Abstract
Progressive supranuclear palsy (PSP) is the most common atypical neurodegenerative parkinsonian disorder (1,2). It was first described as a discrete clinicopathological entity by Steele et al. (3) in 1964 (Fig. 1), but there are several previous clinical descriptions of patients who may have had this disease (see Chapter 2). Clinically, PSP typically presents at middle-to-late age with progressive unexplained prominent postural instability with falls, supranuclear vertical gaze palsy, pseudobulbar palsy, levodopa-unresponsive parkinsonism, and frontal cognitive disturbances (3–5). Neuropathologically, PSP is characterized by the presence of neurofibrillary tangles in neurons and glia in specific basal ganglia and brainstem areas (6) (Fig.2). Neurofibrillary tangles are abnormal aggregates of tau protein that are also the main pathologic feature in corticobasal degeneration (CBD), Pick’s disease, frontotemporal dementia with parkinsonism associated to chromosome 17 abnormalities (FTDP-17), and Alzheimer’s disease (AD). Hence, as all these disorders, PSP is considered a “tauopathy” (see also Chapter 4).
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
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):1284–1288.
Schrag A, Ben-Shlomo Y, Quinn NP. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet 1999;354(9192):1771–1775.
Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy. Arch Neurol 1964;10: 333–359.
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):1–9.
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):1702–1706.
Hauw JJ, Daniel SE, Dickson D, Horoupian DS, Jellinger K, Lantos PL, et al. Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 1994;44(11): 2015–2019.
Maher ER, Lees AJ. The clinical features and natural history of the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 1986;36(7):1005–1008.
Walsh JS, Welch HG, Larson EB. Survival of outpatients with Alzheimer-type dementia. Ann Intern Med 1990;113(6):429–434.
Tolosa E, Valldeoriola F, Marti MJ. Clinical diagnosis and diagnostic criteria of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). J Neural Transm Suppl 1994;42:15–31.
Litvan I, Mangone CA, McKee A, Verny M, Parsa A, Jellinger K, et al. Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study. J Neurol Neurosurg Psychiatry 1996;60(6):615–620.
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(4):427–431.
Santacruz P, Uttl B, Litvan I, Grafman J. Progressive supranuclear palsy: a survey of the disease course. Neurology 1998;50(6):1637–1647.
Wenning GK, Ebersbach G, Verny M, Chaudhuri KR, Jellinger K, McKee A, et al. Progression of falls in postmortem-confirmed parkinsonian disorders. Mov Disord 1999;14(6):947–950.
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):910–916.
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. J Neurol 1999; 246Suppl 2:II1–II5.
Litvan I, Campbell G, Mangone CA, Verny M, McKee A, Chaudhuri KR, et al. Which clinical features differentiate progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) from related disorders? A clinicopathological study. Brain 1997;120(Pt 1):65–74.
Collins SJ, Ahlskog JE, Parisi JE, Maraganore DM. Progressive supranuclear palsy: neuropathologically based diagnostic clinical criteria. J Neurol Neurosurg Psychiatry 1995;58(2):167–173.
Barclay CL, Lang AE. Dystonia in progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 1997; 62(4):352–356.
Pillon B, Dubois B, Lhermitte F, Agid Y. Heterogeneity of cognitive impairment in progressive supranuclear palsy, Parkinson’s disease, and Alzheimer’s disease. Neurology 1986;36(9):1179–1185.
Grafman J, Litvan I, Gomez C, Chase TN. Frontal lobe function in progressive supranuclear palsy. Arch Neurol 1990;47(5):553–558.
Grafman J, Weingartner H, Newhouse PA, Thompson K, Lalonde F, Litvan I, et al. Implicit learning in patients with Alzheimer’s disease. Pharmacopsychiatry 1990;23(2):94–101.
Pillon B, Dubois B, Ploska A, Agid Y. Severity and specificity of cognitive impairment in Alzheimer’s, Huntington’s, and Parkinson’s diseases and progressive supranuclear palsy. Neurology 1991; 41(5):634–643.
