Paraneoplastic neurological syndrome: An evolving story

Jiraporn Jitprapaikulsan, Pritikanta Paul, Smathorn Thakolwiboon, Shivam Om Mittal, Sean J. Pittock, Divyanshu Dubey

Abstract

Paraneoplastic neurological syndrome (PNS) comprises a group of neurological disorders that result from a misguided immune response to the nervous system triggered by a distant tumor. These disorders frequently manifest before the diagnosis of the underlying neoplasm. Since the first reported case in 1888 by Oppenheim, the knowledge in this area has evolved rapidly. Several classic PNS have been described, such as limbic encephalitis, paraneoplastic cerebellar degeneration, encephalomyelitis, opsoclonus-myoclonus, sensory neuronopathy, Lambert-Eaton Myasthenic syndrome, and chronic gastrointestinal dysmotility. It is now recognized that PNS can have varied nonclassical manifestations that extend beyond the traditional syndromic descriptions. Multiple onconeural antibodies with high specificity for certain tumor types and neurological phenotypes have been discovered over the past 3 decades. Increasing use of immune checkpoint inhibitors (ICIs) has led to increased recognition of neurologic ICI-related adverse events. Some of these resemble PNS. In this article, we review the clinical, oncologic, and immunopathogenic associations of PNS.

Original languageEnglish (US)
Pages (from-to)362-374
Number of pages13
JournalNeuro-Oncology Practice
Volume8
Issue number4
DOIs
StatePublished - Aug 1 2021

Keywords

  • autoimmune neurological syndrome
  • immune checkpoint inhibitor
  • paraneoplastic neurological syndrome

ASJC Scopus subject areas

  • Medicine (miscellaneous)
  • Oncology
  • Neurology

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