Received: 26.09.2022; Accepted: 11.10.2022; Published on-line: 27.10.2022
Neuroophthalmic abnormalities in supranuclear palsy
L. V. Venger 1, I. V. Khubetova 2
1 Odesa National Medical University
2 Odesa regional clinical hospital
TO CITE THIS ARTICLE: Venger LV, Khubetova IV. Neuroophthalmic abnormalities in supranuclear palsy. J.ophthalmol.(Ukraine).2022;5:37-41. http://doi.org/10.31288/oftalmolzh202253741
Background: Progressive supranuclear palsy (PSP) is the most common atypical parkinsonism with various movement disorders and oculomotoric abnormalities.
Purpose: To identify major neuroophthalmic manifestations in different clinical phenotypes of PSP.
Material and Methods: The study was conducted at the Odesa regional clinical hospital in 2011 to 2021.
Twenty one patients with PSP (including one patient with PSP combined with Hallervorden–Spatz disease and levodopa-induced hyperkynesis) underwent an examination. This included a classical neurological examination and comprehensive neuroophthalmic examination with high-field magnetic resonance imaging (MRI) of the brain and spine and videonystagmography testing. Mean patient age was 53.2 ± 1.1 years, and most patients were women (13 [61.9%]). Statistical analysis included frequency analysis.
Results: PSP-P was the most common phenotype of PSP (52.4%), followed by PSP-PGF (9.5%), PSP-OM (9.5%), PSP-PI (9.5%), PSP-CBS (4.8%), PSP-SL (4.8%), PSP-F (4.8%) and PSP+GSD (4.8%). All patients showed changes in velocity and amplitude of vertical saccades. Diplopia was, however, observed in only 12 (57.1%) patients.
Conclusion: Neurological and neuroophthalmic examination is decisive in establishing the diagnosis of progressive supranuclear palsy and selecting a treatment strategy. In the current study, PSP-P was the most common (52.4%) phenotype, the frequency of PSP-OM variant was 9.5%.
Keywords: progressive supranuclear palsy, neuroophthalmology, diagnostic assessment, phenotype
1.Tagai K, Ono M, Kubota M, Kitamura S et al. High-Contrast in Vivo Imaging of Tau Pathologies in Alzheimer’s and Non-Alzheimer’s Disease Tauopathies. Neuron. 2021 Jan 6;109(1):42-58.e8.
2.Dickson DW, Kouri N, Murray ME, Josephs KA. Neuropathology of frontotemporal lobar degeneration-tau (FTLD-tau). J Mol Neurosci. 2011 Nov;45(3):384-9.
3.Robinson JL, Yan N, Caswell C, Xie SX, Suh E, Van Deerlin VM et al. Primary Tau Pathology, Not Copathology, Correlates With Clinical Symptoms in PSP and CBD. J Neuropathol Exp Neurol. 2020 Mar 1;79(3):296-304.
4.Levin J, Kurz A, Arzberger T, Giese A, Höglinger GU. The Differential Diagnosis and Treatment of Atypical Parkinsonism. Dtsch Arztebl Int. 2016 Feb 5;113(5):61-9.
5.De Pablo-Fernández E, Lees AJ, Holton JL, Warner TT. Prognosis and Neuropathologic Correlation of Clinical Subtypes of Parkinson Disease. JAMA Neurol. 2019 Apr 1;76(4):470-479.
6.Williams DR, Holton JL, Strand C, Pittman A, de Silva R, Lees AJ, Revesz T. Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson’s syndrome. Brain. 2007 Jun;130(Pt 6):1566-76.
7.Sakae N, Josephs KA, Litvan I, Murray ME, Duara R, Uitti RJ, Wszolek ZK, Graff-Radford NR, Dickson DW. Neuropathologic basis of frontotemporal dementia in progressive supranuclear palsy. Mov Disord. 2019 Nov;34(11):1655-1662.
8.Tsuboi Y, Josephs KA, Boeve BF, Litvan I, Caselli RJ, Caviness JN et al. Increased tau burden in the cortices of progressive supranuclear palsy presenting with corticobasal syndrome. Mov Disord. 2005 Aug;20(8):982-8.
9.Przewodowska D, Marzec W, Madetko N. Novel Therapies for Parkinsonian Syndromes-Recent Progress and Future Perspectives. Front Mol Neurosci. 2021 Aug 26;14:720220.
10.Boxer AL, Yu JT, Golbe LI, Litvan I, Lang AE, Höglinger GU. Advances in progressive supranuclear palsy: new diagnostic criteria, biomarkers, and therapeutic approaches. Lancet Neurol. 2017 Jul;16(7):552-563.
11.Kovacs GG, Lukic MJ, Irwin DJ, Arzberger T, Respondek G, Lee EB et al. Distribution patterns of tau pathology in progressive supranuclear palsy. Acta Neuropathol. 2020 Aug;140(2):99-119.
12.Choi JH, Kim H, Shin JH, Lee JY, Kim HJ, Kim JM, Jeon B. Eye movements and association with regional brain atrophy in clinical subtypes of progressive supranuclear palsy. J Neurol. 2021 Mar;268(3):967-977.
13.Tolnay M, Clavaguera F. Argyrophilic grain disease: a late-onset dementia with distinctive features among tauopathies. Neuropathology. 2004 Dec;24(4):269-83.
14.Narasimhan S, Changolkar L, Riddle DM, Kats A, Stieber A, Weitzman SA et al. Human tau pathology transmits glial tau aggregates in the absence of neuronal tau. J Exp Med. 2020 Feb 3;217(2):e20190783.
15.Friedman DI, Jankovic J, McCrary JA. Neuro-ophthalmic Findings in Progressive Supranuclear Palsy. J Clin Neuroophthalmol. 1992 June; 12 (2): 104-109.
16.Progressive Supranuclear Palsy https://eyewiki.aao.org/Progressive_Supranuclear_Palsy.
17.Muratova TM, Venger LV, Khramtsov DM, Vorokhta IuM, Teliushchenko VD. Neuro-ophthalmological abnormalities in patients with ischemic stroke in the setting of a stroke center of a university clinic. J Ophthalmol (Ukraine). 2020;5:56-61.
18.Agarwal S, Gilbert R. Progressive Supranuclear Palsy. 2021 Apr 11. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan.
19.Mastaglia FL, Grainger KM. Internuclear ophthalmoplegia in progressive supranuclear palsy. J Neurol Sci. 1975 Jul;25(3):303-8.
Venger LV: Conceptualization, Data Curation, Formal Analysis, Investigation, Methodology, Project administration, Writing – original draft, Writing – review & editing
Khubetova IV: Conceptualization, Data Curation, Formal Analysis, Investigation, Methodology, Project administration, Supervision, Validation, Visualization, Writing – original draft
All authors reviewed the results and approved the final version of the manuscript.
Funding sources: No stated funding sources.
Conflict of interest: The authors state that they have no conflict of interest that might bias this work.
The study involved human subjects, was approved by the Ethics Committee, and adhered to the tenets of the Declaration of Helsinki. Informed consent was obtained from all participants. This study did not include animal experiments.