Publications - Keith A. Josephs, M.D.

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1. 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]
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2. 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]
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3. 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]
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4. 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]
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5. 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]
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6. 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]
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7. Bruns MB, Josephs KA. Neuropsychiatry of corticobasal degeneration and progressive supranuclear palsy. Int Rev Psychiatry. 2013 Apr; 25(2):197-209.
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8. 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.
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9. Whitwell JL, Duffy JR, Strand EA, Xia R, Mandrekar J, Machulda MM, Senjem ML, Lowe VJ, Jack Jr CR, Josephs KA. Distinct regional anatomic and functional correlates of neurodegenerative apraxia of speech and aphasia: An MRI and FDG-PET study. Brain Lang. 2013 Mar 28; 125(3):245-52. [Epub ahead of print]
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10. 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]
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11. 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.
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12. 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.
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13. 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.
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14. 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.
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15. Adeli A, Whitwell JL, Duffy JR, Strand EA, Josephs KA. Ideomotor apraxia in agrammatic and logopenic variants of primary progressive aphasia. J Neurol. 2013 Jan 29. [Epub ahead of print]
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16. 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.
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17. 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.
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18. 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.
19. 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.
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20. 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.
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21. Whitwell JL, Josephs KA. Recent advances in the imaging of frontotemporal dementia. Curr Neurol Neurosci Rep. 2012 Dec; 12(6):715-23.
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22. 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.
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23. 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.
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24. 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.
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25. 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.
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26. 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.
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27. 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.
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28. 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.
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29. 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.
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30. 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.
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31. 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.
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32. 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.
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33. 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.
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34. Graff-Radford J, Josephs KA. Primary progressive aphasia and transient global amnesia. Arch Neurol. 2012 Mar; 69(3):401-4.
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35. 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.
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36. 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.
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37. 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.
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38. 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.
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39. 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.
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40. 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.
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41. 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.
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42. 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.
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43. 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.
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44. 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.
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45. 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.
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46. Hassan A, Whitwell JL, Josephs KA. The corticobasal syndrome-Alzheimer's disease conundrum. Expert Rev Neurother. 2011 Nov; 11(11):1569-78.
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47. 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.
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48. 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.
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49. 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.
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50. 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.
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51. 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.
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52. Savica R, Rabinstein AA, Josephs KA. Ifosfamide associated myoclonus-encephalopathy syndrome. J Neurol. 2011 Sep; 258(9):1729-31. Epub 2011 Mar 12.
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53. 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.
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54. 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.
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55. 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.
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56. 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.
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57. 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.
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58. 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.
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59. 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.
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60. 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.
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61. 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.
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62. 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.
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63. 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.
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64. 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.
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65. 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.
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66. 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.
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67. 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.
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68. 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.
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69. 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.
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70. 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.
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71. 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.
72. 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.
73. 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.
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74. Whitwell JL, Josephs KA. Neuroimaging in frontotemporal lobar degeneration--predicting molecular pathology. Nat Rev Neurol. 2011; 8(3):131-42. Epub 2012 Jan 31.
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75. 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.
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76. 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.
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77. 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.
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78. 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.
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79. Josephs KA. Dementia and the TAR DNA binding protein 43. Clin Pharmacol Ther. 2010 Oct; 88(4):555-8. Epub 2010 Aug 25.
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80. 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.
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81. 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.
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82. 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.
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83. 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.
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84. 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.
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85. 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.
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86. 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.
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87. 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
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88. Hassan A, Whitwell JL, Boeve BF, Jack CR Jr, Parisi JE, Dickson DW, Josephs KA. Symmetric corticobasal degeneration (S-CBD). Parkinsonism Relat Disord. 2010 Mar; 16(3):208-14. Epub 2009 Dec 16.
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89. Claassen DO, Josephs KA, Peller PJ. The stripe of primary lateral sclerosis: focal primary motor cortex hypometabolism seen on fluorodeoxyglucose F18 positron emission tomography. Arch Neurol. 2010 Jan; 67(1):122-5.
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90. Dickson DW, Fujishiro H, Orr C, DelleDonne A, Josephs KA, Frigerio R, Burnett M, Parisi JE, Klos KJ, Ahlskog JE. Neuropathology of non-motor features of Parkinson disease. Parkinsonism Relat Disord. 2009 Dec; 15 Suppl 3:S1-5.
