Progressive Supranuclear Palsy and Corticobasal Degeneration

Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders: Second Edition

Volumn , Issue , 2011, Pages 135-155

  Dickson, Dennis W ^a   Hauw, Jean Jaques ^b   Agid, Yves ^c   Litvan, Irene ^d  

^a MAYO CLINIC   (United States)

^b Anatomopathologie Neurologique Hôpital   (France)

^c PITIÉ SALPÊTRIÈRE HOSPITAL   (France)

^d University of Louisville School of Medicine   (United States)

Author keywords

"cortical basal ganglionic degeneration" (CBGD); Alzheimer's type pathology; Astrocytic lesions; Asymmetrical atrophy in PSP CBS; CBD, akinetic rigid parkinsonism; Corticobasal syndrome (CBS); Progressive supranuclear palsy (PSP) and CBD; Pure akinesia and gait failure (PAGF); Richardson's syndrome; Tau phosphorylation and dephosphorylation pathways

Indexed keywords

EID: 84885973796     PISSN: None     EISSN: None     Source Type: Book    
DOI: 10.1002/9781444341256.ch15     Document Type: Chapter

References (222)

1. Scaravilli T, Tolosa E, Ferrer I. Progressive supranuclear palsy and corticobasal degeneration: lumping versus splitting. Mov Disord 20 Suppl 2005; 12: S21-S28.
2. 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. Arch Neurol 1964; 10: 333-359.
3. Dickson DW, Ahmed Z, Algom AA, Tsuboi Y, Josephs KA. Neuropathology of variants of progressive supranuclear palsy. Curr Opin Neurol 2010; 23: 394-400.
4. Gibb WR, Luthert PJ, Marsden CD. Corticobasal degeneration. Brain 1989; 112 (Pt 5): 1171-1192.
5. Grimes DA, Lang AE, Bergeron CB. Dementia as the most common presentation of cortical-basal ganglionic degeneration. Neurology 1999; 53: 1969-1974.
6. Dickson DW. Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration. J Neurol 1999; 246 Suppl 2: II6-15.
7. Williams DR, Lees AJ, Wherrett JR, Steele JC. J. Clifford Richardson and 50 years of progressive supranuclear palsy. Neurology 2008; 70: 566-573.
8. Williams DR, de Silva R, Paviour DC et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism. Brain 2005; 128: 1247-1258.
9. Williams DR, Holton JL, Strand K, Revesz T, Lees AJ. Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy. Mov Disord 2007; 22: 2235-2241.
10. Tsuboi Y, Josephs KA, Boeve BF et al. Increased tau burden in the cortices of progressive supranuclear palsy presenting with corticobasal syndrome. Mov Disord 2005; 20: 982-988.
11. Josephs KA, Boeve BF, Duffy JR et al. Atypical progressive supranuclear palsy underlying progressive apraxia of speech and nonfluent aphasia. Neurocase 2005; 11: 283-296.
12. Josephs KA, Katsuse O, Beccano-Kelly DA et al. Atypical progressive supranuclear palsy with corticospinal tract degeneration. J Neuropathol Exp Neurol 2006; 65: 396-405.
13. Rebeiz JJ, Kolodny EH, Richardson EP Jr. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 1968; 18: 20-33.
14. Arima K, Uesugi H, Fujita I et al. Corticonigral degeneration with neuronal achromasia presenting with primary progressive aphasia: ultrastructural and immunocytochemical studies. J Neurol Sci 1994; 127: 186-197.
15. Dickson DW, Yen SH, Suzuki KI, Davies P, Garcia JH, Hirano A. Ballooned neurons in select neurodegenerative diseases contain phosphorylated neurofilament epitopes. Acta Neuropathol 1986; 71: 216-223.
16. Paulus W, Selim M. Corticonigral degeneration with neuronal achromasia and basal neurofibrillary tangles. Acta Neuropathol 1990; 81: 89-94.
17. Wang LN, Kowall NW, Richardson EP Jr. Phosphorylated neurofilament epitopes in the achromasic neurons of corticonigral degeneration. Chin Med J (Engl) 1991; 104: 1011-1017.
18. Bergeron C, Pollanen MS, Weyer L, Black SE, Lang AE. Unusual clinical presentations of cortical-basal ganglionic degeneration. Ann Neurol 1996; 40: 893-900.
19. Grimes DA, Bergeron CB, Lang AE. Motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration. Mov Disord 1999; 14: 674-680.
20. Riley DE, Lang AE, Lewis A et al. Cortical-basal ganglionic degeneration. Neurology 1990; 40: 1203-1212.
21. Borroni B, Alberici A, Agosti C, Cosseddu M, Padovani A. Pattern of behavioral disturbances in corticobasal degeneration syndrome and progressive supranuclear palsy. Int Psychogeriatr 2009; 21: 463-468.
22. Hu WT, Mandrekar JN, Parisi JE et al. Clinical features of pathologic subtypes of behavioral-variant frontotemporal dementia. Arch Neurol 2007; 64: 1611-1616.
23. Kertesz A, Martinez-Lage P, Davidson W, Muñoz DG. The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology 2000; 55: 1368-1375.
24. Graham NL, Bak T, Patterson K, Hodges JR. Language function and dysfunction in corticobasal degeneration. Neurology 2003; 61: 493-499.
25. 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; 21: 688-692.
26. Wadia PM, Lang AE. The many faces of corticobasal degeneration. Parkinsonism Relat Disord 13 Suppl 2007; 3: S336-S340.