Pillon B, Deweer B, Michon A, Malapani C, Agid Y, Dubois B. Are explicit memory disorders of progressive supranuclear palsy related to damage to striatofrontal circuits? Comparison with Alzheimer’s, Parkinson’s, and Huntington’s diseases. Neurology 1994;44(7):1264–1270.
Litvan I, Mega MS, Cummings JL, Fairbanks L. Neuropsychiatric aspects of progressive supranuclear palsy. Neurology 1996;47(5):1184–1189.
Litvan I, Paulsen JS, Mega MS, Cummings JL. Neuropsychiatric assessment of patients with hyperkinetic and hypokinetic movement disorders. Arch Neurol 1998;55(10):1313–1319.
Robbins TW, James M, Owen AM, Lange KW, Lees AJ, Leigh PN, et al. Cognitive deficits in progressive supranuclear palsy, Parkinson’s disease, and multiple system atrophy in tests sensitive to frontal lobe dysfunction. J Neurol Neurosurg Psychiatry 1994;57(1):79–88.
Verny M, Duyckaerts C, Agid Y, Hauw JJ. The significance of cortical pathology in progressive supranuclear palsy. Clinico-pathological data in 10 cases. Brain 1996;119(Pt 4):1123–1136.
Goetz CG, Leurgans S, Lang AE, Litvan I. Progression of gait, speech and swallowing deficits in progressive supranuclear palsy. Neurology 2003;60(6):917–922.
Siderowf A, Quinn NP. Progressive supranuclear palsy: setting the scene for therapeutic trials. Neurology 2003;60(6):892–893.
Lopez OL, Litvan I, Catt KE, Stowe R, Klunk W, Kaufer DI, et al. Accuracy of four clinical diagnostic criteria for the diagnosis of neurodegenerative dementias. Neurology 1999;53(6):1292–1299.
Davis PH, Bergeron C, McLachlan DR. Atypical presentation of progressive supranuclear palsy. Ann Neurol 1985;17(4):337–343.
Matsuo H, Takashima H, Kishikawa M, Kinoshita I, Mori M, Tsujihata M, et al. Pure akinesia: an atypical manifestation of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 1991;54(5):397–400.
Daniel SE, de Bruin VM, Lees AJ. The clinical and pathological spectrum of Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy): a reappraisal. Brain 1995;118(Pt 3):759–770.
Bergeron C, Pollanen MS, Weyer L, Lang AE. Cortical degeneration in progressive supranuclear palsy. A comparison with cortical-basal ganglionic degeneration. J Neuropathol Exp Neurol 1997;56(6):726–734.
Boeve B, Dickson D, Duffy J, Bartleson J, Trenerry M, Petersen R. Progressive nonfluent aphasia and subsequent aphasic dementia associated with atypical progressive supranuclear palsy pathology. Eur Neurol 2003; 49(2):72–78.
Bower JH, Maraganore DM, McDonnell SK, Rocca WA. Incidence and distribution of parkinsonism in Olmsted County, Minnesota, 1976–1990. Neurology 1999;52(6):1214–1220.
Mastaglia FL, Grainger K, Kee F, Sadka M, Lefroy R. Progressive supranuclear palsy (the Steele-Richarson-Olszewski syndrome) clinical and electrophysiological observations in eleven cases. Proc Aust Assoc Neurol 1973;10:35–44.
Davis PH, Golbe LI, Duvoisin RC, Schoenberg BS. Risk factors for progressive supranuclear palsy. Neurology 1988;38(10):1546–1552.
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 2001;124(Pt 7):1438–1449.
Gilman S, Low P, Quinn N, Albanese A, Ben-Shlomo Y, Fowler C, et al. Consensus statement on the diagnosis of multiple system atrophy. American Autonomic Society and American Academy of Neurology. Clin Auton Res 1998; 8(6):359–362.
Litvan I, Hauw JJ, Bartko JJ, Lantos PL, Daniel SE, Horoupian DS, et al. Validity and reliability of the preliminary NINDS neuropathologic criteria for progressive supranuclear palsy and related disorders. J Neuropathol Exp Neurol 1996;55(1):97–105.