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91. Josephs KA, Whitwell JL, Knopman DS, Boeve BF, Vemuri P, Senjem ML, Parisi JE, Ivnik RJ, Dickson DW, Petersen RC, Jack CR Jr. Two distinct subtypes of right temporal variant frontotemporal dementia. Neurology. 2009 Nov 3; 73(18):1443-50.
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92. Hu WT, Seelaar H, Josephs KA, Knopman DS, Boeve BF, Sorenson EJ, McCluskey L, Elman L, Schelhaas HJ, Parisi JE, Kuesters B, Lee VM, Trojanowski JQ, Petersen RC, van Swieten JC, Grossman M. Survival profiles of patients with frontotemporal dementia and motor neuron disease. Arch Neurol. 2009 Nov; 66(11):1359-64.
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93. Whitwell JL, Przybelski SA, Weigand SD, Ivnik RJ, Vemuri P, Gunter JL, Senjem ML, Shiung MM, Boeve BF, Knopman DS, Parisi JE, Dickson DW, Petersen RC, Jack CR Jr, Josephs KA. Distinct anatomical subtypes of the behavioural variant of frontotemporal dementia: a cluster analysis study. Brain. 2009 Nov; 132(Pt 11):2932-46. Epub 2009 Sep 17.
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94. Cobb SA, Wider C, Ross OA, Mata IF, Adler CH, Rajput A, Rajput AH, Wu RM, Hauser R, Josephs KA, Carr J, Gwinn K, Heckman MG, Aasly JO, Lynch T, Uitti RJ, Wszolek ZK, Kapatos G, Farrer MJ. GCH1 in darly-onset Parkinson's disease. Mov Disord. 2009 Oct 30; 24(14):2070-5.
95. Cobb SA, Wider C, Ross OA, Mata IF, Adler CH, Rajput A, Rajput AH, Wu RM, Hauser R, Josephs KA, Carr J, Gwinn K, Heckman MG, Aasly JO, Lynch T, Uitti RJ, Wszolek ZK, Kapatos G, Farrer MJ. GCH1 in early-onset Parkinson's disease. Mov Disord. 2009 Oct 30; 24(14):2070-5.
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96. Whitwell JL, Jack CR Jr, Boeve BF, Senjem ML, Baker M, Ivnik RJ, Knopman DS, Wszolek ZK, Petersen RC, Rademakers R, Josephs KA. Atrophy patterns in IVS10+16, IVS10+3, N279K, S305N, P301L, and V337M MAPT mutations. Neurology. 2009 Sep 29; 73(13):1058-65.
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97. Frigerio R, Fujishiro H, Maraganore DM, Klos KJ, DelleDonne A, Heckman MG, Crook JE, Josephs KA, Parisi JE, Boeve BF, Dickson DW, Ahlskog JE. Comparison of risk factor profiles in incidental Lewy body disease and Parkinson disease. Arch Neurol. 2009 Sep; 66(9):1114-9.
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98. Josephs KA, Stroh A, Dugger B, Dickson DW. Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes. Acta Neuropathol. 2009 Sep; 118(3):349-58. Epub 2009 May 20.
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99. Hu WT, Rippon GW, Boeve BF, Knopman DS, Petersen RC, Parisi JE, Josephs KA. Alzheimer's disease and corticobasal degeneration presenting as corticobasal syndrome. Mov Disord. 2009 Jul 15; 24(9):1375-9.
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100. Mateen FJ, Josephs KA. The clinical spectrum of stereotypies in frontotemporal lobar degeneration. Mov Disord. 2009 Jun 15; 24(8):1237-40.
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101. Vilarino-Guell C, Wider C, Soto-Ortolaza AI, Cobb SA, Kachergus JM, Keeling BH, Dachsel JC, Hulihan MM, Dickson DW, Wszolek ZK, Uitti RJ, Graff-Radford NR, Boeve BF, Josephs KA, Miller B, Boylan KB, Gwinn K, Adler CH, Aasly JO, Hentati F, Destee A, Characterization of DCTN1 genetic variability in neurodegeneration. Neurology. 2009 Jun 9; 72(23):2024-8.
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102. Kelley BJ, Haidar W, Boeve BF, Baker M, Graff-Radford NR, Krefft T, Frank AR, Jack CR Jr, Shiung M, Knopman DS, Josephs KA, Parashos SA, Rademakers R, Hutton M, Pickering-Brown S, Adamson J, Kuntz KM, Dickson DW, Parisi JE, Smith GE, Ivnik RJ, Petersen Prominent phenotypic variability associated with mutations in Progranulin. Neurobiol Aging. 2009 May; 30(5):739-51. Epub 2007 Oct 18.