27. Jellinger KA, Grazer A, Petrovic K et al. Four-repeat tauopathy clinically presenting as posterior cortical atrophy: atypical corticobasal degeneration? Acta Neuropathol 2010 (epub ahead of print).
28. Tang-Wai DF, Graff-Radford NR, Boeve BF et al. Clinical, genetic, and neuropathologic characteristics of posterior cortical atrophy. Neurology 2004; 63: 1168-1174.
29. Hassan A, Whitwell JL, Boeve BF et al. Symmetric corticobasal degeneration (S-CBD). Parkinsonism Relat Disord 2010; 16: 208-214.
30. Shiozawa M, Fukutani Y, Sasaki K et al. Corticobasal degeneration: an autopsy case clinically diagnosed as progressive supranuclear palsy. Clin Neuropathol 2000; 19: 192-199.
31. Nath U, Ben-Shlomo Y, Thomson RG et al. The prevalence of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) in the UK. Brain 2001; 124: 1438-1449.
32. Golbe LI. The epidemiology of PSP. J Neural Transm Suppl 1994; 42: 263-273.
33. Schrag A, Ben-Shlomo Y, Quinn NP. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet 1999; 354: 1771-1775.
34. 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: 1284-1288.
35. Litvan I, Grimes DA, Lang AE. Phenotypes and prognosis: clinicopathologic studies of corticobasal degeneration. Adv Neurol 2000; 82: 183-196.
36. Togasaki DM, Tanner CM. Epidemiologic aspects. In: Litvan I, Goetz CG, Lang AE (eds) Corticobasal degeneration. Philadelphia: Lippincott, Williams & Wilkins, 2000, pp. 53-59.
37. Winter Y, Bezdolnyy Y, Katunina E et al. Incidence of Parkinson's disease and atypical parkinsonism: Russian population-based study. Mov Disord 2010; 25: 349-356.
38. Davis PH, Golbe LI, Duvoisin RC, Schoenberg BS. Risk factors for progressive supranuclear palsy. Neurology 1988; 38: 1546-1552.
39. Ghika J, Bogousslavsky J. Presymptomatic hypertension is a major feature in the diagnosis of progressive supranuclear palsy. Arch Neurol 1997; 54: 1104-1108.
40. Colosimo C, Osaki Y, Vanacore N, Lees AJ. Lack of association between progressive supranuclear palsy and arterial hypertension: a clinicopathological study. Mov Disord 2003; 18: 694-697.
41. Sibon I, Macia F, Vital A, Delacourte A, Tison F. Hypertension and progressive supranuclear palsy: is everything so clear? Mov Disord 2004; 19: 1259-1261.
42. Caparros-Lefebvre D, Elbaz A. Possible relation of atypical parkinsonism in the French West Indies with consumption of tropical plants: a case-control study. Caribbean Parkinsonism Study Group. Lancet 1999; 354: 281-286.
43. Caparros-Lefebvre D, Sergeant N, Lees A et al. Guadeloupean parkinsonism: a cluster of progressive supranuclear palsy-like tauopathy. Brain 2002; 125: 801-811.
44. Vanacore N, Bonifati V, Fabbrini G et al. Smoking habits in multiple system atrophy and progressive supranuclear palsy. European Study Group on Atypical Parkinsonisms. Neurology 2000; 54: 114-119.
45. Vidal JS, Vidailhet M, Derkinderen P, de Gaillarbois TD, Tzourio C, Alpérovitch A. Risk factors for progressive supranuclear palsy: a case-control study in France. J Neurol Neurosurg Psychiatry 2009; 80: 1271-1274.
46. Brown J, Lantos PL, Roques P, Fidani L, Rossor MN. Familial dementia with swollen achromatic neurons and corticobasal inclusion bodies: a clinical and pathological study. J Neurol Sci 1996; 135: 21-30.
47. De Yébenes JG, Sarasa JL, Daniel SE, Lees AJ. Familial progressive supranuclear palsy. Description of a pedigree and review of the literature. Brain 1995; 118 (Pt 5): 1095-1103.
48. Gazeley S, Maguire JA. Familial progressive supranuclear palsy. Clin Neuropathol 1996; 15: 215-220.
49. Rojo A, Pernaute RS, Fontán A et al. Clinical genetics of familial progressive supranuclear palsy. Brain 1999; 122 (Pt 7): 1233-1245.
50. Bugiani O, Murrell JR, Giaccone G et al. Frontotemporal dementia and corticobasal degeneration in a family with a P301S mutation in tau. J Neuropathol Exp Neurol 1999; 58: 667-677.
51. Stanford PM, Halliday GM, Brooks WS et al. Progressive supranuclear palsy pathology caused by a novel silent mutation in exon 10 of the tau gene: expansion of the disease phenotype caused by tau gene mutations. Brain 2000; 123 (Pt 5): 880-893.
52. Baba Y, Baker MC, Le Ber I et al. Clinical and genetic features of families with frontotemporal dementia and parkinsonism linked to chromosome 17 with a P301S tau mutation. J Neural Transm 2007; 114: 947-950.
53. Conrad C, Amano N, Andreadis A et al. Differences in a dinucleotide repeat polymorphism in the tau gene between Caucasian and Japanese populations: implication for progressive supranuclear palsy. Neurosci Lett 1998; 250: 135-137.
54. Conrad C, Andreadis A, Trojanowski JQ et al. Genetic evidence for the involvement of tau in progressive supranuclear palsy. Ann Neurol 1997; 41: 277-281.
55. Higgins JJ, Golbe LI, De Biase A, Jankovic J, Factor SA, Adler RL. An extended 5 '-tau susceptibility haplotype in progressive supranuclear palsy. Neurology 2000; 55: 1364-1367.
