
Spectrum of Dystonia in Progressive Supranuclear Palsy: A Prospective Observational Study and Review of Literature
By: Kartika Gulati and Sanjay Pandey
References
- 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. Archives of neurology. 1964 Apr 1;10(4):333–359. DOI: 10.1001/archneur.1964.00460160003001
- Kukkle PL, Neupane R, Pantelyat A, Wills AM, Jabbari E, Dopper EG, Kovacs GG, Hoglinger G, Aiba I, Litvan I, Ganguly J. Progressive supranuclear palsy—a global review. Movement Disorders Clinical Practice. 2026 Mar;13(3):611–622. DOI: 10.1002/mdc3.70338
- Erkkinen MG, Kim MO, Geschwind MD. Clinical neurology and epidemiology of the major neurodegenerative diseases. Cold Spring Harbor perspectives in biology. 2018 Apr 1;10(4):
a033118 . DOI: 10.1101/cshperspect.a033118 - Currens L, Pantelyat A. Progressive supranuclear palsy diagnosis and treatment. Current Treatment Options in Neurology. 2024 Apr;26(4):97–114. DOI: 10.1007/s11940-024-00784-9
- Giagkou N, Höglinger GU, Stamelou M. Progressive supranuclear palsy. International review of neurobiology. 2019 Jan 1;149:49–86. DOI: 10.1016/bs.irn.2019.10.013
- Höglinger GU, Respondek G, Stamelou M, Kurz C, Josephs KA, Lang AE, Mollenhauer B, Müller U, Nilsson C, Whitwell JL, Arzberger T. Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria. Movement disorders. 2017 Jun;32(6):853–864. DOI: 10.1002/mds.26987
- Grimm MJ, Respondek G, Stamelou M, Arzberger T, Ferguson L, Gelpi E, Giese A, Grossman M, Irwin DJ, Pantelyat A, Rajput A. How to apply the movement disorder society criteria for diagnosis of progressive supranuclear palsy. Movement disorders. 2019 Aug;34(8):1228–1232. DOI: 10.1002/mds.27666
- Ali F, Martin PR, Botha H, Ahlskog JE, Bower JH, Masumoto JY, Maraganore D, Hassan A, Eggers S, Boeve BF, Knopman DS. Sensitivity and specificity of diagnostic criteria for progressive supranuclear palsy. Movement Disorders. 2019 Aug;34(8):1144–1153. DOI: 10.1002/mds.27619
- Mahale RR, Krishnan S, Divya KP, Jisha VT, Kishore A. Subtypes of PSP and prognosis: a retrospective analysis. Annals of Indian Academy of Neurology. 2021 Jan 1;24(1):56–62. DOI: 10.4103/aian.AIAN_611_20
- Pillai KS, Rajeswari P, Kamble RB, Gopalan Nair Santhamma S, Chacko M, Jayakrishnan V, Ramachandran R, Avarachan A, Kishore A. Multimodality Brain Imaging Markers in Progressive Supranuclear Palsy Subtypes and Parkinson’s Disease. Movement Disorders Clinical Practice. 2025 May;12(5):664–669. DOI: 10.1002/mdc3.14346
- Rajput A, Rajput AH. Progressive supranuclear palsy: clinical features, pathophysiology and management. Drugs & aging. 2001 Dec;18(12):913–925. DOI: 10.2165/00002512-200118120-00003
- Kaasinen V, Kangassalo N, Gardberg M, Isotalo J, Karhu J, Parkkola R, Sonninen P. Midbrain-to-pons ratio in autopsy-confirmed progressive supranuclear palsy: replication in an independent cohort. Neurological Sciences. 2015 Jul;36(7):1251–1253. DOI: 10.1007/s10072-015-2184-3
- Massey LA, Jäger HR, Paviour DC, O’Sullivan SS, Ling H, Williams DR, Kallis C, Holton J, Revesz T, Burn DJ, Yousry T. The midbrain to pons ratio: a simple and specific MRI sign of progressive supranuclear palsy. Neurology. 2013 May 14;80(20):1856–1861. DOI: 10.1212/WNL.0b013e318292a2d2
