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Paraneoplastic neurologic syndrome

MedGen UID:
155656
•Concept ID:
C0751911
•
Disease or Syndrome
Synonyms: Nervous System Paraneoplastic Syndromes; Neurologic Syndrome, Paraneoplastic; Neurologic Syndromes, Paraneoplastic; Paraneoplastic Neurologic Syndrome; Paraneoplastic Neurologic Syndromes; Paraneoplastic Syndromes, Nervous System; Syndrome, Paraneoplastic Neurologic; Syndromes, Paraneoplastic Neurologic
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018215
Orphanet: ORPHA36388

Definition

Paraneoplastic neurological syndromes (PNS) can be defined as remote effects of cancer that are not caused by the tumor and its metastasis, or by infection, ischemia or metabolic disruptions. [from ORDO]

Professional guidelines

PubMed

Comprehensive Analysis of Paraneoplastic Neurologic Syndrome and PNS-CARE Diagnostic Criteria in Clinical Practice.
Zhao-Fleming H, Rezk M, Shah S, Gupta P, Zekeridou A, Flanagan EP, Pittock SJ, McKeon A, Dubey D
Neurol Neuroimmunol Neuroinflamm 2024 Dec;11(6):e200316. Epub 2024 Sep 25 doi: 10.1212/NXI.0000000000200316. PMID: 39321395Free PMC Article
Evaluation of the Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes in China.
Cai MT, Qiao S, Lai QL, Zheng Y, Yang F, Fang GL, Shen CH, Zhang YX, Ding MP
Front Immunol 2022;13:790400. Epub 2022 Jan 31 doi: 10.3389/fimmu.2022.790400. PMID: 35173717Free PMC Article

Recent clinical studies

Etiology

Paraneoplastic Neurologic Disorders.
Zekeridou A
Continuum (Minneap Minn) 2024 Aug 1;30(4):1021-1051. doi: 10.1212/CON.0000000000001449. PMID: 39088287
Clinical approach to diagnosis of paraneoplastic neurologic syndromes.
Graus F
Handb Clin Neurol 2024;200:79-96. doi: 10.1016/B978-0-12-823912-4.00007-4. PMID: 38494298
Paraneoplastic neurologic syndrome and autoantibody accompaniments of germ cell tumors.
Hammami MB, Rezk M, Dubey D
Handb Clin Neurol 2024;200:431-445. doi: 10.1016/B978-0-12-823912-4.00001-3. PMID: 38494295
Identification of SKOR2 IgG as a novel biomarker of paraneoplastic neurologic syndrome.
Rezk M, Pittock SJ, Kapadia RK, Knight AM, Guo Y, Gupta P, LaFrance-Corey RG, Zekeridou A, McKeon A, Dasari S, Mills JR, Dubey D
Front Immunol 2023;14:1243946. Epub 2023 Sep 18 doi: 10.3389/fimmu.2023.1243946. PMID: 37795104Free PMC Article
Immune-mediated disorders.
Höftberger R, Lassmann H
Handb Clin Neurol 2017;145:285-299. doi: 10.1016/B978-0-12-802395-2.00020-1. PMID: 28987176

Diagnosis

Paraneoplastic Neurologic Disorders.
Zekeridou A
Continuum (Minneap Minn) 2024 Aug 1;30(4):1021-1051. doi: 10.1212/CON.0000000000001449. PMID: 39088287
Identification of SKOR2 IgG as a novel biomarker of paraneoplastic neurologic syndrome.
Rezk M, Pittock SJ, Kapadia RK, Knight AM, Guo Y, Gupta P, LaFrance-Corey RG, Zekeridou A, McKeon A, Dasari S, Mills JR, Dubey D
Front Immunol 2023;14:1243946. Epub 2023 Sep 18 doi: 10.3389/fimmu.2023.1243946. PMID: 37795104Free PMC Article
Paraneoplastic Disorders of the Nervous System.
Flanagan EP
Continuum (Minneap Minn) 2020 Dec;26(6):1602-1628. doi: 10.1212/CON.0000000000000941. PMID: 33273174
Imaging Review of Paraneoplastic Neurologic Syndromes.
Madhavan AA, Carr CM, Morris PP, Flanagan EP, Kotsenas AL, Hunt CH, Eckel LJ, Lindell EP, Diehn FE
AJNR Am J Neuroradiol 2020 Dec;41(12):2176-2187. Epub 2020 Oct 22 doi: 10.3174/ajnr.A6815. PMID: 33093137Free PMC Article
Immune-mediated disorders.
Höftberger R, Lassmann H
Handb Clin Neurol 2017;145:285-299. doi: 10.1016/B978-0-12-802395-2.00020-1. PMID: 28987176

