Paraneoplastic Neurologic Disorders

Author:

Gilligan Michael,McGuigan Christopher,McKeon AndrewORCID

Abstract

Abstract Purpose of Review To provide an overview and highlight recent updates in the field of paraneoplastic neurologic disorders. Recent Findings The prevalence of paraneoplastic neurologic disorders is greater than previously reported and the incidence has been rising over time, due to improved recognition in the era of antibody biomarkers. Updated diagnostic criteria that are broadly inclusive and also contain diagnostic risk for clinical presentations (high and intermediate) and diagnostic antibodies (high, intermediate, and low) have replaced the original 2004 criteria. Antibody biomarkers continue to be characterized (e.g., KLHL-11 associated with seminoma in men with brainstem encephalitis). Some paraneoplastic antibodies also provide insight into likely immunotherapy response and prognosis. The rise of immune checkpoint inhibitors as cancer therapeutics has been associated with newly observed immune-mediated adverse effects including paraneoplastic neurological disorders. The therapeutic approach to paraneoplastic neurologic disorders is centered around cancer care and trials of immune therapy. Summary The field of paraneoplastic neurologic disorders continues to be advanced by the identification of novel antibody biomarkers which have diagnostic utility, and give insight into likely treatment responses and outcomes.

Funder

NINDS

ICAT

Publisher

Springer Science and Business Media LLC

Subject

Neurology (clinical),General Neuroscience

Reference140 articles.

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3. Lai M, Hughes EG, Peng X, Zhou L, Gleichman AJ, Shu H, et al. AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Ann Neurol. 2009;65:424–34. https://doi.org/10.1002/ana.21589.

4. • Fang B, McKeon A, Hinson SR, Kryzer TJ, Pittock SJ, Aksamit AJ, et al. Autoimmune glial fibrillary acidic protein astrocytopathy: a novel meningoencephalomyelitis. JAMA Neurol. 2016;73:1297–307. https://doi.org/10.1001/jamaneurol.2016.2549. Although more often an idiopathic autoimmune disorder, GFAP astrocytopathy can occur as a paraneoplastic disorder in approximately one third of cases.

5. •• Vogrig A, Gigli GL, Segatti S, Corazza E, Marini A, Bernardini A, et al. Epidemiology of paraneoplastic neurological syndromes: a population-based study. J Neurol. 2020;267:26–35. https://doi.org/10.1007/s00415-019-09544-1. The first epidemiological study of paraneoplastic neurologic disorders which highlighted a much higher prevalence of these disorders than previously reported.

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