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Progressive supranuclear palsy (PSP)

Authors
Stewart A Factor, DO
Christine Doss Esper, MD
Section Editor
Howard I Hurtig, MD
Deputy Editor
John F Dashe, MD, PhD

INTRODUCTION

Symptoms and signs of parkinsonism (ie, tremor, bradykinesia, rigidity, and postural instability) can be prominent in neurodegenerative disorders other than idiopathic Parkinson disease, particularly in atypical parkinsonian disorders, which include corticobasal degeneration, multiple system atrophy, and progressive supranuclear palsy.

Progressive supranuclear palsy (PSP), also known as Steele Richardson Olszewski syndrome, is an uncommon but not rare parkinsonian syndrome. Characteristic features of PSP include vertical supranuclear gaze palsy and postural instability with unexplained falls. This topic will review specifically the clinical aspects of PSP. Other neurodegenerative parkinsonian syndromes are discussed separately. (See "Clinical manifestations of Parkinson disease" and "Corticobasal degeneration" and "Multiple system atrophy: Clinical features and diagnosis" and "Diagnosis and differential diagnosis of Parkinson disease", section on 'Differential diagnosis'.)

HISTORICAL BACKGROUND

In 1964, Steele, Richardson, and Olszewski were the first to describe PSP when their seminal report of nine cases with autopsy confirmation was published [1]. As a result of their pioneering work, some have referred to the disease as the Steele-Richardson-Olszewski syndrome. Since that time, hundreds of additional cases have been recorded, and the disease is now a well-recognized atypical parkinsonian syndrome (or Parkinson-plus disorder). As originally described, PSP is characterized by progressive supranuclear ophthalmoplegia, gait disorder and postural instability, dysarthria, dysphagia, rigidity, and frontal cognitive disturbance. The authors of the original report speculated whether PSP was a primary disorder of the central nervous system or a postinfectious (viral) syndrome [2]. The consistent pathologic features of PSP consist of neuronal loss, globose neurofibrillary tangles, and gliosis mainly in the basal ganglia, cerebellum, brainstem, and to a lesser extent, the cerebral cortex [3]. A universally accepted set of diagnostic criteria for PSP was proposed in 1996 [4]. (See 'Diagnostic criteria' below.)

PSP is now recognized to encompass a number of phenotypic variants. The two most common are Richardson syndrome (the classic form of PSP) and PSP-parkinsonism.

EPIDEMIOLOGY

PSP is the most common of the degenerative forms of atypical parkinsonism [5,6]. An early study found that the population prevalence of PSP was 1.39 per 100,000 in the United States [7]. Although this study has been criticized because of methodologic flaws, it established that PSP was not as rare as had previously been believed. Later reports from other countries demonstrated an even higher prevalence, 6.4 per 100,000 in the United Kingdom [6,8] and 5.8 per 100,000 in western Japan [9].

                      

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Literature review current through: Nov 2016. | This topic last updated: Thu Jun 30 00:00:00 GMT+00:00 2016.
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References
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  1. 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.
  2. Kristensen MO. Progressive supranuclear palsy--20 years later. Acta Neurol Scand 1985; 71:177.
  3. Golbe LI. Progressive Supranuclear Palsy. Curr Treat Options Neurol 2001; 3:473.
  4. 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.
  5. 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.
  6. Schrag A, Ben-Shlomo Y, Quinn NP. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet 1999; 354:1771.
  7. Golbe LI, Davis PH, Schoenberg BS, Duvoisin RC. Prevalence and natural history of progressive supranuclear palsy. Neurology 1988; 38:1031.
  8. 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.
  9. Kawashima M, Miyake M, Kusumi M, et al. Prevalence of progressive supranuclear palsy in Yonago, Japan. Mov Disord 2004; 19:1239.
