations in the progranulin (PGRN) gene to subjects with mutations in the microtubule-associated protein tau (MAPT) gene...
  58. doi request reprint Progranulin gene mutation with an unusual clinical and neuropathologic presentation
    Christian Wider
    Department of Neurology, Mayo Clinic, Jacksonville, Florida, USA
    Mov Disord 23:1168-73. 2008
    ..This study further expands the clinical and pathological spectrum of PGRN mutations, and suggests the diagnosis could be missed in some individuals with atypical presentations...
  59. pmc Neuroimaging comparison of primary progressive apraxia of speech and progressive supranuclear palsy
    J L Whitwell
    Department of Radiology, Mayo Clinic, Rochester, MN 55905, USA
    Eur J Neurol 20:629-37. 2013
    ..We aimed to compare, for the first time, atrophy patterns, as well as white matter tract degeneration, between these two syndromes...
  60. pmc MRI correlates of neurofibrillary tangle pathology at autopsy: a voxel-based morphometry study
    J L Whitwell
    Department of Radiology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA
    Neurology 71:743-9. 2008
    ..We aimed to determine whether patterns of gray matter atrophy from antemortem MRI correlate with Braak staging of NFT pathology...
  61. pmc Midbrain atrophy is not a biomarker of progressive supranuclear palsy pathology
    J L Whitwell
    Department of Radiology, Mayo Clinic, Rochester, MN, USA
    Eur J Neurol 20:1417-22. 2013
    ..The aim of the present study was to determine whether midbrain atrophy is a useful biomarker of PSP pathology, or whether it is only associated with typical PSPS...
  62. pmc Altered functional connectivity in asymptomatic MAPT subjects: a comparison to bvFTD
    J L Whitwell
    Department of Radiology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 77:866-74. 2011
    ....
  63. doi request reprint Sporadic adult-onset leukoencephalopathy with neuroaxonal spheroids mimicking cerebral MS
    B M Keegan
    Department of Neurology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA
    Neurology 70:1128-33. 2008
    ..The authors present clinical, radiologic, and pathologic features of adult-onset, sporadic cases mimicking cerebral-type progressive MS...
  64. ncbi request reprint The stripe of primary lateral sclerosis: focal primary motor cortex hypometabolism seen on fluorodeoxyglucose F18 positron emission tomography
    Daniel O Claassen
    Departments of Neurology, Mayo Clinic and Mayo College of Medicine, Rochester, Minnesota 55905, USA
    Arch Neurol 67:122-5. 2010
    ..Primary lateral sclerosis (PLS) is a progressive upper motor neuron neurodegenerative condition. The diagnosis is made using clinical history, objective neurological assessment, and exclusion of other neurodegenerative disorders...
  65. ncbi request reprint Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal changes: broadening the clinical picture to include progressive supranuclear palsy
    D C Paviour
    The Sara Koe PSP Research Centre, Institute of Neurology, London, UK
    Brain 127:2441-51. 2004
    ..FTLD-U or FTLD-MND should be considered in the differential diagnosis of progressive frontal dementia with an akinetic rigid syndrome and supranuclear gaze palsy or Steele-Richardson-Olszewski disease...
  66. ncbi request reprint Pathologically confirmed corticobasal degeneration presenting with visuospatial dysfunction
    D F Tang-Wai
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 61:1134-5. 2003
    ..Underlying CBD should be considered in the differential diagnosis of patients with findings reflecting posterior cerebral dysfunction...
  67. ncbi request reprint Brain metal concentrations in chronic liver failure patients with pallidal T1 MRI hyperintensity
    K J Klos
    Department of Neurology, Mayo Clinic, Rochester, MN, USA
    Neurology 67:1984-9. 2006
    ..Manganese accumulation may be responsible for the imaging and clinical findings...
  68. ncbi request reprint Neurofilament inclusion body disease: a new proteinopathy?
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN, USA
    Brain 126:2291-303. 2003
    ..We propose the term neurofilament inclusion body disease for this entity...