Mailliot C, Sergeant N, Bussiere T, Caillet-Boudin ML, Delacourte A, Buee L. Phosphorylation of specific sets of tau isoforms reflects different neurofibrillary degeneration processes. FEBS Lett 1998;433(3): 201–204.
Buee L, Delacourte A. Comparative biochemistry of tau in progressive supranuclear palsy, corticobasal degeneration, FTDP-17 and Pick’s disease. Brain Pathol 1999;9(4):681–693.
Goedert M, Crowther RA, Spillantini MG. Tau mutations cause frontotemporal dementias. Neuron 1998; 21(5):955–958.
Sergeant N, Wattez A, Delacourte A. Neurofibrillary degeneration in progressive supranuclear palsy and corticobasal degeneration: tau pathologies with exclusively “exon 10” isoforms. J Neurochem 1999; 72(3):1243–1249.
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. Ann Neurol 1999;46(3):325–332.
Ruberg M, Javoy-Agid F, Hirsch E, Scatton B, R LH, Hauw JJ, et al. Dopaminergic and cholinergic lesions in progressive supranuclear palsy. Ann Neurol 1985;18(5):523–529.
Agid Y, Javoy-Agid F, Ruberg M, Pillon B, Dubois B, Duyckaerts C, et al. Progressive supranuclear palsy: anatomoclinical and biochemical considerations. Adv Neurol 1987;45:191–206.
Hirsch EC, Graybiel AM, Duyckaerts C, Javoy-Agid F. Neuronal loss in the pedunculopontine tegmental nucleus in Parkinson disease and in progressive supranuclear palsy. Proc Natl Acad Sci U S A 1987;84(16): 5976–5980.
Javoy-Agid F. Cholinergic and peptidergic systems in PSP. J Neural Transm Suppl 1994;42:205–218.
Kasashima S, Oda Y. Cholinergic neuronal loss in the basal forebrain and mesopontine tegmentum of progressive supranuclear palsy and corticobasal degeneration. Acta Neuropathol (Berl) 2003;105(2):117–124.
Frattali CM, Grafman J, Patronas N, Makhlouf F, Litvan I. Language disturbances in corticobasal degeneration. Neurology 2000;54(4):990–992.
Golbe L. Progressive supranuclear palsy in the molecular age. Lancet 2000;356:870–871.
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. Hum Mol Genet 1999;8(4):711–715.
Di Maria E, Tabaton M, Vigo T, Abbruzzese G, Bellone E, Donati C, et al. Corticobasal degeneration shares a common genetic background with progressive supranuclear palsy. Ann Neurol 2000;47(3):374–377.
D’Souza I, Poorkaj P, Hong M, Nochlin D, Lee VM, Bird TD, et al. Missense and silent tau gene mutations cause frontotemporal dementia with parkinsonism-chromosome 17 type, by affecting multiple alternative RNA splicing regulatory elements. Proc Natl Acad Sci USA 1999;96(10):5598–5603.
Iijima M, Tabira T, Poorkaj P, Schellenberg GD, Trojanowski JQ, Lee VM, et al. A distinct familial presenile dementia with a novel missense mutation in the tau gene. Neuroreport 1999;10(3):497–501.
Grover A, Houlden H, Baker M, Adamson J, Lewis J, Prihar G, et al. 5’ splice site mutations in tau associated with the inherited dementia FTDP-17 affect a stem-loop structure that regulates alternative splicing of exon 10. J Biol Chem 1999;274(21):15134–15143.
Bird TD, Nochlin D, Poorkaj P, Cherrier M, Kaye J, Payami H, et al. A clinical pathological comparison of three families with frontotemporal dementia and identical mutations in the tau gene (P301L). Brain 1999; 122(Pt 4):741–756.
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. J Neuropathol Exp Neurol 1999;58(6): 667–677.
van Swieten JC, Stevens M, Rosso SM, Rizzu P, Joosse M, de Koning I, et al. Phenotypic variation in hereditary frontotemporal dementia with tau mutations. Ann Neurol 1999;46(4):617–626.