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103. Whitwell JL, Jack CR Jr, Boeve BF, Senjem ML, Baker M, Rademakers R, Ivnik RJ, Knopman DS, Wszolek ZK, Petersen RC, Josephs KA. Voxel-based morphometry patterns of atrophy in FTLD with mutations in MAPT or PGRN. Neurology. 2009 Mar 3; 72(9):813-20.
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104. Kelley BJ, Boeve BF, Josephs KA. Rapidly progressive young-onset dementia. Cogn Behav Neurol. 2009 Mar; 22(1):22-7.
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105. Josephs KA, Ahlskog JE, Parisi JE, Boeve BF, Crum BA, Giannini C, Petersen RC. Rapidly progressive neurodegenerative dementias. Arch Neurol. 2009 Feb; 66(2):201-7.
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106. McKeon A, Ahlskog JE, Bower JH, Josephs KA, Matsumoto JY. Psychogenic tremor: long-term prognosis in patients with electrophysiologically confirmed disease. Mov Disord. 2009 Jan 15; 24(1):72-6.
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107. Cooper AD, Josephs KA. Photophobia, visual hallucinations, and REM sleep behavior disorder in progressive supranuclear palsy and corticobasal degeneration: a prospective study. Parkinsonism Relat Disord. 2009 Jan; 15(1):59-61. Epub 2008 Mar 06.
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108. Whitwell JL, Jack CR Jr, Senjem ML, Parisi JE, Boeve BF, Knopman DS, Dickson DW, Petersen RC, Josephs KA. MRI correlates of protein deposition and disease severity in postmortem frontotemporal lobar degeneration. Neurodegener Dis. 2009; 6(3):106-17. Epub 2009 Mar 19.
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109. Kelley BJ, Boeve BF, Josephs KA. Cognitive and noncognitive neurological features of young-onset dementia. Dement Geriatr Cogn Disord. 2009; 27(6):564-71. Epub 2009 Jul 11.
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110. Yurchenko A, Lapid MI, Josephs KA. Frontotemporal dementia in older adults: Diagnostic and therapeutic challenges. Clinical Geriatrics. 2009; 17(1):26-31.
111. Whitwell JL, Josephs KA. Imaging in familial frontotemporal lobar degeneration with mutations in MAPT or PGRN. European Neurological Journal. 2009; 1:25-31.
112. Rademakers R, Eriksen JL, Baker M, Robinson T, Ahmed Z, Lincoln SJ, Finch N, Rutherford NJ, Crook RJ, Josephs KA, Boeve BF, Knopman DS, Petersen RC, Parisi JE, Caselli RJ, Wszolek ZK, Uitti RJ, Feldman H, Hutton ML, Mackenzie IR, Graff-Radford NR, Common variation in the miR-659 binding-site of GRN is a major risk factor for TDP43-positive frontotemporal dementia. Hum Mol Genet. 2008 Dec 1; 17(23):3631-42. Epub 2008 Aug 21.
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113. Czarnecki K, Duffy JR, Nehl CR, Cross SA, Molano JR, Jack CR Jr, Shiung MM, Josephs KA, Boeve BF. Very early semantic dementia with progressive temporal lobe atrophy: an 8-year longitudinal study. Arch Neurol. 2008 Dec; 65(12):1659-63.
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114. Josephs KA, Duffy JR. Apraxia of speech and nonfluent aphasia: a new clinical marker for corticobasal degeneration and progressive supranuclear palsy. Curr Opin Neurol. 2008 Dec; 21(6):688-92.
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115. Josephs KA, Whitwell JL, Jack CR Jr. Anatomic correlates of stereotypies in frontotemporal lobar degeneration. Neurobiol Aging. 2008 Dec; 29(12):1859-63. Epub 2007 Jun 14.
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116. Josephs KA, Whitwell JL, Vemuri P, Senjem ML, Boeve BF, Knopman DS, Smith GE, Ivnik RJ, Petersen RC, Jack CR Jr. The anatomic correlate of prosopagnosia in semantic dementia. Neurology. 2008 Nov 11; 71(20):1628-33.
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117. Kelley BJ, Boeve BF, Josephs KA. Young-onset dementia: demographic and etiologic characteristics of 235 patients. Arch Neurol. 2008 Nov; 65(11):1502-8.
