Publications

  1. Whitwell JL, Jack CR Jr, Parisi JE, Gunter JL, Weigand SD, Boeve BF, Ahlskog JE, Petersen RC, Dickson DW, Josephs KA. Midbrain atrophy is not a biomarker of progressive supranuclear palsy pathology. Eur J Neurol. 2013 Jun 07. [Epub ahead of print] 2329984
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  2. Adeli A, Whitwell JL, Duffy JR, Strand EA, Josephs KA. Ideomotor apraxia in agrammatic and logopenic variants of primary progressive aphasia. J Neurol. 2013 Jun; 260(6):1594-600. Epub 2013 Jan 29. 2299564
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  3. Whitwell JL, Duffy JR, Strand EA, Xia R, Mandrekar J, Machulda MM, Senjem ML, Lowe VJ, Jack CR Jr, Josephs KA. Distinct regional anatomic and functional correlates of neurodegenerative apraxia of speech and aphasia: An MRI and FDG-PET study. Brain Lang. 2013 Jun; 125(3):245-52. Epub 2013 Mar 28. 2324764
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  4. van Blitterswijk M, Baker MC, Bieniek KF, Knopman DS, Josephs KA, Boeve B, Caselli R, Wszolek ZK, Petersen R, Graff-Radford NR, Boylan KB, Dickson DW, Rademakers R. Profilin-1 mutations are rare in patients with amyotrophic lateral sclerosis and frontotemporal dementia. Amyotroph Lateral Scler Frontotemporal Degener. 2013 May 02. [Epub ahead of print] 2320458
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  5. Ravenscroft TA, Baker MC, Rutherford NJ, Neumann M, Mackenzie IR, Josephs KA, Boeve BF, Petersen R, Halliday GM, Kril J, van Swieten JC, Seeley WW, Dickson DW, Rademakers R. Mutations in protein N-arginine methyltransferases are not the cause of FTLD-FUS. Neurobiol Aging. 2013 Apr 28. [Epub ahead of print] 2320469
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  6. Graff-Radford J, Rubin MN, Jones DT, Aksamit AJ, Ahlskog JE, Knopman DS, Petersen RC, Boeve BF, Josephs KA. The alien limb phenomenon. J Neurol. 2013 Apr 10. [Epub ahead of print] 2315377
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  7. Josephs KA, Xia R, Mandrekar J, Gunter JL, Senjem ML, Jack CR Jr, Whitwell JL. Modeling trajectories of regional volume loss in progressive supranuclear palsy. Mov Disord. 2013 Apr 08. [Epub ahead of print] 2315359
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  8. Machulda MM, Whitwell JL, Duffy JR, Strand EA, Dean PM, Senjem ML, Jack CR Jr, Josephs KA. Identification of an atypical variant of logopenic progressive aphasia. Brain Lang. 2013 Apr 06. [Epub ahead of print] 2315379
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  9. Whitwell JL, Jack CR Jr, Duffy JR, Strand EA, Gunter JL, Senjem ML, Murphy MC, Kantarci K, Machulda MM, Lowe VJ, Josephs KA. Microbleeds in the logopenic variant of primary progressive aphasia. Alzheimers Dement. 2013 Apr 03. [Epub ahead of print] 2315017
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  10. Bruns MB, Josephs KA. Neuropsychiatry of corticobasal degeneration and progressive supranuclear palsy. Int Rev Psychiatry. 2013 Apr; 25(2):197-209. 2319155
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  11. Whitwell JL, Duffy JR, Strand EA, Machulda MM, Senjem ML, Gunter JL, Kantarci K, Eggers SD, Jack CR Jr, Josephs KA. Neuroimaging comparison of primary progressive apraxia of speech and progressive supranuclear palsy. Eur J Neurol. 2013 Apr; 20(4):629-37. Epub 2012 Oct 18. 2278392
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  12. Josephs KA, Dickson DW, Murray ME, Senjem ML, Parisi JE, Petersen RC, Jack CR Jr, Whitwell JL. Quantitative neurofibrillary tangle density and brain volumetric MRI analyses in Alzheimer's disease presenting as logopenic progressive aphasia. Brain Lang. 2013 Mar 27. [Epub ahead of print] 2314150
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  13. Coon EA, Daube JR, Dejesus-Hernandez M, Adeli A, Savica R, Parisi JE, Dickson DW, Josephs KA, Baker MC, Johnson KA, Ivnik RJ, Petersen RC, Knopman DS, Boylan KB, Rademakers R, Boeve BF. Clinical and electrophysiologic variability in amyotrophic lateral sclerosis within a kindred harboring the C9ORF72 repeat expansion. Amyotroph Lateral Scler Frontotemporal Degener. 2013 Mar; 14(2):132-7. Epub 2012 Oct 04. 2274544
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  14. Whitwell JL, Xu J, Mandrekar J, Boeve BF, Knopman DS, Parisi JE, Senjem ML, Dickson DW, Petersen RC, Rademakers R, Jack CR Jr, Josephs KA. Frontal asymmetry in behavioral variant frontotemporal dementia: clinicoimaging and pathogenetic correlates. Neurobiol Aging. 2013 Feb; 34(2):636-9. Epub 2012 Apr 11. 2238961
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  15. Josephs KA, Whitwell JL, Murray ME, Parisi JE, Graff-Radford NR, Knopman DS, Boeve BF, Senjem ML, Rademakers R, Jack CR Jr, Petersen RC, Dickson DW. Corticospinal tract degeneration associated with TDP-43 type C pathology and semantic dementia. Brain. 2013 Feb; 136(Pt 2):455-70. Epub 2013 Jan 28. 2299568