Rojo A, Pernaute RS, Fontan A, Ruiz PG, Honnorat J, Lynch T, et al. Clinical genetics of familial progressive supranuclear palsy. Brain 1999;122(Pt 7):1233–1245.
Morris HR, Katzenschlager R, Janssen JC, Brown JM, Ozansoy M, Quinn N, et al. Sequence analysis of tau in familial and sporadic progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 2002;72(3):388–390.
Swerdlow RH, Golbe LI, Parks JK, Cassarino DS, Binder DR, Grawey AE, et al. Mitochondrial dysfunction in cybrid lines expressing mitochondrial genes from patients with progressive supranuclear palsy. J Neurochem 2000; 75(4):1681–1684.
Albers DS, Beal MF. Mitochondrial dysfunction in progressive supranuclear palsy. Neurochem Int 2002; 40(6):559–564.
Di Monte DA, Harati Y, Jankovic J, Sandy MS, Jewell SA, Langston JW. Muscle mitochondrial ATP production in progressive supranuclear palsy. J Neurochem 1994;62(4):1631–1634.
Jenner P, Dexter DT, Sian J, Schapira AH, Marsden CD. Oxidative stress as a cause of nigral cell death in Parkinson’s disease and incidental Lewy body disease. The Royal Kings and Queens Parkinson’s Disease Research Group. Ann Neurol 1992;32(Suppl):S82–S87.
Jenner P. Oxidative stress in Parkinson’s disease and other neurodegenerative disorders. Pathol Biol (Paris) 1996;44(1):57–64.
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(2):881–884.
Albers DS, Augood SJ, Park LC, Browne SE, Martin DM, Adamson J, et al. Frontal lobe dysfunction in progressive supranuclear palsy: evidence for oxidative stress and mitochondrial impairment. J Neurochem 2000; 74(2):878–881.
Odetti P, Garibaldi S, Norese R, Angelini G, Marinelli L, Valentini S, et al. Lipoperoxidation is selectively involved in progressive supranuclear palsy. J Neuropathol Exp Neurol 2000;59(5):393–397.
Ferrer I, Blanco R, Carmona M, Puig B. Phosphorylated mitogen-activated protein kinase (MAPK/ERK-P), protein kinase of 38 kDa (p38-P), stress-activated protein kinase (SAPK/JNK-P), and calcium/calmodulin-dependent kinase II (CaM kinase II) are differentially expressed in tau deposits in neurons and glial cells in tauopathies. J Neural Transm 2001;108(12):1397–1415.
Ferrer I, Blanco R, Carmona M, Ribera R, Goutan E, Puig B, 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(2):144–158.
Drache B, Diehl GE, Beyreuther K, Perlmutter LS, Konig G. Bcl-xl-specific antibody labels activated microglia associated with Alzheimer’s disease and other pathological states. J Neurosci Res 1997;47(1): 98–108.
Lippa CF, Flanders KC, Kim ES, Croul S. TGF-beta receptors-I and-II immunoexpression in Alzheimer’s disease: a comparison with aging and progressive supranuclear palsy. Neurobiol Aging 1998;19(6): 527–533.
Lippa CF, Smith TW, Flanders KC. Transforming growth factor-beta: neuronal and glial expression in CNS degenerative diseases. Neurodegeneration 1995;4(4):425–432.
Uchihara T, Mitani K, Mori H, Kondo H, Yamada M, Ikeda K. Abnormal cytoskeletal pathology peculiar to corticobasal degeneration is different from that of Alzheimer’s disease or progressive supranuclear palsy. Acta Neuropathol (Berl) 1994;88(4):379–383.
McGeer PL, Rogers J. Anti-inflammatory agents as a therapeutic approach to Alzheimer’s disease. Neurology 1992;42(2):447–449.
Halliday G, Robinson SR, Shepherd C, Kril J. Alzheimer’s disease and inflammation: a review of cellular and therapeutic mechanisms. Clin Exp Pharmacol Physiol 2000;27(1–2):1–8.
Mackenzie IR. Anti-inflammatory drugs and Alzheimer-type pathology in aging. Neurology 2000;54(3): 732–734.