  10. Mastaglia FL, Grainger K, Kee F, et al. Progressive supranuclear palsy (the Steele-Richardson-Olszewski syndrome) clinical and electrophysiological observations in eleven cases. Proc Aust Assoc Neurol 1973; 10:35.
  11. Radhakrishnan K, Thacker AK, Maloo JC, et al. Descriptive epidemiology of some rare neurological diseases in Benghazi, Libya. Neuroepidemiology 1988; 7:159.
  12. Rajput AH, Offord KP, Beard CM, Kurland LT. Epidemiology of parkinsonism: incidence, classification, and mortality. Ann Neurol 1984; 16:278.
  13. Bower JH, Maraganore DM, McDonnell SK, Rocca WA. Incidence and distribution of parkinsonism in Olmsted County, Minnesota, 1976-1990. Neurology 1999; 52:1214.
  14. Wenning GK, Litvan I, Tolosa E. Milestones in atypical and secondary Parkinsonisms. Mov Disord 2011; 26:1083.
  15. Maher ER, Lees AJ. The clinical features and natural history of the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 1986; 36:1005.
  16. Collins SJ, Ahlskog JE, Parisi JE, Maraganore DM. Progressive supranuclear palsy: neuropathologically based diagnostic clinical criteria. J Neurol Neurosurg Psychiatry 1995; 58:167.
  17. De Bruin VM, Lees AJ. Subcortical neurofibrillary degeneration presenting as Steele-Richardson-Olszewski and other related syndromes: a review of 90 pathologically verified cases. Mov Disord 1994; 9:381.
  18. Litvan I, Agid Y, Jankovic J, et al. Accuracy of clinical criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Neurology 1996; 46:922.
  19. Santacruz P, Uttl B, Litvan I, Grafman J. Progressive supranuclear palsy: a survey of the disease course. Neurology 1998; 50:1637.
  20. 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.
  21. Frasca J, Blumbergs PC, Henschke P, Burns RJ. A clinical and pathological study of progressive supranuclear palsy. Clin Exp Neurol 1991; 28:79.
  22. Baba Y, Putzke JD, Whaley NR, et al. Progressive supranuclear palsy: phenotypic sex differences in a clinical cohort. Mov Disord 2006; 21:689.
  23. Davis PH, Golbe LI, Duvoisin RC, Schoenberg BS. Risk factors for progressive supranuclear palsy. Neurology 1988; 38:1546.
  24. Golbe LI, Rubin RS, Cody RP, et al. Follow-up study of risk factors in progressive supranuclear palsy. Neurology 1996; 47:148.
  25. Caparros-Lefebvre D, Golbe LI, Deramecourt V, et al. A geographical cluster of progressive supranuclear palsy in northern France. Neurology 2015; 85:1293.
  26. McCrank E. PSP risk factors. Neurology 1990; 40:1637.
  27. McCrank E, Rabheru K. Four cases of progressive supranuclear palsy in patients exposed to organic solvents. Can J Psychiatry 1989; 34:934.
  28. Litvan I, Lees PS, Cunningham CR, et al. Environmental and occupational risk factors for progressive supranuclear palsy: Case-control study. Mov Disord 2016; 31:644.
  29. 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.
  30. Rojo A, Pernaute RS, Fontán A, et al. Clinical genetics of familial progressive supranuclear palsy. Brain 1999; 122 ( Pt 7):1233.
  31. Donker Kaat L, Boon AJ, Azmani A, et al. Familial aggregation of parkinsonism in progressive supranuclear palsy. Neurology 2009; 73:98.
  32. Höglinger GU, Melhem NM, Dickson DW, et al. Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy. Nat Genet 2011; 43:699.
  33. Pittman AM, Myers AJ, Duckworth J, et al. The structure of the tau haplotype in controls and in progressive supranuclear palsy. Hum Mol Genet 2004; 13:1267.
  34. 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.
  35. 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.
  36. Zabetian CP, Hutter CM, Factor SA, et al. Association analysis of MAPT H1 haplotype and subhaplotypes in Parkinson's disease. Ann Neurol 2007; 62:137.
