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Paraneoplastic cerebellar degeneration

MedGen UID:
98273
•Concept ID:
C0393534
•
Disease or Syndrome
Synonyms: Cerebellar Degeneration, Paraneoplastic; Cerebellar Syndrome, Paraneoplastic; Cerebellar Syndromes, Paraneoplastic; Degeneration, Paraneoplastic Cerebellar; Paraneoplastic Cerebellar Degeneration; Paraneoplastic Cerebellar Syndrome; Paraneoplastic Cerebellar Syndromes
SNOMED CT: Paraneoplastic cerebellar degeneration (192877007); Cerebellar ataxia due to neoplasia (192877007)
 
Monarch Initiative: MONDO:0044877

Definition

A rare, immune-mediated disorder characterized by cerebellar degeneration due to the presence of an often undetected malignancy (usually carcinoma or lymphoma) in an anatomic site other than the cerebellum. Signs and symptoms include progressive ataxia, dysarthria, and nystagmus. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVParaneoplastic cerebellar degeneration

Professional guidelines

PubMed

Treatment of Primary Autoimmune Cerebellar Ataxia with Mycophenolate.
Hadjivassiliou M, Grunewald RA, Shanmugarajah PD, Sarrigiannis PG, Zis P, Skarlatou V, Hoggard N
Cerebellum 2020 Oct;19(5):680-684. doi: 10.1007/s12311-020-01152-4. PMID: 32524518Free 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
Updates in the Diagnosis and Treatment of Paraneoplastic Neurologic Syndromes.
Grativvol RS, Cavalcante WCP, Castro LHM, Nitrini R, Simabukuro MM
Curr Oncol Rep 2018 Nov 10;20(11):92. doi: 10.1007/s11912-018-0721-y. PMID: 30415318

Recent clinical studies

Etiology

Updates in the Diagnosis and Treatment of Paraneoplastic Neurologic Syndromes.
Grativvol RS, Cavalcante WCP, Castro LHM, Nitrini R, Simabukuro MM
Curr Oncol Rep 2018 Nov 10;20(11):92. doi: 10.1007/s11912-018-0721-y. PMID: 30415318
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
Parkinsonism & related disorders. Ataxias.
Klockgether T
Parkinsonism Relat Disord 2007;13 Suppl 3:S391-4. doi: 10.1016/S1353-8020(08)70036-1. PMID: 18267270
Cancer-associated neuromusculoskeletal syndromes. Recognizing the rheumatic-neoplastic connection.
Marmur R, Kagen L
Postgrad Med 2002 Apr;111(4):95-8, 101-2. doi: 10.3810/pgm.2002.04.1162. PMID: 11985137

Diagnosis

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
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 opsoclonus–myoclonus syndrome.
Sahu JK, Prasad K
Pract Neurol 2011 Jun;11(3):160-6. doi: 10.1136/practneurol-2011-000017. PMID: 21551110
Navigating the paraneoplastic neurological syndromes.
Giannopoulou C
Eur J Nucl Med Mol Imaging 2003 Mar;30(3):333-8. doi: 10.1007/s00259-002-1005-0. PMID: 12722733
Anti-CV2 autoantibodies and paraneoplastic neurological syndromes.
Rogemond V, Honnorat J
Clin Rev Allergy Immunol 2000 Aug;19(1):51-9. doi: 10.1385/CRIAI:19:1:51. PMID: 11064826

Therapy

CD8 T-cell-mediated cerebellitis directed against Purkinje cell antigen after ipilimumab for small cell lung cancer.
Hardwick M, Nolan L, Nicoll JAR, Jogai S, Arriola E, Joseph-Pietras D, Norman J, Ottensmeier CHH, Galea I
Neuropathol Appl Neurobiol 2022 Feb;48(2):e12755. Epub 2021 Aug 8 doi: 10.1111/nan.12755. PMID: 34309064
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
Advances in Therapies of Cerebellar Disorders: Immune-mediated Ataxias.
Hadjivassiliou M
CNS Neurol Disord Drug Targets 2019;18(6):423-431. doi: 10.2174/1871527317666171221110548. PMID: 29268693
Gross cerebellar paraneoplastic neurological disorder in a patient with an occult breast cancer.
Poudel CK, Achar KN
BMJ Case Rep 2013 Apr 16;2013 doi: 10.1136/bcr-2012-008208. PMID: 23595173Free PMC Article
Positional periodic alternating vertical nystagmus with PCA-Tr antibodies in Hodgkin lymphoma.
Eggers SD, Pittock SJ, Shepard NT, Habermann TM, Neff BA, Klebig RR
Neurology 2012 May 29;78(22):1800-2. Epub 2012 May 16 doi: 10.1212/WNL.0b013e3182583085. PMID: 22592362Free PMC Article

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
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
T cells presenting viral antigens or autoantigens induce cytotoxic T cell anergy.
Blachère NE, Orange DE, Gantman EC, Santomasso BD, Couture GC, Ramirez-Montagut T, Fak J, O'Donovan KJ, Ru Z, Parveen S, Frank MO, Moore MJ, Darnell RB
JCI Insight 2017 Nov 2;2(21) doi: 10.1172/jci.insight.96173. PMID: 29093272Free PMC Article
Unusual paraneoplastic neurological syndrome secondary to a well differentiated pancreatic neuroendocrine tumor: a case report and review of the literature.
Brizzi MP, Sonetto C, Tampellini M, Di Maio M, Volante M, Scagliotti GV
BMC Cancer 2015 Nov 18;15:914. doi: 10.1186/s12885-015-1923-4. PMID: 26582328Free PMC Article
Paraneoplastic syndromes affecting the central nervous system.
Posner JB, Dalmau JO
Annu Rev Med 1997;48:157-66. doi: 10.1146/annurev.med.48.1.157. PMID: 9046952

Clinical prediction guides

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
Immune-mediated acquired ataxias.
Hadjivassiliou M
Handb Clin Neurol 2012;103:189-99. doi: 10.1016/B978-0-444-51892-7.00011-5. PMID: 21827889
Detection of anti-brain serum antibodies using a semi-quantitative immunohistological method.
Boscolo S, Passoni M, Baldas V, Cancelli I, Hadjivassiliou M, Ventura A, Tongiorgi E
J Immunol Methods 2006 Feb 20;309(1-2):139-49. Epub 2006 Jan 4 doi: 10.1016/j.jim.2005.11.020. PMID: 16413571
Paraneoplastic antibodies detected by isoelectric focusing of cerebrospinal fluid and serum.
Storstein A, Monstad SE, Honnorat J, Vedeler CA
J Neuroimmunol 2004 Oct;155(1-2):150-4. doi: 10.1016/j.jneuroim.2004.05.014. PMID: 15342206
Paraneoplastic cerebellar disease. Remission with excision of the primary tumor.
Kearsley JH, Johnson P, Halmagyi GM
Arch Neurol 1985 Dec;42(12):1208-10. doi: 10.1001/archneur.1985.04060110090026. PMID: 4062621

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
Clinical spectrum and diagnostic pitfalls of neurologic syndromes with Ri antibodies.
Simard C, Vogrig A, Joubert B, Muñiz-Castrillo S, Picard G, Rogemond V, Ducray F, Berzero G, Psimaras D, Antoine JC, Desestret V, Honnorat J
Neurol Neuroimmunol Neuroinflamm 2020 May;7(3) Epub 2020 Mar 13 doi: 10.1212/NXI.0000000000000699. PMID: 32170042Free PMC Article

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