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118. Whitwell JL, Josephs KA, Murray ME, Kantarci K, Przybelski SA, Weigand SD, Vemuri P, Senjem ML, Parisi JE, Knopman DS, Boeve BF, Petersen RC, Dickson DW, Jack CR Jr. MRI correlates of neurofibrillary tangle pathology at autopsy: a voxel-based morphometry study. Neurology. 2008 Sep 2; 71(10):743-9.
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119. Claassen DO, Parisi JE, Giannini C, Boeve BF, Dickson DW, Josephs KA. Frontotemporal dementia mimicking dementia with Lewy bodies. Cogn Behav Neurol. 2008 Sep; 21(3):157-63.
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120. Fujishiro H, Ahn TB, Frigerio R, DelleDonne A, Josephs KA, Parisi JE, Eric Ahlskog J, Dickson DW. Glial cytoplasmic inclusions in neurologically normal elderly: prodromal multiple system atrophy? Acta Neuropathol. 2008 Sep; 116(3):269-75. Epub 2008 Jun 14.
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121. Vemuri P, Whitwell JL, Kantarci K, Josephs KA, Parisi JE, Shiung MS, Knopman DS, Boeve BF, Petersen RC, Dickson DW, Jack CR Jr. Antemortem MRI based STructural Abnormality iNDex (STAND)-scores correlate with postmortem Braak neurofibrillary tangle stage. Neuroimage. 2008 Aug 15; 42(2):559-67. Epub 2008 May 20.
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122. DelleDonne A, Klos KJ, Fujishiro H, Ahmed Z, Parisi JE, Josephs KA, Frigerio R, Burnett M, Wszolek ZK, Uitti RJ, Ahlskog JE, Dickson DW. Incidental Lewy body disease and preclinical Parkinson disease. Arch Neurol. 2008 Aug; 65(8):1074-80.
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123. Hu WT, Josephs KA, Knopman DS, Boeve BF, Dickson DW, Petersen RC, Parisi JE. Temporal lobar predominance of TDP-43 neuronal cytoplasmic inclusions in Alzheimer disease. Acta Neuropathol. 2008 Aug; 116(2):215-20. Epub 2008 Jul 01.
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124. Josephs KA, Lin WL, Ahmed Z, Stroh DA, Graff-Radford NR, Dickson DW. Frontotemporal lobar degeneration with ubiquitin-positive, but TDP-43-negative inclusions. Acta Neuropathol. 2008 Aug; 116(2):159-67. Epub 2008 Jun 14.
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125. Milone M, Brunetti-Pierri N, Tang LY, Kumar N, Mezei MM, Josephs K, Powell S, Simpson E, Wong LJ. Sensory ataxic neuropathy with ophthalmoparesis caused by POLG mutations. Neuromuscul Disord. 2008 Aug; 18(8):626-32. Epub 2008 Jun 27.
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126. Josephs KA. Frontotemporal dementia and related disorders: deciphering the enigma. Ann Neurol. 2008 Jul; 64(1):4-14.
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127. Kantarci K, Knopman DS, Dickson DW, Parisi JE, Whitwell JL, Weigand SD, Josephs KA, Boeve BF, Petersen RC, Jack CR Jr. Alzheimer disease: postmortem neuropathologic correlates of antemortem 1H MR spectroscopy metabolite measurements. Radiology. 2008 Jul; 248(1):210-20.
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128. Fujishiro H, Frigerio R, Burnett M, Klos KJ, Josephs KA, Delledonne A, Parisi JE, Ahlskog JE, Dickson DW. Cardiac sympathetic denervation correlates with clinical and pathologic stages of Parkinson's disease. Mov Disord. 2008 Jun 15; 23(8):1085-92.
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129. Wider C, Uitti RJ, Wszolek ZK, Fang JY, Josephs KA, Baker MC, Rademakers R, Hutton ML, Dickson DW. Progranulin gene mutation with an unusual clinical and neuropathologic presentation. Mov Disord. 2008 Jun 15; 23(8):1168-73.
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130. Josephs KA, Whitwell JL, Knopman DS, Hu WT, Stroh DA, Baker M, Rademakers R, Boeve BF, Parisi JE, Smith GE, Ivnik RJ, Petersen RC, Jack CR Jr, Dickson DW. Abnormal TDP-43 immunoreactivity in AD modifies clinicopathologic and radiologic phenotype. Neurology. 2008 May 6; 70(19 Pt 2):1850-7. Epub 2008 Apr 09.