  37. Baker KB, Montgomery EB Jr. Performance on the PD test battery by relatives of patients with progressive supranuclear palsy. Neurology 2001; 56:25.
  38. Boeve BF. Progressive supranuclear palsy. Parkinsonism Relat Disord 2012; 18 Suppl 1:S192.
  39. Litvan I. Update on progressive supranuclear palsy. Curr Neurol Neurosci Rep 2004; 4:296.
  40. Litvan I, Mangone CA, McKee A, 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; 60:615.
  41. Troost BT, Daroff RB. The ocular motor defects in progressive supranuclear palsy. Ann Neurol 1977; 2:397.
  42. Broderick M, Riley DE. Parkinson's-plus disorders. In: Parkinson's disease: Diagnosis and clinical management, 2nd ed, Factor SA, Weiner WJ (Eds), Demos Medical Publishing, New York 2008. p.727.
  43. Barclay CL, Lang AE. Dystonia in progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 1997; 62:352.
  44. Rafal RD, Friedman JH. Limb dystonia in progressive supranuclear palsy. Neurology 1987; 37:1546.
  45. Dubois B, Pillon B, Legault F, et al. Slowing of cognitive processing in progressive supranuclear palsy. A comparison with Parkinson's disease. Arch Neurol 1988; 45:1194.
  46. 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; 36:1179.
  47. Golbe LI, Boeve BF, Keegan BM, Parisi JE. An 81-year-old man with imbalance and memory impairment. Neurology 2007; 68:1147.
  48. Brown RG, Lacomblez L, Landwehrmeyer BG, et al. Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. Brain 2010; 133:2382.
  49. Pharr V, Uttl B, Stark M, et al. Comparison of apraxia in corticobasal degeneration and progressive supranuclear palsy. Neurology 2001; 56:957.
  50. Litvan I, Mega MS, Cummings JL, Fairbanks L. Neuropsychiatric aspects of progressive supranuclear palsy. Neurology 1996; 47:1184.
  51. Schrag A, Sheikh S, Quinn NP, et al. A comparison of depression, anxiety, and health status in patients with progressive supranuclear palsy and multiple system atrophy. Mov Disord 2010; 25:1077.
  52. Fukui T, Lee E, Hosoda H, Okita K. Obsessive-compulsive behavior as a symptom of dementia in progressive supranuclear palsy. Dement Geriatr Cogn Disord 2010; 30:179.
  53. Aldrich MS, Foster NL, White RF, et al. Sleep abnormalities in progressive supranuclear palsy. Ann Neurol 1989; 25:577.
  54. Boeve BF, Silber MH, Parisi JE, et al. Synucleinopathy pathology and REM sleep behavior disorder plus dementia or parkinsonism. Neurology 2003; 61:40.
  55. Respondek G, Stamelou M, Kurz C, et al. The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases. Mov Disord 2014; 29:1758.
  56. 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.
  57. Schofield EC, Hodges JR, Macdonald V, et al. Cortical atrophy differentiates Richardson's syndrome from the parkinsonian form of progressive supranuclear palsy. Mov Disord 2011; 26:256.
  58. Mizusawa H, Mochizuki A, Ohkoshi N, et al. Progressive supranuclear palsy presenting with pure akinesia. Adv Neurol 1993; 60:618.
  59. Williams DR, Holton JL, Strand K, et al. Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy. Mov Disord 2007; 22:2235.
  60. Riley DE, Fogt N, Leigh RJ. The syndrome of 'pure akinesia' and its relationship to progressive supranuclear palsy. Neurology 1994; 44:1025.
  61. Matsuo H, Takashima H, Kishikawa M, et al. Pure akinesia: an atypical manifestation of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 1991; 54:397.
  62. Facheris MF, Maniak S, Scaravilli F, et al. Pure akinesia as initial presentation of PSP: a clinicopathological study. Parkinsonism Relat Disord 2008; 14:517.