- Rafal RD, Friedman JH. Limb dystonia in progressive supranuclear palsy. Neurology. 1987 Sep;37(9):1546–1549. DOI: 10.1212/WNL.37.9.1546
- Wagle-Shukla A, Rajan A, Jabarkheel Z, McFarland N. Prevalence estimates and clinical characterization for tremor and dystonia in Progressive Supranuclear Palsy (P3. 8–005). Neurology. 2019 Apr 9;92(15_supplement):P3–8. DOI: 10.1212/WNL.92.15_supplement.P3.8-005
- Barclay CL, Lang AE. Dystonia in progressive supranuclear palsy. Journal of Neurology, Neurosurgery & Psychiatry. 1997 Apr 1;62(4):352–356. DOI: 10.1136/jnnp.62.4.352
- Godeiro-Junior C, Felício AC, Barsottini OG, Aguiar PM, Silva S, Borges V, Ferraz HB. Clinical features of dystonia in atypical parkinsonism. Arquivos de neuro-psiquiatria. 2008;66:800–804. DOI: 10.1590/S0004-282X2008000600004
- Marsili L, Bologna M, Kojovic M, Berardelli A, Espay AJ, Colosimo C. Dystonia in atypical parkinsonian disorders. Parkinsonism & Related Disorders. 2019 Sep 1;66:25–33. DOI: 10.1016/j.parkreldis.2019.07.030
- Rivest J, Quinn N, Marsden CD. Dystonia in Parkinson’s disease, multiple system atrophy, and progressive supranuclear palsy. Neurology. 1990 Oct;40(10):1571–1578. DOI: 10.1212/WNL.40.10.1571
- Guasp M, Molina-Porcel L, Painous C, Caballol N, Camara A, Perez-Soriano A, Sánchez-Gómez A, Garrido A, Muñoz E, Marti MJ, Valldeoriola F. Association of PSP phenotypes with survival: a brain-bank study. Parkinsonism & related disorders. 2021 Mar 1;84:77–81. DOI: 10.1016/j.parkreldis.2021.01.015
- Chatterjee K, Choudhury S, Kumar H. Relevance of subtype classification of PSP. Annals of Indian Academy of Neurology. 2021 Jan 1;24(1):7. DOI: 10.4103/aian.AIAN_1039_20
- Constantinides VC, Paraskevas GP, Paraskevas PG, Stefanis L, Kapaki E. Corticobasal degeneration and corticobasal syndrome: A review. Clinical Parkinsonism & Related Disorders. 2019 Jan 1;1:66–71. DOI: 10.1016/j.prdoa.2019.08.005
- Wilson D, Le Heron C, Anderson T. Corticobasal syndrome: a practical guide. Practical Neurology. 2021 Aug 1;21(4):276–285. DOI: 10.1007/s11910-021-01109-y
- Chung EJ, Kim SJ. Levodopa-induced facial dystonia in a case of progressive supranuclear palsy. J Mov Disord. 2012 May;5(1):28–32. DOI: 10.1002/mds.25037
- Crespi J, Bråthen G, Quist-Paulsen P, Pagonabarraga J, Roig-Arnall C. Facial Dystonia with Facial Grimacing and Vertical Gaze Palsy with “Round the Houses” Sign in a 29-Year-Old Woman. Neuroophthalmology. 2016 Jan 19;40(1):31–34. DOI: 10.1002/mds.26350
- Giza E, Katsarou Z, Dagklis I, Bostantjopoulou S. Lingual dystonia as a new clinical feature in corticobasal syndrome. J Neurol Sci. 2015;353(1–2):187–188. DOI: 10.1378/chest.14-0970
- Ishii N, Mochizuki H. Alternating flexed-extended posturing in progressive supranuclear palsy. Neurol Int. 2019 Sep 18;11(3):8257. DOI: 10.1016/j.parkreldis.2011.03.007
- Janati A. Progressive supranuclear palsy: report of a case with torticollis, blepharospasm, and dysfluency. Am J Med Sci. 1986 Dec;292(6):391–392. DOI: 10.5334/tohm.350