56. Baker M, Litvan I, Houlden H et al. Association of an extended haplotype in the tau gene with progressive supranuclear palsy. Hum Mol Genet 1999; 8: 711-715.
57. Stefansson H, Helgason A, Thorleifsson G et al. A common inversion under selection in Europeans. Nat Genet 2005; 37: 129-137.
58. Webb A, Miller B, Bonasera S, Boxer A, Karydas A, Wilhelmsen KC. Role of the tau gene region chromosome inversion in progressive supranuclear palsy, corticobasal degeneration, and related disorders. Arch Neurol 2008; 65: 1473-1478.
59. Pittman AM, Myers AJ, Abou-Sleiman P et al. Linkage disequilibrium fine mapping and haplotype association analysis of the tau gene in progressive supranuclear palsy and corticobasal degeneration. J Med Genet 2005; 42: 837-846.
60. Rademakers R, Melquist S, Cruts M et al. High-density SNP haplotyping suggests altered regulation of tau gene expression in progressive supranuclear palsy. Hum Mol Genet 2005; 14: 3281-3292.
61. Myers AJ, Pittman AM, Zhao AS, et al. (2007) The MAPT H1c risk haplotype is associated with increased expression of tau and especially of 4 repeat containing transcripts. Neurobiol Dis 25: 561-570
62. Golbe LI, Ohman-Strickland PA. A clinical rating scale for progressive supranuclear palsy. Brain 2007; 130: 1552-1565.
63. Litvan I, Grimes DA, Lang AE et al. Clinical features differentiating patients with postmortem confirmed progressive supranuclear palsy and corticobasal degeneration. J Neurol 1999; 246 Suppl 2: II1-II5.
64. Santacruz P, Uttl B, Litvan I, Grafman J. Progressive supranuclear palsy: a survey of the disease course. Neurology 1998; 50: 1637-1647.
65. Verny M, Jellinger KA, Hauw JJ, Bancher C, Litvan I, Agid Y. Progressive supranuclear palsy: a clinicopathological study of 21 cases. Acta Neuropathol 1996; 91: 427-431.
66. Josephs KA, Dickson DW. Diagnostic accuracy of progressive supranuclear palsy in the Society for Progressive Supranuclear Palsy brain bank. Mov Disord 2003; 18: 1018-1026.
67. 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; 59: 1597-1601.
68. Winikates J, Jankovic J. Vascular progressive supranuclear palsy. J Neural Transm Suppl 1994; 42: 189-201.
69. Williams DR, Holton JL, Strand C et al. Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson's syndrome. Brain 2007; 130: 1566-1576.
70. Williams DR, Lees AJ. What features improve the accuracy of the clinical diagnosis of progressive supranuclear palsy-parkinsonism (PSP-P)? Mov Disord 2010; 25: 357-362.
71. Imai H, Narabayashi H, Sakata E. "Pure akinesia" and the later added supranuclear ophthalmoplegia. Adv Neurol 1987; 45: 207-212.
72. Riley DE, Fogt N, Leigh RJ. The syndrome of "pure akinesia" and its relationship to progressive supranuclear palsy. Neurology 1994; 44: 1025-1029.
73. 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; 131: 460-472.
74. Kanazawa M, Shimohata T, Toyoshima Y et al. Cerebellar involvement in progressive supranuclear palsy: A clinicopathological study. Mov Disord 2009; 24: 1312-1318.
75. Wenning GK, Litvan I, Jankovic J et al. Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. J Neurol Neurosurg Psychiatry 1998; 64: 184-189.
76. Doody RS, Jankovic J. The alien hand and related signs. J Neurol Neurosurg Psychiatry 1992; 55: 806-810.
77. Boeve BF, Maraganore DM, Parisi JE et al. Pathologic heterogeneity in clinically diagnosed corticobasal degeneration. Neurology 1999; 53: 795-800.
78. Chand P, Grafman J, Dickson D, Ishizawa K, Litvan I. Alzheimer's disease presenting as corticobasal syndrome. Mov Disord 2006; 21: 2018-2022.
79. Horoupian DS, Wasserstein PH. Alzheimer's disease pathology in motor cortex in dementia with Lewy bodies clinically mimicking corticobasal degeneration. Acta Neuropathol 1999; 98: 317-322.
80. Kertesz A, McMonagle P, Blair M, Davidson W, Muñoz DG. The evolution and pathology of frontotemporal dementia. Brain 2005; 128: 1996-2005.
81. Lang AE, Bergeron C, Pollanen MS, Ashby P. Parietal Pick's disease mimicking cortical-basal ganglionic degeneration. Neurology 1994; 44: 1436-1440.
82. Litvan I, Agid Y, Goetz C et al. Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study. Neurology 1997; 48: 119-125.
83. McKhann GM, Albert MS, Grossman M, Miller B, Dickson D, Trojanowski JQ. Clinical and pathological diagnosis of frontotemporal dementia: report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol 2001; 58: 1803-1809.
84. Neary D, Snowden JS, Gustafson L et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998; 51: 1546-1554.
85. Hu WT, Parisi JE, Knopman DS et al. Clinical features and survival of 3R and 4R tauopathies presenting as behavioral variant frontotemporal dementia. Alzheimer Dis Assoc Disord 2007; 21: S39-S43.
86. Mackenzie IR, Baborie A, Pickering-Brown S et al. Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: classifi-cation and relation to clinical phenotype. Acta Neuropathol 2006; 112: 539-549.