  63. Factor SA, Jennings DL, Molho ES, Marek KL. The natural history of the syndrome of primary progressive freezing gait. Arch Neurol 2002; 59:1778.
  64. Compta Y, Valldeoriola F, Tolosa E, et al. Long lasting pure freezing of gait preceding progressive supranuclear palsy: a clinicopathological study. Mov Disord 2007; 22:1954.
  65. Factor SA, Higgins DS, Qian J. Primary progressive freezing gait: a syndrome with many causes. Neurology 2006; 66:411.
  66. Cordato NJ, Halliday GM, McCann H, et al. Corticobasal syndrome with tau pathology. Mov Disord 2001; 16:656.
  67. Boeve B, Dickson D, Duffy J, et al. Progressive nonfluent aphasia and subsequent aphasic dementia associated with atypical progressive supranuclear palsy pathology. Eur Neurol 2003; 49:72.
  68. 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.
  69. Rohrer JD, Paviour D, Bronstein AM, et al. Progressive supranuclear palsy syndrome presenting as progressive nonfluent aphasia: a neuropsychological and neuroimaging analysis. Mov Disord 2010; 25:179.
  70. Santos-Santos MA, Mandelli ML, Binney RJ, et al. Features of Patients With Nonfluent/Agrammatic Primary Progressive Aphasia With Underlying Progressive Supranuclear Palsy Pathology or Corticobasal Degeneration. JAMA Neurol 2016; 73:733.
  71. Ahmed Z, Josephs KA, Gonzalez J, et al. Clinical and neuropathologic features of progressive supranuclear palsy with severe pallido-nigro-luysial degeneration and axonal dystrophy. Brain 2008; 131:460.
  72. Kanazawa M, Shimohata T, Toyoshima Y, et al. Cerebellar involvement in progressive supranuclear palsy: A clinicopathological study. Mov Disord 2009; 24:1312.
  73. Iwasaki Y, Mori K, Ito M, et al. An autopsied case of progressive supranuclear palsy presenting with cerebellar ataxia and severe cerebellar involvement. Neuropathology 2013; 33:561.
  74. Kanazawa M, Tada M, Onodera O, et al. Early clinical features of patients with progressive supranuclear palsy with predominant cerebellar ataxia. Parkinsonism Relat Disord 2013; 19:1149.
  75. Koga S, Josephs KA, Ogaki K, et al. Cerebellar ataxia in progressive supranuclear palsy: An autopsy study of PSP-C. Mov Disord 2016; 31:653.
  76. Shimohata T, Kanazawa M, Yoshida M, et al. Clinical and imaging findings of progressive supranuclear palsy with predominant cerebellar ataxia. Mov Disord 2016; 31:760.
  77. Graber JJ, Staudinger R. Teaching NeuroImages: "Penguin" or "hummingbird" sign and midbrain atrophy in progressive supranuclear palsy. Neurology 2009; 72:e81.
  78. Kato N, Arai K, Hattori T. Study of the rostral midbrain atrophy in progressive supranuclear palsy. J Neurol Sci 2003; 210:57.
  79. Shukla R, Sinha M, Kumar R, Singh D. 'Hummingbird' sign in progressive supranuclear palsy. Ann Indian Acad Neurol 2009; 12:133.
  80. Tsuboi Y, Slowinski J, Josephs KA, et al. Atrophy of superior cerebellar peduncle in progressive supranuclear palsy. Neurology 2003; 60:1766.
  81. Hauw JJ, Daniel SE, Dickson D, et al. Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 1994; 44:2015.
  82. Scaravilli T, Pramstaller PP, Salerno A, et al. Neuronal loss in Onuf's nucleus in three patients with progressive supranuclear palsy. Ann Neurol 2000; 48:97.
  83. Takahashi M, Weidenheim KM, Dickson DW, Ksiezak-Reding H. Morphological and biochemical correlations of abnormal tau filaments in progressive supranuclear palsy. J Neuropathol Exp Neurol 2002; 61:33.