Arima K, Murayama S, Oyanagi S, Akashi T, Inose T. Presenile dementia with progressive supranuclear palsy tangles and Pick bodies: an unusual degenerative disorder involving the cerebral cortex, cerebral nuclei, and brain stem nuclei. Acta Neuropathol (Berl) 1992;84(2):128–134.
Imran MB, Kawashima R, Awata S, Sato K, Kinomura S, Ono S, et al. Parametric mapping of cerebral blood flow deficits in Alzheimer’s disease: a SPECT study using HMPAO and image standardization technique. J Nucl Med 1999;40(2):244–249.
McGeer PL, Schulzer M, McGeer EG. Arthritis and anti-inflammatory agents as possible protective factors for Alzheimer’s disease: a review of 17 epidemiologic studies. Neurology 1996;47(2):425–432.
Golbe LI, Rubin RS, Cody RP, Belsh JM, Duvoisin RC, Grosmann C, et al. Follow-up study of risk factors in progressive supranuclear palsy. Neurology 1996;47(1):148–154.
Caparros-Lefebvre D, Sergeant N, Lees A, Camuzat A, Daniel S, Lannuzel A, et al. Guadeloupean parkinsonism: a cluster of progressive supranuclear palsy-like tauopathy. Brain 2002;125(Pt 4):801–811.
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(9175):281–286.
Kikuchi T, Tottori K, Uwahodo Y, Hirose T, Miwa T, Oshiro Y, et al. 7-(4-[4-(2,3-Dichlorophenyl)-1-piperazinyl]butyloxy)-3,4-dihydro-2(1H)-qui nolinone (OPC-14597), a new putative antipsychotic drug with both presynaptic dopamine autoreceptor agonistic activity and postsynaptic D2 receptor antagonistic activity. J Pharmacol Exp Ther 1995;274(1):329–336.
Soto-Otero R, Mendez-Alvarez E, Hermida-Ameijeiras A, Munoz-Patino AM, Labandeira-Garcia JL. Autoxidation and neurotoxicity of 6-hydroxydopamine in the presence of some antioxidants: potential implication in relation to the pathogenesis of Parkinson’s disease. J Neurochem 2000;74(4):1605–1612.
Kotake Y, Tasaki Y, Makino Yea. 1-Benzyl-1,2,3,4-Tetrahydroisoquinoline as a parkinsonism-inducing agent: a novel endogenous amine in mouse brain and parkinsonian CSF. J Neurochem 1995;65:2633–2638.
Kawai H, Makino Y, Hirobe M, Ohta S. Novel Endogenous 1,2,3,4-tetrahydroisoquinoline derivative: uptake by dopamine transporter and activity to induce parkinsonism. J Neurochem 1998;70:745–751.
Tasaki Y, Makino Y, Ohta S, Hirobe M. 1-Methyl-1,2,3,4-tetrahydroisoquioline, decreasing in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-treated mouse, prevents parkinsonims-like behavior abnormalities. J Neurochemi 1991;57:1940–1943.
Kotake T, Yoshida M, Ogawa Mea. Chronic Administration of 1-benzyl-1,2,3,4-tetrahydroisoquinoline, an endogenous amine in the brain, induces parkinsonism in a primate. Neuroscience Letters 1996;217:69–71.
Nagatsu T, Yoshida M. An edogenous substance of the brain, tetrahydroisoquinoline, produces parkinsonism in primates with decreased dopamine, tyrosine hydroxylase and biopterin in the nigrostriatal regions. Neurosci Lett. 1988;87:178–182.
Yoshida M, Niwa T, Nagatsu T. Parkinsonism in monkeys produced by chronic administration of an endogenous substance of the brain, tetrahydroisoquinolone: the behavioral and biochemical changes. Neuroscie Lett. 1990; 119:109–113.
Lannuzel A, Michel PP, Abaul MJ, Caparros-Lefebvre D, Ruberg M. Neurotoxic effects of alkaloids from Annona muricata (sour-sop) on dopaminergic neurons: potential role in etiology of atypical parkinsonism in French West Indies. Mov Disord 2000;13(Suppl 3):28 [Abstract].