87. Mott RT, Dickson DW, Trojanowski JQ et al. Neuropathologic, biochemical, and molecular characterization of the frontotemporal dementias. J Neuropathol Exp Neurol 2005; 64: 420-428.
88. Josephs KA, Petersen RC, Knopman DS et al. Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology 2006; 66: 41-48.
89. Takao M, Tsuchiya K, Mimura M et al. Corticobasal degeneration as cause of progressive non-fluent aphasia: clinical, radiological and pathological study of an autopsy case. Neuropathology 2006; 26: 569-578.
90. Duffy JR, Peach RK, Strand EA. Progressive apraxia of speech as a sign of motor neuron disease. Am J Speech Lang Pathol 2007; 16: 198-208.
91. Ogar J, Slama H, Dronkers N, Amici S, Gorno-Tempini ML. Apraxia of speech: an overview. Neurocase 2005; 11: 427-432.
92. Dickson DW, Josephs KA, Amador-Ortiz C. TDP-43 in differential diagnosis of motor neuron disorders. Acta Neuropathol 2007; 114: 71-79.
93. Takahashi T, Amano N, Hanihara T et al. Corticobasal degeneration: widespread argentophilic threads and glia in addition to neurofibrillary tangles. Similarities of cytoskeletal abnormalities in corticobasal degeneration and progressive supranuclear palsy. J Neurol Sci 1996; 138: 66-77.
94. Tsuchiya K, Murayama S, Mitani K et al. Constant and severe involvement of Betz cells in corticobasal degeneration is not consistent with pyramidal signs: a clinicopathological study of ten autopsy cases. Acta Neuropathol 2005; 109: 353-366.
95. Kato N, Arai K, Hattori T. Study of the rostral midbrain atrophy in progressive supranuclear palsy. J Neurol Sci 2003; 210: 57-60.
96. Paviour DC, Thornton JS, Lees AJ, Jäger HR. Diffusion-weighted magnetic resonance imaging differentiates Parkinsonian variant of multiple-system atrophy from progressive supranuclear palsy. Mov Disord 2007; 22: 68-74.
97. Slowinski J, Imamura A, Uitti RJ et al. MR imaging of brainstem atrophy in progressive supranuclear palsy. J Neurol 2008; 255: 37-44.
98. Koyama M, Yagishita A, Nakata Y, Hayashi M, Bandoh M, Mizutani T. Imaging of corticobasal degeneration syndrome. Neuroradiology 2007; 49: 905-912.
99. Soliveri P, Monza D, Paridi D et al. Cognitive and magnetic resonance imaging aspects of corticobasal degeneration and progressive supranuclear palsy. Neurology 1999; 53: 502-507.
100. Yamauchi H, Fukuyama H, Nagahama Y et al. Atrophy of the corpus callosum, cortical hypometabolism, and cognitive impairment in corticobasal degeneration. Arch Neurol 1998; 55: 609-614.
101. Nagahama Y, Fukuyama H, Turjanski N et al. Cerebral glucose metabolism in corticobasal degeneration: comparison with progressive supranuclear palsy and normal controls. Mov Disord 1997; 12: 691-696.
102. Okuda B, Tachibana H, Kawabata K, Takeda M, Sugita M. Cerebral blood flow in corticobasal degeneration and progressive supranuclear palsy. Alzheimer Dis Assoc Disord 2000; 14: 46-52.
103. Sawle GV, Brooks DJ, Marsden CD, Frackowiak RS. Corticobasal degeneration. A unique pattern of regional cortical oxygen hypometabolism and striatal fluorodopa uptake demonstrated by positron emission tomography. Brain 1991; 114 (Pt 1B): 541-556.
104. Brücke T, Djamshidian S, Bencsits G, Pirker W, Asenbaum S, Podreka I. SPECT and PET imaging of the dopaminergic system in Parkinson 's disease. J Neurol 247 Suppl 2000; 4: IV/2-7.
105. Blin J, Vidailhet MJ, Pillon B, Dubois B, Feve JR, Agid Y. Corticobasal degeneration: decreased and asymmetrical glucose consumption as studied with PET. Mov Disord 1992; 7: 348-354.
106. Hirano S, Shinotoh H, Shimada H et al. Cholinergic imaging in corticobasal syndrome, progressive supranuclear palsy and frontotemporal dementia. Brain 2010; 133: 2058-2068.
107. Arai H, Morikawa Y, Higuchi M et al. Cerebrospinal fluid tau levels in neurodegenerative diseases with distinct tau-related pathology. Biochem Biophys Res Commun 1997; 236: 262-264.
108. Borroni B, Gardoni F, Parnetti L et al. Pattern of Tau forms in CSF is altered in progressive supranuclear palsy. Neurobiol Aging 2009; 30: 34-40.
109. Constantinescu R, Zetterberg H, Holmberg B, Rosengren L. Levels of brain related proteins in cerebrospinal fluid: an aid in the differential diagnosis of parkinsonian disorders. Parkinsonism Relat Disord 2009; 15: 205-212.
110. Mitani K, Furiya Y, Uchihara T et al. Increased CSF tau protein in corticobasal degeneration. J Neurol 1998; 245: 44-46.
111. Süssmuth SD, Uttner I, Landwehrmeyer B et al. Differential pattern of brain-specific CSF proteins tau and amyloid-beta in Parkinsonian syndromes. Mov Disord 2010; 25: 1284-1288.
112. Urakami K, Mori M, Wada K et al. A comparison of tau protein in cerebrospinal fluid between corticobasal degeneration and progressive supranuclear palsy. Neurosci Lett 1999; 259: 127-129.
113. 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 Neurol Sci 2001; 183: 95-98.
