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Progressive Supranuclear Palsy and Corticobasal Degeneration

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Abstract:

Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are commonly referred to as “atypical parkinsonian syndromes” or “frontotemporal dementias.” Clinically, they are characterized by an akinetic-rigid syndrome and cognitive impairment combined with a variety of so-called “plus features.” On pathological grounds, their hallmarks are certain neurodegenerative lesion patterns coupled with tau protein-positive intracellular protein aggregates in both neuronal and glial cells bodies or their processes. The pathogenesis of these disorders is not fully elucidated yet. Both genetic and environmental factors may be contributory. Here we review briefly the clinical presentation, comment on the neuropathology, and discuss in more detail the current state of pathogenetic concepts of these disorders with a special focus on recent genetic findings.

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Abbreviations

AD:

Alzheimer's disease

CBD:

corticobasal degeneration

FTDP-17:

frontotemporal dementia with parkinsonism linked to chromosome 17

3R-tau:

3 repeat tau

4R-tau:

4 repeat tau

PD:

Parkinson's disease

PiD:

Pick's disease

PSP:

progressive supranuclear palsy

TIQs:

tetrahydroisoquinolines

References

  • Arima K, Nakamura M, Sunohara N, Ogawa M, Anno M, et al. 1997. Ultrastructural characterization of the tau-immunoreactive tubules in the oligodendroglial perikarya and their inner loop processes in progressive supranuclear palsy. Acta Neuropathol (Berl) 93(6): 558–566.

    Article  CAS  Google Scholar 

  • Armstrong RA, Cairns NJ, Lantos PL. 2000. A quantitative study of the pathological lesions in the neocortex and hippocampus of twelve patients with corticobasal degeneration. Exp Neurol 163(2): 348–356.

    Article  CAS  PubMed  Google Scholar 

  • Armstrong R, Lantos PL, Cairns NJ. 2005. Overlap between neurodegenerative disorders. Neuropathol 25: 111–124.

    Article  Google Scholar 

  • Baker M, Litvan I, Houlden H, Adamson J, Dickson D, et al. 1999. Association of an extended haplotype in the tau gene with progressive supranuclear palsy. Hum Mol Genet 8: 711–715.

    Article  CAS  PubMed  Google Scholar 

  • Bergeron C, Pollanen MS, Weyer L, Lang AE. 1997. Cortical degeneration in progressive supranuclear palsy. A comparison with cortical-basal ganglionic degeneration. J Neuropathol Exp Neurol 56(6): 726–734.

    Article  CAS  PubMed  Google Scholar 

  • Boeve BF, Maraganore DM, Parisi JE, Ahlskog JE, Graff-Radford N, et al. 1999. Pathologic heterogeneity in clinically diagnosed corticobasal degeneration. Neurology 53(4): 795–800.

    Article  CAS  PubMed  Google Scholar 

  • Boeve BF, Lang AE, Litvan I. 2003. Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol 54 (Suppl. 5): S15–S19.

    Article  PubMed  Google Scholar 

  • Bower J, Maraganore D, McDonnell S, Rocca W. 1997. Incidence of progressive supranuclear palsy and multiple system atrophy in Olmsted County, Minnesota, 1976 to 1990. Neurology 49: 1284–1288.

    Article  CAS  PubMed  Google Scholar 

  • Buee L, Delacourte A. 1999. Comparative biochemistry of tau in progressive supranuclear palsy, corticobasal degeneration, FTDP-17 and Pick's disease. Brain Pathol 9: 681–693.

    Article  CAS  PubMed  Google Scholar 

  • Bugiani O, Murrell JR, Giaccone G, Hasegawa M, Ghigo G, et al. 1999. Frontotemporal dementia and corticobasal degeneration in a family with a P301S mutation in tau. J Neuropathol 58: 667–677.

    Article  CAS  Google Scholar 

  • Bugiani O, Mancardi GL, Brusa A, Ederli A. 1979. The fine structure of subcortical neurofibrillary tangles in progressive supranuclear palsy. Acta Neuropathol 45: 147–152.

    Article  CAS  PubMed  Google Scholar 

  • Burn DJ, Lees AJ. 2002. Progressive supranuclear palsy: Where are we now? Lancet Neurol 1(6): 359–369.

    Article  PubMed  Google Scholar 

  • Caparros-Lefebvre D, Elbaz A. 1999. Possible relation of atypical parkinsonism in the French West Indies with consumption of tropical plants: A case-control study. Caribbean Parkinsonism Study Group. Lancet 354(9175): 281–286.

    Article  CAS  PubMed  Google Scholar 

  • Caparros-Lefebvre D, Sergeant N, Lees A, Camuzat A, Daniel S, et al. 2002. Guadeloupean parkinsonism: A cluster of progressive supranuclear palsy-like tauopathy. Brain 125(Pt 4): 801–811.

    Article  PubMed  Google Scholar 

  • Case Records of the Massachusetts General Hospital 1993. Case 38–1985: Case of corticonigral degeneration with neuronal achromasia. N Eng J Med 329: 1560–1567.

    Google Scholar 

  • Castellani R, Smith MA, Richey PL, Kalaria R, Gambetti P, et al. 1995. Evidence for oxidative stress in Pick disease and corticobasal degeneration. Brain Res 696(1–2): 268–271.