  84. Feany MB, Dickson DW. Neurodegenerative disorders with extensive tau pathology: a comparative study and review. Ann Neurol 1996; 40:139.
  85. Gearing M, Olson DA, Watts RL, Mirra SS. Progressive supranuclear palsy: neuropathologic and clinical heterogeneity. Neurology 1994; 44:1015.
  86. Scaravilli T, Tolosa E, Ferrer I. Progressive supranuclear palsy and corticobasal degeneration: lumping versus splitting. Mov Disord 2005; 20 Suppl 12:S21.
  87. Houlden H, Baker M, Morris HR, et al. Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype. Neurology 2001; 56:1702.
  88. Hutton M, Lendon CL, Rizzu P, et al. Association of missense and 5'-splice-site mutations in tau with the inherited dementia FTDP-17. Nature 1998; 393:702.
  89. Higgins JJ, Golbe LI, De Biase A, et al. An extended 5'-tau susceptibility haplotype in progressive supranuclear palsy. Neurology 2000; 55:1364.
  90. 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.
  91. Warren NM, Piggott MA, Lees AJ, Burn DJ. The basal ganglia cholinergic neurochemistry of progressive supranuclear palsy and other neurodegenerative diseases. J Neurol Neurosurg Psychiatry 2007; 78:571.
  92. Pascual J, Berciano J, Grijalba B, et al. Dopamine D1 and D2 receptors in progressive supranuclear palsy: an autoradiographic study. Ann Neurol 1992; 32:703.
  93. Pierot L, Desnos C, Blin J, et al. D1 and D2-type dopamine receptors in patients with Parkinson's disease and progressive supranuclear palsy. J Neurol Sci 1988; 86:291.
  94. Juncos JL, Hirsch EC, Malessa S, et al. Mesencephalic cholinergic nuclei in progressive supranuclear palsy. Neurology 1991; 41:25.
  95. Zweig RM, Whitehouse PJ, Casanova MF, et al. Loss of pedunculopontine neurons in progressive supranuclear palsy. Ann Neurol 1987; 22:18.
  96. Suzuki M, Desmond TJ, Albin RL, Frey KA. Cholinergic vesicular transporters in progressive supranuclear palsy. Neurology 2002; 58:1013.
  97. Holemans S, Javoy F, Agid Y, et al. [3H]MK-801 binding to NMDA glutamatergic receptors in Parkinson's disease and progressive supranuclear palsy. Brain Res 1991; 565:154.
  98. 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.
  99. Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol 2009; 8:270.
  100. Mendell JR, Engel WK, Chase TN. Modification by L-dopa of a case of progressive supranuclear palsy. With evidence of defective cerebral dopamine metabolism. Lancet 1970; 1:593.
  101. 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.
  102. Borroni B, Malinverno M, Gardoni F, et al. Tau forms in CSF as a reliable biomarker for progressive supranuclear palsy. Neurology 2008; 71:1796.
  103. 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; 58:1076.
  104. Oba H, Yagishita A, Terada H, et al. New and reliable MRI diagnosis for progressive supranuclear palsy. Neurology 2005; 64:2050.
  105. Looi JC, Macfarlane MD, Walterfang M, et al. Morphometric analysis of subcortical structures in progressive supranuclear palsy: In vivo evidence of neostriatal and mesencephalic atrophy. Psychiatry Res 2011; 194:163.
  106. Quattrone A, Nicoletti G, Messina D, et al. MR imaging index for differentiation of progressive supranuclear palsy from Parkinson disease and the Parkinson variant of multiple system atrophy. Radiology 2008; 246:214.
  107. Morelli M, Arabia G, Salsone M, et al. Accuracy of magnetic resonance parkinsonism index for differentiation of progressive supranuclear palsy from probable or possible Parkinson disease. Mov Disord 2011; 26:527.
