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Chapter 14 - The cognitive neurology of corticobasal degeneration and progressive supranuclear palsy

Published online by Cambridge University Press:  01 December 2016

Bruce L. Miller
Affiliation:
University of California, San Francisco
Bradley F. Boeve
Affiliation:
Mayo Clinic, Minnesota
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Print publication year: 2016

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References

Rebeiz, JJ, Kolodny, EH, Richardson, EP Jr. Corticodentatonigral degeneration with neuronal achromasia. Archives of Neurology. 1968;18(1):2033.CrossRefGoogle ScholarPubMed
Gibb, WR, Luthert, PJ, Marsden, CD. Corticobasal degeneration. Brain: A Journal of Neurology. 1989;112(Pt 5):1171–92.CrossRefGoogle ScholarPubMed
Riley, DE, Lang, AE, Lewis, A, Resch, L, Ashby, P, Hornykiewicz, O, et al. Cortical-basal ganglionic degeneration. Neurology. 1990;40(8):1203–12.CrossRefGoogle ScholarPubMed
Constantinidis, J, Richard, J, Tissot, R. Pick’s disease. Histological and clinical correlations. European Neurology. 1974;11(4):208–17.CrossRefGoogle ScholarPubMed
Lee, SE, Rabinovici, GD, Mayo, MC, Wilson, SM, Seeley, WW, DeArmond, SJ, et al. Clinicopathological correlations in corticobasal degeneration. Annals of Neurology. 2011;70(2):327–40.CrossRefGoogle ScholarPubMed
Vanvoorst, WA, Greenaway, MC, Boeve, BF, Ivnik, RJ, Parisi, JE, Eric Ahlskog, J, et al. Neuropsychological findings in clinically atypical autopsy confirmed corticobasal degeneration and progressive supranuclear palsy. Parkinsonism & Related Disorders. 2008;14(4):376–8.CrossRefGoogle ScholarPubMed
Grimes, DA, Lang, AE, Bergeron, CB. Dementia as the most common presentation of cortical-basal ganglionic degeneration. Neurology. 1999;53(9):1969–74.CrossRefGoogle ScholarPubMed
Murray, R, Neumann, M, Forman, MS, Farmer, J, Massimo, L, Rice, A, et al. Cognitive and motor assessment in autopsy-proven corticobasal degeneration. Neurology. 2007;68(16):1274–83.CrossRefGoogle ScholarPubMed
Kertesz, A, McMonagle, P, Blair, M, Davidson, W, Munoz, DG. The evolution and pathology of frontotemporal dementia. Brain: A Journal of Neurology. 2005;128(Pt 9):19962005.CrossRefGoogle ScholarPubMed
Geda, YE, Boeve, BF, Negash, S, Graff-Radford, NR, Knopman, DS, Parisi, JE, et al. Neuropsychiatric features in 36 pathologically confirmed cases of corticobasal degeneration. The Journal of Neuropsychiatry and Clinical Neurosciences. 2007;19(1):7780.CrossRefGoogle ScholarPubMed
Wakabayashi, K, Oyanagi, K, Makifuchi, T, Ikuta, F, Homma, A, Homma, Y, et al. Corticobasal degeneration: etiopathological significance of the cytoskeletal alterations. Acta Neuropathologica. 1994;87(6):545–53.CrossRefGoogle Scholar
Grundke-Iqbal, I, Iqbal, K, Quinlan, M, Tung, YC, Zaidi, MS, Wisniewski, HM. Microtubule-associated protein tau. A component of Alzheimer paired helical filaments. The Journal of Biological Chemistry. 1986;261(13):6084–9.Google ScholarPubMed
Witman, GB, Cleveland, DW, Weingarten, MD, Kirschner, MW. Tubulin requires tau for growth onto microtubule initiating sites. Proceedings of the National Academy of Sciences of the USA. 1976;73(11):4070–4.Google ScholarPubMed