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131. Josephs KA. Review of Frontotemporal dementia syndromes. Journal of Neurology, Neurosurgery & Psychiatry. 2008 May; 79(5):614.
132. Dickson DW, Fujishiro H, DelleDonne A, Menke J, Ahmed Z, Klos KJ, Josephs KA, Frigerio R, Burnett M, Parisi JE, Ahlskog JE. Evidence that incidental Lewy body disease is pre-symptomatic Parkinson's disease. Acta Neuropathol. 2008 Apr; 115(4):437-44. Epub 2008 Feb 09.
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133. Josephs KA, Whitwell JL, Parisi JE, Knopman DS, Boeve BF, Geda YE, Jack CR Jr, Petersen RC, Dickson DW. Argyrophilic grains: a distinct disease or an additive pathology? Neurobiol Aging. 2008 Apr; 29(4):566-73. Epub 2006 Dec 26.
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134. Keegan BM, Giannini C, Parisi JE, Lucchinetti CF, Boeve BF, Josephs KA. Sporadic adult-onset leukoencephalopathy with neuroaxonal spheroids mimicking cerebral MS. Neurology. 2008 Mar 25; 70(13 Pt 2):1128-33. Epub 2008 Feb 20.
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135. Whitwell JL, Jack CR Jr, Pankratz VS, Parisi JE, Knopman DS, Boeve BF, Petersen RC, Dickson DW, Josephs KA. Rates of brain atrophy over time in autopsy-proven frontotemporal dementia and Alzheimer disease. Neuroimage. 2008 Feb 1; 39(3):1034-40. Epub 2007 Oct 10.
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136. Josephs KA, Whitwell JL, Ahmed Z, Shiung MM, Weigand SD, Knopman DS, Boeve BF, Parisi JE, Petersen RC, Dickson DW, Jack CR Jr. Beta-amyloid burden is not associated with rates of brain atrophy. Ann Neurol. 2008 Feb; 63(2):204-12.
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137. Ahmed Z, Josephs KA, Gonzalez J, DelleDonne A, Dickson DW. Clinical and neuropathologic features of progressive supranuclear palsy with severe pallido-nigro-luysial degeneration and axonal dystrophy. Brain. 2008 Feb; 131(Pt 2):460-72. Epub 2007 Dec 24.
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138. Josephs KA, Whitwell JL, Dickson DW, Boeve BF, Knopman DS, Petersen RC, Parisi JE, Jack CR Jr. Voxel-based morphometry in autopsy proven PSP and CBD. Neurobiol Aging. 2008 Feb; 29(2):280-9. Epub 2006 Nov 13.
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139. Czarnecki K, Kumar N, Josephs KA. Parkinsonism and tardive antecollis in frontotemporal dementia--increased sensitivity to newer antipsychotics? Eur J Neurol. 2008 Feb; 15(2):199-201.
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140. Josephs KA, Whitwell JL, Duffy JR, Vanvoorst WA, Strand EA, Hu WT, Boeve BF, Graff-Radford NR, Parisi JE, Knopman DS, Dickson DW, Jack CR Jr, Petersen RC. Progressive aphasia secondary to Alzheimer disease vs FTLD pathology. Neurology. 2008 Jan 1; 70(1):25-34.
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141. Boeve BF, Josephs KA, Drubach DA. Current and future management of the corticobasal syndrome and corticobasal degeneration. Handb Clin Neurol. 2008; 89:533-48.
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142. Josephs KA. Clinical aspects of TDP-43 proteinopathy, neurofilament inclusion body disease and dementias lacking distinctive proteinopathy. Handb Clin Neurol. 2008; 89:377-82.
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143. McKeon A, Josephs KA, Klos KJ, Hecksel K, Bower JH, Michael Bostwick J, Eric Ahlskog J. Unusual compulsive behaviors primarily related to dopamine agonist therapy in Parkinson's disease and multiple system atrophy. Parkinsonism Relat Disord. 2007 Dec; 13(8):516-9. Epub 2007 Jun 04.
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144. Josephs KA. Capgras syndrome and its relationship to neurodegenerative disease. Arch Neurol. 2007 Dec; 64(12):1762-6.
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145. Geschwind MD, Josephs KA, Parisi JE, Keegan BM. A 54-year-old man with slowness of movement and confusion. Neurology. 2007 Nov 6; 69(19):1881-7.
