Progressive Supranuclear Palsy and Corticobasal Degeneration: Novel Clinical Concepts and Advances in Biomarkers

Overview

Journal Arq Neuropsiquiatr

Publisher Thieme

Specialties Neurology
Psychiatry

Date 2022 Aug 17

PMID 35976324

Authors

Jacy Bezerra Parmera

Marcos Castello Barbosa de Oliveira

Roberta Diehl Rodrigues

Artur Martins Coutinho

Affiliations

Soon will be listed here.

Abstract

Background: Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are sporadic adult-onset primary tauopathies clinically classified among the atypical parkinsonian syndromes. They are intrinsically related with regard to their clinical features, pathology, biochemistry, and genetic risk factors.

Objectives: This review highlights the current knowledge on PSP and CBD, focusing on evolving clinical concepts, new diagnostic criteria, and advances in biomarkers.

Methods: We performed a non-systematic literature review through the PubMed database. The search was restricted to articles written in English, published from 1964 to date.

Results: Clinicopathologic and in vivo biomarkers studies have broadened PSP and CBD clinical phenotypes. They are now recognized as a range of motor and behavioral syndromes associated with underlying 4R-tauopathy neuropathology. The Movement Disorders Society PSP diagnostic criteria included clinical variants apart from the classical description, increasing diagnostic sensitivity. Meanwhile, imaging biomarkers have explored the complexity of symptoms and pathological processes related to corticobasal syndrome and CBD.

Conclusions: In recent years, several prospective or clinicopathologic studies have assessed clinical, radiological, and fluid biomarkers that have helped us gain a better understanding of the complexity of the 4R-tauopathies, mainly PSP and CBD.

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References

Steele J, Richardson J, 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. DOI: 10.1001/archneur.1964.00460160003001. View

Parmera J, Coutinho A, Aranha M, Studart-Neto A, de Godoi Carneiro C, de Almeida I . FDG-PET Patterns Predict Amyloid Deposition and Clinical Profile in Corticobasal Syndrome. Mov Disord. 2020; 36(3):651-661. DOI: 10.1002/mds.28373. View

Boeve B, Lang A, Litvan I . Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol. 2003; 54 Suppl 5:S15-9. DOI: 10.1002/ana.10570. View

Nicoletti G, Tonon C, Lodi R, Condino F, Manners D, Malucelli E . Apparent diffusion coefficient of the superior cerebellar peduncle differentiates progressive supranuclear palsy from Parkinson's disease. Mov Disord. 2008; 23(16):2370-6. DOI: 10.1002/mds.22279. View

Ouchi H, Toyoshima Y, Tada M, Oyake M, Aida I, Tomita I . Pathology and sensitivity of current clinical criteria in corticobasal syndrome. Mov Disord. 2013; 29(2):238-44. DOI: 10.1002/mds.25746. View

Ling H, OSullivan S, Holton J, Revesz T, Massey L, Williams D . Does corticobasal degeneration exist? A clinicopathological re-evaluation. Brain. 2010; 133(Pt 7):2045-57. DOI: 10.1093/brain/awq123. View

Josephs K, Whitwell J, Tacik P, Duffy J, Senjem M, Tosakulwong N . [18F]AV-1451 tau-PET uptake does correlate with quantitatively measured 4R-tau burden in autopsy-confirmed corticobasal degeneration. Acta Neuropathol. 2016; 132(6):931-933. PMC: 5107140. DOI: 10.1007/s00401-016-1618-1. View

Hoglinger G, Respondek G, Stamelou M, Kurz C, Josephs K, Lang A . Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria. Mov Disord. 2017; 32(6):853-864. PMC: 5516529. DOI: 10.1002/mds.26987. View

Rebeiz J, Kolodny E, Richardson Jr E . Corticodentatonigral degeneration with neuronal achromasia: a progressive disorder of late adult life. Trans Am Neurol Assoc. 1967; 92:23-6. View

10.

Lemos J, Pereira D, Almendra L, Rebelo D, Patricio M, Castelhano J . Cortical control of vertical and horizontal saccades in progressive supranuclear palsy: An exploratory fMRI study. J Neurol Sci. 2017; 373:157-166. DOI: 10.1016/j.jns.2016.12.049. View

11.

Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin R . 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. DOI: 10.1212/wnl.47.1.1. View

12.

Burrell J, Hornberger M, Villemagne V, Rowe C, Hodges J . Clinical profile of PiB-positive corticobasal syndrome. PLoS One. 2013; 8(4):e61025. PMC: 3618463. DOI: 10.1371/journal.pone.0061025. View

13.

Whitwell J, Tosakulwong N, Botha H, Ali F, Clark H, Duffy J . Brain volume and flortaucipir analysis of progressive supranuclear palsy clinical variants. Neuroimage Clin. 2020; 25:102152. PMC: 6961761. DOI: 10.1016/j.nicl.2019.102152. View

14.

Brendel M, Barthel H, van Eimeren T, Marek K, Beyer L, Song M . Assessment of 18F-PI-2620 as a Biomarker in Progressive Supranuclear Palsy. JAMA Neurol. 2020; 77(11):1408-1419. PMC: 7341407. DOI: 10.1001/jamaneurol.2020.2526. View

15.

Rosskopf J, Gorges M, Muller H, Lule D, Uttner I, Ludolph A . Intrinsic functional connectivity alterations in progressive supranuclear palsy: Differential effects in frontal cortex, motor, and midbrain networks. Mov Disord. 2017; 32(7):1006-1015. DOI: 10.1002/mds.27039. View

16.

Hellwig S, Amtage F, Kreft A, Buchert R, Winz O, Vach W . [¹⁸F]FDG-PET is superior to [¹²³I]IBZM-SPECT for the differential diagnosis of parkinsonism. Neurology. 2012; 79(13):1314-22. DOI: 10.1212/WNL.0b013e31826c1b0a. View

17.

Boxer A, Qureshi I, Ahlijanian M, Grundman M, Golbe L, Litvan I . Safety of the tau-directed monoclonal antibody BIIB092 in progressive supranuclear palsy: a randomised, placebo-controlled, multiple ascending dose phase 1b trial. Lancet Neurol. 2019; 18(6):549-558. DOI: 10.1016/S1474-4422(19)30139-5. View

18.

Lee S, Rabinovici G, Mayo M, Wilson S, Seeley W, DeArmond S . Clinicopathological correlations in corticobasal degeneration. Ann Neurol. 2011; 70(2):327-40. PMC: 3154081. DOI: 10.1002/ana.22424. View

19.

Quattrone A, Nicoletti G, Messina D, Fera F, Condino F, Pugliese P . MR imaging index for differentiation of progressive supranuclear palsy from Parkinson disease and the Parkinson variant of multiple system atrophy. Radiology. 2007; 246(1):214-21. DOI: 10.1148/radiol.2453061703. View

20.

Hoglinger G . Is it Useful to Classify Progressive Supranuclear Palsy and Corticobasal Degeneration as Different Disorders? No. Mov Disord Clin Pract. 2018; 5(2):141-144. PMC: 6174469. DOI: 10.1002/mdc3.12582. View