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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

Paraneoplastic cerebellar and brainstem disorders.
Abbatemarco JR, Vedeler CA, Greenlee JE
Handb Clin Neurol 2024;200:173-191. doi: 10.1016/B978-0-12-823912-4.00030-X. PMID: 38494276
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
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
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
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

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
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
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
Paraneoplastic cerebellar degeneration.
Vernino S
Handb Clin Neurol 2012;103:215-23. doi: 10.1016/B978-0-444-51892-7.00013-9. PMID: 21827891

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
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
Paraneoplastic cerebellar degeneration associated with cerebellar hypermetabolism: Case report.
Abdulaziz ATA, Yu XQ, Zhang L, Jiang XY, Zhou D, Li JM
Medicine (Baltimore) 2018 Jun;97(24):e10717. doi: 10.1097/MD.0000000000010717. PMID: 29901574Free PMC Article
Anti-Tr antibodies as markers of paraneoplastic cerebellar degeneration and Hodgkin's disease.
Bernal F, Shams'ili S, Rojas I, Sanchez-Valle R, Saiz A, Dalmau J, Honnorat J, Sillevis Smitt P, Graus F
Neurology 2003 Jan 28;60(2):230-4. doi: 10.1212/01.wnl.0000041495.87539.98. PMID: 12552036

Prognosis

Pathogenesis and immunopathology of paraneoplastic disorders.
Quinot V, Höftberger R
Handb Clin Neurol 2024;200:33-54. doi: 10.1016/B978-0-12-823912-4.00027-X. PMID: 38494287
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
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
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 neuropathology of the adult cerebellum.
Koeppen AH
Handb Clin Neurol 2018;154:129-149. doi: 10.1016/B978-0-444-63956-1.00008-4. PMID: 29903436Free PMC Article

Clinical prediction guides

Paraneoplastic Cerebellar Degeneration With P/Q-VGCC vs Yo Autoantibodies.
Winklehner M, Bauer J, Endmayr V, Schwaiger C, Ricken G, Motomura M, Yoshimura S, Shintaku H, Ishikawa K, Tsuura Y, Iizuka T, Yokota T, Irioka T, Höftberger R
Neurol Neuroimmunol Neuroinflamm 2022 Jul;9(4) Epub 2022 Jul 7 doi: 10.1212/NXI.0000000000200006. PMID: 36070310Free PMC Article
Expression of cerebellar degeneration-related proteins CDR2 and CDR2L in human and rat brain tissue.
Raspotnig M, Kråkenes T, Herdlevær I, Haugen M, Vedeler C
J Neuroimmunol 2022 Jan 15;362:577766. Epub 2021 Nov 11 doi: 10.1016/j.jneuroim.2021.577766. PMID: 34823119
Paraneoplastic Cerebellar Degeneration: The Importance of Including CDR2L as a Diagnostic Marker.
Herdlevær I, Haugen M, Mazengia K, Totland C, Vedeler C
Neurol Neuroimmunol Neuroinflamm 2021 Mar;8(2) Epub 2021 Feb 2 doi: 10.1212/NXI.0000000000000963. PMID: 33531379Free PMC Article
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

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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