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  16. Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, Boxer AL, Dickson DW, Grossman M, Hallett M, Josephs KA, Kertesz A, Lee SE, Miller BL, Reich SG, Riley DE, Tolosa E, Troster AI, Vidailhet M, Weiner WJ. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013 Jan 29; 80(5):496-503. 2299551
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  17. Chang FCF, Josephs KA. Levodopa responsiveness in adult-onset lower limb dystonia is associated with the development of Parkinson's disease. Proceedings of the Royal Society B: Biological Sciences. 2013; 280(1760). 2329824
  18. Madhavan A, Whitwell JL, Weigand SD, Duffy JR, Strand EA, Machulda MM, Tosakulwong N, Senjem ML, Gunter JL, Lowe VJ, Petersen RC, Jack CR Jr, Josephs KA. FDG PET and MRI in logopenic primary progressive aphasia versus dementia of the Alzheimer's type. PLoS One. 2013; 8(4):e62471. Epub 2013 Apr 23. 2319958
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  19. Chang FC, Josephs KA. Levodopa Responsiveness in Adult-onset Lower Limb Dystonia is Associated with the Development of Parkinson's Disease. Tremor Other Hyperkinet Mov (N Y). 2013; 3. Epub 2013 Apr 18. 2318477
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  20. Whitwell JL, Josephs KA. Imaging brain atrophy in progressive supranuclear palsy and corticobasal syndromes: potential for diagnosis and monitoring of disease progression. Neurodegenerative Disease Management. 2012 Dec; 2(6):589-598. 2289929
  21. Graff-Radford J, Boeve BF, Drubach DA, Knopman DS, Ahlskog JE, Golden EC, Drubach DI, Petersen RC, Josephs KA. Limb immobilization and corticobasal syndrome. Parkinsonism Relat Disord. 2012 Dec; 18(10):1097-9. Epub 2012 Jun 20. 2252670
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  22. Whitwell JL, Josephs KA. Recent advances in the imaging of frontotemporal dementia. Curr Neurol Neurosci Rep. 2012 Dec; 12(6):715-23. 2272961
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  23. Whitwell JL, Xu J, Mandrekar J, Gunter JL, Jack CR Jr, Josephs KA. Imaging measures predict progression in progressive supranuclear palsy. Mov Disord. 2012 Dec; 27(14):1801-4. Epub 2012 Mar 13. 2235382
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  24. Josephs KA, Eggers SD, Jack CR Jr, Whitwell JL. Neuroanatomical correlates of the progressive supranuclear palsy corticobasal syndrome hybrid. Eur J Neurol. 2012 Nov; 19(11):1440-6. Epub 2012 Apr 23. 2240724
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  25. Duffy JR, Josephs KA. The diagnosis and understanding of apraxia of speech: why including neurodegenerative etiologies may be important. J Speech Lang Hear Res. 2012 Oct; 55(5):S1518-22. 2281277
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  26. Whitwell JL, Dickson DW, Murray ME, Weigand SD, Tosakulwong N, Senjem ML, Knopman DS, Boeve BF, Parisi JE, Petersen RC, Jack CR Jr, Josephs KA. Neuroimaging correlates of pathologically defined subtypes of Alzheimer's disease: a case-control study. Lancet Neurol. 2012 Oct; 11(10):868-77. Epub 2012 Sep 03. 2268559
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  27. Rutherford NJ, Carrasquillo MM, Li M, Bisceglio G, Menke J, Josephs KA, Parisi JE, Petersen RC, Graff-Radford NR, Younkin SG, Dickson DW, Rademakers R. TMEM106B risk variant is implicated in the pathologic presentation of Alzheimer disease. Neurology. 2012 Aug 14; 79(7):717-8. Epub 2012 Aug 01. 2261734
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  28. Sundal C, Van Gerpen JA, Nicholson AM, Wider C, Shuster EA, Aasly J, Spina S, Ghetti B, Roeber S, Garbern J, Borjesson-Hanson A, Tselis A, Swerdlow RH, Miller BB, Fujioka S, Heckman MG, Uitti RJ, Josephs KA, Baker M, Andersen O, Rademakers R, Dickson DW, MRI characteristics and scoring in HDLS due to CSF1R gene mutations. Neurology. 2012 Aug 7; 79(6):566-74. Epub 2012 Jul 25. 2261710
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  29. Rubin M, Graff-Radford J, Boeve B, Josephs KA, Aksamit AJ. The alien limb phenomenon and Creutzfeldt-Jakob disease. Parkinsonism Relat Disord. 2012 Aug; 18(7):842-6. Epub 2012 May 08. 2244800
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  30. Graff-Radford J, Duffy JR, Strand EA, Josephs KA. Parkinsonian motor features distinguish the agrammatic from logopenic variant of primary progressive aphasia. Parkinsonism Relat Disord. 2012 Aug; 18(7):890-2. Epub 2012 May 08. 2244736
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  31. Graff-Radford J, Drubach DA, Strand EA, Josephs KA. Fluorodeoxyglucose F18 PET in progressive emotional dysprosody. Neurology. 2012 Jul 31; 79(5):480-1. Epub 2012 Jul 18. 2259046
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  32. Coon EA, Whitwell JL, Jack CR Jr, Josephs KA. Primary lateral sclerosis as progressive supranuclear palsy: diagnosis by diffusion tensor imaging. Mov Disord. 2012 Jun; 27(7):903-6. Epub 2012 Apr 19. 2240747