- Modreanu R, Özdemir G, Buhmann C, Hauptmann B. Levodopa-induced dystonia in a patient with possible progressive supranuclear palsy with progressive gait freezing. J Neurol Sci. 2018 May 15;388:139–140. DOI: 10.1002/mds.1120
- Noda K, Kawajiri S, Tomizawa Y, Hattori N, Okuma Y. Pisa syndrome in progressive supranuclear palsy: A case report. Parkinsonism Relat Disord. 2015 Sep;21(9):1115–1116. DOI: 10.1111/jsr.13050
- Onder H, Comoglu S. Levodopa-Induced Jaw and Facial Dystonia in a Case of Progressive Supranuclear Palsy with Blepharospasm. Mov Disord Clin Pract. 2023 Sep 25;10(12):1806–1808. DOI: 10.1212/WNL.59.4.585
- Panegyres PK, Hillman D, Dunne JW. Laryngeal dystonia causing upper airway obstruction in progressive supranuclear palsy. J Clin Neurosci. 2007 Apr;14(4):380–381. DOI: 10.1002/mdc3.13112
- Tan EK, Chan LL, Wong MC. Levodopa-induced oromandibular dystonia in progressive supranuclear palsy. Clinical neurology and neurosurgery. 2003 Apr 1;105(2):132–134. DOI: 10.1111/ane.13209
- Suresh TG, Rao TV. Progressive supranuclear palsy. Report of 14 cases with special reference to unusual features. J Assoc Physicians India. 1991 Jun;39(6):471–475.
- Suzuki T, Makifuchi T, Fukuhara N. Lower Cranial Dystonia with Inflated Cheeks: A Case of Dystonic Respiratory Failure. Intern Med. 2023 Jun 1;62(11):1671–1674. DOI: 10.2169/internalmedicine.9554-22
- Zhang W, Mao W, Xu E, Chhetri JK, Chan P. Progressive supranuclear palsy presenting with hyperkinetic movement disorder and hemiplegic dystonia: a case report. Int J Neurosci. 2020 Dec;130(12):1278–1281. DOI: 10.1080/00207454.2020.1732965
- Stamelou M, Alonso-Canovas A, Bhatia KP. Dystonia in corticobasal degeneration: a review of the literature on 404 pathologically proven cases. Movement disorders. 2012 May;27(6):696–702. DOI: 10.1002/mds.24992
- Rinne JO, Lee MS, Thompson PD, Marsden CD. Corticobasal degeneration: a clinical study of 36 cases. Brain. 1994 Oct 1;117(5):1183–1196. DOI: 10.1093/brain/117.5.1183
- Vanek Z, Jankovic J. Dystonia in corticobasal degeneration. Movement disorders: official journal of the Movement Disorder Society. 2001 Mar;16(2):252–257. DOI: 10.1002/mds.1038
- Yoon WT. Comparison of dystonia between Parkinson’s disease and atypical parkinsonism: the clinical usefulness of dystonia distribution and characteristics in the differential diagnosis of parkinsonism. Neurologia i Neurochirurgia Polska. 2018 Jan 1;52(1):48–53. DOI: 10.1016/j.pjnns.2017.11.004
- 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 Mar 25;60(6):910–916. DOI: 10.1212/01.WNL.0000052991.70149.68
- Marsili L, Colosimo C.
Dystonia in multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration . In Dystonia and Dystonic Syndromes 2015 (pp. 89–99). Vienna: Springer Vienna. DOI: 10.1007/978-3-7091-1516-9_7 - Lamberti P, De Mari M, Zenzola A, Aniello MS, Defazio G. Frequency of apraxia of eyelid opening in the general population and in patients with extrapyramidal disorders. Neurological Sciences. 2002 Sep;23(Suppl 2):s81–s82. DOI: 10.1007/s100720200080
- Yoon WT, Chung EJ, Lee SH, Kim BJ, Lee WY.
