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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]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVParaneoplastic neurologic syndrome

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Disorder by Site
      - Disorder of nervous system
        Degenerative Disease of Nervous System, Unspecified
        Paraneoplastic neurologic syndrome
        Anti-NMDA receptor encephalitis
        Cancer-associated retinopathy
        Eaton-Lambert syndrome
        Limbic encephalitis
        Myasthenia gravis
        Adult-onset myasthenia gravis
        Juvenile myasthenia gravis
        Neonatal myasthenia gravis
        Transient neonatal myasthenia gravis
        Opsoclonus-myoclonus syndrome
        Paraneoplastic cerebellar degeneration
        Paraneoplastic isolated brainstem encephalitis
        Paraneoplastic polyneuropathy
        Paraneoplastic Subacute Sensory Neuronopathy
        Paraneoplastic sensory ganglionopathy
        Transverse myelitis
        Neuromyelitis optica
        Neuromyelitis optica spectrum disorder with anti-AQP4 antibodies
        Neuromyelitis optica spectrum disorder with anti-MOG antibodies
        Neuromyelitis optica spectrum disorder without anti-MOG and without anti-AQP4 antibodies

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: 39321395 Free 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: 35173717 Free PMC Article

See all (2)

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

Case report: PCA-2-associated encephalitis with different clinical phenotypes: a two-case series and literature review.

Li X, Lang Y, Ma D, Bai J, Shen P, Wang X, Cui L
Front Immunol 2024;15:1431585. Epub 2024 Jul 12 doi: 10.3389/fimmu.2024.1431585. PMID: 39072315 Free PMC Article

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: 37795104 Free PMC Article

CV2/CRMP5-antibody-related Paraneoplastic Neurologic Syndrome Associated with Gastrointestinal Stromal Tumor.

Fukushima H, Shibuya T, Awatsu T, Kurosawa T, Haga K, Murakami T, Serizawa N, Mori A, Hojo M, Yao T, Hattori N, Nagahara A
Intern Med 2022 May 15;61(10):1497-1501. Epub 2021 Oct 19 doi: 10.2169/internalmedicine.8425-21. PMID: 34670904 Free 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

See all (22)

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.

CV2/CRMP5-antibody-related Paraneoplastic Neurologic Syndrome Associated with Gastrointestinal Stromal Tumor.

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

Paraneoplastic disorders of the central and peripheral nervous systems.

Didelot A, Honnorat J
Handb Clin Neurol 2014;121:1159-79. doi: 10.1016/B978-0-7020-4088-7.00078-X. PMID: 24365410

See all (49)

Therapy

Worsening of anti-Hu paraneoplastic neurological syndrome related to anti-PD-1 treatment: Case report and review of literature.

Raibagkar P, Ho D, Gunturu KS, Srinivasan J
J Neuroimmunol 2020 Apr 15;341:577184. Epub 2020 Feb 3 doi: 10.1016/j.jneuroim.2020.577184. PMID: 32058173

(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: 31548813 Free 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

See all (11)

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.

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: 32170042 Free PMC Article

See all (20)

Clinical prediction guides

Clinical characteristics and immunotherapy response in paraneoplastic neurologic syndrome patients with increased number of high-risk antibodies.

Wang G, Chen M, Gao F, Guo M, Li M, He Q, Jiang J, Huang C, Chen X, Xu R
Front Immunol 2024;15:1520493. Epub 2025 Jan 9 doi: 10.3389/fimmu.2024.1520493. PMID: 39850901 Free PMC Article

Comprehensive Analysis of Paraneoplastic Neurologic Syndrome and PNS-CARE Diagnostic Criteria in Clinical Practice.

Paraneoplastic Neurologic Disorders.

Zekeridou A
Continuum (Minneap Minn) 2024 Aug 1;30(4):1021-1051. doi: 10.1212/CON.0000000000001449. PMID: 39088287

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: 32744608 Free PMC Article

Clinical spectrum and diagnostic pitfalls of neurologic syndromes with Ri antibodies.

See all (11)

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: 35075066 Free PMC Article

Clinical spectrum and diagnostic pitfalls of neurologic syndromes with Ri antibodies.

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: 31548813 Free 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

See all (4)

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Paraneoplastic neurologic syndrome

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

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