    Article  CAS  PubMed  Google Scholar 

  • Conrad C, Andreadis A, Trojanowski JQ, Dickson DW, Kang D, et al. 1997. Genetic evidence for the involvement of τ in progressive supranuclear palsy. Ann Neurol 41: 277–281.

    Article  CAS  PubMed  Google Scholar 

  • Cordato NJ, Halliday GM, Harding AJ, Hely MA, Morris JG. 2000. Regional brain atrophy in progressive supranuclear palsy and Lewy body disease. Ann Neurol 47(6): 718–728.

    Article  CAS  PubMed  Google Scholar 

  • Couchie D, Mavilia C, Georgieff IS, Liem RK, Shelanski ML, et al. 1992. Primary structure of high molecular weight tau present in the peripheral nervous system. Proc Natl Acad Sci USA 89(10): 4378–4381.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Cummings JL, Litvan I. 2000. Neuropsychiatric aspects of corticobasal degeneration. “Corticobasal Degeneration”. Advances in Neurology, Vol. 82. Litvan I, Goetz CG, Lang AE, editors. Philadelphia: Lippincott Williams & Wilkins; pp. 147–152.

    Google Scholar 

  • Cruz-Sanchez FF, Rossi ML, Cardozo A, Picardo A, Tolosa E. 1992. Immunohistological study of grumose degeneration of the dentate nucleus in progressive supranuclear palsy. J Neurol Sci 110(1–2): 228–231.

    Article  CAS  PubMed  Google Scholar 

  • Davis PH, Golbe LI, Duvoisin RC, Schoenberg BS. 1988. Risk factors for progressive supranuclear palsy. Neurology 38(10): 1546–1552.

    Article  CAS  PubMed  Google Scholar 

  • Delisle MB, Murrell JR, Richardson R, Trofatter JA, Rascol O, et al. 1999. A mutation at codon 279 (N279K) in exon 10 of the tau gene causes a tauopathy with dementia and progressive supranuclear palsy. Acta Neuropathol 98: 62–77.

    Article  CAS  PubMed  Google Scholar 

  • de Silva R, Hope A, Pittman A, Weale ME, Morris HR, et al. 2003. Strong association of the Saitohin gene Q7 variant with progressive supranuclear palsy. Neurology 61: 407–409.

    Article  CAS  PubMed  Google Scholar 

  • de Yebenese JG, Sarasa JL, Daniel SE, Lees AL. 1995. Familial progressive supranuclear palsy: Description of a pedigree and review of the literature. Brain 118: 1095–1103.

    Article  Google Scholar 

  • Dickson DW. 1999. Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration. J Neurol 246: II6–15.

    Article  PubMed  Google Scholar 

  • Dickson DW, Bergeron C, Chin SS, Duyckaerts C, Horoupian D, et al. 2002. Office of rare diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol 61(11): 935–946.

    Article  CAS  PubMed  Google Scholar 

  • Dickson DW, Liu WK, Ksiezak-Reding H, Yen SH. 2000. Neuropathologic and molecular considerations. Adv Neurol 82: 9–27.

    CAS  PubMed  Google Scholar 

  • di Maria E, Tabaton M, Vigo T, Abbruzzese G, Bellone E, et al. 2000. Corticobasal degeneration shares a common genetic background with progressive supranuclear palsy. Ann Neurol 47(3): 374-377.

    Article  CAS  PubMed  Google Scholar 

  • Drache B, Diehl GE, Beyreuther K, Perlmutter LS, Konig G. 1997. Bcl-xl-specific antibody labels activated microglia associated with Alzheimer's disease and other pathological states. J Neurosci Res 47(1): 98–108.

    Article  CAS  PubMed  Google Scholar 

  • Feany MB, Dickson DW. 1995. Widespread cytoskeletal pathology characterizes corticobasal degeneration. Am J Pathol 146: 1388–1396.

    CAS  PubMed  PubMed Central  Google Scholar 

  • Feany MB, Dickson DW. 1996. Neurodegenerative disorders with extensive tau pathology: A comparative study and review. Ann Neurol 40(2): 139–148.

    Article  CAS  PubMed  Google Scholar 

  • Feany MB, Mattiace LA, Dickson DW. 1996. Neuropathologic overlap of progressive supranuclear palsy, Pick's disease and corticobasal degeneration. J Neuropathol Exp Neurol 55: 53–67.

    Article  CAS  PubMed  Google Scholar 

  • Flament S, Delacourte A, Verny M, Hauw JJ, Javoy-Agid F. 1991. Abnormal tau proteins in progressive supranuclear palsy: Similarities and differences with the neurofibrillary degeneration of the Alzheimer type. Acta Neuropathol 81: 591–596.

    Article  CAS  PubMed  Google Scholar 

  • Forman MS, Farmer J, Johnson JK, Clark CM, Arnold SE, et al. 2006. Frontotemporal dementia: Clinicopathological correlations. Ann Neurol 59(6): 952–962.

    Article  PubMed  PubMed Central  Google Scholar 

  • Friedrich MJ. 1999. Pesticide study aids Parkinson research. JAMA 282: 2200.

    Article  CAS  PubMed  Google Scholar 

  • Georgieff IS, Liem RK, Mellado W, Nunez J, Shelanski ML. 1991. High molecular weight tau: Preferential localization in the peripheral nervous system. J Cell Sci 100(1): 55–60.

