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Atypical progressive supranuclear palsy syndrome

MedGen UID:
1779597
Concept ID:
C5548371
Disease or Syndrome
Synonyms: atypical progressive supranuclear palsy syndrome; Atypical PSP syndrome; atypical PSP syndrome
SNOMED CT: Atypical progressive supranuclear palsy syndrome (1156790008)
 
Monarch Initiative: MONDO:0020488
Orphanet: ORPHA99750

Definition

A form or progressive supranuclear palsy syndrome (PSP), a rare late-onset neurodegenerative disease, characterized by an underlying PSP-tau pathology, that does not conform to the classic presentation of PSP. The clinical phenotype is variable and comprises PSP with predominant Parkinsonism (PSP-P), PSP with progressive gait freezing (PSP-PGF), PSP with predominant corticobasal syndrome (PSP-CBS), PSP with predominant speech/language disorder (PSP-SL), PSP with predominant frontal presentation (PSP-F), PSP with predominant ocular motor dysfunction (PSP-OM), and PSP with predominant postural instability (PSP-PI). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAtypical progressive supranuclear palsy syndrome

Professional guidelines

PubMed

Meyer PT, Frings L, Rücker G, Hellwig S
J Nucl Med 2017 Dec;58(12):1888-1898. Epub 2017 Sep 14 doi: 10.2967/jnumed.116.186403. PMID: 28912150
Boxer AL, Yu JT, Golbe LI, Litvan I, Lang AE, Höglinger GU
Lancet Neurol 2017 Jul;16(7):552-563. Epub 2017 Jun 13 doi: 10.1016/S1474-4422(17)30157-6. PMID: 28653647Free PMC Article
Levin J, Kurz A, Arzberger T, Giese A, Höglinger GU
Dtsch Arztebl Int 2016 Feb 5;113(5):61-9. doi: 10.3238/arztebl.2016.0061. PMID: 26900156Free PMC Article

Recent clinical studies

Etiology

Parmera JB, Oliveira MCB, Rodrigues RD, Coutinho AM
Arq Neuropsiquiatr 2022 May;80(5 Suppl 1):126-136. doi: 10.1590/0004-282X-ANP-2022-S134. PMID: 35976324Free PMC Article
Wen Y, Zhou Y, Jiao B, Shen L
J Parkinsons Dis 2021;11(1):93-105. doi: 10.3233/JPD-202302. PMID: 33104043Free PMC Article
Jiang C, Hopfner F, Katsikoudi A, Hein R, Catli C, Evetts S, Huang Y, Wang H, Ryder JW, Kuhlenbaeumer G, Deuschl G, Padovani A, Berg D, Borroni B, Hu MT, Davis JJ, Tofaris GK
J Neurol Neurosurg Psychiatry 2020 Jul;91(7):720-729. Epub 2020 Apr 9 doi: 10.1136/jnnp-2019-322588. PMID: 32273329Free PMC Article
Jabbari E, Holland N, Chelban V, Jones PS, Lamb R, Rawlinson C, Guo T, Costantini AA, Tan MMX, Heslegrave AJ, Roncaroli F, Klein JC, Ansorge O, Allinson KSJ, Jaunmuktane Z, Holton JL, Revesz T, Warner TT, Lees AJ, Zetterberg H, Russell LL, Bocchetta M, Rohrer JD, Williams NM, Grosset DG, Burn DJ, Pavese N, Gerhard A, Kobylecki C, Leigh PN, Church A, Hu MTM, Woodside J, Houlden H, Rowe JB, Morris HR
JAMA Neurol 2020 Mar 1;77(3):377-387. doi: 10.1001/jamaneurol.2019.4347. PMID: 31860007Free PMC Article
Meyer PT, Frings L, Rücker G, Hellwig S
J Nucl Med 2017 Dec;58(12):1888-1898. Epub 2017 Sep 14 doi: 10.2967/jnumed.116.186403. PMID: 28912150

Diagnosis

Aludin S, Schmill LA
Rofo 2021 Dec;193(12):1403-1410. Epub 2021 May 25 doi: 10.1055/a-1460-8795. PMID: 34034347
Giagkou N, Höglinger GU, Stamelou M
Int Rev Neurobiol 2019;149:49-86. Epub 2019 Nov 21 doi: 10.1016/bs.irn.2019.10.013. PMID: 31779824
Risacher SL, Saykin AJ
Handb Clin Neurol 2019;167:191-227. doi: 10.1016/B978-0-12-804766-8.00012-1. PMID: 31753134Free PMC Article
Boxer AL, Yu JT, Golbe LI, Litvan I, Lang AE, Höglinger GU
Lancet Neurol 2017 Jul;16(7):552-563. Epub 2017 Jun 13 doi: 10.1016/S1474-4422(17)30157-6. PMID: 28653647Free PMC Article
Levin J, Kurz A, Arzberger T, Giese A, Höglinger GU
Dtsch Arztebl Int 2016 Feb 5;113(5):61-9. doi: 10.3238/arztebl.2016.0061. PMID: 26900156Free PMC Article