Buee Scherrer, V, Hof, PR, Buee, L, Leveugle, B, Vermersch, P, Perl, DP, et al. Hyperphosphorylated tau proteins differentiate corticobasal degeneration and Pick’s disease. Acta Neuropathologica. 1996;91(4):351–9.CrossRefGoogle Scholar
Cairns, NJ, Bigio, EH, Mackenzie, IR, Neumann, M, Lee, VM, Hatanpaa, KJ, et al. Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathologica. 2007;114(1):522.CrossRefGoogle ScholarPubMed
Dickson, DW, Bergeron, C, Chin, SS, Duyckaerts, C, Horoupian, D, Ikeda, K, et al. Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. Journal of Neuropathology and Experimental Neurology. 2002;61(11):935–46.CrossRefGoogle Scholar
Komori, T, Arai, N, Oda, M, Nakayama, H, Mori, H, Yagishita, S, et al. Astrocytic plaques and tufts of abnormal fibers do not coexist in corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathologica. 1998;96(4):401–8.Google ScholarPubMed
Feany, MB, Dickson, DW. Widespread cytoskeletal pathology characterizes corticobasal degeneration. American Journal of Pathology. 1995;146(6):1388–96.Google ScholarPubMed
Togasaki, DM, Tanner, CM. Epidemiologic aspects. Advances in Neurology. 2000;82:53–9.CrossRefGoogle ScholarPubMed
Nath, U, Burn, DJ. The epidemiology of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Parkinsonism & Related Disorders. 2000;6(3):145–53.Google ScholarPubMed
Morimatsu, M, Negoro, K. [Provisional diagnostic criteria of corticobasal degeneration (CBD) and the survey of patients with CBD in Japan]. Rinsho Shinkeigaku. 2002;42(11):1150–3.CrossRefGoogle ScholarPubMed
Wenning, GK, Litvan, I, Jankovic, J, Granata, R, Mangone, CA, McKee, A, et al. Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. Journal of Neurology, Neurosurgery, and Psychiatry. 1998;64(2):184–9.CrossRefGoogle ScholarPubMed
Mahapatra, RK, Edwards, MJ, Schott, JM, Bhatia, KP. Corticobasal degeneration. The Lancet Neurology. 2004;3(12):736–43.CrossRefGoogle Scholar
Bugiani, O, Murrell, JR, Giaccone, G, Hasegawa, M, Ghigo, G, Tabaton, M, et al. Frontotemporal dementia and corticobasal degeneration in a family with a P301S mutation in tau. Journal of Neuropathology and Experimental Neurology. 1999;58(6):667–77.Google ScholarPubMed
Spillantini, MG, Yoshida, H, Rizzini, C, Lantos, PL, Khan, N, Rossor, MN, et al. A novel tau mutation (N296N) in familial dementia with swollen achromatic neurons and corticobasal inclusion bodies. Annals of Neurology. 2000;48(6):939–43.CrossRefGoogle ScholarPubMed
Rossi, G, Marelli, C, Farina, L, Laura, M, Maria Basile, A, Ciano, C, et al. The G389R mutation in the MAPT gene presenting as sporadic corticobasal syndrome. Movement Disorders: Official Journal of the Movement Disorder Society. 2008;23(6):892–5.CrossRefGoogle ScholarPubMed
Yu, CE, Bird, TD, Bekris, LM, Montine, TJ, Leverenz, JB, Steinbart, E, et al. The spectrum of mutations in progranulin: a collaborative study screening 545 cases of neurodegeneration. Archives of Neurology. 2010;67(2):161–70.CrossRefGoogle ScholarPubMed
Rohrer, JD, Beck, J, Warren, JD, King, A, Al Sarraj, S, Holton, J, et al. Corticobasal syndrome associated with a novel 1048_1049insG progranulin mutation. Journal of Neurology, Neurosurgery, and Psychiatry. 2009;80(11):1297–8.CrossRefGoogle ScholarPubMed
