Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Mar 6;9:F1000 Faculty Rev-167.
doi: 10.12688/f1000research.21309.1. eCollection 2020.

Paraneoplastic Diseases of the Central Nervous System

Free PMC article

Paraneoplastic Diseases of the Central Nervous System

Jonathan Galli et al. F1000Res. .
Free PMC article


Paraneoplastic neurological syndromes are nonmetastatic complications of malignancy secondary to immune-mediated neuronal dysfunction or death. Pathogenesis may occur from cell surface binding of antineuronal antibodies leading to dysfunction of the target protein, or from antibodies binding against intracellular antigens which ultimately leads to cell death. There are several classical neurological paraneoplastic phenotypes including subacute cerebellar degeneration, limbic encephalitis, encephalomyelitis, and dorsal sensory neuropathy. The patient's clinical presentations may be suggestive to the treating clinician as to the specific underlying paraneoplastic antibody. Specific antibodies often correlate with the specific underlying tumor type, and malignancy screening is essential in all patients with paraneoplastic neurological disease. Prompt initiation of immunotherapy is essential in the treatment of patients with paraneoplastic neurological disease, often more effective in cell surface antibodies in comparison to intracellular antibodies, as is removal of the underlying tumor.

Keywords: antibody-mediated; immunotherapy; neurological; paraneoplastic.

Conflict of interest statement

No competing interests were disclosed.No competing interests were disclosed.No competing interests were disclosed.

Similar articles

See all similar articles


    1. Furneaux HM, Rosenblum MK, Dalmau J, et al. : Selective expression of Purkinje-cell antigens in tumor tissue from patients with paraneoplastic cerebellar degeneration. N Engl J Med. 1990;322(26):1844–51. 10.1056/NEJM199006283222604 - DOI - PubMed
    1. Dropcho EJ, Furneaux H, Chen YT, et al. : Expression of the CDR brain protein by tumors from patients with paraneoplastic cerebellar degeneration. Ann Neurol. 1988;24:121–2.
    1. Manley GT, Smitt PS, Dalmau J, et al. : Hu antigens: Reactivity with hu antibodies, tumor expression, and major immunogenic sites. Ann Neurol. 1995;38(1):102–10. 10.1002/ana.410380117 - DOI - PubMed
    1. Dalmau J, Tüzün E, Wu HY, et al. : Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61(1):25–36. 10.1002/ana.21050 - DOI - PMC - PubMed
    2. F1000 Recommendation

    1. Wilkinson PC, Zeromski J: Immunofluorescent detection of antibodies against neurones in sensory carcinomatous neuropathy. Brain. 1965;88(3):529–83. 10.1093/brain/88.3.529 - DOI - PubMed

Grant support

This research was supported by a Merit Review award from the US Department of Veterans Affairs (JEG).

LinkOut - more resources