Friedrich MJ. Pesticide study aids Parkinson research. JAMA 1999;282:2200.
Makino Y, Ohta S, Tachikawa O, Hirobe M. Presence of Tetrahydroisoquinoline and 1-Methyl-Tetrahydro-Isoquinoline in Foods: Compounds Related to Parkinson’s Disease. Life Sciences 1988; 43:373–378.
Niwa T, Takeda N, Sasaoka T, Kaneda T, hashizume Y, Yoshizumi H, Tatematsu A, et al. Detection of tetrahydroisoquinoline in parkinsonian brain as an endogenous amine by use of gas chromatography-mass spectrometry. J Chromatogr 1989;491:397–403.
Spencer PS, Kisby GE, Ross SM, Roy DN, Hugon J, Ludolph AC, et al. Guam ALS-PDC: possible causes. Science 1993;262(5135):825–826.
Zhang ZX, Anderson DW, Mantel N, Roman GC. Motor neuron disease on Guam: geographic and familial occurrence, 1956–85. Acta Neurol Scand 1996;94(1):51–59.
Zhang ZX, Anderson DW, Mantel N. Geographic patterns of parkinsonism-dementia complex on Guam, 1956 through 1985. Arch Neurol 1990;47(10):1069–1074.
McCrank E. PSP risk factors. Neurology 1990;40(10):1637.
McCrank E, Rabheru K. Four cases of progressive supranuclear palsy in patients exposed to organic solvents. Can J Psychiatry 1989;34(9):934–936.
Tetrud J, Langston J, Irwin I, Snow B. Parkinsonism caused by petroleum waste ingestion. Neurology 1994;44: 1051–1054.
Pezzoli G, Ricciardi S, Masotto C, Mariani CB, Carenzi A. n-Hexane induces parkinsonism in rodents. Brain Res 1990;531(1–2):355–357.
Pezzoli G, Barbieri S, Ferrante C, Zecchinelli A, Foa V. Parkinsonism due to n-hexane exposure [letter]. Lancet 1989;2(8667):874.
Geddes JF, Vowles GH, Nicoll JA, Revesz T. Neuronal cytoskeletal changes are an early consequence of repetitive head injury. Acta Neuropathol (Berl) 1999;98:171–178.
Bhatt M, Desai J, Mankodi A, Elias M, Wadia N. Posttraumatic akinetic-rigid syndrome resembling Parkinson’s disease: a report on three patients. Mov Disord 2000;15(2):313–317.
Goetz CG, Pappert EJ. Trauma and movement disorders. Neurol Clin 1992;10(4):907–919.
Gomez-Tortosa E, Newell K, Irizarry MC, Albert M, Growdon JH, Hyman BT. Clinical and quantitative pathologic correlates of dementia with Lewy bodies. Neurology 1999;53(6):1284–1291.
O’Meara ES, Kukull WA, Sheppard L, Bowen JD, McCormick WC, Teri L, et al. Head injury and risk of Alzheimer’s disease by apolipoprotein E genotype. Am J Epidemiol 1997;146:373–384.
Nemetz PN, Leibson C, Naessens JM, Beard M, Kokmen E, Annegers JF, et al. Traumatic brain injury and time to onset of Alzheimer’s disease: a population-based study. Am J Epidemiol 1999;149:32–40.
Guo Z, Cupples LA, Kurz A, Auerbach SH, Volicer L, Chui H, et al. Head injury and the risk of AD in the MIRAGE study. Neurology 2000;54:1316–1323.
Kofler M, Muller J, Wenning GK, Reggiani L, Hollosi P, Bosch S, et al. The auditory startle reaction in parkinsonian disorders. Mov Disord 2001;16(1):62–71.
Mehta KM, Ott A, Kalmijn S, Slooter AJ, vanDuijn CM, Hofman A, et al. Head trauma and risk of dementia and Alzheimer’s disease: the Rotterdam Study. Neurology 1999;53:1959–1962.