Masellis, M, Momeni, P, Meschino, W, Heffner, R Jr., Elder, J, Sato, C, et al. Novel splicing mutation in the progranulin gene causing familial corticobasal syndrome. Brain: a Journal of Neurology. 2006;129(Pt 11):3115–23.CrossRefGoogle Scholar
Conrad, C, Amano, N, Andreadis, A, Xia, Y, Namekataf, K, Oyama, F, et al. Differences in a dinucleotide repeat polymorphism in the tau gene between Caucasian and Japanese populations: implication for progressive supranuclear palsy. Neuroscience Letters. 1998;250(2):135–7.CrossRefGoogle ScholarPubMed
Evans, W, Fung, HC, Steele, J, Eerola, J, Tienari, P, Pittman, A, et al. The tau H2 haplotype is almost exclusively Caucasian in origin. Neuroscience Letters. 2004;369(3):183–5.CrossRefGoogle ScholarPubMed
Baker, M, Litvan, I, Houlden, H, Adamson, J, Dickson, D, Perez-Tur, J, et al. Association of an extended haplotype in the tau gene with progressive supranuclear palsy. Human Molecular Genetics. 1999;8(4):711–15.CrossRefGoogle Scholar
Houlden, H, Baker, M, Morris, HR, MacDonald, N, Pickering-Brown, S, Adamson, J, et al. Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype. Neurology. 2001;56(12):1702–6.CrossRefGoogle ScholarPubMed
Verpillat, P, Camuzat, A, Hannequin, D, Thomas-Anterion, C, Puel, M, Belliard, S, et al. Association between the extended tau haplotype and frontotemporal dementia. Archives of Neurology. 2002;59(6):935–9.CrossRefGoogle ScholarPubMed
Hughes, A, Mann, D, Pickering-Brown, S. Tau haplotype frequency in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Experimental Neurology. 2003;181(1):1216.CrossRefGoogle ScholarPubMed
Sobrido, MJ, Abu-Khalil, A, Weintraub, S, Johnson, N, Quinn, B, Cummings, JL, et al. Possible association of the tau H1/H1 genotype with primary progressive aphasia. Neurology. 2003;60(5):862–4.CrossRefGoogle ScholarPubMed
Kertesz, A, Martinez-Lage, P, Davidson, W, Munoz, DG. The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology. 2000;55(9):1368–75.CrossRefGoogle ScholarPubMed
Graham, NL, Bak, TH, Hodges, JR. Corticobasal degeneration as a cognitive disorder. Movement Disorders: Official Journal of the Movement Disorder Society. 2003;18(11):1224–32.CrossRefGoogle ScholarPubMed
Pillon, B, Blin, J, Vidailhet, M, Deweer, B, Sirigu, A, Dubois, B, et al. The neuropsychological pattern of corticobasal degeneration: comparison with progressive supranuclear palsy and Alzheimer’s disease. Neurology. 1995;45(8):1477–83.CrossRefGoogle ScholarPubMed
Belfor, N, Amici, S, Boxer, AL, Kramer, JH, Gorno-Tempini, ML, Rosen, HJ, et al. Clinical and neuropsychological features of corticobasal degeneration. Mechanisms of Ageing and Development. 2006;127(2):203–7.CrossRefGoogle ScholarPubMed
Gorno-Tempini, ML, Hillis, AE, Weintraub, S, Kertesz, A, Mendez, M, Cappa, SF, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011;76(11):1006–14.CrossRefGoogle ScholarPubMed
Boeve, BF, Maraganore, DM, Parisi, JE, Ahlskog, JE, Graff-Radford, N, Caselli, RJ, et al. Pathologic heterogeneity in clinically diagnosed corticobasal degeneration. Neurology. 1999;53(4):795800.CrossRefGoogle Scholar