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146. Hu WT, Mandrekar JN, Parisi JE, Knopman DS, Boeve BF, Petersen RC, Hutton M, Dickson DW, Josephs KA. Clinical features of pathologic subtypes of behavioral--variant frontotemporal dementia. Arch Neurol. 2007 Nov; 64(11):1611-6.
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147. Josephs KA, Dickson DW. Hippocampal sclerosis in tau-negative frontotemporal lobar degeneration. Neurobiol Aging. 2007 Nov; 28(11):1718-22. Epub 2006 Aug 22.
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148. Josephs KA, Dickson DW. Frontotemporal lobar degeneration with upper motor neuron disease/ primary lateral sclerosis. Neurology. 2007 Oct 30; 69(18):1800-1.
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149. Rademakers R, Baker M, Gass J, Adamson J, Huey ED, Momeni P, Spina S, Coppola G, Karydas AM, Stewart H, Johnson N, Hsiung GY, Kelley B, Kuntz K, Steinbart E, Wood EM, Yu CE, Josephs K, Sorenson E, Womack KB, Weintraub S, Pickering-Brown SM, Schofield PR, Phenotypic variability associated with progranulin haploinsufficiency in patients with the common 1477C-->T (Arg493X) mutation: an international initiative. Lancet Neurol. 2007 Oct; 6(10):857-68.
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150. Hu WT, Parisi JE, Knopman DS, Boeve BF, Dickson DW, Ahlskog JE, Petersen RC, Josephs KA. Clinical features and survival of 3R and 4R tauopathies presenting as behavioral variant frontotemporal dementia. Alzheimer Dis Assoc Disord. 2007 Oct-Dec; 21(4):S39-43.
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151. Rascovsky K, Hodges JR, Kipps CM, Johnson JK, Seeley WW, Mendez MF, Knopman D, Kertesz A, Mesulam M, Salmon DP, Galasko D, Chow TW, Decarli C, Hillis A, Josephs K, Kramer JH, Weintraub S, Grossman M, Gorno-Tempini ML, Miller BM. Diagnostic criteria for the behavioral variant of frontotemporal dementia (bvFTD): current limitations and future directions. Alzheimer Dis Assoc Disord. 2007 Oct-Dec; 21(4):S14-8.
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152. McKeon A, Pittock SJ, Glass GA, Josephs KA, Bower JH, Lennon VA, Ahlskog JE. Whole-body tremulousness: isolated generalized polymyoclonus. Arch Neurol. 2007 Sep; 64(9):1318-22.
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153. Josephs KA. Frontotemporal lobar degeneration. Neurol Clin. 2007 Aug; 25(3):683-96, vi.
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154. Dickson DW, Josephs KA, Amador-Ortiz C. TDP-43 in differential diagnosis of motor neuron disorders. Acta Neuropathol. 2007 Jul; 114(1):71-9. Epub 2007 Jun 14.
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155. Whitwell JL, Jack CR Jr, Kantarci K, Weigand SD, Boeve BF, Knopman DS, Drubach DA, Tang-Wai DF, Petersen RC, Josephs KA. Imaging correlates of posterior cortical atrophy. Neurobiol Aging. 2007 Jul; 28(7):1051-61. Epub 2006 Jun 23.
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156. Whitwell JL, Jack CR Jr, Parisi JE, Knopman DS, Boeve BF, Petersen RC, Ferman TJ, Dickson DW, Josephs KA. Rates of cerebral atrophy differ in different degenerative pathologies. Brain. 2007 Apr; 130(Pt 4):1148-58. Epub 2007 Mar 08.
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157. Frigerrio R, Glass G, Klos K, Ahlskog J, et al. Do Incidental Lewy Body Disease (ILBD) and Parkinson's disease (PD) Have Similar Risk Factor Profiles? An Exploratory Study. Neurology. 2007 Mar 20; 68(Suppl 1):A259.
158. Whitwell JL, Weigand SD, Shiung MM, Boeve BF, Ferman TJ, Smith GE, Knopman DS, Petersen RC, Benarroch EE, Josephs KA, Jack CR Jr. Focal atrophy in dementia with Lewy bodies on MRI: a distinct pattern from Alzheimer's disease. Brain. 2007 Mar; 130(Pt 3):708-19. Epub 2007 Jan 31.