    Article  CAS  PubMed  Google Scholar 

  • Gerhard A, Banati RB, Cagnin A, Brooks DJ. 2001. In vivo imaging of activated microglia with [11C]PK11195 positron emission tomography (PET) in idiopathic and atypical Parkinson's disease. Neurology 56: 270.

    Article  Google Scholar 

  • Gerhard A, Watts J, Trender-Gerhard I, Turkheimer F, Banati RB, et al. 2004. In vivo imaging of microglial activation with [11C](R)-PK11195 PET in corticobasal degeneration. Mov Disord 19(10): 1221–1226.

    Article  PubMed  Google Scholar 

  • Gibb WRG, Luthert PJ, Marsden CD. 1989. Corticobasal degeneration. Brain 112: 1171–1192.

    Article  PubMed  Google Scholar 

  • Goedert M, Jakes R, Spillantini MG, Hasegawa M, Smith MJ, et al. (1996) Assembly of microtubule-associated protein tau into Alzheimer-like filaments induced by sulfated glycosaminoglycans. Nature 282: 550–553.

    Article  Google Scholar 

  • Goedert M, Spillantini MG, Crowther RA. 1992. Cloning of a big tau microtubule-associated protein characteristic of the peripheral nervous system. Proc Natl Acad Sci USA 89(5): 1983–1987.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Goedert M, Spillantini MG, Jakes R, Rutherford D, Crowther RA. 1989. Multiple isoforms of human microtubule-associated protein tau: Sequences and localization in neurofibrillary tangles in Alzheimer's disease. Neuron 3: 519–526.

    Article  CAS  PubMed  Google Scholar 

  • Golbe LI. 2000. Progressive supranuclear palsy in the molecular age. Lancet 356(9233): 870–871.

    Article  CAS  PubMed  Google Scholar 

  • Golbe LI, Rubin RS, Cody RP, Belsh JM, Duvoisin RC, et al. 1996. Follow-up study of risk factors in progressive supranuclear palsy. Neurology 47(1): 148–154.

    Article  CAS  PubMed  Google Scholar 

  • Gong CX, Liu F, Grundke-Iqbal I, Iqbal K. 2005. Post-translational modifications of tau protein in Alzheimer's disease. J Neural Transm 112(6): 813–838.

    Article  CAS  PubMed  Google Scholar 

  • Graham NL, Bak TH, Hodges JR. 2003. Corticobasal degeneration as a cognitive disorder. Mov Disord 18(11): 1224–1232.

    Article  PubMed  Google Scholar 

  • Greenberg SG, Davis P. 1990. A preparation of Alzheimer paired helical filaments that displays distinct tau proteins by polyacrylamide gel electrophoresis. Proc Natl Acad Sci USA 87(15): 5827–5831.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Grimes DA, Lang AE, Bergeron CB. 1999. Dementia as the most common presentation of cortical-basal ganglionic degeneration. Neurology 53(9): 1969–1974.

    Article  CAS  PubMed  Google Scholar 

  • Halliday GM, Hardman CD, Cordato NJ, Hely MA, Morris JG. 2000. A role for the substantia nigra pars reticulata in the gaze palsy of progressive supranuclear palsy. Brain 123 (Pt 4): 724–732.

    Article  PubMed  Google Scholar 

  • Hardy J, Pittman A, Myers A, Gwinn-Hardy K, Fung HC, 2005. Evidence suggesting that Homo neanderthalensis contributed the H2 MAPT haplotype to Homo sapiens. 32(4): 582–585.

    Google Scholar 

  • Hartzler AW, Zhu X, Siedlak SL, Castellani RJ, Avila J, et al. 2002. The p38 pathway is activated in Pick disease and progressive supranuclear palsy: A mechanistic link between mitogenic pathways, oxidative stress, and tau. Neurobiol Aging 23(5): 855–859.

    Article  CAS  PubMed  Google Scholar 

  • Hauw JJ, Daniel SE, Dickson D, Horoupian DS, Jellinger K, et al. 1994. Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 44(11): 2015–2019.

    Article  CAS  PubMed  Google Scholar 

  • Higuchi Y, Iwaki T, Tateishi J. 1995. Neurodegeneration in the limbic and paralimbic system in progressive supranuclear palsy. Neuropathol Appl Neurobiol 21: 246–254.

    Article  CAS  PubMed  Google Scholar 

  • Hof PR, Nimchinsky EA, Buee-Scherrer V, Buee L, Nasrallah J, et al. 1994. Amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam: Quantitative neuropathology, immunohistochemical analysis of neuronal vulnerability, and comparison with related neurodegenerative disorders. Acta Neuropathol 88(5): 397–404.

    Article  CAS  PubMed  Google Scholar 

  • Horiguchi T, Uryu K, Giasson BI, Ischiropoulos H, Light Foot R, et al. 2003. Nitration of tau protein is linked to neurodegeneration in tauopathies. Am J Pathol 163(3): 1021–1031.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Houlden H, Baker M, Morris HR, Mac Donald N, Pickering-Brown S, et al. 2001. Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype. Neurology 56: 1702–1706.

    Article  CAS  PubMed  Google Scholar 

  • Hughes AJ, Daniel SE, Ben-Shlomo Y, Lees AJ. 2002. The accuracy of diagnosis of parkinsonian syndromes in a specialist movement disorder service. Brain 125(Pt 4): 861–870.