Josephs, KA, Dickson, DW. Diagnostic accuracy of progressive supranuclear palsy in the Society for Progressive Supranuclear Palsy brain bank. Movement Disorders: Official Journal of the Movement Disorder Society. 2003;18(9):1018–26.CrossRefGoogle ScholarPubMed
Josephs, KA, Petersen, RC, Knopman, DS, Boeve, BF, Whitwell, JL, Duffy, JR, et al. Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology. 2006;66(1):41–8.CrossRefGoogle ScholarPubMed
Rohrer, JD, Geser, F, Zhou, J, Gennatas, ED, Sidhu, M, Trojanowski, JQ, et al. TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia. Neurology. 2010;75(24):2204–11.CrossRefGoogle ScholarPubMed
Rascovsky, K, Hodges, JR, Knopman, D, Mendez, MF, Kramer, JH, Neuhaus, J, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain: A Journal of Neurology. 2011;134(Pt 9):2456–77.CrossRefGoogle ScholarPubMed
Litvan, I, Agid, Y, Goetz, C, Jankovic, J, Wenning, GK, Brandel, JP, et al. Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study. Neurology. 1997;48(1):119–25.CrossRefGoogle ScholarPubMed
Mackenzie, IR, Neumann, M, Bigio, EH, Cairns, NJ, Alafuzoff, I, Kril, J, et al. Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathologica. 2010;119(1):14.CrossRefGoogle ScholarPubMed
Josephs, KA, Duffy, JR, Strand, EA, Whitwell, JL, Layton, KF, Parisi, JE, et al. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain: A Journal of Neurology. 2006;129(Pt 6):1385–98.CrossRefGoogle ScholarPubMed
Armstrong, MJ, Litvan, I, Lang, AE, Bak, TH, Bhatia, KP, Borroni, B, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013;80(5):496503.CrossRefGoogle ScholarPubMed
Ling, H, O’Sullivan, SS, Holton, JL, Revesz, T, Massey, LA, Williams, DR, et al. Does corticobasal degeneration exist? A clinicopathological re-evaluation. Brain: A Journal of Neurology. 2010;133(Pt 7):2045–57.CrossRefGoogle ScholarPubMed
Hu, WT, Rippon, GW, Boeve, BF, Knopman, DS, Petersen, RC, Parisi, JE, et al. Alzheimer’s disease and corticobasal degeneration presenting as corticobasal syndrome. Movement Disorders: Official Journal of the Movement Disorder Society. 2009;24(9):1375–9.CrossRefGoogle ScholarPubMed
Shelley, BP, Hodges, JR, Kipps, CM, Xuereb, JH, Bak, TH. Is the pathology of corticobasal syndrome predictable in life? Movement Disorders: Official Journal of the Movement Disorder Society. 2009;24(11):1593–9.CrossRefGoogle ScholarPubMed
Rivaud-Pechoux, S, Vidailhet, M, Gallouedec, G, Litvan, I, Gaymard, B, Pierrot-Deseilligny, C. Longitudinal ocular motor study in corticobasal degeneration and progressive supranuclear palsy. Neurology. 2000;54(5):1029–32.CrossRefGoogle ScholarPubMed
Garbutt, S, Matlin, A, Hellmuth, J, Schenk, AK, Johnson, JK, Rosen, H, et al. Oculomotor function in frontotemporal lobar degeneration, related disorders and Alzheimer’s disease. Brain: A Journal of Neurology. 2008;131(Pt 5):1268–81.CrossRefGoogle ScholarPubMed
Vidailhet, M, Rivaud, S, Gouider-Khouja, N, Pillon, B, Bonnet, AM, Gaymard, B, et al. Eye movements in parkinsonian syndromes. Annals of Neurology. 1994;35(4):420–6.CrossRefGoogle ScholarPubMed
Rottach, KG, Riley, DE, DiScenna, AO, Zivotofsky, AZ, Leigh, RJ. Dynamic properties of horizontal and vertical eye movements in Parkinsonian syndromes. Annals of Neurology. 1996;39(3):368–77.Google ScholarPubMed