  108. Morelli M, Arabia G, Novellino F, et al. MRI measurements predict PSP in unclassifiable parkinsonisms: a cohort study. Neurology 2011; 77:1042.
  109. Massey LA, Jäger HR, Paviour DC, et al. The midbrain to pons ratio: a simple and specific MRI sign of progressive supranuclear palsy. Neurology 2013; 80:1856.
  110. Karimi M, Perlmutter JS. MRI measures predict progressive supranuclear palsy: clinically useful? Neurology 2011; 77:1028.
  111. Mishina M, Ishii K, Mitani K, et al. Midbrain hypometabolism as early diagnostic sign for progressive supranuclear palsy. Acta Neurol Scand 2004; 110:128.
  112. Blin J, Baron JC, Dubois B, et al. Positron emission tomography study in progressive supranuclear palsy. Brain hypometabolic pattern and clinicometabolic correlations. Arch Neurol 1990; 47:747.
  113. Foster NL, Gilman S, Berent S, et al. Cerebral hypometabolism in progressive supranuclear palsy studied with positron emission tomography. Ann Neurol 1988; 24:399.
  114. Goffinet AM, De Volder AG, Gillain C, et al. Positron tomography demonstrates frontal lobe hypometabolism in progressive supranuclear palsy. Ann Neurol 1989; 25:131.
  115. Brooks DJ, Ibanez V, Sawle GV, et al. Striatal D2 receptor status in patients with Parkinson's disease, striatonigral degeneration, and progressive supranuclear palsy, measured with 11C-raclopride and positron emission tomography. Ann Neurol 1992; 31:184.
  116. Valls-Solé J. Neurophysiological characterization of parkinsonian syndromes. Neurophysiol Clin 2000; 30:352.
  117. Rivaud-Péchoux S, Vidailhet M, Gallouedec G, et al. Longitudinal ocular motor study in corticobasal degeneration and progressive supranuclear palsy. Neurology 2000; 54:1029.
  118. Murphy MA, Friedman JH, Tetrud JW, Factor SA. Neurodegenerative disorders mimicking progressive supranuclear palsy: a report of three cases. J Clin Neurosci 2005; 12:941.
  119. Dubinsky RM, Jankovic J. Progressive supranuclear palsy and a multi-infarct state. Neurology 1987; 37:570.
  120. Weiner WJ. A differential diagnosis of Parkinsonism. Rev Neurol Dis 2005; 2:124.
  121. David NJ, Mackey EA, Smith JL. Further observations in progressive supranuclear palsy. Neurology 1968; 18:349.
  122. Testa D, Monza D, Ferrarini M, et al. Comparison of natural histories of progressive supranuclear palsy and multiple system atrophy. Neurol Sci 2001; 22:247.
  123. Golbe LI, Ohman-Strickland PA. A clinical rating scale for progressive supranuclear palsy. Brain 2007; 130:1552.
  124. O'Sullivan SS, Massey LA, Williams DR, et al. Clinical outcomes of progressive supranuclear palsy and multiple system atrophy. Brain 2008; 131:1362.
  125. Winter Y, Spottke AE, Stamelou M, et al. Health-related quality of life in multiple system atrophy and progressive supranuclear palsy. Neurodegener Dis 2011; 8:438.
  126. Burn DJ, Warren NM. Toward future therapies in progressive supranuclear palsy. Mov Disord 2005; 20 Suppl 12:S92.
  127. Krack P, Marion MH. "Apraxia of lid opening," a focal eyelid dystonia: clinical study of 32 patients. Mov Disord 1994; 9:610.
  128. Sosner J, Wall GC, Sznajder J. Progressive supranuclear palsy: clinical presentation and rehabilitation of two patients. Arch Phys Med Rehabil 1993; 74:537.
  129. Kompoliti K, Goetz CG, Litvan I, et al. Pharmacological therapy in progressive supranuclear palsy. Arch Neurol 1998; 55:1099.