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  33. Perry DC, Whitwell JL, Boeve BF, Pankratz VS, Knopman DS, Petersen RC, Jack CR Jr, Josephs KA. Voxel-based morphometry in patients with obsessive-compulsive behaviors in behavioral variant frontotemporal dementia. Eur J Neurol. 2012 Jun; 19(6):911-7. Epub 2012 Jan 28. 2221005
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  34. Josephs KA, Duffy JR, Strand EA, Machulda MM, Senjem ML, Master AV, Lowe VJ, Jack CR Jr, Whitwell JL. Characterizing a neurodegenerative syndrome: primary progressive apraxia of speech. Brain. 2012 May; 135(Pt 5):1522-36. Epub 2012 Mar 01. 2228993
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  35. Savica R, Parisi JE, Wold LE, Josephs KA, Ahlskog JE. High school football and risk of neurodegeneration: a community-based study. Mayo Clin Proc. 2012 Apr; 87(4):335-40. 2236799
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  36. Graff-Radford J, Josephs KA. Primary progressive aphasia and transient global amnesia. Arch Neurol. 2012 Mar; 69(3):401-4. 2235289
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  37. Whitwell JL, Weigand SD, Boeve BF, Senjem ML, Gunter JL, DeJesus-Hernandez M, Rutherford NJ, Baker M, Knopman DS, Wszolek ZK, Parisi JE, Dickson DW, Petersen RC, Rademakers R, Jack CR Jr, Josephs KA. Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics. Brain. 2012 Mar; 135(Pt 3):794-806. 2227679
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  38. Boeve BF, Boylan KB, Graff-Radford NR, DeJesus-Hernandez M, Knopman DS, Pedraza O, Vemuri P, Jones D, Lowe V, Murray ME, Dickson DW, Josephs KA, Rush BK, Machulda MM, Fields JA, Ferman TJ, Baker M, Rutherford NJ, Adamson J, Wszolek ZK, Adeli A, Savica R, Characterization of frontotemporal dementia and/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72. Brain. 2012 Mar; 135(Pt 3):765-83. 2227707
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  39. Whitwell JL, Xu J, Mandrekar JN, Gunter JL, Jack CR Jr, Josephs KA. Rates of brain atrophy and clinical decline over 6 and 12-month intervals in PSP: determining sample size for treatment trials. Parkinsonism Relat Disord. 2012 Mar; 18(3):252-6. Epub 2011 Nov 13. 2206838
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  40. Mateen FJ, Josephs KA, Parisi JE, Drubach DA, Caselli RJ, Kantarci K, Jack C Jr, Boeve BF. Steroid-responsive encephalopathy subsequently associated with Alzheimer's disease pathology: a case series. Neurocase. 2012 Feb; 18(1):1-12. Epub 2011 Jun 30. 2227609
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  41. Coon EA, Whitwell JL, Parisi JE, Dickson DW, Josephs KA. Right temporal variant frontotemporal dementia with motor neuron disease. J Clin Neurosci. 2012 Jan; 19(1):85-91. Epub 2011 Nov 02. 2203458
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  42. Savica R, Kumar N, Ahlskog JE, Josephs KA, Matsumoto JY, McKeon A. Parkinsonism and dropped head: dystonia, myopathy or both? Parkinsonism Relat Disord. 2012 Jan; 18(1):30-4. Epub 2011 Aug 27. 2187828
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  43. Graff-Radford J, Whitwell JL, Geda YE, Josephs KA. Clinical and imaging features of Othello's syndrome. Eur J Neurol. 2012 Jan; 19(1):38-46. Epub 2011 Apr 25. 2161975
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  44. Hassan A, Parisi JE, Josephs KA. Autopsy-proven progressive supranuclear palsy presenting as behavioral variant frontotemporal dementia. Neurocase. 2012; 18(6):478-88. Epub 2011 Dec 20. 2213797
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  45. Murray ME, DeJesus-Hernandez M, Rutherford NJ, Baker M, Duara R, Graff-Radford NR, Wszolek ZK, Ferman TJ, Josephs KA, Boylan KB, Rademakers R, Dickson DW. Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72. Acta Neuropathol. 2011 Dec; 122(6):673-90. Epub 2011 Nov 15. 2206597
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  46. Graff-Radford J, Whitwell JL, Trenerry MR, Ahlskog JE, Jensen MD, Jack CR Jr, Josephs KA. Focal brain atrophy in gastric bypass patients with cognitive complaints. J Clin Neurosci. 2011 Dec; 18(12):1671-6. Epub 2011 Nov 15. 2206626
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  47. Whitwell JL, Jack CR Jr, Parisi JE, Knopman DS, Boeve BF, Petersen RC, Dickson DW, Josephs KA. Imaging signatures of molecular pathology in behavioral variant frontotemporal dementia. J Mol Neurosci. 2011 Nov; 45(3):372-8. Epub 2011 May 10. 2163837
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  48. Dickson DW, Kouri N, Murray ME, Josephs KA. Neuropathology of frontotemporal lobar degeneration-tau (FTLD-tau). J Mol Neurosci. 2011 Nov; 45(3):384-9. Epub 2011 Jul 01. 2173542
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  49. Kouri N, Murray ME, Hassan A, Rademakers R, Uitti RJ, Boeve BF, Graff-Radford NR, Wszolek ZK, Litvan I, Josephs KA, Dickson DW. Neuropathological features of corticobasal degeneration presenting as corticobasal syndrome or Richardson syndrome. Brain. 2011 Nov; 134(Pt 11):3264-75. Epub 2011 Sep 20. 2192280