Rivaud-Pechoux, S, Vidailhet, M, Brandel, JP, Gaymard, B. Mixing pro- and antisaccades in patients with Parkinsonian syndromes. Brain: A Journal of Neurology. 2007;130(Pt 1):256–64.Google ScholarPubMed
Boxer, AL, Garbutt, S, Seeley, WW, Jafari, A, Heuer, HW, Mirsky, J, et al. Saccade abnormalities in autopsy-confirmed frontotemporal lobar degeneration and Alzheimer disease. Archives of Neurology. 2012;69(4):509–17.CrossRefGoogle ScholarPubMed
Groschel, K, Hauser, TK, Luft, A, Patronas, N, Dichgans, J, Litvan, I, et al. Magnetic resonance imaging-based volumetry differentiates progressive supranuclear palsy from corticobasal degeneration. NeuroImage. 2004;21(2):714–24.CrossRefGoogle ScholarPubMed
Hassan, A, Whitwell, JL, Boeve, BF, Jack, CR Jr., Parisi, JE, Dickson, DW, et al. Symmetric corticobasal degeneration (S-CBD). Parkinsonism & Related Disorders. 2010;16(3):208–14.CrossRefGoogle ScholarPubMed
Josephs, KA, Whitwell, JL, Dickson, DW, Boeve, BF, Knopman, DS, Petersen, RC, et al. Voxel-based morphometry in autopsy proven PSP and CBD. Neurobiology of Aging. 2008;29(2):280–9.CrossRefGoogle ScholarPubMed
Steele, JC, Richardson, JC, Olszewski, J. Progressive supranuclear palsy. A heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Archives of Neurology. 1964;10:333–59.3.0.CO;2-V>CrossRefGoogle Scholar
Chambers, CB, Lee, JM, Troncoso, JC, Reich, S, Muma, NA. Overexpression of four-repeat tau mRNA isoforms in progressive supranuclear palsy but not in Alzheimer’s disease. Annals of Neurology. 1999;46(3):325–32.Google ScholarPubMed
Steele, JC. Progressive supranuclear palsy. Brain: A Journal of Neurology. 1972;95(4):693704.CrossRefGoogle ScholarPubMed
David, NJ, Mackey, EA, Smith, JL. Further observations in progressive supranuclear palsy. Neurology. 1968;18(4):349–56.CrossRefGoogle ScholarPubMed
Litvan, I, Agid, Y, Calne, D, Campbell, G, Dubois, B, Duvoisin, RC, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology. 1996;47(1):19.CrossRefGoogle ScholarPubMed
Osaki, Y, Ben-Shlomo, Y, Lees, AJ, Daniel, SE, Colosimo, C, Wenning, G, et al. Accuracy of clinical diagnosis of progressive supranuclear palsy. Movement Disorders: Official Journal of the Movement Disorder Society. 2004;19(2):181–9.CrossRefGoogle ScholarPubMed
Litvan, I, Agid, Y, Jankovic, J, Goetz, C, Brandel, JP, Lai, EC, et al. Accuracy of clinical criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Neurology. 1996;46(4):922–30.CrossRefGoogle Scholar
Hughes, AJ, Daniel, SE, Ben-Shlomo, Y, Lees, AJ. The accuracy of diagnosis of Parkinsonian syndromes in a specialist movement disorder service. Brain: A Journal of Neurology. 2002;125(Pt 4):861–70.CrossRefGoogle ScholarPubMed
Daniel, SE, de Bruin, VM, Lees, AJ. The clinical and pathological spectrum of Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy): a reappraisal. Brain: a Journal of Neurology. 1995;118 ( Pt 3):759–70.CrossRefGoogle ScholarPubMed
Litvan, I, Grimes, DA, Lang, AE, Jankovic, J, McKee, A, Verny, M, et al. Clinical features differentiating patients with postmortem confirmed progressive supranuclear palsy and corticobasal degeneration. Journal of Neurology. 1999;246 (Suppl 2):II1–15.CrossRefGoogle ScholarPubMed
