Abstract
A 60-year-old man presented with a 2-year history of progressive difficulty in walking, with occasional unprovoked falls, especially in the backward direction which occurred while walking, sitting without support and getting up from a chair. Family members reported a constant staring expression, reduced volume and output of speech, and change in behaviour—disinterest in routine activities and occasional inappropriate laughing or crying. There was no history of tremor. There was also a history of occasional difficulty swallowing liquids, choking, and spillage of food while eating. There was no family history of Parkinsonism. Though the patient was started on dopaminergic medications, there was no significant improvement.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
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. Arch Neurol. 1964;10:333–59.
Dickson DW, Ahmed Z, Algom AA, Tsuboi Y, Josephs KA. Neuropathology of variants of progressive supranuclear palsy. Curr Opin Neurol. 2010;23(4):394–400.
Armstrong MJ. Progressive supranuclear palsy: an update. Curr Neurol Neurosci Rep. 2018;18(3):12.
Coyle-Gilchrist IT, Dick KM, Patterson K, et al. Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes. Neurology. 2016;86(18):1736–43.
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(Pt 6):1247–58.
Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol. 2009;8(3):270–9.
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):1–9.
Hoglinger GU, Melhem NM, Dickson DW, et al. Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy. Nat Genet. 2011;43(7):699–705.
Lannuzel A, Michel PP, Caparros-Lefebvre D, et al. Toxicity of Annonaceae for dopaminergic neurons: potential role in atypical Parkinsonism in Guadeloupe. Mov Disord. 2002;17(1):84–90.
Hoglinger GU, Lannuzel A, Khondiker ME, et al. The mitochondrial complex I inhibitor rotenone triggers a cerebral tauopathy. J Neurochem. 2005;95(4):930–9.
Litvan I, Hauw JJ, Bartko JJ, et al. Validity and reliability of the preliminary NINDS neuropathologic criteria for progressive supranuclear palsy and related disorders. J Neuropathol Exp Neurol. 1996;55(1):97–105.
Maher ER, Lees AJ. The clinical features and natural history of the Steele–Richardson–Olszewski syndrome (progressive supranuclear palsy). Neurology. 1986;36(7):1005–8.
Golbe LI, Davis PH, Schoenberg BS, Duvoisin RC. Prevalence and natural history of progressive supranuclear palsy. Neurology. 1988;38(7):1031–4.
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(6):615–20.
Ling H. Clinical approach to progressive supranuclear palsy. J Mov Disord. 2016;9(1):3–13.
Ling H, Massey LA, Lees AJ, Brown P, Day BL. Hypokinesia without decrement distinguishes progressive supranuclear palsy from Parkinson’s disease. Brain. 2012;135(Pt 4):1141–53.
Niall Q, Mikko K. Fast micrographia and pallidal pathology. Mov Disord. 2003;18(9):1067–9.
Rivaud-Pechoux S, Vidailhet M, Gallouedec G, et al. Longitudinal ocular motor study in corticobasal degeneration and progressive supranuclear palsy. Neurology. 2000;54(5):1029–32.
Quinn N. The “round the houses” sign in progressive supranuclear palsy. Ann Neurol. 1996;40(6):951.
Troost BT, Daroff RB. The ocular motor defects in progressive supranuclear palsy. Ann Neurol. 1977;2(5):397–403.
Rafal RD, Posner MI, Friedman JH, Inhoff AW, Bernstein E. Orienting of visual attention in progressive supranuclear palsy. Brain. 1988;111(Pt 2):267–80.
Gerstenecker A, Mast B, Duff K, et al. Executive dysfunction is the primary cognitive impairment in progressive supranuclear palsy. Arch Clin Neuropsychol. 2013;28(2):104–13.
Litvan I. Cognitive disturbances in progressive supranuclear palsy. J Neural Transm Suppl. 1994;42:69–78.
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):910–6.
Radicati FG, Martinez Martin P, Fossati C, et al. Non motor symptoms in progressive supranuclear palsy: prevalence and severity. NPJ Parkinsons Dis. 2017;3:35.
Sixel-Doring F, Schweitzer M, Mollenhauer B, Trenkwalder C. Polysomnographic findings, video-based sleep analysis and sleep perception in progressive supranuclear palsy. Sleep Med. 2009;10(4):407–15.
Stamelou M, Bhatia KP. Atypical Parkinsonism: diagnosis and treatment. Neurol Clin. 2015;33(1):39–56.
Stamelou M, Quinn NP, Bhatia KP. “Atypical” atypical Parkinsonism: new genetic conditions presenting with features of progressive supranuclear palsy, corticobasal degeneration, or multiple system atrophy—a diagnostic guide. Mov Disord. 2013;28(9):1184–99.
Stezin A, Lenka A, Jhunjhunwala K, Saini J, Pal PK. Advanced structural neuroimaging in progressive supranuclear palsy: where do we stand? Parkinsonism Relat Disord. 2017;36:19–32.
Longoni G, Agosta F, Kostic VS, et al. MRI measurements of brainstem structures in patients with Richardson’s syndrome, progressive supranuclear palsy-Parkinsonism, and Parkinson’s disease. Mov Disord. 2011;26(2):247–55.
Kato N, Arai K, Hattori T. Study of the rostral midbrain atrophy in progressive supranuclear palsy. J Neurol Sci. 2003;210(1–2):57–60.
Adachi M, Kawanami T, Ohshima H, Sugai Y, Hosoya T. Morning glory sign: a particular MR finding in progressive supranuclear palsy. Magn Reson Med Sci. 2004;3(3):125–32.
Boxer AL, Yu JT, Golbe LI, et al. Advances in progressive supranuclear palsy: new diagnostic criteria, biomarkers, and therapeutic approaches. Lancet Neurol. 2017;16(7):552–63.
Stamelou M, de Silva R, Arias-Carrion O, et al. Rational therapeutic approaches to progressive supranuclear palsy. Brain. 2010;133(Pt 6):1578–90.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2024 Springer Nature Singapore Pte Ltd.
About this chapter
Cite this chapter
Prasad, S., Pal, P.K. (2024). Progressive Supranuclear Palsy. In: Oli, K.K., Shrestha, G.S., Ojha, R., Pal, P.K., Pandey, S., Das, B. (eds) Case-based Approach to Common Neurological Disorders. Springer, Singapore. https://doi.org/10.1007/978-981-99-8676-7_24
Download citation
DOI: https://doi.org/10.1007/978-981-99-8676-7_24
Published:
Publisher Name: Springer, Singapore
Print ISBN: 978-981-99-8675-0
Online ISBN: 978-981-99-8676-7
eBook Packages: MedicineMedicine (R0)