Clinical analysis of blepharospasm and apraxia of eyelid opening in patients with parkinsonism . Journal of clinical neurology (Seoul, Korea). 2005 Oct 20. 1(2):159. DOI: 10.3988/jcn.2005.1.2.159 - Chang KH, Chuang CC, Lyu RK, Chen CM, Ro LS, Chen ST, Wu YR. Clinical characteristics of corticobasal syndrome amongst Chinese in Taiwan. Parkinsonism & Related Disorders. 2007 May 1;13(4):219–223. DOI: 10.1016/j.parkreldis.2006.10.003
- Höglinger GU. Is it useful to classify progressive supranuclear palsy and corticobasal degeneration as different disorders? No. Movement disorders clinical practice. 2018 Mar 6;5(2):141–144. DOI: 10.1002/mdc3.12582
- Oide T, Ohara S, Yazawa M, Inoue K, Itoh N, Tokuda T, Ikeda SI. Progressive supranuclear palsy with asymmetric tau pathology presenting with unilateral limb dystonia. Acta neuropathologica. 2002 Aug;104(2):209–214. DOI: 10.1007/s00401-002-0531-y
- Chahine LM, Rebeiz T, Rebeiz JJ, Grossman M, Gross RG. Corticobasal syndrome: five new things. Neurology: Clinical Practice. 2014 Aug;4(4):304–312. DOI: 10.1212/CPJ.0000000000000026
- Quattrone A, Franzmeier N, Levin J, Petzold GC, Spottke A, Brosseron F, Falkenburger B, Prudlo J, Gasser T, DESCRIBE-PSP Group, The ProPSP Group, Petzold GC. Prospective multicenter evaluation of the MDS “suggestive of PSP” diagnostic criteria. Movement disorders. 2025 Mar;40(3):526–536. DOI: 10.1002/mds.30112
- Stezin A, Holla VV, Chaithra SP, Kamble N, Saini J, Yadav R, Pal PK. Asymmetric limb dystonia in progressive supranuclear palsy: conundrum in nosology. Movement Disorders Clinical Practice. 2019 Apr 5;6(5):415–416. DOI: 10.1002/mdc3.12756
- Chunowski P, Madetko-Alster N, Alster P. Asymmetry in atypical parkinsonian syndromes—a review. Journal of Clinical Medicine. 2024 Sep 28;13(19):5798. DOI: 10.3390/jcm13195798
- 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. DOI: 10.1002/ana.10570
- Mathew R, Bak TH, Hodges JR. Diagnostic criteria for corticobasal syndrome: a comparative study. Journal of Neurology, Neurosurgery & Psychiatry. 2012 Apr 1;83(4):405–410. DOI: 10.1136/jnnp-2011-300875
- Respondek G, Höglinger GU. The phenotypic spectrum of progressive supranuclear palsy. Parkinsonism & related disorders. 2016 Jan 1;22:S34–36. DOI: 10.1016/j.parkreldis.2015.09.041
- Williams DR, Lees AJ. What features improve the accuracy of the clinical diagnosis of progressive supranuclear palsy-parkinsonism (PSP-P)?. Movement Disorders. 2010 Feb 15;25(3):357–362. DOI: 10.1002/mds.22977
- Alster P, Madetko N, Koziorowski D, Friedman A. Progressive supranuclear palsy—parkinsonism predominant (PSP-P)—a clinical challenge at the boundaries of psp and Parkinson’s disease (PD). Frontiers in Neurology. 2020 Mar 10;11:180. DOI: 10.3389/fneur.2020.00180
- Necpál J, Borsek M, Jeleňová B. “Parkinson’s disease” on the way to progressive supranuclear palsy: A review on PSP-parkinsonism. Neurological Sciences. 2021 Dec;42(12):4927–4936. DOI: 10.1007/s10072-021-05601-8