Morris, HR, Gibb, G, Katzenschlager, R, Wood, NW, Hanger, DP, Strand, C, et al. Pathological, clinical and genetic heterogeneity in progressive supranuclear palsy. Brain: A Journal of Neurology. 2002;125(Pt 5):969–75.CrossRefGoogle ScholarPubMed
Wadia, PM, Lang, AE. The many faces of corticobasal degeneration. Parkinsonism & Related Disorders. 2007;13(Suppl 3):S336–40.CrossRefGoogle ScholarPubMed
Lantos, PL. The neuropathology of progressive supranuclear palsy. Journal of Neural Transmission Supplementum. 1994;42:137–52.CrossRefGoogle ScholarPubMed
Williams, DR, de Silva, R, Paviour, DC, Pittman, A, Watt, HC, Kilford, L, et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-Parkinsonism. Brain : A Journal of Neurology. 2005;128(Pt 6):1247–58.CrossRefGoogle ScholarPubMed
Williams, DR, Holton, JL, Strand, C, Pittman, A, de Silva, R, Lees, AJ, et al. Pathological tau burden and distribution distinguishes progressive supranuclear palsy-Parkinsonism from Richardson’s syndrome. Brain: A Journal of Neurology. 2007;130(Pt 6):1566–76.CrossRefGoogle ScholarPubMed
Williams, DR, Holton, JL, Strand, K, Revesz, T, Lees, AJ. Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy. Movement Disorders: Official Journal of the Movement Disorder Society. 2007;22(15):2235–41.CrossRefGoogle ScholarPubMed
Dickson, DW, Ahmed, Z, Algom, AA, Tsuboi, Y, Josephs, KA. Neuropathology of variants of progressive supranuclear palsy. Current Opinion in Neurology. 2010;23(4):394400.CrossRefGoogle ScholarPubMed
Tagliavini, F, Pilleri, G, Bouras, C, Constantinidis, J. The basal nucleus of Meynert in patients with progressive supranuclear palsy. Neuroscience Letters. 1984;44(1):3742.CrossRefGoogle ScholarPubMed
Nishimura, M, Namba, Y, Ikeda, K, Oda, M. Glial fibrillary tangles with straight tubules in the brains of patients with progressive supranuclear palsy. Neuroscience Letters. 1992;143(1–2):35–8.CrossRefGoogle ScholarPubMed
Matsusaka, H, Ikeda, K, Akiyama, H, Arai, T, Inoue, M, Yagishita, S. Astrocytic pathology in progressive supranuclear palsy: significance for neuropathological diagnosis. Acta Neuropathologica. 1998;96(3):248–52.CrossRefGoogle ScholarPubMed
Arima, K, Nakamura, M, Sunohara, N, Ogawa, M, Anno, M, Izumiyama, Y, et al. Ultrastructural characterization of the tau-immunoreactive tubules in the oligodendroglial perikarya and their inner loop processes in progressive supranuclear palsy. Acta Neuropathologica. 1997;93(6):558–66.CrossRefGoogle ScholarPubMed
Nath, U, Ben-Shlomo, Y, Thomson, RG, Morris, HR, Wood, NW, Lees, AJ, et al. The prevalence of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) in the UK. Brain: A Journal of Neurology. 2001;124(Pt 7):1438–49.CrossRefGoogle ScholarPubMed
Schrag, A, Ben-Shlomo, Y, Quinn, NP. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet. 1999;354(9192):1771–5.CrossRefGoogle ScholarPubMed
Bower, JH, Maraganore, DM, McDonnell, SK, Rocca, WA. Incidence of progressive supranuclear palsy and multiple system atrophy in Olmsted County, Minnesota, 1976 to 1990. Neurology. 1997;49(5):1284–8.CrossRefGoogle ScholarPubMed