    Article  PubMed  Google Scholar 

  • Hutton M. 2000. “Missing” tau mutation identified. Ann Neurol 47: 417–418.

    Article  CAS  PubMed  Google Scholar 

  • Ikeda K, Akiyama H, Arai T, Tsuchiya K. 2002. Pick-body-like inclusions in corticobasal degeneration differ from Pick bodies in Pick's disease. Acta Neuropathol (Berl) 103: 115–118.

    Article  CAS  Google Scholar 

  • Ikeda K, Akiyama H, Haga C, Kondo H, Arima K, et al. 1994. Argyrophilic thread-like structure in corticobasal degeneration and supranuclear palsy. Neurosci Lett 174: 157–159.

    Article  CAS  PubMed  Google Scholar 

  • Ishizawa K, Dickson DW. 2001. Microglial activation parallels system degeneration in progressive supranuclear palsy and corticobasal degeneration. J Neuropathol Exp Neurol 60: 647–657.

    Article  CAS  PubMed  Google Scholar 

  • Jellinger KA, Blancher C. 1992. Neuropathology. Progressive Supranuclear Palsy: Clinical and Research Approaches. Litvan I, Agid Y, editors. Oxford: Oxford University Press; pp. 44–88.

    Google Scholar 

  • Jiang Z, Cote J, Kwon JM, Goate AM, Wu JY. 2000. Aberrant splicing of tau pre-mRNA caused by intronic mutations associated with the inherited dementia frontotemporal dementia with Parkinsonism linked to chromosome 17. Mol Cell Biol 20: 4036–4048.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Jiang Y, Tang H, Havlioglu N, Zhang X, Stamm S, et al. 2003. Mutations in tau gene exon 10 associated with FTDP-17 alter the activity of an exonic splicing enhancer to interact with Tra2β. J Biol Chem 278: 18997–19007.

    Article  CAS  PubMed  Google Scholar 

  • Josephs KA, Dickson DW. 2003. Diagnostic accuracy of progressive supranuclear palsy in the Society for Progressive Supranuclear Palsy brain bank. Mov Disord 18(9): 1018–1026.

    Article  PubMed  Google Scholar 

  • Kawai H, Makino Y, Hirobe M, Ohta S. 1998. Novel endogenous 1,2,3,4-tetrahydroisoquinoline derivative: Uptake by dopamine transporter and activity to induce parkinsonism. J Neurochem 70: 745–751.

    Article  CAS  PubMed  Google Scholar 

  • Kertesz A, McMonagle P, Blair M, Davidson W, Munoz DG. 2005. The evolution and pathology of frontotemporal dementia. 128(Pt 9): 1996-2005.

    Google Scholar 

  • Kikuchi K, Nagatsu Y, Makino Y, Mashino T, Ohta S, et al. 1991. Metabolism and penetration through blood-brain barrier of parkinsonism-related compounds 1,2,3,4-tetrahydroisoquinoline and 1-methyl-1,2,3,4-tetrahydroisoquinoline. Drug Metab Dispos 19(1): 257–262.

    CAS  PubMed  Google Scholar 

  • Komori T. 1999. Tau-positive glial inclusions in progressive supranuclear palsy, corticobasal degeneration and Pick's disease. Brain Pathol 9: 663–679.

    Article  CAS  PubMed  Google Scholar 

  • Komori T, Arai N, Oda M, Nakayama H, Mori H, et al. 1998. Astrocytic plaques and tufts of abnormal fibers do not coexist in corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathol (Berl) 96(4): 401–408.

    Article  CAS  Google Scholar 

  • Kondo S, Yamamoto N, Murakami T, Okumura M, Mayeda A, et al. 2000. Tra2β, SF2/ASF and SRp30c modulate the function of an exonic splicing enhancer in exon 10 of tau pre-mRNA. Genes Cells 9: 121–130.

    Article  Google Scholar 

  • Kotake Y, Tasaki Y, Yea. Makino 1995. 1-Benzyl-1,2,3,4-tetrahydroisoquinoline as a parkinsonism-inducing agent: A novel endogenous amine in mouse brain and parkinsonian CSF. J Neurochem 65: 2633–2638.

    Article  CAS  PubMed  Google Scholar 

  • Kotake T, Yoshida M, Ogawa M, Tasaki Y, Hirobe M, et al. 1996. Chronic administration of 1-benzyl-1,2,3,4-tetrahydroisoquinoline, an endogenous amine in the brain, induces parkinsonism in a primate. Neurosci Lett 217: 69–71.

    Article  CAS  PubMed  Google Scholar 

  • Ksiezak-Reding H, Morgan K, Mattiace LA, Davies P, Liu W-K, et al. 1994. Ultrastructure and biochemical composition of paired helical filaments in corticobasal degeneration. Am J Pathol 145: 1496–1508.

    CAS  PubMed  PubMed Central  Google Scholar 

  • Kumar R, Bergeron C, Lang AE. 2002. Corticobasal degeneration. Parkinson's Disease and Movement Disorders, Jankovic J, Tolosa E, editors. 4th edition. Philadelphia: Lippincott Williams & Wilkins; pp. 185–198.

    Google Scholar 

  • Lang AE. 2003. Corticobasal degeneration: Selected developments. Mov Disord 18(Suppl. 6): S51–S56.