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159. Whitwell JL, Jack CR Jr, Baker M, Rademakers R, Adamson J, Boeve BF, Knopman DS, Parisi JF, Petersen RC, Dickson DW, Hutton ML, Josephs KA. Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations. Arch Neurol. 2007 Mar; 64(3):371-6.
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160. Josephs KA, Ahmed Z, Katsuse O, Parisi JF, Boeve BF, Knopman DS, Petersen RC, Davies P, Duara R, Graff-Radford NR, Uitti RJ, Rademakers R, Adamson J, Baker M, Hutton ML, Dickson DW. Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations. J Neuropathol Exp Neurol. 2007 Feb; 66(2):142-51.
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161. Miller BL, Josephs KA, Parisi JE, Keegan BM. A 34-year-old man with progressive behavioral and language disturbance. Neurology. 2007 Jan 2; 68(1):68-74.
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162. Whitwell JL, Josephs KA. Voxel-based morphometry and its application to movement disorders. Parkinsonism Relat Disord. 2007; 13 Suppl 3:S406-16.
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163. Klos KJ, Ahlskog JE, Kumar N, Cambern S, Butz J, Burritt M, Fealey RD, Cowl CT, Parisi JE, Josephs KA. Brain metal concentrations in chronic liver failure patients with pallidal T1 MRI hyperintensity. Neurology. 2006 Dec 12; 67(11):1984-9.
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164. Boeve BF, Baker M, Dickson DW, Parisi JE, Giannini C, Josephs KA, Hutton M, Pickering-Brown SM, Rademakers R, Tang-Wai D, Jack CR Jr, Kantarci K, Shiung MM, Golde T, Smith GE, Geda YE, Knopman DS, Petersen RC. Frontotemporal dementia and parkinsonism associated with the IVS1+1G->A mutation in progranulin: a clinicopathologic study. Brain. 2006 Nov; 129(Pt 11):3103-14. Epub 2006 Oct 09.
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165. Josephs KA, Whitwell JL, Jack CR, Parisi JE, Dickson DW. Frontotemporal lobar degeneration without lobar atrophy. Arch Neurol. 2006 Nov; 63(11):1632-8.
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166. Gass J, Cannon A, Mackenzie IR, Boeve B, Baker M, Adamson J, Crook R, Melquist S, Kuntz K, Petersen R, Josephs K, Pickering-Brown SM, Graff-Radford N, Uitti R, Dickson D, Wszolek Z, Gonzalez J, Beach TG, Bigio E, Johnson N, Weintraub S, Mesulam M, White Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration. Hum Mol Genet. 2006 Oct 15; 15(20):2988-3001. Epub 2006 Sep 01.
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167. Klos KJ, Chandler M, Kumar N, Ahlskog JE, Josephs KA. Neuropsychological profiles of manganese neurotoxicity. Eur J Neurol. 2006 Oct; 13(10):1139-41.
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168. Josephs KA, Whitwell JL, Boeve BF, Knopman DS, Tang-Wai DF, Drubach DA, Jack CR Jr, Petersen RC. Visual hallucinations in posterior cortical atrophy. Arch Neurol. 2006 Oct; 63(10):1427-32.
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169. Barnes J, Whitwell JL, Frost C, Josephs KA, Rossor M, Fox NC. Measurements of the amygdala and hippocampus in pathologically confirmed Alzheimer disease and frontotemporal lobar degeneration. Arch Neurol. 2006 Oct; 63(10):1434-9.
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170. Hu WT, Murray JA, Greenaway MC, Parisi JE, Josephs KA. Cognitive impairment and celiac disease. Arch Neurol. 2006 Oct; 63(10):1440-6.
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171. Baba Y, Putzke JD, Tsuboi Y, Josephs KA, Thomas N, Wszolek ZK, Dickson DW. Effect of MAPT and APOE on prognosis of progressive supranuclear palsy. Neurosci Lett. 2006 Sep 11; 405(1-2):116-9. Epub 2006 Jul 12.
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172. Glass GA, Josephs KA, Ahlskog JE. Respiratory insufficiency as the primary presenting symptom of multiple-system atrophy. Arch Neurol. 2006 Jul; 63(7):978-81.
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173. Josephs KA, Duffy JR, Strand EA, Whitwell JL, Layton KF, Parisi JE, Hauser MF, Witte RJ, Boeve BF, Knopman DS, Dickson DW, Jack CR Jr, Petersen RC. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain. 2006 Jun; 129(Pt 6):1385-98. Epub 2006 Apr 13.