Maher, ER, Lees, AJ. The clinical features and natural history of the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology. 1986;36(7):1005–8.CrossRefGoogle ScholarPubMed
Golbe, LI, Davis, PH, Schoenberg, BS, Duvoisin, RC. Prevalence and natural history of progressive supranuclear palsy. Neurology. 1988;38(7):1031–4.CrossRefGoogle ScholarPubMed
Donker Kaat, L, Boon, AJ, Azmani, A, Kamphorst, W, Breteler, MM, Anar, B, et al. Familial aggregation of parkinsonism in progressive supranuclear palsy. Neurology. 2009;73(2):98105.CrossRefGoogle ScholarPubMed
Dickson, DW, Rademakers, R, Hutton, ML. Progressive supranuclear palsy: pathology and genetics. Brain Pathology. 2007;17(1):7482.CrossRefGoogle ScholarPubMed
Hoglinger, GU, Melhem, NM, Dickson, DW, Sleiman, PM, Wang, LS, Klei, L, et al. Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy. Nature Genetics. 2011;43(7):699705.CrossRefGoogle ScholarPubMed
Golbe, LI, Davis, PH, Lepore, FE. Eyelid movement abnormalities in progressive supranuclear palsy. Movement Disorders: Official Journal of the Movement Disorder Society. 1989;4(4):297302.CrossRefGoogle ScholarPubMed
Nath, U, Ben-Shlomo, Y, Thomson, RG, Lees, AJ, Burn, DJ. Clinical features and natural history of progressive supranuclear palsy: a clinical cohort study. Neurology. 2003;60(6):910–16.Google ScholarPubMed
Donker Kaat, L, Boon, AJ, Kamphorst, W, Ravid, R, Duivenvoorden, HJ, van Swieten, JC. Frontal presentation in progressive supranuclear palsy. Neurology. 2007;69(8):723–9.CrossRefGoogle ScholarPubMed
Kertesz, A, McMonagle, P. Behavior and cognition in corticobasal degeneration and progressive supranuclear palsy. Journal of the Neurological Sciences. 2010;289(1–2):138–43.CrossRefGoogle ScholarPubMed
Litvan, I, Mega, MS, Cummings, JL, Fairbanks, L. Neuropsychiatric aspects of progressive supranuclear palsy. Neurology. 1996;47(5):1184–9.CrossRefGoogle ScholarPubMed
Pillon, B, Gouider-Khouja, N, Deweer, B, Vidailhet, M, Malapani, C, Dubois, B, et al. Neuropsychological pattern of striatonigral degeneration: comparison with Parkinson’s disease and progressive supranuclear palsy. Journal of Neurology, Neurosurgery, and Psychiatry. 1995;58(2):174–9.CrossRefGoogle ScholarPubMed
Arai, H, Morikawa, Y, Higuchi, M, Matsui, T, Clark, CM, Miura, M, et al. Cerebrospinal fluid tau levels in neurodegenerative diseases with distinct tau-related pathology. Biochemical and Biophysical Research Communications. 1997;236(2):262–4.Google ScholarPubMed
Noguchi, M, Yoshita, M, Matsumoto, Y, Ono, K, Iwasa, K, Yamada, M. Decreased beta-amyloid peptide42 in cerebrospinal fluid of patients with progressive supranuclear palsy and corticobasal degeneration. Journal of the Neurological Sciences. 2005;237(1–2):61–5.CrossRefGoogle ScholarPubMed
Urakami, K, Wada, K, Arai, H, Sasaki, H, Kanai, M, Shoji, M, et al. Diagnostic significance of tau protein in cerebrospinal fluid from patients with corticobasal degeneration or progressive supranuclear palsy. Journal of the Neurological Sciences. 2001;183(1):95–8.Google ScholarPubMed
Mitani, K, Furiya, Y, Uchihara, T, Ishii, K, Yamanouchi, H, Mizusawa, H, et al. Increased CSF tau protein in corticobasal degeneration. Journal of Neurology. 1998;245(1):44–6.CrossRefGoogle ScholarPubMed