    Article  PubMed  Google Scholar 

  • Lang A, Riley D, Bergeron C. 1994. Cortical-basal degeneration. Neurodegenerative Diseases. Calne D, editor. Philadelphia: Saunders; pp. 877–894.

    Google Scholar 

  • Lannuzel A, Michel PP, Abaul MJ, Caparros-Lefebvre D, Ruberg M. 2000. Neurotoxic effects of alkaloids from Annona muricata (soursop) on dopaminergic neurons: Potential role in etiology of atypical parkinsonism in French West Indies. Mov Disord 13(Suppl. 3): 28.

    Google Scholar 

  • Lee V, Goedert M, Tojanowski JQ. 2001. Neurodegenerative tauopathies. Annu Rev Neurosci 24: 1121–1159.

    Article  CAS  PubMed  Google Scholar 

  • Lippa CF, Flanders KC, Kim ES, Croul S. 1998. TGF-β receptors-I and -II immunoexpression in Alzheimer's disease: A comparison with aging and progressive supranuclear palsy. Neurobiol Aging 19(6): 527–533.

    Article  CAS  PubMed  Google Scholar 

  • Litvan I. 2003. Update on epidemiological aspects of progressive supranuclear palsy. Mov Disord 18 (Suppl. 6): S43-S50.

    Article  PubMed  Google Scholar 

  • Litvan I. 2005a. Progressive supranuclear palsy. Scientific Basis for Treatment of Parkinson's disease. Galvez-Jimenez N, editor. London, NewYork: Taylor & Francis; pp. 267–278.

    Google Scholar 

  • Litvan I. 2005b. Progressive supranuclear palsy and corticobasal degeneration. Animal Models of Movement Disorders. LeDoux M, editor. Amsterdam: Elsevier; pp. 505–514.

    Chapter  Google Scholar 

  • Litvan I, Baker M, Hutton M. 2001. Tau genotype: No effect on onset, symptom severity, or survival in progressive supranuclear palsy. Neurology 57: 138–140.

    Article  CAS  PubMed  Google Scholar 

  • Litvan I, Agid Y, Calne D, Campbell G, Dubois B, et al. 1996a. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): Report of the NINDS-SPSP international workshop. Neurology 47(1): 1–9.

    Article  CAS  PubMed  Google Scholar 

  • Litvan I, Mangone CA, McKee A, Verny M, Parsa A, et al. 1996b. Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: A clinicopathological study. J Neurol Neurosurg Psychiat 60: 615–620.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Mackenzie IRA, Hudson LP. 1995. Achromatic neurons in the cortex of progressive supranuclear palsy. Acta Neuropathol (Berl) 90: 615–619.

    Article  CAS  Google Scholar 

  • Mahapatra RK, Edwards MJ, Schott JM, Bhatia KP. 2004. Corticobasal degeneration. Lancet Neurol 3(12): 736–743.

    Article  PubMed  Google Scholar 

  • Makino Y, Ohta S, Tachikawa O, Hirobe M. 1988. Presence of tetrahydroisoquinoline and 1-methyl-tetrahydro-isoquinoline in foods: Compounds related to Parkinson's disease. Life Sci 43: 373–378.

    Article  CAS  PubMed  Google Scholar 

  • Makino Y, Ohta S, Tasaki Y, Tachikawa O, Kashiwasake M, et al. 1990. A novel and neurotoxic tetrahydroisoquinoline derivative in vivo: Formation of 1,3-dimethyl-1,2,3,4-tetrahydroisoquinoline, a condensation product of amphetamines, in brains of rats under chronic ethanol treatment. J Neurochem 55: 963–969.

    Article  CAS  PubMed  Google Scholar 

  • Maraganore D, Ahlskog J, Petersen R. 1992. Progressive asymmetric rigidity with apraxia: A distinct clinical entity. Mov Disord 7(Suppl. 1): 80.

    Google Scholar 

  • McCrank E. 1990. PSP risk factors. Neurology 40(10): 1637.

    Article  CAS  PubMed  Google Scholar 

  • McCrank E, Rabheru K. 1989. Four cases of progressive supranuclear palsy in patients exposed to organic solvents. Can J Psychiatry 34(9): 934–936.

    Article  CAS  PubMed  Google Scholar 

  • Miller ML, Johnson JVW. 1995. Transglutaminase cross-linking of the τ protein. J Neurochem 65: 1760–1770.

    Article  CAS  PubMed  Google Scholar 

  • Morimatsu M, Negoro K. 2002. Provisional diagnostic criteria of corticobasal degeneration (CBD) and the survey of patients with CBD in Japan. Rinsho Shinkeigaku 42(11): 1150–1153.

    PubMed  Google Scholar 

  • Morris HR, Vaughan JR, Datta SR, Bandopadhyay R, Rohan De Silva HA, et al. 2000. Multiple system atrophy/progressive supranuclear palsy: α-Synuclein, synphilin, tau, and APOE. Neurology 55(12): 1918–1920.

    Article  CAS  PubMed  Google Scholar 

  • Morris HR, Gibb G, Katzenschlager R, Wood NW, Hanger DP, et al. 2002. Pathological, clinical and genetic heterogeneity in progressive supranuclear palsy. Brain 125: 969–975.

    Article  CAS  PubMed  Google Scholar 

  • Morris HR, Osaki Y, Holton J, Lees AJ, Wood NW, et al. 2003. Tau exon 10+16 mutation FTDP-17 presenting clinically as sporadic young onset PSP. Neurology 61: 102–104.