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  50. Hassan A, Whitwell JL, Josephs KA. The corticobasal syndrome-Alzheimer's disease conundrum. Expert Rev Neurother. 2011 Nov; 11(11):1569-78. 2201929
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  51. DeJesus-Hernandez M, Mackenzie IR, Boeve BF, Boxer AL, Baker M, Rutherford NJ, Nicholson AM, Finch NA, Flynn H, Adamson J, Kouri N, Wojtas A, Sengdy P, Hsiung GY, Karydas A, Seeley WW, Josephs KA, Coppola G, Geschwind DH, Wszolek ZK, Feldman H, Knopman Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron. 2011 Oct 20; 72(2):245-56. Epub 2011 Sep 21. 2196787
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  52. Mateen FJ, Josephs KA, Trenerry MR, Felmlee-Devine MD, Weaver AL, Carone M, White RD. Long-term cognitive outcomes following out-of-hospital cardiac arrest: a population-based study. Neurology. 2011 Oct 11; 77(15):1438-45. Epub 2011 Sep 14. 2190763
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  53. Savica R, Rabinstein AA, Josephs KA. Ifosfamide associated myoclonus-encephalopathy syndrome. J Neurol. 2011 Sep; 258(9):1729-31. Epub 2011 Mar 12. 2152022
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  54. Whitwell JL, Avula R, Master A, Vemuri P, Senjem ML, Jones DT, Jack CR Jr, Josephs KA. Disrupted thalamocortical connectivity in PSP: a resting-state fMRI, DTI, and VBM study. Parkinsonism Relat Disord. 2011 Sep; 17(8):599-605. Epub 2011 Jun 12. 2169967
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  55. Rascovsky K, Hodges JR, Knopman D, Mendez MF, Kramer JH, Neuhaus J, van Swieten JC, Seelaar H, Dopper EG, Onyike CU, Hillis AE, Josephs KA, Boeve BF, Kertesz A, Seeley WW, Rankin KP, Johnson JK, Gorno-Tempini ML, Rosen H, Prioleau-Latham CE, Lee A, Kipps Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011 Sep; 134(Pt 9):2456-77. Epub 2011 Aug 02. 2199838
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  56. Whitwell JL, Jack CR Jr, Przybelski SA, Parisi JE, Senjem ML, Boeve BF, Knopman DS, Petersen RC, Dickson DW, Josephs KA. Temporoparietal atrophy: a marker of AD pathology independent of clinical diagnosis. Neurobiol Aging. 2011 Sep; 32(9):1531-41. Epub 2009 Nov 14. 1972842
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  57. Whitwell JL, Josephs KA, Avula R, Tosakulwong N, Weigand SD, Senjem ML, Vemuri P, Jones DT, Gunter JL, Baker M, Wszolek ZK, Knopman DS, Rademakers R, Petersen RC, Boeve BF, Jack CR Jr. Altered functional connectivity in asymptomatic MAPT subjects: a comparison to bvFTD. Neurology. 2011 Aug 30; 77(9):866-74. Epub 2011 Aug 17. 2184829
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  58. Josephs KA, Hodges JR, Snowden JS, Mackenzie IR, Neumann M, Mann DM, Dickson DW. Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol. 2011 Aug; 122(2):137-53. Epub 2011 May 26. 2166910
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  59. Savica R, Matsumoto JY, Josephs KA, Ahlskog JE, Stead M, Lee KH, Klassen BT. Deep brain stimulation in benign tremulous parkinsonism. Arch Neurol. 2011 Aug; 68(8):1033-6. 2181953
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  60. Whitwell JL, Weigand SD, Gunter JL, Boeve BF, Rademakers R, Baker M, Knopman DS, Wszolek ZK, Petersen RC, Jack CR Jr, Josephs KA. Trajectories of brain and hippocampal atrophy in FTD with mutations in MAPT or GRN. Neurology. 2011 Jul 26; 77(4):393-8. Epub 2011 Jul 13. 2176118
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  61. Cornelius JR, Pittock SJ, McKeon A, Lennon VA, Aston PA, Josephs KA, Tippmann-Peikert M, Silber MH. Sleep manifestations of voltage-gated potassium channel complex autoimmunity. Arch Neurol. 2011 Jun; 68(6):733-8. 2170004
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  62. Whitwell JL, Master AV, Avula R, Kantarci K, Eggers SD, Edmonson HA, Jack CR Jr, Josephs KA. Clinical correlates of white matter tract degeneration in progressive supranuclear palsy. Arch Neurol. 2011 Jun; 68(6):753-60. 2169959
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  63. Coon EA, Sorenson EJ, Whitwell JL, Knopman DS, Josephs KA. Predicting survival in frontotemporal dementia with motor neuron disease. Neurology. 2011 May 31; 76(22):1886-93. 2167783
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  64. Frigerio R, Fujishiro H, Ahn TB, Josephs KA, Maraganore DM, DelleDonne A, Parisi JE, Klos KJ, Boeve BF, Dickson DW, Ahlskog JE. Incidental Lewy body disease: do some cases represent a preclinical stage of dementia with Lewy bodies? Neurobiol Aging. 2011 May; 32(5):857-63. Epub 2009 Jun 26. 1951014
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  65. Li X, Lapid MI, Burton MC, Josephs KA. Psychoses as the presenting manifestation of Creutzfeldt-Jakob disease in an elderly male. Gen Hosp Psychiatry. 2011 May-Jun; 33(3):302.e7-9. Epub 2011 Mar 24. 2166915