Launer LJ, Andersen K, Dewey ME, Letenneur L, Ott A, Amaducci LA, et al. Rates and risk factors for dementia and Alzheimer’s disease: results from EURODEM pooled analyses. EURODEM Incidence Research Group and Work Groups. European Studies of Dementia. Neurology 1999;52:78–84.
Luukinen H, Viramo P, Koski K, Laippala P, Kivela SL. Head injuries and cognitive decline among older adults—a population-based study. Neurology 1999;52:557–562.
Vanacore N, Bonifati V, Fabbrini G, Colosimo C, Marconi R, Nicholl D, et al. Smoking habits in multiple system atrophy and progressive supranuclear palsy. European Study Group on Atypical Parkinsonisms. Neurology 2000; 54(1):114–119.
Fujii C, Harado S, Ohkoshi N, Hayashi A, Yoshizawa K. Cross-cultural traits for personality of patients with Parkinson’s disease in Japan. Am J Med Genet 2000;96:1–3.
Nefzger M, Quadfasel F, Karl V. A retrospective study of smoking in parkinson’s disease. Am J Epidemiol 1968;88:149–158.
Morens D, Grandinetti A, Reed D, White L, Ross G. Cigarette smoking and protection from Parkinson’s disease: false association or etiologic clue? Neurology 1995;45:1041–1051.
Dubinsky RM, Jankovic J. Progressive supranuclear palsy and a multi-infarct state. Neurology 1987; 37(4):570–576.
Winikates J, Jankovic J. Vascular progressive supranuclear palsy. J Neural Transm Suppl 1994;42: 189–201.
Gearing M, Olson DA, Watts RL, Mirra SS. Progressive supranuclear palsy: neuropathologic and clinical heterogeneity. Neurology 1994;44(6):1015–1024.
Fabbrini G, Vanacore N, Bonifati V, Colosimo C, Meco G. Presymptomatic hypertension in progressive supranuclear palsy. Study Group on Atypical Parkinsonisms [letter]. Arch Neurol 1998;55(8):1153–1155.
Ghika J, Bogousslavsky J. Presymptomatic hypertension is a major feature in the diagnosis of progressive supranuclear palsy. Arch Neurol 1997;54(9):1104–1108.
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): 922–930.
Boeve BF, Lang AE, Litvan I. Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol 2003;54(Suppl 5):S15–S19.
Boeve BF, Maraganore DM, Parisi JE, Ivnik RJ, Westmoreland BF, Dickson DW, et al. Corticobasal degeneration and frontotemporal dementia presentations in a kindred with nonspecific histopathology. Dement Geriatr Cogn Disord 2002;13(2):80–90.
Vidailhet M, Rivaud S, Gouider-Khouja N, Pillon B, Bonnet AM, Gaymard B, et al. Eye movements in parkinsonian syndromes. Ann Neurol 1994;35(4):420–426.
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): 1029–1032.
Schrag A, Good CD, Miszkiel K, Morris HR, Mathias CJ, Lees AJ, et al. Differentiation of atypical parkinsonian syndromes with routine MRI. Neurology 2000;54(3):697–702.
Yekhlef F, Ballan G, Macia F, Delmer O, Sourgen C, Tison F. Routine MRI for the differential diagnosis of Parkinson’s disease, MSA, PSP, and CBD. J Neural Transm 2003;110(2):151–169.
Savoiardo M. Differential diagnosis of Parkinson’s disease and atypical parkinsonian disorders by magnetic resonance imaging. Neurol Sci 2003;24(Suppl 1):S35–S37.
Warmuth-Metz M, Naumann M, Csoti I, Solymosi L. Measurement of the midbrain diameter on routine magnetic resonance imaging: a simple and accurate method of differentiating between Parkinson disease and progressive supranuclear palsy. Arch Neurol 2001;58(7):1076–1079.
Pillon B, Dubois B, Agid Y. Testing cognition may contribute to the diagnosis of movement disorders. Neurology 1996;46:329–334.
Johnson R, Jr., Litvan I, Grafman J. Progressive supranuclear palsy: altered sensory processing leads to degraded cognition. Neurology 1991;41(8):1257–1262.