- DeLong MR, Wichmann T. Circuits and circuit disorders of the basal ganglia. Archives of neurology. 2007 Jan 1;64(1):20–24. DOI: 10.1001/archneur.64.1.20
- Jankovic J, Tintner R. Dystonia and parkinsonism. Parkinsonism & related disorders. 2001 Oct 1;8(2):109–121. DOI: 10.1016/S1353-8020(01)00025-6
- Morales-Briceno H, Fung VS, Bhatia KP, Balint B. Parkinsonism and dystonia: Clinical spectrum and diagnostic clues. Journal of the Neurological Sciences. 2022 Feb 15;433:120016. DOI: 10.1016/j.jns.2021.120016
- Palleis C, Quattrone A, Dehsarvi A, Roemer-Cassiano SN, Bernhardt AM, AL-108-231 Investigators, the PASSPORT Study Group, Aiba I, Antonini A, Apetauerova D, Azulay JP, Martinez EB. Brain networks route neurodegeneration patterns in patients with progressive supranuclear palsy. Movement Disorders. 2025 Oct;40(10):2102–2115. DOI: 10.1002/mds.30257
- Spinelli EG, Ghirelli A, Bottale I, Basaia S, Canu E, Castelnovo V, Volontè MA, Galantucci S, Magnani G, Caso F, Cecchetti G. Stepwise functional brain architecture correlates with atrophy in progressive supranuclear palsy. Movement Disorders. 2024 Sep;39(9):1493–1503. DOI: 10.1002/mds.29887
- Lang AE. Treatment of progressive supranuclear palsy and corticobasal degeneration. Movement disorders: official journal of the Movement Disorder Society. 2005 Aug;20(S12):S83–S91. DOI: 10.1002/mds.20545
- Jankovic J. An update on new and unique uses of botulinum toxin in movement disorders. Toxicon. 2018 Jun 1;147:84–88. DOI: 10.1016/j.toxicon.2017.09.003
- Ling H. Clinical approach to progressive supranuclear palsy. Journal of movement disorders. 2016 Jan 25;9(1):3–13. DOI: 10.14802/jmd.15060
- Rowe JB, Holland N, Rittman T. Progressive supranuclear palsy: diagnosis and management. Practical Neurology. 2021 Oct 1;21(5):376–383. DOI: 10.1136/practneurol-2020-002794
- Müller J, Wenning GK, Wissel J, Seppi K, Poewe W. Botulinum toxin treatment in atypical parkinsonian disorders associated with disabling focal dystonia. Journal of neurology. 2002 Mar;249(3):300–304. DOI: 10.1007/s004150200009
- Balint B, Neo S, Magrinelli F, Mulroy E, Latorre A, Stamelou M, Morris HR, Batla A, Bhatia KP. Ethnic differences in atypical parkinsonism—is South Asian PSP different?. Movement disorders clinical practice. 2024 Nov;11(11):1355–1364. DOI: 10.1002/mdc3.14182
- Wrigley S, Cullinane PW, Parmera JB, Arca VM, Sifontes Valladares W, Rivera-Sánchez M, Curless T, Chajed LP, Burrows M, Revesz T, Morris HR. Clinical diagnosis of progressive supranuclear palsy (PSP): a clinicopathological comparison of patients with confirmed PSP and clinical mimics. Movement Disorders. 2025 Oct;40(10):2116–2128. DOI: 10.1002/mds.30261
DOI: https://doi.org/10.5334/tohm.1243 | Journal eISSN: 2160-8288
Language: English
Page range: 48 - 48
Submitted on: Jun 1, 2026
Accepted on: Aug 7, 2026
Published on: Aug 19, 2026
Published by: Ubiquity Press
In partnership with: Paradigm Publishing Services
© 2026 Kartika Gulati, Sanjay Pandey, published by Ubiquity Press
This work is licensed under the Creative Commons Attribution 4.0 License.