114. Litvan I, Agid Y, Calne D 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: 1-9.
115. Lopez OL, Litvan I, Catt KE et al. Accuracy of four clinical diagnostic criteria for the diagnosis of neurodegenerative dementias. Neurology 1999; 53: 1292-1299.
116. Kumar RVR, Bergeron C, Pollanen MS, Lang AE. Cortical basal ganglionic degneration. In: Jankovic J, Tolosa E (eds) Parkinson's Disease and Movement Disorders. Baltimore, MD: Williams and Wilkins, 1998, pp. 297-316.
117. Shelley BP, Hodges JR, Kipps CM, Xuereb JH, Bak TH. Is the pathology of corticobasal syndrome predictable in life? Mov Disord 2009; 24: 1593-1599
118. Stover NP, Watts RL. Corticobasal degeneration. Semin Neurol 2001; 21: 49-58.
119. Watts RL, Mirra SS Corticobasal ganglionic degeneration. In: Marsden CD, Fahn S (eds) Movement disorders. London: Butterworth, 1994, pp. 282-299.
120. Dickson DW, Bergeron C, Chin SS et al. Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol 2002; 61: 935-946.
121. Braak E, Braak H, Mandelkow EM. A sequence of cytoskeleton changes related to the formation of neurofibrillary tangles and neuropil threads. Acta Neuropathol 1994; 87: 554-567.
122. Komori T, Arai N, Oda M et al. Astrocytic plaques and tufts of abnormal fibers do not coexist in corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathol 1998; 96: 401-408.
123. Nishimura M, Namba Y, Ikeda K, Oda M. Glial fibrillary tangles with straight tubules in the brains of patients with progressive supranuclear palsy. Neurosci Lett 1992; 143: 35-38.
124. Nishimura T, Ikeda K, Akiyama H et al. Immunohistochemical investigation of tau-positive structures in the cerebral cortex of patients with progressive supranuclear palsy. Neurosci Lett 1995; 201: 123-126.
125. Yamada T, McGeer PL, McGeer EG. Appearance of paired nucleated, Tau-positive glia in patients with progressive supranuclear palsy brain tissue. Neurosci Lett 1992; 135: 99-102.
126. Togo T, Dickson DW. Tau accumulation in astrocytes in progressive supranuclear palsy is a degenerative rather than a reactive process. Acta Neuropathol 2002; 104: 398-402.
127. Feany MB, Dickson DW. Widespread cytoskeletal pathology characterizes corticobasal degeneration. Am J Pathol 1995; 146: 1388-1396.
128. Clark AW, Manz HJ, White CL 3rd, Lehmann J, Miller D, Coyle JT. Cortical degeneration with swollen chromatolytic neurons: its relationship to Pick's disease. J Neuropathol Exp Neurol 1986; 45: 268-284.
129. Fujino Y, Delucia MW, Davies P, Dickson DW. Ballooned neurones in the limbic lobe are associated with Alzheimer type pathology and lack diagnostic specificity. Neuropathol Appl Neurobiol 2004; 30: 676-682.
130. Tolnay M, Probst A. Ballooned neurons expressing alphaB-crystallin as a constant feature of the amygdala in argyrophilic grain disease. Neurosci Lett 1998; 246: 165-168.
131. Halliday GM, Davies L, McRitchie DA, Cartwright H, Pamphlett R, Morris JG. Ubiquitin-positive achromatic neurons in corticobasal degeneration. Acta Neuropathol 1995; 90: 68-75.
132. Lowe J, Errington DR, Lennox G et al. Ballooned neurons in several neurodegenerative diseases and stroke contain alpha B crystallin. Neuropathol Appl Neurobiol 1992; 18: 341-350.
133. Mackenzie IR, Hudson LP. Achromatic neurons in the cortex of progressive supranuclear palsy. Acta Neuropathol 1995; 90: 615-619.
134. Togo T, Dickson DW. Ballooned neurons in progressive supranuclear palsy are usually due to concurrent argyrophilic grain disease. Acta Neuropathol 2002; 104: 53-56.
135. Yamada T, McGeer PL. Oligodendroglial microtubular masses: an abnormality observed in some human neurodegenerative diseases. Neurosci Lett 1990; 120: 163-166.
136. Braak H, Braak E. Argyrophilic grains: characteristic pathology of cerebral cortex in cases of adult onset dementia without Alzheimer changes. Neurosci Lett 1987; 76: 124-127.
137. Oyanagi K, Takahashi H, Wakabayashi K, Ikuta F. Selective decrease of large neurons in the neostriatum in progressive supranuclear palsy. Brain Res 1988; 458: 218-223.
138. Oyanagi K, Tsuchiya K, Yamazaki M, Ikeda K. Substantia nigra in progressive supranuclear palsy, corticobasal degeneration, and parkinsonism-dementia complex of Guam: specific pathological features. J Neuropathol Exp Neurol 2001; 60: 393-402.
139. Ishizawa K, Dickson DW. Microglial activation parallels system degeneration in progressive supranuclear palsy and corticobasal degeneration. J Neuropathol Exp Neurol 2001; 60: 647-657.
140. Ikeda K, Akiyama H, Kondo H et al. Thorn-shaped astrocytes: possibly secondarily induced tau-positive glial fibrillary tangles. Acta Neuropathol 1995; 90: 620-625.
141. Schultz C, Ghebremedhin E, Del Tredici K, Rüb U, Braak H. High prevalence of thorn-shaped astrocytes in the aged human medial temporal lobe. Neurobiol Aging 2004; 25: 397-405.