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  66. Hassan A, Bower JH, Kumar N, Matsumoto JY, Fealey RD, Josephs KA, Ahlskog JE. Dopamine agonist-triggered pathological behaviors: surveillance in the PD clinic reveals high frequencies. Parkinsonism Relat Disord. 2011 May; 17(4):260-4. Epub 2011 Feb 09. 2147579
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  67. Kouri N, Whitwell JL, Josephs KA, Rademakers R, Dickson DW. Corticobasal degeneration: a pathologically distinct 4R tauopathy. Nat Rev Neurol. 2011 May; 7(5):263-72. Epub 2011 Apr 12. 2160276
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  68. Wicklund MR, Mokri B, Drubach DA, Boeve BF, Parisi JE, Josephs KA. Frontotemporal brain sagging syndrome: an SIH-like presentation mimicking FTD. Neurology. 2011 Apr 19; 76(16):1377-82. 2160250
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  69. Vemuri P, Simon G, Kantarci K, Whitwell JL, Senjem ML, Przybelski SA, Gunter JL, Josephs KA, Knopman DS, Boeve BF, Ferman TJ, Dickson DW, Parisi JE, Petersen RC, Jack CR Jr. Antemortem differential diagnosis of dementia pathology using structural MRI: Differential-STAND. Neuroimage. 2011 Mar 15; 55(2):522-31. Epub 2010 Dec 31. 2141759
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  70. Claassen DO, Lowe VJ, Peller PJ, Petersen RC, Josephs KA. Amyloid and glucose imaging in dementia with Lewy bodies and multiple systems atrophy. Parkinsonism Relat Disord. 2011 Mar; 17(3):160-5. Epub 2010 Dec 31. 2141217
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  71. Passov V, Gavrilova RH, Strand E, Cerhan JH, Josephs KA. Sporadic corticobasal syndrome with progranulin mutation presenting as progressive apraxic agraphia. Arch Neurol. 2011 Mar; 68(3):376-80. 2153023
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  72. Josephs KA, Whitwell JL, Eggers SD, Senjem ML, Jack CR Jr. Gray matter correlates of behavioral severity in progressive supranuclear palsy. Mov Disord. 2011 Feb 15; 26(3):493-8. Epub 2011 Jan 12. 2157430
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  73. Hassan A, Bower JH, Kumar N, Matsumoto JY, Fealey RD, Josephs KA, Ahlskog JE. Corrigendum to "Dopamine agonist-triggered pathological behaviors: Surveillance in the PD clinic reveals high frequencies" [Parkinsonism Relat Disord 17 (2011) 260-264]. Parkinsonism Relat Disord. 2011; 17(6):499. 2176642
  74. Tomiyasu A, Nakamura M, Ichiba M, Ueno S, Saiki S, Morimoto M, Kobal J, Kageyama Y, Inui T, Wakabayashi K, Yamada T, Kanemori Y, Jung HH, Tanaka H, Orimo S, Afawi Z, Blatt I, Aasly J, Ujike H, Babovic-Vuksanovic D, Josephs KA, Tohge R, Rodrigues GR, Dupre Novel pathogenic mutations and copy number variations in the VPS13A Gene in patients with chorea-acanthocytosis. American Journal of Medical Genetics, Part B: Neuropsychiatric Genetics. 2011; 156(5):620-31. 2176584
  75. Kocerha J, Kouri N, Baker M, Finch N, DeJesus-Hernandez M, Gonzalez J, Chidamparam K, Josephs KA, Boeve BF, Graff-Radford NR, Crook J, Dickson DW, Rademakers R. Altered microRNA expression in frontotemporal lobar degeneration with TDP-43 pathology caused by progranulin mutations. BMC Genomics. 2011; 12:527. Epub 2011 Oct 27. 2209904
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  76. Whitwell JL, Josephs KA. Neuroimaging in frontotemporal lobar degeneration--predicting molecular pathology. Nat Rev Neurol. 2011; 8(3):131-42. Epub 2012 Jan 31. 2221003
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  77. Whitwell JL, Jack CR Jr, Parisi JE, Senjem ML, Knopman DS, Boeve BF, Rademakers R, Baker M, Petersen RC, Dickson DW, Josephs KA. Does TDP-43 type confer a distinct pattern of atrophy in frontotemporal lobar degeneration? Neurology. 2010 Dec 14; 75(24):2212-20. 2136002
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  78. Graff-Radford J, Ahlskog JE, Bower JH, Josephs KA. Dopamine agonists and Othello's syndrome. Parkinsonism Relat Disord. 2010 Dec; 16(10):680-2. Epub 2010 Sep 09. 2114854
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  79. Whitwell JL, Jack CR Jr, Boeve BF, Parisi JE, Ahlskog JE, Drubach DA, Senjem ML, Knopman DS, Petersen RC, Dickson DW, Josephs KA. Imaging correlates of pathology in corticobasal syndrome. Neurology. 2010 Nov 23; 75(21):1879-87. 2124966
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  80. Josephs KA. Dementia and the TAR DNA binding protein 43. Clin Pharmacol Ther. 2010 Oct; 88(4):555-8. Epub 2010 Aug 25. 2112328
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  81. Flanagan EP, McKeon A, Lennon VA, Boeve BF, Trenerry MR, Tan KM, Drubach DA, Josephs KA, Britton JW, Mandrekar JN, Lowe V, Parisi JE, Pittock SJ. Autoimmune dementia: clinical course and predictors of immunotherapy response. Mayo Clin Proc. 2010 Oct; 85(10):881-97. 2118657
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  82. Claassen DO, Josephs KA, Ahlskog JE, Silber MH, Tippmann-Peikert M, Boeve BF. REM sleep behavior disorder preceding other aspects of synucleinopathies by up to half a century. Neurology. 2010 Aug 10; 75(6):494-9. Epub 2010 Jul 28. 2109321