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174. Cannon A, Baker M, Boeve B, Josephs K, Knopman D, Petersen R, Parisi J, Dickison D, Adamson J, Snowden J, Neary D, Mann D, Hutton M, Pickering-Brown SM. CHMP2B mutations are not a common cause of frontotemporal lobar degeneration. Neurosci Lett. 2006 May 1; 398(1-2):83-4. Epub 2006 Jan 23.
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175. Klos KJ, Ahlskog JE, Josephs KA, Apaydin H, Parisi JE, Boeve BF, DeLucia MW, Dickson DW. Alpha-synuclein pathology in the spinal cords of neurologically asymptomatic aged individuals. Neurology. 2006 Apr 11; 66(7):1100-2.
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176. Josephs KA, Parisi JE, Knopman DS, Boeve BF, Petersen RC, Dickson DW. Clinically undetected motor neuron disease in pathologically proven frontotemporal lobar degeneration with motor neuron disease. Arch Neurol. 2006 Apr; 63(4):506-12.
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177. Josephs KA, Katsuse O, Beccano-Kelly DA, Lin WL, Uitti RJ, Fujino Y, Boeve BF, Hutton ML, Baker MC, Dickson DW. Atypical progressive supranuclear palsy with corticospinal tract degeneration. J Neuropathol Exp Neurol. 2006 Apr; 65(4):396-405.
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178. Ozawa T, Healy DG, Abou-Sleiman PM, Ahmadi KR, Quinn N, Lees AJ, Shaw K, Wullner U, Berciano J, Moller JC, Kamm C, Burk K, Josephs KA, Barone P, Tolosa E, Goldstein DB, Wenning G, Geser F, Holton JL, Gasser T, Revesz T, Wood NW, European MSA study group. The alpha-synuclein gene in multiple system atrophy. J Neurol Neurosurg Psychiatry. 2006 Apr; 77(4):464-7.
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179. Murali HR, Josephs KA, Wijdicks EF. Guillain-Barre syndrome in an immunocompromised patient and coccidioidomycosis infection. Rev Neurol Dis. 2006 Spring; 3(2):82-4.
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180. 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.
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181. Josephs KA, Matsumoto JY, Ahlskog JE. Benign tremulous parkinsonism. Arch Neurol. 2006 Mar; 63(3):354-7.
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182. Crum BA, Josephs KA. Varied electrophysiologic patterns in spinocerebellar ataxia type 2. Eur J Neurol. 2006 Feb; 13(2):194-7.
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183. 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.
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184. 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.
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185. Shamim A, Siddiqui BK, Josephs KA. The corticobasal syndrome triggered by central pontine myelinolysis. Eur J Neurol. 2006 Jan; 13(1):82-4.
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186. 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.
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187. Ridha B, Josephs KA. Young-onset dementia: a practical approach to diagnosis. Neurologist. 2006 Jan; 12(1):2-13.
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188. 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.
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189. 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.
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190. 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.
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191. 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.
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192. 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.
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193. 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.
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194. 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.
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195. 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.
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196. 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.
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197. 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.
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198. 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.
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199. 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.
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200. 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.
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201. Stead M, Josephs KA. Successful treatment of status migrainosus after electroconvulsive therapy with dihydroergotamine. Headache. 2005 Apr; 45(4):378-80.
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202. 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.
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203. 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.
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204. 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.
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205. 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.
206. Josephs KA, Matsumoto JY, Lindor NM. Heterozygous Niemann-Pick disease type C presenting with tremor. Neurology. 2004 Dec 14; 63(11):2189-90.
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207. 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.
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208. 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.
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209. 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.
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210. 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.
211. 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.
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212. 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.
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213. Josephs KA, Rubino FA, Dickson DW. Nonvasculitic autoimmune inflammatory meningoencephalitis. Neuropathology. 2004 Jun; 24(2):149-52.
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214. Josephs KA, Tsuboi Y, Dickson DW. Creutzfeldt-Jakob disease presenting as progressive supranuclear palsy. Eur J Neurol. 2004 May; 11(5):343-6.
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215. 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.
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216. 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.
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217. 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.
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218. 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.
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219. 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.
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220. 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.
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221. 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.
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222. 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.
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223. 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.
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224. 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.
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225. 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.
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226. Travis LH, Josephs KA, Bower JH. 25-year-old woman with incoordination and decreased balance. Mayo Clin Proc. 2001 May; 76(5):551-4.
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227. 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.
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