142. Ishizawa K, Lin WL, Tiseo P, Honer WG, Davies P, Dickson DW. A qualitative and quantitative study of grumose degeneration in progressive supranuclear palsy. J Neuropathol Exp Neurol 2000; 59: 513-524.
143. Hanihara T, Amano N, Takahashi T, Itoh Y, Yagishita S. Hypertrophy of the inferior olivary nucleus in patients with progressive supranuclear palsy. Eur Neurol 1998; 39: 97-102.
144. Katsuse O, Dickson DW. Inferior olivary hypertrophy is uncommon in progressive supranuclear palsy. Acta Neuropathol 2004; 108: 143-146.
145. Feany MB, Dickson DW. Neurodegenerative disorders with extensive tau pathology: a comparative study and review. Ann Neurol 1996; 40: 139-148.
146. Feany MB, Mattiace LA, Dickson DW. Neuropathologic overlap of progressive supranuclear palsy, Pick's disease and corticobasal degeneration. J Neuropathol Exp Neurol 1996; 55: 53-67.
147. Hauw JJ, Daniel SE, Dickson D et al. Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 1994; 44: 2015-2019.
148. Litvan I, Hauw JJ, Bartko JJ et al. Validity and reliability of the preliminary NINDS neuropathologic criteria for progressive supranuclear palsy and related disorders. J Neuropathol Exp Neurol 1996; 55: 97-105.
149. 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; 61: 1579-1584.
150. Tsuboi Y, Josephs KA, Cookson N, Dickson DW. APOE E4 is a determinant for Alzheimer type pathology in progressive supranuclear palsy. Neurology 2003; 60: 240-245.
151. Hyman BT, Trojanowski JQ. Consensus recommendations for the postmortem diagnosis of Alzheimer disease from the National Institute on Aging and the Reagan Institute Working Group on diagnostic criteria for the neuropathological assessment of Alzheimer disease. J Neuropathol Exp Neurol 1997; 56: 1095-1097.
152. Mori H, Oda M, Komori T et al. Lewy bodies in progressive supranuclear palsy. Acta Neuropathol 2002; 104: 273-278.
153. Tsuboi Y, Ahlskog JE, Apaydin H, Parisi JE, Dickson DW. Lewy bodies are not increased in progressive supranuclear palsy compared with normal controls. Neurology 2001; 57: 1675-1678.
154. Uchikado H, DelleDonne A, Ahmed Z, Dickson DW. Lewy bodies in progressive supranuclear palsy represent an independent disease process. J Neuropathol Exp Neurol 2006; 65: 387-395.
155. Uchikado H, DelleDonne A, Togo T, Dickson DW. Lewy bodies in corticobasal degeneration represent and independent disease process. Neuropathology 2006; 26: A52.
156. Braak H, Braak E. Argyrophilic grain disease: frequency of occurrence in different age categories and neuropathological diagnostic criteria. J Neural Transm 1998; 105: 801-819.
157. Togo T, Sahara N, Yen SH et al. Argyrophilic grain disease is a sporadic 4-repeat tauopathy. J Neuropathol Exp Neurol 2002; 61: 547-556.
158. Tolnay M, Spillantini MG, Goedert M, Ulrich J, Langui D, Probst A. Argyrophilic grain disease: widespread hyperphosphorylation of tau protein in limbic neurons. Acta Neuropathol 1997; 93: 477-484.
159. Saito Y, Ruberu NN, Sawabe M et al. Staging of argyrophilic grains: an age-associated tauopathy. J Neuropathol Exp Neurol 2004; 63: 911-918.
160. Botez G, Probst A, Ipsen S, Tolnay M. Astrocytes expressing hyperphosphorylated tau protein without glial fibrillary tangles in argyrophilic grain disease. Acta Neuropathol 1999; 98: 251-256.
161. Martinez-Lage P, Muñoz DG. Prevalence and disease associations of argyrophilic grains of Braak. J Neuropathol Exp Neurol 1997; 56: 157-164.
162. Togo T, Cookson N, Dickson DW. Argyrophilic grain disease: neuropathology, frequency in a dementia brain bank and lack of relationship with apolipoprotein E. Brain Pathol 2002; 12: 45-52.
163. Ghatak NR, Nochlin D, Hadfield MG. Neurofibrillary pathology in progressive supranuclear palsy. Acta Neuropathol 1980; 52: 73-76.
164. Montpetit V, Clapin DF, Guberman A. Substructure of 20 nm filaments of progressive supranuclear palsy. Acta Neuropathol 1985; 68: 311-318.
165. Nishimura M, Namba Y, Ikeda K, Akiguchi I, Oda M. Neuro fibrillary tangles in the neurons of spinal dorsal root ganglia of patients with progressive supranuclear palsy. Acta Neuropathol 1993; 85: 453-457.
166. Tellez-Nagel I, Wisniewski HM. Ultrastructure of neurofibrillary tangles in Steele-Richardson-Olszewski syndrome. Arch Neurol 1973; 29: 324-327.
167. Arima K, Nakamura M, Sunohara N et al. Ultrastructural characterization of the tau-immunoreactive tubules in the oligodendroglial perikarya and their inner loop processes in progressive supranuclear palsy. Acta Neuropathol 1997; 93: 558-566.
168. Ikeda K, Akiyama H, Haga C, Kondo H, Arima K, Oda T. Argyrophilic thread-like structure in corticobasal degeneration and supranuclear palsy. Neurosci Lett 1994; 174: 157-159.
169. Ksiezak-Reding H, Morgan K, Mattiace LA et al. Ultrastructure and biochemical composition of paired helical filaments in corticobasal degeneration. Am J Pathol 1994; 145: 1496-1508.