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  83. Dickson DW, Ahmed Z, Algom AA, Tsuboi Y, Josephs KA. Neuropathology of variants of progressive supranuclear palsy. Curr Opin Neurol. 2010 Aug; 23(4):394-400. 2105106
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  84. Josephs KA, Whitwell JL, Boeve BF, Knopman DS, Petersen RC, Hu WT, Parisi JE, Dickson DW, Jack CR Jr. Anatomical differences between CBS-corticobasal degeneration and CBS-Alzheimer's disease. Mov Disord. 2010 Jul 15; 25(9):1246-52. 2106732
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  85. Josephs KA, Whitwell JL, Parisi JE, Petersen RC, Boeve BF, Jack CR Jr, Dickson DW. Caudate atrophy on MRI is a characteristic feature of FTLD-FUS. Eur J Neurol. 2010 Jul; 17(7):969-75. Epub 2010 Mar 03. 2106755
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  86. Urwin H, Josephs KA, Rohrer JD, Mackenzie IR, Neumann M, Authier A, Seelaar H, Van Swieten JC, Brown JM, Johannsen P, Nielsen JE, Holm IE, FReJA Consortium, Dickson DW, Rademakers R, Graff-Radford NR, Parisi JE, Petersen RC, Hatanpaa KJ, White CL 3rd, FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathol. 2010 Jul; 120(1):33-41. Epub 2010 May 20. 2143932
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  87. Josephs KA, Duffy JR, Fossett TR, Strand EA, Claassen DO, Whitwell JL, Peller PJ. Fluorodeoxyglucose F18 positron emission tomography in progressive apraxia of speech and primary progressive aphasia variants. Arch Neurol. 2010 May; 67(5):596-605. 1999722
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  88. Whitwell JL, Avula R, Senjem ML, Kantarci K, Weigand SD, Samikoglu A, Edmonson HA, Vemuri P, Knopman DS, Boeve BF, Petersen RC, Josephs KA, Jack CR Jr. Gray and white matter water diffusion in the syndromic variants of frontotemporal dementia. Neurology. 2010 Apr 20; 74(16):1279-87. 1997847
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  89. Gendron TF, Josephs KA, Petrucelli L. Review: transactive response DNA-binding protein 43 (TDP-43): mechanisms of neurodegeneration. Neuropathol Appl Neurobiol. 2010 Apr; 36(2):97-112. Epub 2010 Feb 19 1990507
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  182. Josephs KA, Mandrekar JN, Dickson DW. The relationship between histopathological features of progressive supranuclear palsy and disease duration. Parkinsonism Relat Disord. 2006 Mar; 12(2):109-12. Epub 2005 Dec 06. 1752216
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  183. Josephs KA, Matsumoto JY, Ahlskog JE. Benign tremulous parkinsonism. Arch Neurol. 2006 Mar; 63(3):354-7. 1754342
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  184. Crum BA, Josephs KA. Varied electrophysiologic patterns in spinocerebellar ataxia type 2. Eur J Neurol. 2006 Feb; 13(2):194-7. 1750492
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  185. Josephs KA, Petersen RC, Knopman DS, Boeve BF, Whitwell JL, Duffy JR, Parisi JE, Dickson DW. Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology. 2006 Jan 10; 66(1):41-8. 1744205
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  186. Whitwell JL, Jack CR Jr, Senjem ML, Josephs KA. Patterns of atrophy in pathologically confirmed FTLD with and without motor neuron degeneration. Neurology. 2006 Jan 10; 66(1):102-4. 1744206
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  187. Shamim A, Siddiqui BK, Josephs KA. The corticobasal syndrome triggered by central pontine myelinolysis. Eur J Neurol. 2006 Jan; 13(1):82-4. 1744231
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  188. Josephs KA, Whitwell JL, Boeve BF, Shiung MM, Gunter JL, Parisi JE, Dickson DW, Jack CR. Rates of cerebral atrophy in autopsy-confirmed progressive supranuclear palsy. Ann Neurol. 2006 Jan; 59(1):200-3. 1744182
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  189. Ridha B, Josephs KA. Young-onset dementia: a practical approach to diagnosis. Neurologist. 2006 Jan; 12(1):2-13. 1792934
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  190. Klos KJ, Josephs KA, Parisi JE, Dickson DW. Alpha-synuclein immunohistochemistry in two cases of co-occurring idiopathic Parkinson's disease and motor neuron disease. Mov Disord. 2005 Nov; 20(11):1515-20. 1736552
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  191. Klos KJ, Bower JH, Josephs KA, Matsumoto JY, Ahlskog JE. Pathological hypersexuality predominantly linked to adjuvant dopamine agonist therapy in Parkinson's disease and multiple system atrophy. Parkinsonism Relat Disord. 2005 Sep; 11(6):381-6. 2194047
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  192. Dodd ML, Klos KJ, Bower JH, Geda YE, Josephs KA, Ahlskog JE. Pathological gambling caused by drugs used to treat Parkinson disease. Arch Neurol. 2005 Sep; 62(9):1377-81. 1730125
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  193. Klos KJ, Ahlskog JE, Josephs KA, Fealey RD, Cowl CT, Kumar N. Neurologic spectrum of chronic liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging: probable manganese neurotoxicity. Arch Neurol. 2005 Sep; 62(9):1385-90. 1730126