  130. Birdi S, Rajput AH, Fenton M, et al. Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases. Mov Disord 2002; 17:1255.
  131. Rajput A, Rajput AH. Progressive supranuclear palsy: clinical features, pathophysiology and management. Drugs Aging 2001; 18:913.
  132. Tan EK, Chan LL, Wong MC. Levodopa-induced oromandibular dystonia in progressive supranuclear palsy. Clin Neurol Neurosurg 2003; 105:132.
  133. Defazio G, De Mari M, De Salvia R, et al. "Apraxia of eyelid opening" induced by levodopa therapy and apomorphine in atypical parkinsonism (possible progressive supranuclear palsy): a case report. Clin Neuropharmacol 1999; 22:292.
  134. Lang AE. Treatment of progressive supranuclear palsy and corticobasal degeneration. Mov Disord 2005; 20 Suppl 12:S83.
  135. Rajrut AH, Uitti RJ, Fenton ME, George D. Amantadine effectiveness in multiple system atrophy and progressive supranuclear palsy. Parkinsonism Relat Disord 1997; 3:211.
  136. Jackson JA, Jankovic J, Ford J. Progressive supranuclear palsy: clinical features and response to treatment in 16 patients. Ann Neurol 1983; 13:273.
  137. Nieforth KA, Golbe LI. Retrospective study of drug response in 87 patients with progressive supranuclear palsy. Clin Neuropharmacol 1993; 16:338.
  138. Daniele A, Moro E, Bentivoglio AR. Zolpidem in progressive supranuclear palsy. N Engl J Med 1999; 341:543.
  139. Ghika J, Tennis M, Hoffman E, et al. Idazoxan treatment in progressive supranuclear palsy. Neurology 1991; 41:986.
  140. Engel PA. Treatment of progressive supranuclear palsy with amitriptyline: therapeutic and toxic effects. J Am Geriatr Soc 1996; 44:1072.
  141. Newman GC. Treatment of progressive supranuclear palsy with tricyclic antidepressants. Neurology 1985; 35:1189.
  142. Duncombe AS, Lees AJ. Methysergide in progressive supranuclear palsy. Neurology 1985; 35:936.
  143. Barclay CL, Duff J, Sandor P, Lang AE. Limited usefulness of electroconvulsive therapy in progressive supranuclear palsy. Neurology 1996; 46:1284.
  144. Hauser RA, Trehan R. Initial experience with electroconvulsive therapy for progressive supranuclear palsy. Mov Disord 1994; 9:467.
  145. Okun MS, Tagliati M, Pourfar M, et al. Management of referred deep brain stimulation failures: a retrospective analysis from 2 movement disorders centers. Arch Neurol 2005; 62:1250.
  146. Brusa L, Iani C, Ceravolo R, et al. Implantation of the nucleus tegmenti pedunculopontini in a PSP-P patient: safe procedure, modest benefits. Mov Disord 2009; 24:2020.
  147. Domínguez JM, Fuertes A, Orozco L, et al. Evidence for irreversible inhibition of glycogen synthase kinase-3β by tideglusib. J Biol Chem 2012; 287:893.
  148. Gold M, Lorenzl S, Stewart AJ, et al. Critical appraisal of the role of davunetide in the treatment of progressive supranuclear palsy. Neuropsychiatr Dis Treat 2012; 8:85.
  149. Tolosa E, Litvan I, Höglinger GU, et al. A phase 2 trial of the GSK-3 inhibitor tideglusib in progressive supranuclear palsy. Mov Disord 2014; 29:470.
  150. Boxer AL, Lang AE, Grossman M, et al. Davunetide in patients with progressive supranuclear palsy: a randomised, double-blind, placebo-controlled phase 2/3 trial. Lancet Neurol 2014; 13:676.
  151. Poewe W, Mahlknecht P, Krismer F. Therapeutic advances in multiple system atrophy and progressive supranuclear palsy. Mov Disord 2015; 30:1528.