170. Ksiezak-Reding H, Tracz E, Yang LS, Dickson DW, Simon M, Wall JS. Ultrastructural instability of paired helical filaments from corticobasal degeneration as examined by scanning transmission electron microscopy. Am J Pathol 1996; 149: 639-651.
171. Yang L, Ksiezak-Reding H. Ubiquitin immunoreactivity of paired helical filaments differs in Alzheimer's disease and corticobasal degeneration. Acta Neuropathol 1998; 96: 520-526.
172. Mori H, Nishimura M, Namba Y, Oda M. Corticobasal degeneration: a disease with widespread appearance of abnormal tau and neurofi-brillary tangles, and its relation to progressive supranuclear palsy. Acta Neuropathol 1994; 88: 113-121.
173. Buée L, Delacourte A. Comparative biochemistry of tau in progressive supranuclear palsy, corticobasal degeneration, FTDP-17 and Pick's disease. Brain Pathol 1999; 9: 681-693.
174. Sergeant N, Bretteville A, Hamdane M et al. Biochemistry of Tau in Alzheimer's disease and related neurological disorders. Expert Rev Proteomics 2008; 5: 207-224.
175. Liu WK, Le TV, Adamson J et al. Relationship of the extended tau haplotype to tau biochemistry and neuropathology in progressive supranuclear palsy. Ann Neurol 2001; 50: 494-502.
176. Arai T, Ikeda K, Akiyama H et al. Identification of amino-terminally cleaved tau fragments that distinguish progressive supranuclear palsy from corticobasal degeneration. Ann Neurol 2004; 55: 72-79.
177. Hornykiewicz O, Shannak K. Brain monoamines in progressive supranuclear palsy-comparison with idiopathic Parkinson's disease. J Neural Transm Suppl 1994; 42: 219-227.
178. Jellinger K, Riederer P, Tomonaga M. Progressive supranuclear palsy: clinico-pathological and biochemical studies. J Neural Transm Suppl 1980; (16): 111-128.
179. Murphy KE, Karaconji T, Hardman CD, Halliday GM. Excessive dopamine neuron loss in progressive supranuclear palsy. Mov Disord 2008; 23: 607-610.
180. Landwehrmeyer B, Palacios JM. Alterations of neurotransmitter receptors and neurotransmitter transporters in progressive supranuclear palsy. J Neural Transm Suppl 1994; 42: 229-246.
181. Warren NM, Piggott MA, Greally E, Lake M, Lees AJ, Burn DJ. Basal ganglia cholinergic and dopaminergic function in progressive supranuclear palsy. Mov Disord 2007; 22: 1594-1600.
182. Denk F, Wade-Martins R. Knock-out and transgenic mouse models of tauopathies. Neurobiol Aging 2009; 30: 1-13.
183. Javoy-Agid F. Cholinergic and peptidergic systems in PSP. J Neural Transm Suppl 1994; 42: 205-218.
184. Kasashima S, Oda Y. Cholinergic neuronal loss in the basal forebrain and mesopontine tegmentum of progressive supranuclear palsy and corticobasal degeneration. Acta Neuropathol 2003; 105: 117-124.
185. Malessa S, Hirsch EC, Cervera P et al. Progressive supranuclear palsy: loss of choline-acetyltransferase-like immunoreactive neurons in the pontine reticular formation. Neurology 1991; 41: 1593-1597.
186. Suzuki M, Desmond TJ, Albin RL, Frey KA. Cholinergic vesicular transporters in progressive supranuclear palsy. Neurology 2002; 58: 1013-1018.
187. Levy R, Ruberg M, Herrero MT et al. Alterations of GABAergic neurons in the basal ganglia of patients with progressive supranuclear palsy: an in situ hybridization study of GAD67 messenger RNA. Neurology 1995; 45: 127-134.
188. Taquet H, Javoy-Agid F, Mauborgne A et al. Brain neuropeptides in progressive supranuclear palsy. Brain Res 1987; 411: 178-182.
189. Marshall EF, Perry RH, Perry EK et al. Striatal dopaminergic loss without parkinsonism in a case of corticobasal degeneration. Acta Neurol Scand 1997; 95: 287-292.
190. Götz J, Deters N, Doldissen A et al. A decade of tau transgenic animal models and beyond. Brain Pathol 2007; 17: 91-103.
191. Noble W, Hanger DP, Gallo JM. Transgenic mouse models of tauopathy in drug discovery. CNS Neurol Disord Drug Targets 2010; 9: 403-428.
192. Paquet D, Schmid B, Haass C. Transgenic zebrafish as a novel animal model to study tauopathies and other neurodegenerative disorders in vivo. Neurodegener Dis 2010; 7: 99-102.
193. Brandt R, Gergou A, Wacker I, Fath T, Hutter H. A Caenorhabditis elegans model of tau hyperphosphorylation: induction of develop-mental defects by transgenic overexpression of Alzheimer's disease-like modified tau. Neurobiol Aging 2009; 30: 22-33.
194. Wentzell J, Kretzschmar D. Alzheimer's Disease and tauopathy studies in flies and worms. Neurobiol Dis 2010; 40: 21-28.
195. Iijima-Ando K, Iijima K. Transgenic Drosophila models of Alzheimer's disease and tauopathies. Brain Struct Funct 2010; 214: 245-262.
196. Khurana V. Modeling Tauopathy in the fruit fly Drosophila melanogaster. J Alzheimers Dis 2008; 15: 541-553.
197. Ko LW, DeTure M, Sahara N, Chihab R, Vega IE, Yen SH. Recent advances in experimental modeling of the assembly of tau filaments. Biochim Biophys Acta 2005; 1739: 125-139.
