U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Nervous System Paraneoplastic Syndromes

MedGen UID:
155656
•Concept ID:
C0751911
•
Disease or Syndrome
Synonyms: Neurologic Syndrome, Paraneoplastic; Neurologic Syndromes, Paraneoplastic; Paraneoplastic Neurologic Syndrome; Paraneoplastic Neurologic Syndromes; Paraneoplastic Syndromes, Nervous System; Syndrome, Paraneoplastic Neurologic; Syndromes, Paraneoplastic Neurologic

Definition

Degenerative or inflammatory conditions affecting the central or peripheral nervous system that develop in association with a systemic neoplasm without direct invasion by tumor. They may be associated with circulating antibodies that react with the affected neural tissue. (Intern Med 1996 Dec;35(12):925-9) [from MeSH]

Professional guidelines

PubMed

The radiographic diagnosis and treatment of paraneoplastic central nervous system disease.
Glantz MJ, Biran H, Myers ME, Gockerman JP, Friedberg MH
Cancer 1994 Jan 1;73(1):168-75. doi: 10.1002/1097-0142(19940101)73:1<168::aid-cncr2820730129>3.0.co;2-z. PMID: 8275420

Recent clinical studies

Etiology

Paraneoplastic Neurologic Disorders.
Gilligan M, McGuigan C, McKeon A
Curr Neurol Neurosci Rep 2023 Mar;23(3):67-82. Epub 2023 Feb 13 doi: 10.1007/s11910-023-01250-w. PMID: 36781586Free PMC Article
Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes.
Graus F, Vogrig A, Muñiz-Castrillo S, Antoine JG, Desestret V, Dubey D, Giometto B, Irani SR, Joubert B, Leypoldt F, McKeon A, Prüss H, Psimaras D, Thomas L, Titulaer MJ, Vedeler CA, Verschuuren JJ, Dalmau J, Honnorat J
Neurol Neuroimmunol Neuroinflamm 2021 Jul;8(4) Epub 2021 May 18 doi: 10.1212/NXI.0000000000001014. PMID: 34006622Free PMC Article
Paraneoplastic neurological syndromes.
Iorio R, Spagni G, Masi G
Semin Diagn Pathol 2019 Jul;36(4):279-292. Epub 2019 Jun 21 doi: 10.1053/j.semdp.2019.06.005. PMID: 31253539
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 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.
Gilligan M, McGuigan C, McKeon A
Curr Neurol Neurosci Rep 2023 Mar;23(3):67-82. Epub 2023 Feb 13 doi: 10.1007/s11910-023-01250-w. PMID: 36781586Free PMC Article
Paraneoplastic neurological syndromes: a practical approach to diagnosis and management.
Binks S, Uy C, Honnorat J, Irani SR
Pract Neurol 2022 Feb;22(1):19-31. Epub 2021 Sep 11 doi: 10.1136/practneurol-2021-003073. PMID: 34510016
Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes.
Graus F, Vogrig A, Muñiz-Castrillo S, Antoine JG, Desestret V, Dubey D, Giometto B, Irani SR, Joubert B, Leypoldt F, McKeon A, Prüss H, Psimaras D, Thomas L, Titulaer MJ, Vedeler CA, Verschuuren JJ, Dalmau J, Honnorat J
Neurol Neuroimmunol Neuroinflamm 2021 Jul;8(4) Epub 2021 May 18 doi: 10.1212/NXI.0000000000001014. PMID: 34006622Free 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
Rhombencephalitis / brainstem encephalitis.
Jubelt B, Mihai C, Li TM, Veerapaneni P
Curr Neurol Neurosci Rep 2011 Dec;11(6):543-52. doi: 10.1007/s11910-011-0228-5. PMID: 21956758

Therapy

Immune-mediated epilepsy with GAD65 antibodies.
Li X, Guo Q, Zheng Z, Wang X, Liu S
J Neuroimmunol 2020 Apr 15;341:577189. Epub 2020 Feb 14 doi: 10.1016/j.jneuroim.2020.577189. PMID: 32087461
The psychopharmacology of primary and metastatic brain tumors and paraneoplastic syndromes.
Madhusoodanan S, Ting MB, Wilson SY
Handb Clin Neurol 2019;165:269-283. doi: 10.1016/B978-0-444-64012-3.00016-2. PMID: 31727217
Paraneoplastic neurological syndromes in the era of immune-checkpoint inhibitors.
Graus F, Dalmau J
Nat Rev Clin Oncol 2019 Sep;16(9):535-548. doi: 10.1038/s41571-019-0194-4. PMID: 30867573
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
Paraneoplastic neurological syndromes--diagnosis and management.
Blaes F
Curr Pharm Des 2012;18(29):4518-25. doi: 10.2174/138161212802502323. PMID: 22612756