    Article  CAS  PubMed  Google Scholar 

  • Nicholl DJ, Bennett P, Hiller L, Bonifati V, Vanacore N, et al. 1999. A study of five candidate genes in Parkinson's disease and related neurodegenerative disorders. European Study Group on Atypical Parkinsonism. Neurology 53(7): 1415–1421.

    Article  CAS  PubMed  Google Scholar 

  • Niwa T, Takeda N, Sasaoka T, Kaneda T, et al. 1989a. Detection of tetrahydroisoquinoline, in parkinsonian brain as an endogenous amine by use of gas chromatography-mass spectrometry. J Chromatogr 491: 397–403.

    Article  CAS  PubMed  Google Scholar 

  • Niwa T, Yoshizumi H, Aea. Tatematsu 1989b. Presence of tetrahydroisoquinoline, a parkinsonism-related compound, in foods. J Chromatogr 493: 347–352.

    Article  CAS  PubMed  Google Scholar 

  • Oyanagi K, Tsuchiya K, Yamazaki M, Ikeda K. 2001. Substantia nigra in progressive supranuclear palsy, corticobasal degeneration, and parkinsonism-dementia complex of Guam: Specific pathological features. J Neuropathol Exp Neurol 60: 393–402.

    Article  CAS  PubMed  Google Scholar 

  • Pastor P, Pastor E, Carnero C, Vela R, Garcia T, et al. 2001. Familial atypical progressive supranuclear palsy associated with homozygosity for del N296 mutation in the tau gene. Ann Neurol 49: 263–267.

    Article  CAS  PubMed  Google Scholar 

  • Perez M, Valpuesta JM, Medine M, Montejo de Garcini E, Avila J. 1996. Polymerization of tau into filaments in the presence of heparin: The minimal sequence requirement for tau-tau interactions. J Neurochem 67: 1183–1190.

    Article  CAS  PubMed  Google Scholar 

  • Pezzoli G, Ricciardi S, Masotto C, Mariani CB, Carenzi A. 1990. n-Hexane induces parkinsonism in rodents. Brain Res 531(1–2): 355–357.

    Article  CAS  PubMed  Google Scholar 

  • Pezzoli G, Barbieri S, Ferrante C, Zecchinelli A, Foa V. 1989. Parkinsonism due to n-Hexane exposure. Lancet 2(8667): 874.

    Article  CAS  PubMed  Google Scholar 

  • Poewe W, Wenning GK. 2003. Atypical parkinsonism. Neurological Disorders: Course and Treatment. Brandt T, Caplan LR, Dichans J, Diener HC, Kennard C, editors. Amsterdam: Elsevier; pp. 1081–1098.

    Chapter  Google Scholar 

  • Poorkaj P, Muma NA, Zhukareva V, Cochran EJ, Shannon KM, et al. 2002. An R5L mutation in a subject with a progressive supranuclear palsy phenotype. Ann Neurol 52: 511–516.

    Article  PubMed  Google Scholar 

  • Powell HC, London GW, Lampert PW. 1974. Neurofibrillary tangles in progressive supranuclear palsy: Electron microscopic observations. J Neuropathol Exp Neurol 33: 98–106.

    Article  CAS  PubMed  Google Scholar 

  • Rebeiz JJ, Kolodny EH, Richardson EP Jr. 1968. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 18(1): 20–33.

    Article  CAS  PubMed  Google Scholar 

  • Riley DE, Lang AE, Lewis A, Resch L, Ashby P, et al. 1990. Cortical-basal ganglionic degeneration. Neurology 40(8): 1203–1212.

    Article  CAS  PubMed  Google Scholar 

  • Riley DE, Lang AE. 2000. Clinical diagnostic criteria. Adv Neurol 82: 29–34.

    CAS  PubMed  Google Scholar 

  • Riley DE, Moro-de-Casillas ML. 2005. Cortico-basal degeneration. Scientific Basis for the Treatment of Parkinson's Disease. Galvez-Jimenez N, editor. London, NewYork: Taylor & Francis; pp. 279–289.

    Google Scholar 

  • Rinne JO, Lee MS, Thompson PD, Marsden CD. 1994. Corticobasal degeneration. A clinical study of 36 cases. Brain 117 (Pt 5): 1183–1196.

    Article  PubMed  Google Scholar 

  • Rojo A, Pernaute RS, Fontan A, Ruiz PG, Honnorat J, et al. 1999. Clinical genetics of familial progressive supranuclear palsy. Brain 122: 1233–1245.

    Article  PubMed  Google Scholar 

  • Ros R, Garre PG, Hirano M, Tai YF, Ampuero I, et al. 2005. Genetic linkage of autosomal dominant progressive supranuclear palsy to 1q31.1. Ann Neurol 57: 634–641.

    Article  CAS  PubMed  Google Scholar 

  • Sasaki N, Fukatsu R, Tsuzuki K, Hayashi Y, Yoshida T, et al. 1998. Advanced glycation end products in Alzheimer's disease and other neurodegenerative diseases. Am J Pathol 153(4): 1149–1155.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Schrag A, Ben Shlomo Y, Quinn NP. 1999. Prevalence of progressive supranuclear palsy and multiple system atrophy: A cross-sectional study. Lancet 354(9192): 1771–1775.

