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Striatonigral degeneration

MedGen UID:
124366
Concept ID:
C0270733
Disease or Syndrome
Synonyms: Atrophies, Striatonigral; Atrophy, Striatonigral; Degeneration, Striatonigral; Striatonigral Atrophies; Striatonigral Atrophy; Striatonigral Degeneration; Striatonigral Degenerations
SNOMED CT: SND - Striatonigral degeneration (29618004); Striatonigral degeneration (29618004); Striatonigral atrophy (29618004)
 
Monarch Initiative: MONDO:0003122
OMIM® Phenotypic series: PS271930

Definition

A progressive neurodegenerative disorder caused by a disruption in the connection between the striatum and the substantia nigra. It is a type of multiple system atrophy (MSA). Signs and symptoms include rigidity, instability, impaired speech, and slow movements. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVStriatonigral degeneration

Professional guidelines

PubMed

Jellinger KA
Expert Rev Neurother 2017 Dec;17(12):1189-1208. Epub 2017 Oct 23 doi: 10.1080/14737175.2017.1392239. PMID: 29023182
Brooks DJ
Neurology 1993 Dec;43(12 Suppl 6):S6-16. PMID: 8264913
Bannister R
Curr Opin Neurol Neurosurg 1992 Aug;5(4):487-91. PMID: 1515689

Recent clinical studies

Etiology

Stefanova N
Neurotherapeutics 2023 Jan;20(1):117-126. Epub 2022 Aug 22 doi: 10.1007/s13311-022-01287-8. PMID: 35995919Free PMC Article
Bower PG
Auton Neurosci 2018 May;211:39-42. Epub 2017 Dec 16 doi: 10.1016/j.autneu.2017.12.006. PMID: 29269241
Ozawa T, Onodera O
Proc Jpn Acad Ser B Phys Biol Sci 2017;93(5):251-258. doi: 10.2183/pjab.93.016. PMID: 28496050Free PMC Article
Jellinger KA
Mov Disord 2003 Sep;18 Suppl 6:S2-12. doi: 10.1002/mds.10557. PMID: 14502650
Wenning GK, Quinn NP
Baillieres Clin Neurol 1997 Apr;6(1):187-204. PMID: 9426875

Diagnosis

Stefanova N
Neurotherapeutics 2023 Jan;20(1):117-126. Epub 2022 Aug 22 doi: 10.1007/s13311-022-01287-8. PMID: 35995919Free PMC Article
Hopfner F, Tietz AK, Ruf VC, Ross OA, Koga S, Dickson D, Aguzzi A, Attems J, Beach T, Beller A, Cheshire WP, van Deerlin V, Desplats P, Deuschl G, Duyckaerts C, Ellinghaus D, Evsyukov V, Flanagan ME, Franke A, Frosch MP, Gearing M, Gelpi E, van Gerpen JA, Ghetti B, Glass JD, Grinberg LT, Halliday G, Helbig I, Höllerhage M, Huitinga I, Irwin DJ, Keene DC, Kovacs GG, Lee EB, Levin J, Martí MJ, Mackenzie I, McKeith I, Mclean C, Mollenhauer B, Neumann M, Newell KL, Pantelyat A, Pendziwiat M, Peters A, Molina Porcel L, Rabano A, Matěj R, Rajput A, Rajput A, Reimann R, Scott WK, Seeley W, Selvackadunco S, Simuni T, Stadelmann C, Svenningsson P, Thomas A, Trenkwalder C, Troakes C, Trojanowski JQ, Uitti RJ, White CL, Wszolek ZK, Xie T, Ximelis T, Yebenes J; Alzheimer's Disease Genetics Consortium, Müller U, Schellenberg GD, Herms J, Kuhlenbäumer G, Höglinger G
Mov Disord 2022 Oct;37(10):2110-2121. Epub 2022 Aug 23 doi: 10.1002/mds.29164. PMID: 35997131Free PMC Article
Ryman SG, Poston KL
Parkinsonism Relat Disord 2020 Apr;73:85-93. Epub 2019 Oct 10 doi: 10.1016/j.parkreldis.2019.10.002. PMID: 31629653Free PMC Article
Jellinger KA
J Alzheimers Dis 2018;62(3):1141-1179. doi: 10.3233/JAD-170397. PMID: 28984582Free PMC Article
Jellinger KA
Expert Rev Neurother 2017 Dec;17(12):1189-1208. Epub 2017 Oct 23 doi: 10.1080/14737175.2017.1392239. PMID: 29023182