Takeda M, Tachibana H, Okuda B, Kawabata K, Sugita M. Electrophysiological comparison between corticobasal degeneration and progressive supranuclear palsy. Clin Neurol Neurosurg 1998;100(2):94–98.
Okuda B, Tachibana H, Takeda M, Kawabata K, Sugita M. Asymmetric changes in somatosensory evoked potentials correlate with limb apraxia in corticobasal degeneration. Acta Neurol Scand 1998;97(6):409–412.
Valls-Sole J. Neurophysiological characterization of parkinsonian syndromes. Neurophysiol Clin 2000; 30(6):352–367.
Valldeoriola F, Valls-Sole J, Tolosa ES, Marti MJ. Striated anal sphincter denervation in patients with progressive supranuclear palsy. Mov Disord 1995;10(5):550–555.
Litvan I, Blesa R, Clark K, Nichelli P, Atack JR, Mouradian MM, et al. Pharmacological evaluation of the cholinergic system in progressive supranuclear palsy. Ann Neurol 1994;36(1):55–61.
Kompoliti K, Goetz CG, Litvan I, Jellinger K, Verny M. Pharmacological therapy in progressive supranuclear palsy. Arch Neurol 1998;55(8):1099–1102.
Weiner WJ, Minagar A, Shulman LM. Pramipexole in progressive supranuclear palsy. Neurology 1999; 52(4):873–874.
Litvan I, Gomez C, Atack JR, Gillespie M, Kask AM, Mouradian MM, et al. Physostigmine treatment of progressive supranuclear palsy. Ann Neurol 1989;26(3):404–407.
Foster NL, Aldrich MS, Bluemlein L, White RF, Berent S. Failure of cholinergic agonist RS-86 to improve cognition and movement in PSP despite effects on sleep. Neurology 1989;39(2 Pt 1):257–261.
Frattali CM, Sonies BC, Chi-Fishman G, Litvan I. Effects of physostigmine on swallowing and oral motor functions in patients with progressive supranuclear palsy: a pilot study. Dysphagia 1999;14(3):165–168.
Litvan I, Phipps M, Pharr VL, Hallett M, Grafman J, Salazar A. Randomized placebo-controlled trial of donepezil in patients with progressive supranuclear palsy. Neurology 2001;57(3):467–473.
Pascual J, Berciano J, Gonzalez AM, Grijalba B, Figols J, Pazos A. Autoradiographic demonstration of loss of alpha 2-adrenoceptors in progressive supranuclear palsy: preliminary report. J Neurol Sci 1993;114(2): 165–169.
Ghika J, Tennis M, Hoffman E, Schoenfeld D, Growdon J. Idazoxan treatment in progressive supranuclear palsy. Neurology 1991;41(7):986–991.
Rascol O, Sieradzan K, Peyro-Saint-Paul H, Thalamas C, Brefel-Courbon C, Senard JM, et al. Efaroxan, an alpha-2 antagonist, in the treatment of progressive supranuclear palsy. Mov Disord 1998;13(4):673–676.
Litvan I, Dickson DW, Buttner-Ennever JA, Delacourte A, Hutton M, Dubois B, et al. Research goals in progressive supranuclear palsy. First International Brainstorming Conference on PSP. Mov Disord 2000;15(3): 446–458.
Steele JC. Introduction. In: Litvan I, Agid A, eds. Progressive Supranuclear Palsy: Clinical and Research Aspects. New York: Oxford University Press, 1992:3–14.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2005 Humana Press Inc., Totowa, NJ
About this chapter
Cite this chapter
Litvan, I. (2005). Progressive Supranuclear Palsy. In: Litvan, I. (eds) Atypical Parkinsonian Disorders. Current Clinical Neurology. Humana Press. https://doi.org/10.1385/1-59259-834-X:287
Download citation
DOI: https://doi.org/10.1385/1-59259-834-X:287
Publisher Name: Humana Press
Print ISBN: 978-1-58829-331-2
Online ISBN: 978-1-59259-834-2
eBook Packages: MedicineMedicine (R0)