U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Cerebellar degeneration

MedGen UID:
75496
•Concept ID:
C0262404
•
Disease or Syndrome
Synonyms: Brain degeneration; Cerebellar Degeneration; cerebellar degeneration; cerebellar Degeneration; cerebellum neurodegenerative disease; cerebral degeneration; neurodegenerative disease of cerebellum
SNOMED CT: Cerebellar degeneration (95646004)
 
Monarch Initiative: MONDO:0022687

Definition

Degeneration of the cerebellum. It may be an inherited condition, a paraneoplastic syndrome, or secondary to autoimmune disorders. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Ataxia management: low-tech approaches.
de Silva RN
Pract Neurol 2021 Dec;21(6):466-467. Epub 2021 Nov 5 doi: 10.1136/practneurol-2021-003213. PMID: 34740962
Immune-mediated Cerebellar Ataxias: Practical Guidelines and Therapeutic Challenges.
Mitoma H, Manto M, Hampe CS
Curr Neuropharmacol 2019;17(1):33-58. doi: 10.2174/1570159X16666180917105033. PMID: 30221603Free PMC Article
Treatment of cerebellar ataxia.
Jones TM, Shaw JD, Sullivan K, Zesiewicz TA
Neurodegener Dis Manag 2014;4(5):379-92. doi: 10.2217/nmt.14.27. PMID: 25405651

Recent clinical studies

Etiology

Immune-mediated ataxias: Guide to clinicians.
Meira AT, de Moraes MPM, Ferreira MG, Franklin GL, Rezende Filho FM, Teive HAG, Barsottini OGP, Pedroso JL
Parkinsonism Relat Disord 2023 Dec;117:105861. Epub 2023 Sep 20 doi: 10.1016/j.parkreldis.2023.105861. PMID: 37748994
Paraneoplastic syndromes in ophthalmology.
Bussat A, Langner-Lemercier S, Salmon A, Mouriaux F
J Fr Ophtalmol 2018 May;41(5):e181-e185. Epub 2018 May 18 doi: 10.1016/j.jfo.2018.03.002. PMID: 29784239
The cerebellar cognitive affective/Schmahmann syndrome scale.
Hoche F, Guell X, Vangel MG, Sherman JC, Schmahmann JD
Brain 2018 Jan 1;141(1):248-270. doi: 10.1093/brain/awx317. PMID: 29206893Free PMC Article
CNS syndromes associated with antibodies against metabotropic receptors.
Lancaster E
Curr Opin Neurol 2017 Jun;30(3):354-360. doi: 10.1097/WCO.0000000000000448. PMID: 28306571
Update on neurological paraneoplastic syndromes.
Höftberger R, Rosenfeld MR, Dalmau J
Curr Opin Oncol 2015 Nov;27(6):489-95. doi: 10.1097/CCO.0000000000000222. PMID: 26335665Free PMC Article

Diagnosis

Immune-mediated ataxias: Guide to clinicians.
Meira AT, de Moraes MPM, Ferreira MG, Franklin GL, Rezende Filho FM, Teive HAG, Barsottini OGP, Pedroso JL
Parkinsonism Relat Disord 2023 Dec;117:105861. Epub 2023 Sep 20 doi: 10.1016/j.parkreldis.2023.105861. PMID: 37748994
The hallmarks of aging in Ataxia-Telangiectasia.
Aguado J, Gómez-Inclán C, Leeson HC, Lavin MF, Shiloh Y, Wolvetang EJ
Ageing Res Rev 2022 Aug;79:101653. Epub 2022 May 26 doi: 10.1016/j.arr.2022.101653. PMID: 35644374
Ataxia management: low-tech approaches.
de Silva RN
Pract Neurol 2021 Dec;21(6):466-467. Epub 2021 Nov 5 doi: 10.1136/practneurol-2021-003213. PMID: 34740962
Paraneoplastic syndromes in ophthalmology.
Bussat A, Langner-Lemercier S, Salmon A, Mouriaux F
J Fr Ophtalmol 2018 May;41(5):e181-e185. Epub 2018 May 18 doi: 10.1016/j.jfo.2018.03.002. PMID: 29784239
Neurologic paraneoplastic syndromes.
Dropcho EJ
J Neurol Sci 1998 Jan 8;153(2):264-78. doi: 10.1016/s0022-510x(97)00296-7. PMID: 9511883

Therapy

Overview of treatment strategies in paraneoplastic neurological syndromes.
Kerstens J, Titulaer MJ
Handb Clin Neurol 2024;200:97-112. doi: 10.1016/B978-0-12-823912-4.00015-3. PMID: 38494299
Growth hormone rescue cerebellar degeneration in SCA3 transgenic mice.
Wu S, Liu K, Cheng W, Su S, Lin Y, Lin T, Cheng Y, Chang J, Wu Y, Liu C
Biochem Biophys Res Commun 2020 Aug 20;529(2):467-473. Epub 2020 Jul 2 doi: 10.1016/j.bbrc.2020.05.116. PMID: 32703453
Update on neurological paraneoplastic syndromes.
Höftberger R, Rosenfeld MR, Dalmau J
Curr Opin Oncol 2015 Nov;27(6):489-95. doi: 10.1097/CCO.0000000000000222. PMID: 26335665Free PMC Article
Lambert-Eaton myasthenic syndrome.
Newsom-Davis J
Rev Neurol (Paris) 2004 Feb;160(2):177-80. doi: 10.1016/s0035-3787(04)70888-7. PMID: 15034474
Toxic cerebellar degeneration.
Valsamis MP, Mancall E
Hum Pathol 1973 Dec;4(4):513-20. doi: 10.1016/s0046-8177(73)80063-2. PMID: 4750820