Therapy

Rau A, Schröter N, Rijntjes M, Bamberg F, Jost WH, Zaitsev M, Weiller C, Rau S, Urbach H, Reisert M, Russe MF
Eur Radiol 2023 Oct;33(10):7160-7167. Epub 2023 May 1 doi: 10.1007/s00330-023-09665-2. PMID: 37121929Free PMC Article
Batla A, De Pablo-Fernandez E, Erro R, Reich M, Calandra-Buonaura G, Barbosa P, Balint B, Ling H, Islam S, Cortelli P, Volkmann J, Quinn N, Holton JL, Warner TT, Bhatia KP
Mov Disord 2018 Jul;33(7):1099-1107. doi: 10.1002/mds.27450. PMID: 30153390
Kuzdas-Wood D, Stefanova N, Jellinger KA, Seppi K, Schlossmacher MG, Poewe W, Wenning GK
Prog Neurobiol 2014 Jul;118:19-35. Epub 2014 Mar 2 doi: 10.1016/j.pneurobio.2014.02.007. PMID: 24598411Free PMC Article
Wenning GK, Quinn NP
Baillieres Clin Neurol 1997 Apr;6(1):187-204. PMID: 9426875
Gouider-Khouja N, Vidailhet M, Bonnet AM, Pichon J, Agid Y
Mov Disord 1995 May;10(3):288-94. doi: 10.1002/mds.870100310. PMID: 7651445

Prognosis

Liao S, Chen T, Dai Y, Wang Y, Wu F, Zhong M
Mol Genet Genomic Med 2020 Feb;8(2):e1101. Epub 2019 Dec 26 doi: 10.1002/mgg3.1101. PMID: 31876398Free PMC Article
Ryman SG, Poston KL
Parkinsonism Relat Disord 2020 Apr;73:85-93. Epub 2019 Oct 10 doi: 10.1016/j.parkreldis.2019.10.002. PMID: 31629653Free PMC Article
Cassarino DS, Quezado MM, Ghatak NR, Duray PH
Arch Pathol Lab Med 2003 Sep;127(9):1204-6. doi: 10.5858/2003-127-1204-LPANCS. PMID: 12946221
Costa C, Duyckaerts C
Curr Opin Neurol 1993 Dec;6(6):865-71. doi: 10.1097/00019052-199312000-00007. PMID: 8293161
Kan AE
Pathology 1978 Jan;10(1):45-52. doi: 10.3109/00313027809063478. PMID: 643319

Clinical prediction guides

Campese N, Fanciulli A, Stefanova N, Haybaeck J, Kiechl S, Wenning GK
J Neural Transm (Vienna) 2021 Oct;128(10):1481-1494. Epub 2021 Jul 28 doi: 10.1007/s00702-021-02383-3. PMID: 34319460Free PMC Article
Ryman SG, Poston KL
Parkinsonism Relat Disord 2020 Apr;73:85-93. Epub 2019 Oct 10 doi: 10.1016/j.parkreldis.2019.10.002. PMID: 31629653Free PMC Article
Lee MJ, Kim TH, Mun CW, Shin HK, Son J, Lee JH
J Neurol 2018 Jul;265(7):1540-1547. Epub 2018 Apr 25 doi: 10.1007/s00415-018-8874-z. PMID: 29696500
Yoshida M
Neuropathology 2007 Oct;27(5):484-93. doi: 10.1111/j.1440-1789.2007.00841.x. PMID: 18018485
Wenning GK, Tison F, Scherfler C, Puschban Z, Waldner R, Granata R, Ghorayeb I, Poewe W
Cell Transplant 2000 Mar-Apr;9(2):279-88. doi: 10.1177/096368970000900213. PMID: 10811400

Recent systematic reviews

Ben-Shlomo Y, Wenning GK, Tison F, Quinn NP
Neurology 1997 Feb;48(2):384-93. doi: 10.1212/wnl.48.2.384. PMID: 9040727

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