U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Striatal Degeneration

MedGen UID:
893515
•Concept ID:
CN239279
•
Disease or Syndrome

Professional guidelines

PubMed

Glutaric acidemia type 1: Treatment and outcome of 168 patients over three decades.
Strauss KA, Williams KB, Carson VJ, Poskitt L, Bowser LE, Young M, Robinson DL, Hendrickson C, Beiler K, Taylor CM, Haas-Givler B, Hailey J, Chopko S, Puffenberger EG, Brigatti KW, Miller F, Morton DH
Mol Genet Metab 2020 Nov;131(3):325-340. Epub 2020 Oct 4 doi: 10.1016/j.ymgme.2020.09.007. PMID: 33069577
Glutaric acidemia type 1: outcomes before and after expanded newborn screening.
Viau K, Ernst SL, Vanzo RJ, Botto LD, Pasquali M, Longo N
Mol Genet Metab 2012 Aug;106(4):430-8. Epub 2012 Jun 9 doi: 10.1016/j.ymgme.2012.05.024. PMID: 22728054
Huntington's disease: can mice lead the way to treatment?
Crook ZR, Housman D
Neuron 2011 Feb 10;69(3):423-35. doi: 10.1016/j.neuron.2010.12.035. PMID: 21315254Free PMC Article

Recent clinical studies

Etiology

Mild cognitive impairment in multiple system atrophy: a brain network disorder.
Jellinger KA
J Neural Transm (Vienna) 2023 Oct;130(10):1231-1240. Epub 2023 Aug 15 doi: 10.1007/s00702-023-02682-x. PMID: 37581647
Dopaminergic Dysfunction Is More Symmetric in Dementia with Lewy Bodies Compared to Parkinson's Disease.
Fedorova TD, Knudsen K, Horsager J, Hansen AK, Okkels N, Gottrup H, Vang K, Borghammer P
J Parkinsons Dis 2023;13(4):515-523. doi: 10.3233/JPD-230001. PMID: 37212074Free PMC Article
Exploring the brain-body composition relationship in Huntington's disease.
Turner M, Reyes A, Bartlett DM, Culpin S, Hart NH, Hardt L, Feindel K, Poudel GR, Ziman M, Cruickshank TM
J Musculoskelet Neuronal Interact 2020 Sep 1;20(3):332-338. PMID: 32877970Free PMC Article
The Challenge of Disease-Modifying Therapies in Parkinson's Disease: Role of CSF Biomarkers.
Paolini Paoletti F, Gaetani L, Parnetti L
Biomolecules 2020 Feb 19;10(2) doi: 10.3390/biom10020335. PMID: 32092971Free PMC Article
Glutaric acidemia type 1.
Hedlund GL, Longo N, Pasquali M
Am J Med Genet C Semin Med Genet 2006 May 15;142C(2):86-94. doi: 10.1002/ajmg.c.30088. PMID: 16602100Free PMC Article

Diagnosis

Mild cognitive impairment in multiple system atrophy: a brain network disorder.
Jellinger KA
J Neural Transm (Vienna) 2023 Oct;130(10):1231-1240. Epub 2023 Aug 15 doi: 10.1007/s00702-023-02682-x. PMID: 37581647
Different Generations of Type-B Monoamine Oxidase Inhibitors in Parkinson's Disease: From Bench to Bedside.
Alborghetti M, Nicoletti F
Curr Neuropharmacol 2019;17(9):861-873. doi: 10.2174/1570159X16666180830100754. PMID: 30160213Free PMC Article
18F-FDOPA PET for the diagnosis of parkinsonian syndromes.
Darcourt J, Schiazza A, Sapin N, Dufour M, Ouvrier MJ, Benisvy D, Fontana X, Koulibaly PM
Q J Nucl Med Mol Imaging 2014 Dec;58(4):355-65. Epub 2014 Nov 4 PMID: 25366711
Glutaric acidemia type 1.
Hedlund GL, Longo N, Pasquali M
Am J Med Genet C Semin Med Genet 2006 May 15;142C(2):86-94. doi: 10.1002/ajmg.c.30088. PMID: 16602100Free PMC Article
Brain SPECT imaging in multiple system atrophy.
Cilia R, Marotta G, Benti R, Pezzoli G, Antonini A
J Neural Transm (Vienna) 2005 Dec;112(12):1635-45. doi: 10.1007/s00702-005-0382-5. PMID: 16284908

Therapy

Different Generations of Type-B Monoamine Oxidase Inhibitors in Parkinson's Disease: From Bench to Bedside.
Alborghetti M, Nicoletti F
Curr Neuropharmacol 2019;17(9):861-873. doi: 10.2174/1570159X16666180830100754. PMID: 30160213Free PMC Article
Nigrostriatal dopamine transporter availability in early Parkinson's disease.
Fazio P, Svenningsson P, Cselényi Z, Halldin C, Farde L, Varrone A
Mov Disord 2018 Apr;33(4):592-599. Epub 2018 Feb 13 doi: 10.1002/mds.27316. PMID: 29436751
Can stress trigger Parkinson's disease?
Djamshidian A, Lees AJ
J Neurol Neurosurg Psychiatry 2014 Aug;85(8):878-81. Epub 2013 Nov 20 doi: 10.1136/jnnp-2013-305911. PMID: 24259593
Antioxidants in Huntington's disease.
Johri A, Beal MF
Biochim Biophys Acta 2012 May;1822(5):664-74. Epub 2011 Nov 23 doi: 10.1016/j.bbadis.2011.11.014. PMID: 22138129Free PMC Article
Magnetic resonance imaging of Huntington's disease: preparing for clinical trials.
Klöppel S, Henley SM, Hobbs NZ, Wolf RC, Kassubek J, Tabrizi SJ, Frackowiak RS
Neuroscience 2009 Nov 24;164(1):205-19. Epub 2009 Jan 29 doi: 10.1016/j.neuroscience.2009.01.045. PMID: 19409230Free PMC Article