    Article  CAS  PubMed  Google Scholar 

  • Schweitzer K, Decker E, Zhu L, Miller RE, Mirra SS, et al. 2006. Aberrantly regulated proteins in frontotemporal dementia. Biochem Biophys Res Commun 348(2): 465–472.

    Article  CAS  PubMed  Google Scholar 

  • Smith TW, Lippa CF, de Girolami U. 1992. Immunocytochemical study of ballooned neurons in cortical degeneration with neuronal achromasia. Clin Neuropathol 11(1): 28–35.

    CAS  PubMed  Google Scholar 

  • Soto-Otero R, Mendez-Alvarez E, Sanchez-Sellero I, Cruz-Landeira A, Lopez-Rivadulla Lamas M. 2001. Reduction of rat brain levels of the endogenous dopaminergic proneurotoxins 1,2,3,4-tetrahydroisoquinoline and 1,2,3,4-tetrahydro-β-carboline by cigarette smoke. Neurosci Lett 298: 187–190.

    Article  CAS  PubMed  Google Scholar 

  • Spillantini MG, Goedert M. 2001. Tau and Parkinson disease. JAMA 286: 2324–2326.

    Article  CAS  PubMed  Google Scholar 

  • Spillantini MG, Yoshida H, Rizzini C, Lantos PL, Khan N, et al. 2000. A novel mutation (N296N) in familial dementia with swollen achromatic neurons and corticobasal inclusion bodies. Ann Neurol 48: 939–943.

    Article  CAS  PubMed  Google Scholar 

  • Stanford PM, Halliday GM, Brooks WS, Kwok JB, Store CE, et al. 2000. 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 123: 880–893.

    Article  PubMed  Google Scholar 

  • Steele JC, Richardson JC, Olszewski J. 1964. 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 10: 333–359.

    Article  CAS  PubMed  Google Scholar 

  • Stefansson H, Helgason A, Thorleifsson G, Steinthorsdottir V, Masson G, et al. 2005. A common inversion under selection in Europeans. Nat Genet 37(2): 129–137.

    Article  CAS  PubMed  Google Scholar 

  • Swerdlow RH, Golbe LI, Parks JK, Cassarino DS, Binder DR, et al. 2000. Mitochondrial dysfunction in cybrid lines expressing mitochondrial genes from patients with progressive supranuclear palsy. J Neurochem 75: 1681–1685.

    Article  CAS  PubMed  Google Scholar 

  • Tasaki Y, Makino Y, Ohta S, Hirobe M. 1991. 1-Methyl-1,2,3,4-tetrahydroisoquioline, decreasing in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-treated mouse, prevents parkinsonism-like behavior abnormalities. J Neurochem 57: 1940–1943.

    Article  CAS  PubMed  Google Scholar 

  • Tawana K, Ramsden DB. 2001. Progressive supranuclear palsy. Mol Pathol 54(6): 427–434.

    CAS  PubMed  PubMed Central  Google Scholar 

  • Tetrud JW, Langston JW, Irwin I, Snow B. 1994. Parkinsonism caused by petroleum waste ingestion. Neurology 44(6): 1051–1054.

    Article  CAS  PubMed  Google Scholar 

  • Togo T, Dickson D. 2002. Ballooned neurons in PSP are usually due to concurrent argyrophilic grain disease. Acta Neuropathol (Berl) 104: 5356.

    Google Scholar 

  • Toloso E, Valldeoriola F, Pastor P. 2002. Progressive supranuclear palsy. Parkinson's Disease and Movement Disorders. Jankovic J, Tolosa E, editors. 4th edition. Philadelphia:Lippincott Williams & Wilkins; pp. 152–169.

    Google Scholar 

  • Tsuchiya K, Uchihara T, Oda T, Arima K, Ikeda K, et al. 1997. Basal ganglia lesions in corticobasal degeneration differ from those in Pick's disease and progressive supranuclear palsy: A topographic neuropathological study of six autopsy cases. Neuropathology 17: 208–216.

    Article  Google Scholar 

  • Uchihara T, Mitani K, Mori H, Kondo H, Yamada M, et al. 1994. Abnormal cytoskeletal pathology peculiar to corticobasal degeneration is different from that of Alzheimer's disease or progressive supranuclear palsy. Acta Neuropathol (Berl) 88(4): 379–383.

    Article  CAS  Google Scholar 

  • Uchihara T, Mizusawa H, Tsuchiya K, Kondo H, Oda T, et al. 1998. Discrepancy between tau immunoreactivity and argyrophilia by the Bodian method in neocortical neurons of corticobasal degeneration. Acta Neuropathol 96: 553–557.

    Article  CAS  PubMed  Google Scholar 

  • Vanacore N, Bonifati V, Colosimo C, Fabbrini G, De Michele G, et al. 2001. Epidemiology of progressive supranuclear palsy. ESGAP Consortium. European Study Group on Atypical Parkinsonisms. Neurol Sci 22(1): 101–103.

    Article  CAS  PubMed  Google Scholar 

  • Vanacore N, Bonifati V, Fabbrini G, Colosimo C, Marconi R, et al. 2000. Smoking habits in multiple system atrophy and progressive supranuclear palsy. European Study Group on Atypical Parkinsonisms. Neurology 54(1): 114-119.