Prognosis

Paraneoplastic Neurologic Disorders.
Gilligan M, McGuigan C, McKeon A
Curr Neurol Neurosci Rep 2023 Mar;23(3):67-82. Epub 2023 Feb 13 doi: 10.1007/s11910-023-01250-w. PMID: 36781586Free PMC Article
Paraneoplastic epilepsy.
Serafini A, Lukas RV, VanHaerents S, Warnke P, Tao JX, Rose S, Wu S
Epilepsy Behav 2016 Aug;61:51-58. Epub 2016 Jun 12 doi: 10.1016/j.yebeh.2016.04.046. PMID: 27304613
Stiff-person syndrome: insights into a complex autoimmune disorder.
Baizabal-Carvallo JF, Jankovic J
J Neurol Neurosurg Psychiatry 2015 Aug;86(8):840-8. Epub 2014 Dec 15 doi: 10.1136/jnnp-2014-309201. PMID: 25511790
Paraneoplastic neurological syndromes.
Graus F, Dalmau J
Curr Opin Neurol 2012 Dec;25(6):795-801. doi: 10.1097/WCO.0b013e328359da15. PMID: 23041955Free PMC Article
Rhombencephalitis / brainstem encephalitis.
Jubelt B, Mihai C, Li TM, Veerapaneni P
Curr Neurol Neurosci Rep 2011 Dec;11(6):543-52. doi: 10.1007/s11910-011-0228-5. PMID: 21956758

Clinical prediction guides

Paraneoplastic Neurologic Disorders.
Gilligan M, McGuigan C, McKeon A
Curr Neurol Neurosci Rep 2023 Mar;23(3):67-82. Epub 2023 Feb 13 doi: 10.1007/s11910-023-01250-w. PMID: 36781586Free PMC Article
Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes.
Graus F, Vogrig A, Muñiz-Castrillo S, Antoine JG, Desestret V, Dubey D, Giometto B, Irani SR, Joubert B, Leypoldt F, McKeon A, Prüss H, Psimaras D, Thomas L, Titulaer MJ, Vedeler CA, Verschuuren JJ, Dalmau J, Honnorat J
Neurol Neuroimmunol Neuroinflamm 2021 Jul;8(4) Epub 2021 May 18 doi: 10.1212/NXI.0000000000001014. PMID: 34006622Free 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
Stiff-person syndrome: insights into a complex autoimmune disorder.
Baizabal-Carvallo JF, Jankovic J
J Neurol Neurosurg Psychiatry 2015 Aug;86(8):840-8. Epub 2014 Dec 15 doi: 10.1136/jnnp-2014-309201. PMID: 25511790
Paraneoplastic neurological syndromes.
Graus F, Dalmau J
Curr Opin Neurol 2012 Dec;25(6):795-801. doi: 10.1097/WCO.0b013e328359da15. PMID: 23041955Free PMC Article

Recent systematic reviews

Autoimmune and paraneoplastic neurological disorders: A review of relevant neuroimaging findings.
Akkus S, Elkhooly M, Amatya S, Shrestha K, Sharma K, Kagzi Y, Khan E, Gupta R, Piquet AL, Jaiswal S, Wen S, Tapia M, Samant R, Sista SR, Sriwastava S
J Neurol Sci 2023 Nov 15;454:120830. Epub 2023 Oct 9 doi: 10.1016/j.jns.2023.120830. PMID: 37856996
Paraneoplastic neurological syndromes associated with renal or bladder cancer: case series and PRISMA-IPD systematic review.
Villagrán-García M, Muñiz-Castrillo S, Ciano-Petersen NL, Vogrig A, Farina A, Villard M, Psimaras D, Alentorn A, Gonçalves D, Fabien N, Rogemond V, Joubert B, Honnorat J
J Neurol 2023 Jan;270(1):283-299. Epub 2022 Sep 1 doi: 10.1007/s00415-022-11356-9. PMID: 36050418
Autoimmune paraneoplastic syndromes associated to lung cancer: A systematic review of the literature Part 4: Neurological paraneoplastic syndromes, involving the peripheral nervous system and the neuromuscular junction and muscles.
Ruelle L, Bentea G, Sideris S, El Koulali M, Holbrechts S, Lafitte JJ, Grigoriu B, Sculier C, Meert AP, Durieux V, Berghmans T, Sculier JP
Lung Cancer 2017 Sep;111:150-163. Epub 2017 Jul 25 doi: 10.1016/j.lungcan.2017.07.025. PMID: 28838388
Paraneoplastic syndromes associated with gynecological cancers: A systematic review.
Viau M, Renaud MC, Grégoire J, Sebastianelli A, Plante M
Gynecol Oncol 2017 Sep;146(3):661-671. Epub 2017 Jun 24 doi: 10.1016/j.ygyno.2017.06.025. PMID: 28655412
Imaging Workup of Suspected Classical Paraneoplastic Neurological Syndromes: A Systematic Review and Retrospective Analysis of (18)F-FDG-PET-CT.
Sundermann B, Schröder JB, Warnecke T, Heindel W, Schäfers M, Weckesser M, Buerke B
Acad Radiol 2017 Oct;24(10):1195-1202. Epub 2017 May 24 doi: 10.1016/j.acra.2017.03.022. PMID: 28551401

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • MeSH
      Related Medical Subject Headings
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...