  69. pmc Prominent phenotypic variability associated with mutations in Progranulin
    Brendan J Kelley
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Neurobiol Aging 30:739-51. 2009
    ..Some kindreds with PGRN mutations exhibited lateralized topography of degeneration across all affected individuals...
  70. pmc REM sleep behavior disorder preceding other aspects of synucleinopathies by up to half a century
    D O Claassen
    Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA
    Neurology 75:494-9. 2010
    ..Idiopathic REM sleep behavior disorder (RBD) may be the initial manifestation of synucleinopathies (Parkinson disease [PD], multiple system atrophy [MSA], or dementia with Lewy bodies [DLB])...
  71. ncbi request reprint Alpha-synuclein pathology in the spinal cords of neurologically asymptomatic aged individuals
    K J Klos
    Department of Neurology, Mayo Clinic, Rochester, MN, USA
    Neurology 66:1100-2. 2006
    ..Sparse alpha-synuclein pathology was also detected in the substantia nigra, basal forebrain, amygdala, or cortex in all but two cases...
  72. pmc Patterns of atrophy in pathologically confirmed FTLD with and without motor neuron degeneration
    Jennifer L Whitwell
    Department of Radiology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 66:102-4. 2006
    ..Patterns of atrophy were distinct and different from each other. A localized pattern of frontal lobe atrophy was found in FTLD-MND with a more widespread pattern of atrophy affecting the frontal and temporal lobes in FTLD-U...
  73. ncbi request reprint APOE E4 is a determinant for Alzheimer type pathology in progressive supranuclear palsy
    Yoshio Tsuboi
    Department of Neurology, Mayo Clinic, Jacksonville, FL, USA
    Neurology 60:240-5. 2003
    ..To assess demographic and genetic determinants of Alzheimer type pathology in progressive supranuclear palsy (PSP)...
  74. ncbi request reprint Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
    J Neuropathol Exp Neurol 66:142-51. 2007
    ..On the other hand, there is no histopathologic feature or combination of features that is pathognomonic. Neuronal intranuclear inclusions are virtually always present, but they can be detected in PGRN(-) cases...
  75. pmc Voxel-based morphometry in patients with obsessive-compulsive behaviors in behavioral variant frontotemporal dementia
    D C Perry
    Department of Neurology, Mayo Clinic, Rochester, MN, USA
    Eur J Neurol 19:911-7. 2012
    ..Obsessions and compulsive (OC) behaviors are a frequent feature of behavioral variant frontotemporal dementia (bvFTD), but their structural correlates have not been definitively established...
  76. ncbi request reprint Delusions and hallucinations in dementia with Lewy bodies: worsening with memantine
    B H Ridha
    Dementia Research Centre, National Hospital for Neurology and Neurosurgery, London, WC1N 3BG, UK
    Neurology 65:481-2. 2005
    ..Significant resolution occurred once treatment was discontinued. Caution is required when prescribing memantine to patients with possible DLB...
  77. pmc Characterization of DCTN1 genetic variability in neurodegeneration
    C Vilariño-Güell
    Molecular Genetics Laboratory and Core, Morris K Udall Parkinson s Disease Research Center of Excellence, Mayo Clinic, Department of Neuroscience, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    Neurology 72:2024-8. 2009
    ....
  78. pmc Frontotemporal brain sagging syndrome: an SIH-like presentation mimicking FTD
    M R Wicklund
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 76:1377-82. 2011
    ..We have been evaluating patients with clinical and imaging features partially consistent with bvFTD but with evidence also suggestive of brain sagging, which we refer to as frontotemporal brain sagging syndrome (FBSS)...
  79. ncbi request reprint Neuropsychological profiles of manganese neurotoxicity
    K J Klos
    Department of Neurology, Mayo Clinic, Rochester, MN, USA
    Eur J Neurol 13:1139-41. 2006
    ..Across all three groups, the neuropsychological profiles suggest frontal and subcortical cognitive impairment, with more widespread abnormalities occurring in the non-welding groups...
