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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.
Joubert B, Rostásy K, Honnorat J
Handb Clin Neurol 2018;155:313-332. doi: 10.1016/B978-0-444-64189-2.00021-4. PMID: 29891069
Time Is Cerebellum.
Mitoma H, Manto M, Hampe CS
Cerebellum 2018 Aug;17(4):387-391. doi: 10.1007/s12311-018-0925-6. PMID: 29460203Free 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
Cerebellar ataxia rehabilitation trial in degenerative cerebellar diseases.
Miyai I, Ito M, Hattori N, Mihara M, Hatakenaka M, Yagura H, Sobue G, Nishizawa M; Cerebellar Ataxia Rehabilitation Trialists Collaboration
Neurorehabil Neural Repair 2012 Jun;26(5):515-22. Epub 2011 Dec 2 doi: 10.1177/1545968311425918. PMID: 22140200
Intensive coordinative training improves motor performance in degenerative cerebellar disease.
Ilg W, Synofzik M, Brötz D, Burkard S, Giese MA, Schöls L
Neurology 2009 Dec 1;73(22):1823-30. Epub 2009 Oct 28 doi: 10.1212/WNL.0b013e3181c33adf. PMID: 19864636

Diagnosis

Multiple system atrophy.
Goh YY, Saunders E, Pavey S, Rushton E, Quinn N, Houlden H, Chelban V
Pract Neurol 2023 Jun;23(3):208-221. Epub 2023 Mar 16 doi: 10.1136/pn-2020-002797. PMID: 36927875Free 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
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
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
Cerebellar ataxia rehabilitation trial in degenerative cerebellar diseases.
Miyai I, Ito M, Hattori N, Mihara M, Hatakenaka M, Yagura H, Sobue G, Nishizawa M; Cerebellar Ataxia Rehabilitation Trialists Collaboration
Neurorehabil Neural Repair 2012 Jun;26(5):515-22. Epub 2011 Dec 2 doi: 10.1177/1545968311425918. PMID: 22140200

Therapy

Epidemiology of paraneoplastic neurologic syndromes.
Kadish R, Clardy SL
Handb Clin Neurol 2024;200:57-77. doi: 10.1016/B978-0-12-823912-4.00011-6. PMID: 38494297
Immunological Bases of Paraneoplastic Cerebellar Degeneration and Therapeutic Implications.
Yshii L, Bost C, Liblau R
Front Immunol 2020;11:991. Epub 2020 Jun 2 doi: 10.3389/fimmu.2020.00991. PMID: 32655545Free PMC Article
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
Cerebellar ataxia rehabilitation trial in degenerative cerebellar diseases.
Miyai I, Ito M, Hattori N, Mihara M, Hatakenaka M, Yagura H, Sobue G, Nishizawa M; Cerebellar Ataxia Rehabilitation Trialists Collaboration
Neurorehabil Neural Repair 2012 Jun;26(5):515-22. Epub 2011 Dec 2 doi: 10.1177/1545968311425918. PMID: 22140200
Intensive coordinative training improves motor performance in degenerative cerebellar disease.
Ilg W, Synofzik M, Brötz D, Burkard S, Giese MA, Schöls L
Neurology 2009 Dec 1;73(22):1823-30. Epub 2009 Oct 28 doi: 10.1212/WNL.0b013e3181c33adf. PMID: 19864636

Prognosis

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
Cerebellar ataxia rehabilitation trial in degenerative cerebellar diseases.
Miyai I, Ito M, Hattori N, Mihara M, Hatakenaka M, Yagura H, Sobue G, Nishizawa M; Cerebellar Ataxia Rehabilitation Trialists Collaboration
Neurorehabil Neural Repair 2012 Jun;26(5):515-22. Epub 2011 Dec 2 doi: 10.1177/1545968311425918. PMID: 22140200
Intensive coordinative training improves motor performance in degenerative cerebellar disease.
Ilg W, Synofzik M, Brötz D, Burkard S, Giese MA, Schöls L
Neurology 2009 Dec 1;73(22):1823-30. Epub 2009 Oct 28 doi: 10.1212/WNL.0b013e3181c33adf. PMID: 19864636

Clinical prediction guides

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
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
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
Intensive coordinative training improves motor performance in degenerative cerebellar disease.
Ilg W, Synofzik M, Brötz D, Burkard S, Giese MA, Schöls L
Neurology 2009 Dec 1;73(22):1823-30. Epub 2009 Oct 28 doi: 10.1212/WNL.0b013e3181c33adf. PMID: 19864636
ATM mutations and phenotypes in ataxia-telangiectasia families in the British Isles: expression of mutant ATM and the risk of leukemia, lymphoma, and breast cancer.
Stankovic T, Kidd AM, Sutcliffe A, McGuire GM, Robinson P, Weber P, Bedenham T, Bradwell AR, Easton DF, Lennox GG, Haites N, Byrd PJ, Taylor AM
Am J Hum Genet 1998 Feb;62(2):334-45. doi: 10.1086/301706. PMID: 9463314Free PMC Article

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
Investigating the Clinical Significance and Research Discrepancies of Balance Training in Degenerative Cerebellar Disease: A Systematic Review.
Barbuto S, Kuo SH, Stein J
Am J Phys Med Rehabil 2020 Nov;99(11):989-998. doi: 10.1097/PHM.0000000000001476. PMID: 32467491Free PMC Article

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