Progressive Supranuclear Palsy and Corticobasal Syndrome
- PMID: 36222770
- DOI: 10.1212/CON.0000000000001158
Progressive Supranuclear Palsy and Corticobasal Syndrome
Abstract
Purpose of review: The differential diagnosis of parkinsonism (tremor, rigidity, bradykinesia, and gait difficulty/postural instability) is broad and includes two neurodegenerative conditions that exist on a clinicopathologic spectrum: progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). Early in their clinical course, PSP and CBS may be difficult to distinguish from Parkinson disease and several other illnesses, but it is crucial to do so because of implications for management and prognosis.
Recent findings: Early accurate diagnosis of PSP and CBS remains a challenge because of heterogeneity in presenting symptoms and high frequency of coexisting pathologies (especially Alzheimer disease and vascular disease). It is increasingly recognized that patients with PSP, CBS, and other parkinsonian disorders require multidisciplinary care for optimal outcomes. With the recent proliferation of biomarker studies and therapeutic trials for tauopathies, there is growing hope that better treatments for PSP and CBS are on the horizon.
Summary: Although PSP and CBS currently lack disease-modifying therapies, it is important to diagnose them as early as possible so that the patient can benefit from the many available symptomatic therapies, support groups, and a growing number of clinical trials.
Copyright © 2022 American Academy of Neurology.
Similar articles
-
Sleep disturbances in progressive supranuclear palsy syndrome (PSPS) and corticobasal syndrome (CBS).Neurol Neurochir Pol. 2023;57(3):229-234. doi: 10.5603/PJNNS.a2023.0019. Epub 2023 Mar 17. Neurol Neurochir Pol. 2023. PMID: 36928793 Review.
-
Diagnosis Across the Spectrum of Progressive Supranuclear Palsy and Corticobasal Syndrome.JAMA Neurol. 2020 Mar 1;77(3):377-387. doi: 10.1001/jamaneurol.2019.4347. JAMA Neurol. 2020. PMID: 31860007 Free PMC article.
-
Magnetic Susceptibility in Progressive Supranuclear Palsy Variants, Parkinson's Disease, and Corticobasal Syndrome.Mov Disord. 2023 Dec;38(12):2282-2290. doi: 10.1002/mds.29613. Epub 2023 Sep 29. Mov Disord. 2023. PMID: 37772771 Free PMC article.
-
Presence of Skin α-Synuclein Deposits Discriminates Parkinson's Disease from Progressive Supranuclear Palsy and Corticobasal Syndrome.J Parkinsons Dis. 2022;12(2):585-591. doi: 10.3233/JPD-212904. J Parkinsons Dis. 2022. PMID: 34864689 Free PMC article.
-
Differentiation of progressive supranuclear palsy: clinical, imaging and laboratory tools.Acta Neurol Scand. 2013 May;127(5):362-70. doi: 10.1111/ane.12067. Epub 2013 Feb 13. Acta Neurol Scand. 2013. PMID: 23406296 Review.
Cited by
-
Progressive Supranuclear Palsy Diagnosed After a Severe Fall Trauma in a Patient Who Experienced Episodes of Easy Falling.Cureus. 2024 May 4;16(5):e59643. doi: 10.7759/cureus.59643. eCollection 2024 May. Cureus. 2024. PMID: 38832160 Free PMC article.
-
Corticobasal Syndrome: Are There Central or Peripheral Triggers?Neurol Clin Pract. 2025 Feb;15(1):e200365. doi: 10.1212/CPJ.0000000000200365. Epub 2024 Oct 8. Neurol Clin Pract. 2025. PMID: 39399563
-
The prevalence and incidence of progressive supranuclear palsy and corticobasal syndrome: a systematic review and meta-analysis.J Neurol. 2023 Sep;270(9):4451-4465. doi: 10.1007/s00415-023-11791-2. Epub 2023 Jun 8. J Neurol. 2023. PMID: 37289323 Free PMC article.
-
Primary cilia in Parkinson's disease: summative roles in signaling pathways, genes, defective mitochondrial function, and substantia nigra dopaminergic neurons.Front Aging Neurosci. 2024 Sep 18;16:1451655. doi: 10.3389/fnagi.2024.1451655. eCollection 2024. Front Aging Neurosci. 2024. PMID: 39364348 Free PMC article. Review.
-
Diagnosis and Management of Progressive Corticobasal Syndrome.Curr Treat Options Neurol. 2024 Jul;26(7):319-338. doi: 10.1007/s11940-024-00797-4. Epub 2024 Jun 25. Curr Treat Options Neurol. 2024. PMID: 39886562 Free PMC article.
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;10:333–359. doi:10.1001/archneur.1964.00460160003001 - DOI
-
- Lhermitte J, Levy G, Kyriaco N. Les perturbations de la representation spatiale chez les apraxiques. Rev Neurol (Paris) 1925;2:586–600.
-
- Rebeiz JJ, Kolodny EH, Richardson EP Jr. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 1968;18(1):20–33. doi:10.1001/archneur.1968.00470310034003 - DOI
-
- Schrag A, Ben-Shlomo Y, Quinn NP. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet 1999;354(9192):1771–1775. doi:10.1016/s0140-6736(99)04137-9 - DOI
-
- Jabbari E, Woodside J, Tan MM, et al. The genetic and clinico-pathological profile of early-onset progressive supranuclear palsy. Mov Disord 2019;34(9):1307–1314. doi:10.1002/mds.27786 - DOI
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Medical
Research Materials
Miscellaneous