198. Sydow A, Mandelkow EM. "Prion-like" propagation of mouse and human tau aggregates in an inducible mouse model of tauopathy. Neurodegener Dis 2010; 7: 28-31.
199. Wisniewski T, Boutajangout A. Immunotherapeutic approaches for Alzheimer's disease in transgenic mouse models. Brain Struct Funct 2010; 214: 201-218.
200. Goedert M, Spillantini MG, Jakes R et al. Molecular dissection of the paired helical filament. Neurobiol Aging 1995; 16: 325-334.
201. Iqbal K, Liu F, Gong CX, Alonso Adel C, Grundke-Iqbal I. Mechanisms of tau-induced neurodegeneration. Acta Neuropathol 2009; 118: 53-69.
202. Mandelkow EM, Biernat J, Drewes G et al. Microtubule-associated protein tau, paired helical filaments, and phosphorylation. Ann N Y Acad Sci 1993; 695: 209-216.
203. Trojanowski JQ, Lee VM. Phosphorylation of paired helical filament tau in Alzheimer's disease neurofibrillary lesions: focusing on phosphatases. FASEB J 1995; 9: 1570-1576.
204. Ferrer I, Blanco R, Carmona M, Puig B. Phosphorylated c-MYC expression in Alzheimer disease, Pick's disease, progressive supranuclear palsy and corticobasal degeneration. Neuropathol Appl Neurobiol 2001; 27: 343-351.
205. Jellinger KA, Stadelmann CH. The enigma of cell death in neurodegenerative disorders. J Neural Transm Suppl 2000; (60): 21-36.
206. Tortosa A, Blanco R, Ferrer I. Bcl-2 and Bax protein expression in neurofibrillary tangles in progressive supranuclear palsy. Neuroreport 1998; 9: 1049-1052.
207. Albers DS, Augood SJ, Martin DM, Standaert DG, Vonsattel JP, Beal MF. Evidence for oxidative stress in the subthalamic nucleus in progressive supranuclear palsy. J Neurochem 1999; 73: 881-884.
208. Aoyama K, Matsubara K, Kobayashi S. Aging and oxidative stress in progressive supranuclear palsy. Eur J Neurol 2006; 13: 89-92.
209. Cantuti-Castelvetri I, Keller-McGandy CE, Albers DS et al. Expression and activity of antioxidants in the brain in progressive supranuclear palsy. Brain Res 2002; 930: 170-181.
210. Castellani R, Smith MA, Richey PL, Kalaria R, Gambetti P, Perry G. Evidence for oxidative stress in Pick disease and corticobasal degeneration. Brain Res 1995; 696: 268-271.
211. Fukae J, Takanashi M, Kubo S et al. Expression of 8-oxoguanine DNA glycosylase (OGG1) in Parkinson's disease and related neurodegenerative disorders. Acta Neuropathol 2005; 109: 256-262.
212. Odetti P, Garibaldi S, Norese R et al. Lipoperoxidation is selectively involved in progressive supranuclear palsy. J Neuropathol Exp Neurol 2000; 59: 393-397.
213. Smith MA, Kutty RK, Richey PL et al. Heme oxygenase-1 is associated with the neurofibrillary pathology of Alzheimer's disease. Am J Pathol 1994; 145: 42-47.
214. Takahashi M, Ko LW, Kulathingal J, Jiang P, Sevlever D, Yen SH. Oxidative stress-induced phosphorylation, degradation and aggregation of alpha-synuclein are linked to upregulated CK2 and cathepsin D. Eur J Neurosci 2007; 26: 863-874.
215. Dickey CA, Koren J, Zhang YJ et al. Akt and CHIP coregulate tau degradation through coordinated interactions. Proc Natl Acad Sci USA 2008; 105: 3622-3627.
216. Ferrer I, Barrachina M, Puig B. Glycogen synthase kinase-3 is associated with neuronal and glial hyperphosphorylated tau deposits in Alzheimer's disease, Pick's disease, progressive supranuclear palsy and corticobasal degeneration. Acta Neuropathol 2002; 104: 583-591.
217. Ferrer I, Blanco R, Carmona M et al. Phosphorylated map kinase (ERK1, ERK2) expression is associated with early tau deposition in neurones and glial cells, but not with increased nuclear DNA vulnerability and cell death, in Alzheimer disease, Pick's disease, progressive supranuclear palsy and corticobasal degeneration. Brain Pathol 2001; 11: 144-158.
218. Jicha GA, Weaver C, Lane E et al. cAMP-dependent protein kinase phosphorylations on tau in Alzheimer's disease. J Neurosci 1999; 19: 7486-7494.
219. Takahashi M, Uchikado H, Caprotti D et al. Identification of G-protein coupled receptor kinase 2 in paired helical filaments and neurofibrillary tangles. J Neuropathol Exp Neurol 2006; 65: 1157-1169.
220. Martinez A, Portero-Otin M, Pamplona R, Ferrer I. Protein targets of oxidative damage in human neurodegenerative diseases with abnormal protein aggregates. Brain Pathol 2010; 20: 281-297.
221. Petrucelli L, Dickson D, Kehoe K et al. CHIP and Hsp70 regulate tau ubiquitination, degradation and aggregation. Hum Mol Genet 2004; 13: 703-714.
222. Wang Y, Martinez-Vicente M, Krüger U et al. Tau fragmentation, aggregation and clearance: the dual role of lysosomal processing. Hum Mol Genet 2009; 18: 4153-4170.