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  194. Whitwell JL, Josephs KA, Rossor MN, Stevens JM, Revesz T, Holton JL, Al-Sarraj S, Godbolt AK, Fox NC, Warren JD. Magnetic resonance imaging signatures of tissue pathology in frontotemporal dementia. Arch Neurol. 2005 Sep; 62(9):1402-8. 1730127
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  195. Josephs KA, Knopman DS, Whitwell JL, Boeve BF, Parisi JE, Petersen RC, Dickson DW. Survival in two variants of tau-negative frontotemporal lobar degeneration: FTLD-U vs FTLD-MND. Neurology. 2005 Aug 23; 65(4):645-7. 1730021
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  196. Ridha BH, Josephs KA, Rossor MN. Delusions and hallucinations in dementia with Lewy bodies: Worsening with memantine. Neurology. 2005 Aug 9; 65(3):481-2. 1729975
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  197. Josephs KA, Boeve BF, Duffy JR, Smith GE, Knopman DS, Parisi JE, Petersen RC, Dickson DW. Atypical progressive supranuclear palsy underlying progressive apraxia of speech and nonfluent aphasia. Neurocase. 2005 Aug; 11(4):283-96. 1729949
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  198. Tsuboi Y, Josephs KA, Boeve BF, Litvan I, Caselli RJ, Caviness JN, Uitti RJ, Bott AD, Dickson DW. Increased tau burden in the cortices of progressive supranuclear palsy presenting with corticobasal syndrome. Mov Disord. 2005 Aug; 20(8):982-8. 1730007
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  199. Godbolt AK, Josephs KA, Revesz T, Warrington EK, Lantos P, King A, Fox NC, Al Sarraj S, Holton J, Cipolotti L, Khan MN, Rossor MN. Sporadic and familial dementia with ubiquitin-positive tau-negative inclusions - Clinical features of one histopathological abnormality underlying frontotemporal lobar degeneration. Arch Neurol. 2005 Jul; 62(7):1097-101. 1726823
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  200. Hu WT, Josephs KA, Ahlskog JE, Shin C, Boeve BF, Witte RJ. MRI correlates of alien leg-like phenomenon in corticobasal degeneration. Mov Disord. 2005 Jul; 20(7):870-3. 1729799
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  201. Josephs KA, Ahlskog JE, Klos KJ, Kumar N, Fealey RD, Trenerry MR, Cowl CT. Neurologic manifestations in welders with pallidal MRI T1 hyperintensity. Neurology. 2005 Jun 28; 64(12):2033-9. 1721423
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  202. Rippon GA, Boeve BF, Parisi JE, Dickson DW, Ivnik RI, Jack CR, Hutton M, Baker M, Josephs KA, Knopman DS, Petersen RC. Late-onset frontotemporal dementia associated with progressive supranuclear palsy/argyrophilic grain disease/Alzheimer's disease pathology. Neurocase. 2005 Jun; 11(3):204-11. 1720568
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  203. Josephs KA, Uchikado H, McComb RD, Bashir R, Wszolek Z, Swanson J, Matsumoto J, Shaw G, Dickson DW. Extending the clinicopathological spectrum of neurofilament inclusion disease. Acta Neuropathol (Berl) 2005 Apr; 109(4):427-32. 1720360
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  204. Matthews BR, Jones LK, Saad DA, Aksamit AJ, Josephs KA. Cerebellar ataxia and central nervous system whipple disease. Arch Neurol. 2005 Apr; 62(4):618-20. 1708463
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  205. Knopman DS, Boeve BF, Parisi JE, Dickson DW, Smith GE, Ivnik RJ, Josephs KA, Petersen RC. Antemortem diagnosis of frontotemporal lobar degeneration. Ann Neurol. 2005 Apr; 57(4):480-8. 1705101
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  206. Stead M, Josephs KA. Successful treatment of status migrainosus after electroconvulsive therapy with dihydroergotamine. Headache. 2005 Apr; 45(4):378-80. 1705086
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  207. Janseen JC, Schott JM, Cipolotti L, Fox NC, Scahill RI, Josephs KA, Stevens JM, Rossor MN. Mapping the onset and progression of atrophy in familial frontotemporal lobar degeneration. J Neurol Neurosurg Psychiatry. 2005 Feb; 76(2):162-8. 1424245
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  208. Josephs KA, Matsumoto JY, Lindor NM. Heterozygous Niemann-Pick disease type C presenting with tremor. Neurology. 2004 Dec 14; 63(11):2189-90. 1677623
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  209. Josephs KA, Tang-Wai DF, Edland SD, Knopman DS, Dickson DW, Parisi JE, Petersen RC, Jack CR, Boeve BF. Correlation between antemortem magnetic resonance Imaging findings and pathologically confirmed corticobasal degeneration. Arch Neurol. 2004 Dec; 61(12):1881-4. 1677658
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  210. Josephs KA, Silber MH, Fealey RD, Nippoldt TB, Auger RG, Vernino S. Neurophysiologic studies in Morvan syndrome. J Clin Neurophysiol. 2004 Nov-Dec; 21(6):440-5. 1677766