Therapy

VandeVrede L, Ljubenkov PA, Rojas JC, Welch AE, Boxer AL
Neurotherapeutics 2020 Oct;17(4):1563-1581. doi: 10.1007/s13311-020-00888-5. PMID: 32676851Free PMC Article
Greene P
Continuum (Minneap Minn) 2019 Aug;25(4):919-935. doi: 10.1212/CON.0000000000000751. PMID: 31356287
Jost WH, Lingor P, Tönges L, Schwarz J, Buhmann C, Kassubek J, Schrag A
J Neural Transm (Vienna) 2019 Jul;126(7):925-932. Epub 2019 May 13 doi: 10.1007/s00702-019-02012-0. PMID: 31087195
Meyer PT, Frings L, Rücker G, Hellwig S
J Nucl Med 2017 Dec;58(12):1888-1898. Epub 2017 Sep 14 doi: 10.2967/jnumed.116.186403. PMID: 28912150
Koros C, Stamelou M
Parkinsonism Relat Disord 2016 Jan;22 Suppl 1:S93-5. Epub 2015 Sep 25 doi: 10.1016/j.parkreldis.2015.09.033. PMID: 26459661

Prognosis

Jiang C, Hopfner F, Katsikoudi A, Hein R, Catli C, Evetts S, Huang Y, Wang H, Ryder JW, Kuhlenbaeumer G, Deuschl G, Padovani A, Berg D, Borroni B, Hu MT, Davis JJ, Tofaris GK
J Neurol Neurosurg Psychiatry 2020 Jul;91(7):720-729. Epub 2020 Apr 9 doi: 10.1136/jnnp-2019-322588. PMID: 32273329Free PMC Article
Ali F, Josephs K
Expert Rev Neurother 2018 Jul;18(7):603-616. Epub 2018 Jun 28 doi: 10.1080/14737175.2018.1489241. PMID: 29902389
Meyer PT, Frings L, Rücker G, Hellwig S
J Nucl Med 2017 Dec;58(12):1888-1898. Epub 2017 Sep 14 doi: 10.2967/jnumed.116.186403. PMID: 28912150
Bhatia KP, Stamelou M
Int Rev Neurobiol 2017;134:1285-1301. Epub 2017 Jul 3 doi: 10.1016/bs.irn.2017.06.001. PMID: 28805573
Keener AM, Bordelon YM
Semin Neurol 2016 Aug;36(4):330-4. Epub 2016 Sep 19 doi: 10.1055/s-0036-1585097. PMID: 27643900

Clinical prediction guides

Madetko-Alster N, Otto-Ślusarczyk D, Wiercińska-Drapało A, Koziorowski D, Szlufik S, Samborska-Ćwik J, Struga M, Friedman A, Alster P
Int J Mol Sci 2023 Oct 13;24(20) doi: 10.3390/ijms242015135. PMID: 37894815Free PMC Article
Jiang C, Hopfner F, Katsikoudi A, Hein R, Catli C, Evetts S, Huang Y, Wang H, Ryder JW, Kuhlenbaeumer G, Deuschl G, Padovani A, Berg D, Borroni B, Hu MT, Davis JJ, Tofaris GK
J Neurol Neurosurg Psychiatry 2020 Jul;91(7):720-729. Epub 2020 Apr 9 doi: 10.1136/jnnp-2019-322588. PMID: 32273329Free PMC Article
Agosta F, Sarasso E, Filippi M
Int Rev Neurobiol 2018;142:149-173. Epub 2018 Oct 25 doi: 10.1016/bs.irn.2018.09.002. PMID: 30409252
Ali F, Josephs K
Expert Rev Neurother 2018 Jul;18(7):603-616. Epub 2018 Jun 28 doi: 10.1080/14737175.2018.1489241. PMID: 29902389
Bruns MB, Josephs KA
Int Rev Psychiatry 2013 Apr;25(2):197-209. doi: 10.3109/09540261.2013.766154. PMID: 23611349

Recent systematic reviews

Bougea A
Medicina (Kaunas) 2022 Mar 26;58(4) doi: 10.3390/medicina58040483. PMID: 35454322Free PMC Article
Chang JY, Rukavina K, Lawn T, Chaudhuri KR
J Integr Neurosci 2021 Dec 30;20(4):1067-1078. doi: 10.31083/j.jin2004108. PMID: 34997730
Moretti DV
CNS Neurosci Ther 2019 Feb;25(2):159-174. Epub 2018 Oct 7 doi: 10.1111/cns.13068. PMID: 30294976Free PMC Article
Rana AQ, Qureshi AR, Siddiqui O, Sarfraz Z, Rana R, Shtilbans A
J Neurol 2019 Sep;266(9):2093-2102. Epub 2018 Sep 20 doi: 10.1007/s00415-018-9049-7. PMID: 30238268
Lopez G, Bayulkem K, Hallett M
Acta Neurol Scand 2016 Oct;134(4):242-9. Epub 2016 Jan 6 doi: 10.1111/ane.12546. PMID: 27070344Free PMC Article

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