Verbeek, MM, Abdo, WF, De Jong, D, Horstink, MW, Kremer, BP, Bloem, BR. Cerebrospinal fluid Abeta42 levels in multiple system atrophy. Movement Disorders: Official Journal of the Movement Disorder Society. 2004;19(2):238–40; author reply 40–1.CrossRefGoogle ScholarPubMed
Holmberg, B, Johnels, B, Blennow, K, Rosengren, L. Cerebrospinal fluid Abeta42 is reduced in multiple system atrophy but normal in Parkinson’s disease and progressive supranuclear palsy. Movement Disorders: Official Journal of the Movement Disorder Society. 2003;18(2):186–90.CrossRefGoogle ScholarPubMed
Brettschneider, J, Petzold, A, Sussmuth, SD, Landwehrmeyer, GB, Ludolph, AC, Kassubek, J, et al. Neurofilament heavy-chain NfH(SMI35) in cerebrospinal fluid supports the differential diagnosis of Parkinsonian syndromes. Movement Disorders: Official Journal of the Movement Disorder Society. 2006;21(12):2224–7.CrossRefGoogle ScholarPubMed
Whitwell, JL, Jack, CR Jr., Boeve, BF, Parisi, JE, Ahlskog, JE, Drubach, DA, et al. Imaging correlates of pathology in corticobasal syndrome. Neurology. 2010;75(21):1879–87.CrossRefGoogle ScholarPubMed
Oba, H, Yagishita, A, Terada, H, Barkovich, AJ, Kutomi, K, Yamauchi, T, et al. New and reliable MRI diagnosis for progressive supranuclear palsy. Neurology. 2005;64(12):2050–5.CrossRefGoogle ScholarPubMed
Quattrone, A, Nicoletti, G, Messina, D, Fera, F, Condino, F, Pugliese, P, et al. MR imaging index for differentiation of progressive supranuclear palsy from Parkinson disease and the Parkinson variant of multiple system atrophy. Radiology. 2008;246(1):214–21.Google ScholarPubMed
Brenneis, C, Seppi, K, Schocke, M, Benke, T, Wenning, GK, Poewe, W. Voxel-based morphometry reveals a distinct pattern of frontal atrophy in progressive supranuclear palsy. Journal of Neurology, Neurosurgery, and Psychiatry. 2004;75(2):246–9.CrossRefGoogle ScholarPubMed
Boxer, AL, Geschwind, MD, Belfor, N, Gorno-Tempini, ML, Schauer, GF, Miller, BL, et al. Patterns of brain atrophy that differentiate corticobasal degeneration syndrome from progressive supranuclear palsy. Archives of Neurology. 2006;63(1):81–6.CrossRefGoogle ScholarPubMed
Price, S, Paviour, D, Scahill, R, Stevens, J, Rossor, M, Lees, A, et al. Voxel-based morphometry detects patterns of atrophy that help differentiate progressive supranuclear palsy and Parkinson’s disease. NeuroImage. 2004;23(2):663–9.CrossRefGoogle ScholarPubMed
Okamura, N, Furumoto, S, Fodero-Tavoletti, MT, Mulligan, RS, Harada, R, Yates, P, et al. Non-invasive assessment of Alzheimer’s disease neurofibrillary pathology using 18F-THK5105 PET. Brain: A Journal of Neurology. 2014;137(Pt 6): 1762–71.CrossRefGoogle ScholarPubMed
Kompoliti, K, Goetz, CG, Litvan, I, Jellinger, K, Verny, M. Pharmacological therapy in progressive supranuclear palsy. Archives of Neurology. 1998;55(8):1099–102.Google Scholar
Stamelou, M, de Silva, R, Arias-Carrion, O, Boura, E, Hollerhage, M, Oertel, WH, et al. Rational therapeutic approaches to progressive supranuclear palsy. Brain: A Journal of Neurology. 133(Pt 6):1578–90.Google Scholar

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