Prognosis

Paraneoplastic syndromes in ophthalmology.
Parrado-Carrillo A, Alcubierre R, Camós-Carreras A, Sanchez-Dalmau BF
Arch Soc Esp Oftalmol (Engl Ed) 2022 Jun;97(6):350-357. Epub 2022 Mar 12 doi: 10.1016/j.oftale.2022.03.006. PMID: 35292226
Epigenetic Silencing of CDR1as Drives IGF2BP3-Mediated Melanoma Invasion and Metastasis.
Hanniford D, Ulloa-Morales A, Karz A, Berzoti-Coelho MG, Moubarak RS, Sánchez-Sendra B, Kloetgen A, Davalos V, Imig J, Wu P, Vasudevaraja V, Argibay D, Lilja K, Tabaglio T, Monteagudo C, Guccione E, Tsirigos A, Osman I, Aifantis I, Hernando E
Cancer Cell 2020 Jan 13;37(1):55-70.e15. doi: 10.1016/j.ccell.2019.12.007. PMID: 31935372Free PMC Article
Immune-mediated Cerebellar Ataxias: Practical Guidelines and Therapeutic Challenges.
Mitoma H, Manto M, Hampe CS
Curr Neuropharmacol 2019;17(1):33-58. doi: 10.2174/1570159X16666180917105033. PMID: 30221603Free PMC Article
Ataxia telangiectasia: a review.
Rothblum-Oviatt C, Wright J, Lefton-Greif MA, McGrath-Morrow SA, Crawford TO, Lederman HM
Orphanet J Rare Dis 2016 Nov 25;11(1):159. doi: 10.1186/s13023-016-0543-7. PMID: 27884168Free PMC Article
Paraneoplastic neurological syndromes associated with ovarian tumors.
Zaborowski MP, Spaczynski M, Nowak-Markwitz E, Michalak S
J Cancer Res Clin Oncol 2015 Jan;141(1):99-108. Epub 2014 Jun 26 doi: 10.1007/s00432-014-1745-9. PMID: 24965744Free PMC Article

Clinical prediction guides

Epigenetic Silencing of CDR1as Drives IGF2BP3-Mediated Melanoma Invasion and Metastasis.
Hanniford D, Ulloa-Morales A, Karz A, Berzoti-Coelho MG, Moubarak RS, Sánchez-Sendra B, Kloetgen A, Davalos V, Imig J, Wu P, Vasudevaraja V, Argibay D, Lilja K, Tabaglio T, Monteagudo C, Guccione E, Tsirigos A, Osman I, Aifantis I, Hernando E
Cancer Cell 2020 Jan 13;37(1):55-70.e15. doi: 10.1016/j.ccell.2019.12.007. PMID: 31935372Free PMC Article
Tandem internal models execute motor learning in the cerebellum.
Honda T, Nagao S, Hashimoto Y, Ishikawa K, Yokota T, Mizusawa H, Ito M
Proc Natl Acad Sci U S A 2018 Jul 10;115(28):7428-7433. Epub 2018 Jun 25 doi: 10.1073/pnas.1716489115. PMID: 29941578Free PMC Article
The cerebellar cognitive affective/Schmahmann syndrome scale.
Hoche F, Guell X, Vangel MG, Sherman JC, Schmahmann JD
Brain 2018 Jan 1;141(1):248-270. doi: 10.1093/brain/awx317. PMID: 29206893Free PMC Article
Spinocerebellar ataxias: an update.
Soong BW, Paulson HL
Curr Opin Neurol 2007 Aug;20(4):438-46. doi: 10.1097/WCO.0b013e3281fbd3dd. PMID: 17620880
Distinguishing and treating causes of central vertigo.
Solomon D
Otolaryngol Clin North Am 2000 Jun;33(3):579-601. doi: 10.1016/s0030-6665(05)70228-0. PMID: 10815038

Recent systematic reviews

A Systematic Review on Anti-Yo/PCA-1 Antibody: Beyond Cerebellar Ataxia in Middle-Aged Women with Gynecologic Cancer.
Mendes NT, Ronchi NR, Silva GD
Cerebellum 2023 Dec;22(6):1287-1292. Epub 2022 Nov 5 doi: 10.1007/s12311-022-01492-3. PMID: 36334195
Anti-Tr/DNER Antibody-Associated Cerebellar Ataxia: a Systematic Review.
Campana IG, Silva GD
Cerebellum 2022 Dec;21(6):1085-1091. Epub 2021 Nov 24 doi: 10.1007/s12311-021-01346-4. PMID: 34817790
Cerebellar Degeneration in Epilepsy: A Systematic Review.
Ibdali M, Hadjivassiliou M, Grünewald RA, Shanmugarajah PD
Int J Environ Res Public Health 2021 Jan 8;18(2) doi: 10.3390/ijerph18020473. PMID: 33435567Free PMC Article
Severe Cerebellar Degeneration and Chiari I Malformation - Speculative pathophysiology based on a systematic review.
Joaquim AF
Rev Assoc Med Bras (1992) 2020 Mar;66(3):375-379. doi: 10.1590/1806-9282.66.3.375. PMID: 32520161
Epidemiology of neurodegenerative diseases in sub-Saharan Africa: a systematic review.
Lekoubou A, Echouffo-Tcheugui JB, Kengne AP
BMC Public Health 2014 Jun 26;14:653. doi: 10.1186/1471-2458-14-653. PMID: 24969686Free PMC Article

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

    Reviews

    Related information

    • 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...