  80. pmc Rates of cerebral atrophy differ in different degenerative pathologies
    Jennifer L Whitwell
    Department of Radiology, Mayo Clinic Rochester, Rochester, MN 55905, USA
    Brain 130:1148-58. 2007
    ....
  81. pmc Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN, USA
    Acta Neuropathol 118:349-58. 2009
    ..These findings extend previously described clinicopathological associations of FTLD-TDP subtypes and support the notion that FTLD-TDP subtypes may be distinct clinicopathologic disorders...
  82. pmc Cognitive and noncognitive neurological features of young-onset dementia
    Brendan J Kelley
    Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
    Dement Geriatr Cogn Disord 27:564-71. 2009
    ..Limited data exist regarding clinical features associated with dementia prior to the age of 45...
  83. pmc Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations
    Jennifer L Whitwell
    Division of Behavioral Neurology, Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA
    Arch Neurol 64:371-6. 2007
    ..Mutations in the progranulin gene (PGRN) have recently been identified as a cause of frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) in some families...
  84. ncbi request reprint Creutzfeldt-Jakob disease presenting as progressive supranuclear palsy
    K A Josephs
    Department of Neurology, Mayo Clinic, Jacksonville, FL 32224, USA
    Eur J Neurol 11:343-6. 2004
    ..To our knowledge, this is the first report of cases of CJD with autopsy confirmation that presented with a PSP syndrome...
  85. ncbi request reprint Frontotemporal lobar degeneration and ubiquitin immunohistochemistry
    K A Josephs
    Department of Neurology, Mayo Clinic, Rochester, USA
    Neuropathol Appl Neurobiol 30:369-73. 2004
    ..Therefore in our brain bank series of frontotemporal degeneration, most cases were non-tauopathies with FTLD-U accounting for 62% of all the diagnoses...
  86. ncbi request reprint Effect of MAPT and APOE on prognosis of progressive supranuclear palsy
    Yasuhiko Baba
    Department of Neurology, Mayo Clinic, Jacksonville, FL 32224, USA
    Neurosci Lett 405:116-9. 2006
    ..These results support the assertion that the H1/H1 genotype may contribute to the earlier occurrence of clinical symptoms...
  87. pmc Focal atrophy in dementia with Lewy bodies on MRI: a distinct pattern from Alzheimer's disease
    Jennifer L Whitwell
    Department of Radiology, Mayo Clinic, Rochester, MN 55905, USA
    Brain 130:708-19. 2007
    ..Damage to this network of structures in DLB may affect a number of different neurotransmitter systems which in turn may contribute to a number of the core clinical features of DLB...
  88. doi request reprint Dopamine agonist-triggered pathological behaviors: surveillance in the PD clinic reveals high frequencies
    A Hassan
    Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Parkinsonism Relat Disord 17:260-4. 2011
    ..Compulsive behaviors provoked by dopamine agonists often go undetected in clinical series, especially if not specifically inquired about...
  89. pmc Rapidly progressive young-onset dementia
    Brendan J Kelley
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Cogn Behav Neurol 22:22-7. 2009
    ..To characterize a cohort of individuals who have experienced rapidly progressive dementia with onset before age 45...
  90. ncbi request reprint Alzheimer's disease and corticobasal degeneration presenting as corticobasal syndrome
    William T Hu
    Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
    Mov Disord 24:1375-9. 2009
    ..AD patients with clinical CBS have similar characteristics to CBD patients. Functional brain imaging may have greater utility than the clinical and neuropsychological features in differentiating AD presenting as CBS from CBD...
  91. doi request reprint Young-onset dementia: demographic and etiologic characteristics of 235 patients
    Brendan J Kelley
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Arch Neurol 65:1502-8. 2008
    ..Few data exist regarding the demographics characterizing this population or the etiologic diagnoses among those affected...
