Clinical Features of Progressive Supranuclear Palsy
DOI:
https://doi.org/10.61788/njn.v2i20.08Keywords:
progressive supranuclear palsy, Parkinson's disease, Parkinson's syndrome, diagnosis, differential diagnosisAbstract
Clinical characteristics of progressive supranuclear palsy has been identified in Azerbaijan for the first time. Out of 13 examined patients, 10 has been diagnosed with PSP - Richardson, 2 with PSP - parkinsonism and finally 1 patient with PSP pure akinesia and gait freezing subtypes. Till the Progressive supranuclear palsy diagnose all patients have been treated according to Parkinson disease or Parkinson syndrome diagnose. There has been a gap of about 4 years between the illness manifestation and the correct diagnose. All patients show light dysfunction of central motoneuron and cerebellum system alongside with traditional symptoms of PSP. There has been no record of cognitive activity dysfunction. Along with the study of the clinical features of PSP, the investigation showed the complexity of early diagnosis of the disease.
References
Steele J, Richardson JC, Olszewski J. Progressive supranuclear palsy. A heterogeneous system degeneration involving brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia // Arch Neurol,1964,10:333-359.
Dickson DW, Ahmed Z, Algom AA, Tsuboi Y, Josephs KA. Neuropathology of variants of progressive supranuclear palsy // Curr Opin Neurol 2010;23:394-400.
Lantos PL. The neuropathology of progressive supranuclear palsy // J Neural Transm Suppl 1994;42:137-52.
Lang AE. Clinical heterogeneity in progressive supranuclear palsy: challenges to diagnosis, pathogenesis and future therapies // Mov Disord 2014;29:1707-9.
Josephs KA et al. Neuropathological background of phenotypical variability in frontotemporal dementia // Acta Neuropathol 2011;122:137-53.
Golbe LI, Davis PH, Schoenberg BS, Duvoisin RC. Prevalence and natural history of progressive supranuclear palsy // Neurology 1988;38:1031-1034.
Golbe LI, Ohman-Strickland PA. A clinical rating scale for progressive supranuclear palsy // Brain 2007;130(Pt 6):1552- 1565
Kawashima M, Miyake M, Kusumi M, Adachi Y, Nakashima K. Prevalence of progressive supranuclear palsy in Yonago, Japan // Mov Disord 2004;19:1239-1240.
Schrag A, Ben-Shlomo Y, Quinn NP. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study // Lancet 1999;354:1771-1775.
Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, 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):1247-1258.
Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges // Lancet Neurol 2009;8:270-279.
Ling H, Holton JL, Shaw K, Davey K, Lashley T, Revesz T. Histological evidence of chronic traumatic encephalopathy in a large series of neurodegenerative diseases // Acta Neuropathol 2015;130:891-893.
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.
Hirano A, Kurland LT, Krooth RS, Lessell S. Parkinsonismdementia complex, an endemic disease on the island of Guam. I. Clinical features // Brain 1961;84:642-661.
Hutton M, Lendon CL, Rizzu P, Baker M, Froelich S, Houlden H, et al. Association of missense and 5’-splice-site mutations in tau with the inherited dementia FTDP-17 // Nature 1998;393:702-705.
Morris HR, Osaki Y, Holton J, Lees AJ, Wood NW, Revesz T, et al. Tau exon 10 +16 mutation FTDP-17 presenting clinically as sporadic young onset PSP // Neurology 2003;61: 102-104.
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;47:1-9.
Respondek G,Stamelou M, Kurz C,Ferguson LW, RajputA,Chiu WZ, et al. The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases // Mov Disord. 2014;29(14):1758-66. https://doi.org/10.1002/mds. 26054.
Clavaguera F, Bolmont T, Crowther RA, Abramowski D, Frank S, Probst A, et al. Transmission and spreading of tauopathy in transgenic mouse brain // Nat Cell Biol 2009; 11:909-913.
Rascol O, Sabatini U, Simonetta-Moreau M, Montastruc JL, Rascol A, Clanet M. Square wave jerks in parkinsonian syndromes // J Neurol Neurosurg Psychiatry 1991;54:599-602.
Şirəliyeva R.K., Məmmədbəyli A.K., Əliyev R.R. Nevrologiyada müayinə metodları (şkalalar, testlər, sorğu anketləri). Bakı, «Red N Line» MMC 2020, 342 səh.
Birdi S, Rajput AH, Fenton M, Donat JR, Rozdilsky B, Robinson C, et al. Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases // Mov Disord 2002;17:1255-1264.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2026 National Association of Neurologists
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