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  211. Paviour DC, Lees AJ, Josephs KA, Ozawa T, Ganguly M, Strand C, Godbolt A, Howard RS, Revesz T, Holton JL. Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal changes: broadening the clinical picture to include progressive supranuclear palsy. Brain. 2004 Nov; 127(Part 11):2441-51. 1669276
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  212. Ozawa T, Paviour D, Quinn NP, Josephs KA, Sangha H, Kliford L, Healy DG, Wood NW, Lees AJ, Holton JL, Rvesz T. The spectrum of pathological involvement of the striatonigral and olivopontocerebellar systems in multiple system atrophy: clinicopathological correlations. Brain. 2004 Oct 27; Epub ahead of print. 1667641
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  213. Josephs KA, Tsuboi Y, Cookson N, Watt H, Dickson DW. Apolipoprotein E epsilon 4 is a determinant for Alzheimer-type pathologic features in tauopathies, synucleinopathies, and frontotemporal degeneration. Arch Neurol. 2004 Oct; 61(10):1579-84. 1669389
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  214. Josephs KA, Holton JL, Rossor MN, Godbolt AK, Ozawa T, Strand K, Khan N, Al-Sarraj S, Revesz T. Frontotemporal lobar degeneration and ubiquitin immunohistochemistry. Neuropathology & Applied Neurobiology. 2004 Aug; 30(4):369-73. 1660711
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  215. Josephs KA, Rubino FA, Dickson DW. Nonvasculitic autoimmune inflammatory meningoencephalitis. Neuropathology. 2004 Jun; 24(2):149-52. 1647229
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  216. Josephs KA, Tsuboi Y, Dickson DW. Creutzfeldt-Jakob disease presenting as progressive supranuclear palsy. Eur J Neurol. 2004 May; 11(5):343-6. 1645500
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  217. Josephs KA, Jones AG, Dickson DW. Hippocampal sclerosis and ubiquitin-positive inclusions in dementia lacking distinctive histopathology. Dem and Geriatric Cog Dis. 2004; 17(4):342-5. 1649172
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  218. Whitwell JL, Warren JD, Josephs KA, Godbolt AK, Revesz T, Fox NC, Rossor MN. Voxel-based morphometry in tau-positive and tau-negative frontotemporal lobar degenerations. Neurodegener Dis. 2004; 1(4-5):225-30. 1667643
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  219. Tang-Wai DF, Josephs KA, Boeve BF, Dickson DW, Parisi JE, Petersen RC. Pathologically confirmed corticobasal degeneration presenting with visuospatial dysfunction. Neurology. 2003 Oct 28; 61(8):1134-5. 1422240
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  220. Josephs KA, Holton JL, Rossor MN, Braendgaard H, Ozawa T, Fox NC, Petersen RC, Pearl GS, Ganguly M, Rosa P, Laursen H, Parisi JE, Waldemar G, Quinn NP, Dickson DW, Revesz T. Neurofilament inclusion body disease: a new proteinopathy? Brain. 2003 Oct; 126(Pt 10):2291-303. Epub 2003 Jul 22. 1415056
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  221. Josephs KA, Dickson DW. Diagnostic accuracy of progressive supranuclear palsy in the Society for Progressive Supranuclear Palsy brain bank. Mov Disord. 2003 Sep; 18(9):1018-26. 1415132
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  222. Tsuboi Y, Slowinski J, Josephs KA, Honer WG, Wszolek ZK, Dickson DW. Atrophy of superior cerebellar peduncle in progressive supranuclear palsy. Neurology. 2003 Jun 10; 60(11):1766-9. 1396726
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  223. Josephs KA, Tang Wai DF, Parisi JE. Hippocampal sclerosis in a case of Alzheimer's disease-like dementia with late onset intractable epilepsy. Eur J Neurol. 2003 May; 10(3):333-4. 1341203
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  224. Josephs KA, Van Gerpen MW, Van Gerpen JA. Adult onset Niemann-Pick disease type C presenting with psychosis. J Neurol Neurosurg Psychiatry. 2003 Apr; 74(4):528-9. 1328707
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  225. Tsuboi Y, Josephs KA, Cookson N, Dickson DW. APOE E4 is a determinant for Alzheimer type pathology in progressive supranuclear palsy. Neurology. 2003 Jan 28; 60(2):240-5. 1318262
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  226. Josephs KA, Ishizawa T, Tsuboi Y, Cookson N, Dickson DW. A clinicopathological study of vascular progressive supranuclear palsy: a multi-infarct disorder presenting as progressive supranuclear palsy. Arch Neurol. 2002 Oct; 59(10):1597-601. 1316649
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  227. Josephs KA, Parisi JE, Dickson DW. Alpha-synuclein studies are negative in postencephalic parkinsonism of von Economo. Neurology. 2002 Aug 27; 59(4):645-6. 1315206
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  228. Travis LH, Josephs KA, Bower JH. 25-year-old woman with incoordination and decreased balance. Mayo Clin Proc. 2001 May; 76(5):551-4. 1004959
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  229. Flemming KD, Josephs K, Wijdicks EF. Enlarging vertebrobasilar dolichoectasia with subarachnoid hemorrhage heralded by recurrent ischemia. Case illustration. J Neurosurg. 2000 Mar; 92(3):504. 1011723
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