Therapy

An autoimmune-based, paraneoplastic neurologic syndrome following checkpoint inhibition and concurrent radiotherapy for merkel cell carcinoma: case report.
Sherry AD, Bezzerides M, Khattab MH, Luo G, Ancell KK, Kirschner AN
Strahlenther Onkol 2020 Jul;196(7):664-670. Epub 2020 Jan 31 doi: 10.1007/s00066-020-01582-3. PMID: 32006066Free PMC Article
Increased frequency of anti-Ma2 encephalitis associated with immune checkpoint inhibitors.
Vogrig A, Fouret M, Joubert B, Picard G, Rogemond V, Pinto AL, Muñiz-Castrillo S, Roger M, Raimbourg J, Dayen C, Grignou L, Pallix-Guyot M, Lannoy J, Ducray F, Desestret V, Psimaras D, Honnorat J
Neurol Neuroimmunol Neuroinflamm 2019 Nov;6(6) Epub 2019 Aug 7 doi: 10.1212/NXI.0000000000000604. PMID: 31454760Free PMC Article
Paraneoplastic neurologic syndrome in the PNS Euronetwork database: a European study from 20 centers.
Giometto B, Grisold W, Vitaliani R, Graus F, Honnorat J, Bertolini G; PNS Euronetwork
Arch Neurol 2010 Mar;67(3):330-5. doi: 10.1001/archneurol.2009.341. PMID: 20212230
Paraneoplastic neurologic syndrome associated with occult breast cancer: a case report and review of literature.
Altaha R, Abraham J
Breast J 2003 Sep-Oct;9(5):417-9. doi: 10.1046/j.1524-4741.2003.09514.x. PMID: 12968965
A review of the therapy of paraneoplastic neurologic syndromes.
Das A, Hochberg FH, McNelis S
J Neurooncol 1999 Jan;41(2):181-94. doi: 10.1023/a:1006133102928. PMID: 10222439

Prognosis

Paraneoplastic Neurologic Disorders.
Zekeridou A
Continuum (Minneap Minn) 2024 Aug 1;30(4):1021-1051. doi: 10.1212/CON.0000000000001449. PMID: 39088287
Paraneoplastic neurologic syndrome and autoantibody accompaniments of germ cell tumors.
Hammami MB, Rezk M, Dubey D
Handb Clin Neurol 2024;200:431-445. doi: 10.1016/B978-0-12-823912-4.00001-3. PMID: 38494295
Identification of SKOR2 IgG as a novel biomarker of paraneoplastic neurologic syndrome.
Rezk M, Pittock SJ, Kapadia RK, Knight AM, Guo Y, Gupta P, LaFrance-Corey RG, Zekeridou A, McKeon A, Dasari S, Mills JR, Dubey D
Front Immunol 2023;14:1243946. Epub 2023 Sep 18 doi: 10.3389/fimmu.2023.1243946. PMID: 37795104Free PMC Article
Paraneoplastic Disorders of the Nervous System.
Flanagan EP
Continuum (Minneap Minn) 2020 Dec;26(6):1602-1628. doi: 10.1212/CON.0000000000000941. PMID: 33273174
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

Clinical prediction guides

Comprehensive Analysis of Paraneoplastic Neurologic Syndrome and PNS-CARE Diagnostic Criteria in Clinical Practice.
Zhao-Fleming H, Rezk M, Shah S, Gupta P, Zekeridou A, Flanagan EP, Pittock SJ, McKeon A, Dubey D
Neurol Neuroimmunol Neuroinflamm 2024 Dec;11(6):e200316. Epub 2024 Sep 25 doi: 10.1212/NXI.0000000000200316. PMID: 39321395Free PMC Article
Paraneoplastic Neurologic Disorders.
Zekeridou A
Continuum (Minneap Minn) 2024 Aug 1;30(4):1021-1051. doi: 10.1212/CON.0000000000001449. PMID: 39088287
Evaluation of the Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes in China.
Cai MT, Qiao S, Lai QL, Zheng Y, Yang F, Fang GL, Shen CH, Zhang YX, Ding MP
Front Immunol 2022;13:790400. Epub 2022 Jan 31 doi: 10.3389/fimmu.2022.790400. PMID: 35173717Free PMC Article
Expanded Clinical Phenotype, Oncological Associations, and Immunopathologic Insights of Paraneoplastic Kelch-like Protein-11 Encephalitis.
Dubey D, Wilson MR, Clarkson B, Giannini C, Gandhi M, Cheville J, Lennon VA, Eggers S, Devine MF, Mandel-Brehm C, Kryzer T, Hinson SR, Khazaie K, Hales C, Kattah J, Pavelko KD, Andrews P, Eaton JE, Jitprapaikulsan J, Mills JR, Flanagan EP, Zekeridou A, Leibovich B, Fryer J, Torre M, Kaufman C, Thoreson JB, Sagen J, Linnoila JJ, DeRisi JL, Howe CL, McKeon A, Pittock SJ
JAMA Neurol 2020 Nov 1;77(11):1420-1429. doi: 10.1001/jamaneurol.2020.2231. PMID: 32744608Free PMC Article
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

Recent systematic reviews

Myasthenia Gravis and Myeloproliferative Neoplasms - Mere Association or Paraneoplastic Neurologic Syndrome: A Mini-Review.
Sasi S, Mohamed M, Chitrambika P, Yassin M
Acta Biomed 2022 Jan 19;92(6):e2021437. doi: 10.23750/abm.v92i6.12180. PMID: 35075066Free PMC Article
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
(18) F-Fluorodeoxyglucose positron-emission tomography for the investigation of malignancy in patients with suspected paraneoplastic neurologic syndromes and negative or indeterminate conventional imaging: a retrospective analysis of the Ontario PET Access Program, with systematic review and meta-analysis.
Harlos C, Metser U, Poon R, MacCrostie P, Mason W
Curr Oncol 2019 Aug;26(4):e458-e465. Epub 2019 Aug 1 doi: 10.3747/co.26.4583. PMID: 31548813Free PMC Article
A review of the therapy of paraneoplastic neurologic syndromes.
Das A, Hochberg FH, McNelis S
J Neurooncol 1999 Jan;41(2):181-94. doi: 10.1023/a:1006133102928. PMID: 10222439

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