e-References

e1. Van Zanducke M, Dehaene I. A "cortico-basal degeneration"-like syndrome as first sign of progressive multifocal leukoencephalopathy.ActaNeurolBelg 2000;100:242-245.

e2. Warren JD, Mummery CJ, Al-Din AS, Brown P, Wood NW. Corticobasal degeneration syndrome with basal ganglia calcification: Fahr's disease as a corticobasal look-alike? MovDisord 2002;17:563-567.

e3. Benito-Leon J, Alvarez-Linera J, Louis ED. Neurosyphilis masquerading as corticobasal degeneration.MovDisord 2004;19:1367-1370.

e4. Baba Y, Uitti RJ, Farrer MJ, Wszolek ZK.Sporadic SCA8 mutation resembling corticobasal degeneration. Parkinsonism RelatDisord 2005;11:147-150.

e5. Shamim A, Siddiqui BK, Josephs KA. The corticobasal syndrome triggered by central pontinemyelinolysis. Eur J Neurol 2006;13:82-84.

e6. Rossi G, Marelli C, Farina L, et al. The G389R mutation in the MAPT gene presenting as sporadic corticobasal syndrome.MovDisord 2008;23:892-895.

e7. Chen-Plotkin AS, Yuan W, Anderson C, et al. Corticobasal syndrome and primary progressive aphasia as manifestations of LRRK2 gene mutations. Neurology 2008;70:521-527.

e8. Rubio-Agusti I, Kojovic M, Edwards MJ, et al. Atypical parkinsonism and cerebrotendinousxanthomatosis: Report of a family with corticobasal syndrome and a literature review. MovDisord 2012;27:1769-1774.

e9. Engelen M, Westhoff D, de Gans J, Nederkoorn PJ. A 64-year old man presenting with carotid artery occlusion and corticobasal syndrome: A case report. J Med Case Rep 2011;5:357.

e10. Miyaji Y, Koyama K, Kurokawa T, Mitomi M, Suzuki Y, Kuroiwa Y. Vascular corticobasal syndrome caused by unilateral internal carotid artery occlusion. J Stroke Cerebrovasc Dis 2012.

e11. Lindquist S, Duno M, Batbayli M, et al. Corticobasal and ataxia syndromes widen the spectrum of C9ORF72 hexanucleotide expansion disease. Clin Genet 2012;9999:10.1111/j.1399-0004.2012.01903.x.

e12. Josephs KA, Tang-Wai DF, Edland SD, et al. Correlation between antemortem magnetic resonance imaging findings and pathologically confirmed corticobasal degeneration. Arch Neurol 2004;61:1881-1884.

e13. Grimes DA, Lang AE, Bergeron CB. Dementia as the most common presentation of cortical-basal ganglionic degeneration. Neurology 1999;53:1969-1974.

e14. Dickson DW, Bergeron C, Chin SS, et al. Office of rare diseases neuropathologic criteria for corticobasal degeneration. J NeuropatholExpNeurol 2002;61:935-946.

e15. Cairns NJ, Bigio EH, Mackenzie IR, et al. Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: Consensus of the consortium for frontotemporal lobar degeneration. ActaNeuropathol 2007;114:5-22.

e16. Kouri N, Murray ME, Hassan A, et al. Neuropathological features of corticobasal degeneration presenting as corticobasal syndrome or richardson syndrome. Brain 2011;134:3264-3275.

e17. Arai T, Ikeda K, Akiyama H, et al. Distinct isoforms of tau aggregated in neurons and glial cells in brains of patients with pick's disease, corticobasal degeneration and progressive supranuclear palsy. ActaNeuropathol 2001;101:167-173.

e18. Borroni B, Goldwurm S, Cerini C, et al. Familial aggregation in progressive supranuclear palsy and corticobasal syndrome. Eur J Neurol 2010.

e19. Tuite PJ, Clark HB, Bergeron C, et al. Clinical and pathologic evidence of corticobasal degeneration and progressive supranuclear palsy in familial tauopathy. Arch Neurol 2005;62:1453-1457.

e20. Benussi L, Ghidoni R, Pegoiani E, Moretti DV, Zanetti O, Binetti G. Progranulin Leu271LeufsX10 is one of the most common FTLD and CBS associated mutations worldwide. Neurobiol Dis 2009;33:379-385.

e21. Houlden H, Baker M, Morris HR, et al. Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype. Neurology 2001;56:1702-1706.

e22. Borroni B, Del Bo R, Goldwurm S, et al. VEGF haplotypes are associated with increased risk to progressive supranuclear palsy and corticobasal syndrome. J Alzheimers Dis 2010;21:87-94.