    Article  CAS  PubMed  Google Scholar 

  • Varani L, Spillantini MG, Goedert M, Varani G. 2000. Structural basis for recognition of the RNA major groove in the tau exon 10 splicing regulatory element by aminoglycoside antibiotics. Nucleic Acids Res 28(3): 710–719.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Vermersch P, Robitaille Y, Berneir L, Wattez A, Gauvreau D, et al. 1994. Biochemical mapping of neurofibrillary degeneration in a case of progressive supranuclear palsy: Evidence for general cortical involvement. Acta Neuropathol 87: 572–577.

    Article  CAS  PubMed  Google Scholar 

  • von Bergen M, Barghorn S, Muller SA, Pickhardt M, Biernat J, et al. 2006. The core of tau-paired helical filaments studied by scanning transmission electron microscopy and limited proteolysis. Biochemistry 45(20): 6446–6457.

    Article  CAS  PubMed  Google Scholar 

  • Wakabayashi K, Takahashi H. 1996. Similarities and differences among progressive supranuclear palsy, corticobasal degeneration and Pick's disease. Neuropathology 16: 262–268.

    Article  Google Scholar 

  • Wakabayashi K, Takahashi H. 2004. Pathological heterogeneity in progressive supranuclear palsy and corticobasal degeneration. Neuropathology 24(1): 79–86.

    Article  PubMed  Google Scholar 

  • Watts RL, Mirra SS, Young RR. 1989. Corticobasal ganglionic degeneration (CBDG) with neuronal achromasia: Clinical-pathological study of two cases. Neurology 39: 140.

    Article  Google Scholar 

  • Wenning GK, Litvan I, Jankovic J, Granata R, Mangone CA, et al. 1998. Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. J Neurol Neurosurg Psychiatry 64(2): 184–189.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Williams DR. 2006. Tauopathies: Classification and clinical update on neurodegenerative diseases associated with microtubule-associated protein tau. Intern Med J 36(10): 652–660.

    Article  CAS  PubMed  Google Scholar 

  • Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, et al. 2005. Characteristics of two distinct phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism. Brain 128(6): 1247–1258.

    Article  PubMed  Google Scholar 

  • Yagishita S, Itoh Y, Amano N, Nakano T, Saitoh A. 1979. Ultrastructure of neurofibrillary tangles in progressive supranuclear palsy. Acta Neuropathol 48: 27–30.

    Article  CAS  PubMed  Google Scholar 

  • Yamada T, Moroo I, Koguchi Y, Asahina M, Hirayama K. 1994. Increased concentration of C4d complement protein in the cerebrospinal fluids in progressive supranuclear pals. Acta Neurol Scand 89: 42–46.

    Article  CAS  PubMed  Google Scholar 

  • Yoshida M, Niwa T, Nagatsu T. 1990. Parkinsonism in monkeys produced by chronic administration of an endogenous substance of the brain, tetrahydroisoquinoline: The behavioral and biochemical changes. Neurosci Lett 119: 109–113.

    Article  CAS  PubMed  Google Scholar 

  • Zemaitaitis MO, Lee JM, Troncoso JC, Muma NA. 2000. Transglutaminase-induced cross-linking of tau proteins in progressive supranuclear palsy. J Neuropathol Exp Neurol 59(11): 983–989.

    Article  CAS  PubMed  Google Scholar 

  • Zemaitaitis MO, Kim SY, Halverson RA, Troncoso JC, Lee JM, et al. 2003. Transglutaminase activity, protein, and mRNA expression are increased in progressive supranuclear palsy. J Neuropathol Exp Neurol 62(2): 173–184.

    Article  CAS  PubMed  Google Scholar 

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Authors
  1. F. Geser
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  2. S. W. Scholz
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  3. G. K. Wenning
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Editor information

Editors and Affiliations

  1. Center for Neurochemistry, Nathan S. Kline Institute for Psychiatric Research, 140 Old Orangeburg Road, New York, 10962, Orangeburg, USA

    Abel Lajtha (Director) (Director)

  2. Technion‐Rappaport Family Faculty of Medicine, Efron St., POBox 9697, 31096, Haifa, Israel

    Moussa B. H. Youdim (Professor of Pharmacology Professor of Life Sciences Director of Eve Topf and NPF Centers of Excellence for Neurodegenerative Diseases Research) (Professor of Pharmacology Professor of Life Sciences Director of Eve Topf and NPF Centers of Excellence for Neurodegenerative Diseases Research)

  3. Clinic and Policlinic of Psychiatry and Psychotherapy Dept. Clinical Neurochemistry, University of Würzburg, Füchsleinstr. 15, 97080, Würzburg, Germany

    Peter Riederer

  4. Eve Topf Center for Neurodegenerative Diseases Research and Dept. of Pharmacology, Faculty of Medicine Technion, Efron St., P.O.B. 9697, 31096, Haifa, Israel

    Sylvia A. Mandel

  5. Department of Neurosciences, University of Padova Medical School, Via Giustiniani 5, 35128, Padova, Italy

    Leontino Battistin

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Geser, F., Scholz, S.W., Wenning, G.K. (2007). Progressive Supranuclear Palsy and Corticobasal Degeneration. In: Lajtha, A., Youdim, M.B.H., Riederer, P., Mandel, S.A., Battistin, L. (eds) Handbook of Neurochemistry and Molecular Neurobiology. Springer, New York, NY. https://doi.org/10.1007/978-0-387-30377-2_8

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