  92. ncbi request reprint The relationship between histopathological features of progressive supranuclear palsy and disease duration
    Keith A Josephs
    Department of Neurology, Mayo Clinic Rochester, MN, USA
    Parkinsonism Relat Disord 12:109-12. 2006
    ..Surprisingly, the data suggests that in PSP as duration of illness increases there is a decrease in oligodendroglial tau burden...
  93. pmc Rates of brain atrophy over time in autopsy-proven frontotemporal dementia and Alzheimer disease
    Jennifer L Whitwell
    Department of Radiology, Mayo Clinic, Rochester, MN 55905, USA
    Neuroimage 39:1034-40. 2008
    ..The trajectories of brain and ventricular changes were similar in AD and FTLD-U suggesting that it is independent of pathology, although subjects with FTLD-U show a more rapidly progressive decline...
  94. doi request reprint Cardiac sympathetic denervation correlates with clinical and pathologic stages of Parkinson's disease
    Hiroshige Fujishiro
    Department of Pathology Neuropathology, Mayo Clinic, Jacksonville, Florida, USA
    Mov Disord 23:1085-92. 2008
    ..42, P < 0.05). This study demonstrates that cardiac sympathetic degeneration and alpha-synuclein pathology is present in presymptomatic phase of PD, and that both increase with disease duration and severity...
  95. pmc Clinical and imaging features of Othello's syndrome
    J Graff-Radford
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Eur J Neurol 19:38-46. 2012
    ..Our objective was to document the clinical and imaging features of Othello's syndrome (delusional jealousy)...
  96. pmc Symmetric corticobasal degeneration (S-CBD)
    Anhar Hassan
    Department of Neurology, Mayo Clinic, Rochester, MN, USA
    Parkinsonism Relat Disord 16:208-14. 2010
    ..Asymmetry is also emphasized on neuroimaging...
  97. doi request reprint Neuropathology of non-motor features of Parkinson disease
    Dennis W Dickson
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA
    Parkinsonism Relat Disord 15:S1-5. 2009
    ....
  98. ncbi request reprint Pathological gambling caused by drugs used to treat Parkinson disease
    M Leann Dodd
    Department of Psychiatry and Psychology, Mayo Clinic, Rochester, MN 55905, USA
    Arch Neurol 62:1377-81. 2005
    ..Pathological gambling is a rare potential complication related to treatment of Parkinson disease (PD). However, the etiology of this behavior is poorly understood...
  99. doi request reprint Neuropathology of variants of progressive supranuclear palsy
    Dennis W Dickson
    Department of Neuroscience, Mayo Clinic, Jacksonville, Florida 32224, USA
    Curr Opin Neurol 23:394-400. 2010
    ..This review highlights the range of clinical and pathologic presentations of PSP and its variants...
  100. ncbi request reprint The clinical spectrum of stereotypies in frontotemporal lobar degeneration
    Farrah J Mateen
    Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA
    Mov Disord 24:1237-40. 2009
    ..15%, P = 0.02). Of the 19 patients with stereotypies, three had vocal stereotypies, and three had both vocal and motor stereotypies. Of the 16 subjects with motor stereotypies, 9(56%) were appendicular and 7 (44%) were craniocervical...
  101. ncbi request reprint Alpha-synuclein studies are negative in postencephalic parkinsonism of von Economo
    Keith A Josephs
    Department of Neurology, Division of Movement Disorders, Mayo Clinic, Rochester, MN, USA
    Neurology 59:645-6. 2002

Research Grants2

  1. PIB PET Scanning in Speech and Language Based Dementias
    Keith A Josephs; Fiscal Year: 2010
    ..Results from this grant will ultimately lead to better targeted future treatments for patients with problems with speech and language. ..
  2. Understanding the role of TDP-43 in Alzheimers disease and FTLD
    Keith A Josephs; Fiscal Year: 2010
    ..A better understanding of the role of TDP-43 is important to the development of targeted treatments for both diseases. Alzheimer's disease and frontototemporal lobar degeneration affect over 5 million Americans. ..