e23. Aerts MB, Esselink RA, Bloem BR, Verbeek MM. Cerebrospinal fluid tau and phosphorylated tau protein are elevated in corticobasal syndrome. MovDisord 2011;26:169-173.

e24. 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. J NeurolSci 2005;237:61-65.

e25. Urakami K, Wada K, Arai H, et al. Diagnostic significance of tau protein in cerebrospinal fluid from patients with corticobasal degeneration or progressive supranuclear palsy. J NeurolSci 2001;183:95-98.

e26. Mitani K, Furiya Y, Uchihara T, et al. Increased CSF tau protein in corticobasal degeneration. J Neurol 1998;245:44-46.

e27. Borroni B, Premi E, Agosti C, et al. CSF alzheimer's disease-like pattern in corticobasal syndrome: Evidence for a distinct disorder. J NeurolNeurosurg Psychiatry 2011;82:834-838.

e28. Irwin DJ, McMillan CT, Toledo JB, et al. Comparison of cerebrospinal fluid levels of tau and abeta 1-42 in alzheimer disease and frontotemporal degeneration using 2 analytical platforms. Arch Neurol 2012;69:1018-1025.

e29. Bian H, Van Swieten JC, Leight S, et al. CSF biomarkers in frontotemporal lobar degeneration with known pathology. Neurology 2008;70:1827-1835.

e30. Josephs KA, Whitwell JL, Boeve BF, et al. Anatomical differences between CBS-corticobasal degeneration and CBS-alzheimer's disease.MovDisord 2010;25:1246-1252.

e31. Whitwell JL, Jack CR,Jr, Boeve BF, et al. Imaging correlates of pathology in corticobasal syndrome. Neurology 2010;75:1879-1887.

e32. Josephs KA, Whitwell JL, Dickson DW, et al. Voxel-based morphometry in autopsy proven PSP and CBD.Neurobiol Aging 2008;29:280-289.

e33. Boelmans K, Bodammer NC, Suchorska B, et al. Diffusion tensor imaging of the corpus callosum differentiates corticobasal syndrome from parkinson's disease. Parkinsonism RelatDisord 2010;16:498-502.

e34. Rizzo G, Martinelli P, Manners D, et al. Diffusion-weighted brain imaging study of patients with clinical diagnosis of corticobasal degeneration, progressive supranuclear palsy and parkinson's disease. Brain 2008;131:2690-2700.

e35. Boelmans K, Kaufmann J, Bodammer N, et al. Involvement of motor pathways in corticobasal syndrome detected by diffusion tensor tractography. MovDisord 2009;24:168-175.

e36. Fodero-Tavoletti MT, Okamura N, Furumoto S, et al. 18F-THK523: A novel in vivo tau imaging ligand for alzheimer's disease. Brain 2011;134:1089-1100.

e37. Jensen JR, Cisek K, Funk KE, Naphade S, Schafer KN, Kuret J. Research towards tau imaging. J Alzheimers Dis 2011;26Suppl 3:147-157.

e38. Pirker S, Perju-Dumbrava L, Kovacs GG, Traub-Weidinger T, Asenbaum S, Pirker W. Dopamine D2 receptor SPECT in corticobasal syndrome and autopsy-confirmed corticobasal degeneration. Parkinsonism RelatDisord 2012.

e39. Cilia R, Rossi C, Frosini D, et al. Dopamine transporter SPECT imaging in corticobasal syndrome. PLoS One 2011;6:e18301.

e40. Hellwig S, Amtage F, Kreft A, et al. (1)(8)F]FDG-PET is superior to [(1)(2)(3)I]IBZM-SPECT for the differential diagnosis of parkinsonism. Neurology 2012;79:1314-1322.

e41. Tripathi M, Dhawan V, Peng S, et al. Differential diagnosis of parkinsonian syndromes using F-18 fluorodeoxyglucose positron emission tomography. Neuroradiology 2013;55:483-492.

e42. Mackenzie IR, Neumann M, Bigio EH, et al. Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: Consensus recommendations. ActaNeuropathol 2009;117:15-18.

e43. Kertesz A, Hudson L, Mackenzie IR, Munoz DG.The pathology and nosology of primary progressive aphasia. Neurology 1994;44:2065-2072.

e44. Mackenzie IR, Neumann M, Bigio EH, et al. Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: An update. ActaNeuropathol 2010;119:1-4.

e45. Wadia PM, Lang AE. The many faces of corticobasal degeneration. Parkinsonism RelatDisord 2007;13Suppl 3:S336-40.

e46. Xie SX, Forman MS, Farmer J, et al. Factors associated with survival probability in autopsy-proven frontotemporal lobar degeneration. J NeurolNeurosurg Psychiatry 2008;79:126-129.

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