Prognosis

Phenotypic spectrum of short-chain enoyl-Coa hydratase-1 (ECHS1) deficiency.
Masnada S, Parazzini C, Bini P, Barbarini M, Alberti L, Valente M, Chiapparini L, De Silvestri A, Doneda C, Iascone M, Saielli LA, Cereda C, Veggiotti P, Corbetta C, Tonduti D
Eur J Paediatr Neurol 2020 Sep;28:151-158. Epub 2020 Jul 29 doi: 10.1016/j.ejpn.2020.07.007. PMID: 32800686
Clinical findings of autosomal-dominant striatal degeneration and PDE8B mutation screening in parkinsonism and related disorders.
Ni J, Yi X, Liu Z, Sun W, Yuan Y, Yang J, Jiang H, Shen L, Tang B, Liu Y, Wang J
Parkinsonism Relat Disord 2019 Dec;69:94-98. Epub 2019 Nov 5 doi: 10.1016/j.parkreldis.2019.11.002. PMID: 31726290
Network spread determines severity of degeneration and disconnection in Huntington's disease.
Poudel GR, Harding IH, Egan GF, Georgiou-Karistianis N
Hum Brain Mapp 2019 Oct 1;40(14):4192-4201. Epub 2019 Jun 12 doi: 10.1002/hbm.24695. PMID: 31187915Free PMC Article
A GABBR2 gene variant modifies pathophysiology in Huntington's disease.
Philpott AL, Fitzgerald PB, Bailey NW, Churchyard A, Georgiou-Karistianis N, Cummins TD
Neurosci Lett 2016 May 4;620:8-13. Epub 2016 Mar 23 doi: 10.1016/j.neulet.2016.03.038. PMID: 27033668
Gender differences in Parkinson's disease.
Haaxma CA, Bloem BR, Borm GF, Oyen WJ, Leenders KL, Eshuis S, Booij J, Dluzen DE, Horstink MW
J Neurol Neurosurg Psychiatry 2007 Aug;78(8):819-24. Epub 2006 Nov 10 doi: 10.1136/jnnp.2006.103788. PMID: 17098842Free PMC Article

Clinical prediction guides

Exploring the brain-body composition relationship in Huntington's disease.
Turner M, Reyes A, Bartlett DM, Culpin S, Hart NH, Hardt L, Feindel K, Poudel GR, Ziman M, Cruickshank TM
J Musculoskelet Neuronal Interact 2020 Sep 1;20(3):332-338. PMID: 32877970Free PMC Article
Dual tasking impairments are associated with striatal pathology in Huntington's disease.
Lo J, Reyes A, Pulverenti TS, Rankin TJ, Bartlett DM, Zaenker P, Rowe G, Feindel K, Poudel G, Georgiou-Karistianis N, Ziman MR, Cruickshank TM
Ann Clin Transl Neurol 2020 Sep;7(9):1608-1619. Epub 2020 Aug 14 doi: 10.1002/acn3.51142. PMID: 32794343Free PMC Article
Clinical findings of autosomal-dominant striatal degeneration and PDE8B mutation screening in parkinsonism and related disorders.
Ni J, Yi X, Liu Z, Sun W, Yuan Y, Yang J, Jiang H, Shen L, Tang B, Liu Y, Wang J
Parkinsonism Relat Disord 2019 Dec;69:94-98. Epub 2019 Nov 5 doi: 10.1016/j.parkreldis.2019.11.002. PMID: 31726290
Network spread determines severity of degeneration and disconnection in Huntington's disease.
Poudel GR, Harding IH, Egan GF, Georgiou-Karistianis N
Hum Brain Mapp 2019 Oct 1;40(14):4192-4201. Epub 2019 Jun 12 doi: 10.1002/hbm.24695. PMID: 31187915Free PMC Article
Cell-based treatments for huntington's disease.
Dunnett SB, Rosser AE
Int Rev Neurobiol 2011;98:483-508. doi: 10.1016/B978-0-12-381328-2.00017-1. PMID: 21907097

Recent systematic reviews

The Role of the Cerebellum in Huntington's Disease: a Systematic Review.
Franklin GL, Camargo CHF, Meira AT, Lima NSC, Teive HAG
Cerebellum 2021 Apr;20(2):254-265. Epub 2020 Oct 7 doi: 10.1007/s12311-020-01198-4. PMID: 33029762
Phenotypic spectrum of short-chain enoyl-Coa hydratase-1 (ECHS1) deficiency.
Masnada S, Parazzini C, Bini P, Barbarini M, Alberti L, Valente M, Chiapparini L, De Silvestri A, Doneda C, Iascone M, Saielli LA, Cereda C, Veggiotti P, Corbetta C, Tonduti D
Eur J Paediatr Neurol 2020 Sep;28:151-158. Epub 2020 Jul 29 doi: 10.1016/j.ejpn.2020.07.007. PMID: 32800686

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...