Paraneoplastic Disorders of the Nervous System

Eoin P. Flanagan, MBBCh Neuro-oncology p. 1602-1628 December 2020, Vol.26, No.6 doi: 10.1212/CON.0000000000000941
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PURPOSE OF REVIEW This article reviews paraneoplastic neurologic disorders and includes an overview of the diagnostic approach, the role of autoantibody testing, the pathophysiology of these disorders, and treatment approaches. This article also provides an overview of the emerging clinical scenarios in which paraneoplastic and autoimmune neurologic disorders may occur.

RECENT FINDINGS The number of autoantibodies associated with paraneoplastic neurologic disorders has rapidly expanded over the past 2 decades. These discoveries have improved our ability to diagnose patients with these disorders and have provided insight into their pathogenesis. It is now recognized that these antibodies can be broadly divided into two major categories based on the location of the target antigen: intracellular and cell surface/synaptic. Antibodies to intracellular antigens are almost always accompanied by cancer, respond less well to immunotherapy, and have an unfavorable outcome. In contrast, antibodies to cell surface or synaptic targets are less often accompanied by cancer, generally respond well to immunotherapy, and have a good prognosis. Paraneoplastic and autoimmune neurologic disorders are now being recognized in novel settings, including their occurrence as an immune-related adverse effect of immune checkpoint inhibitor treatment for cancer.

SUMMARY This article discusses when to suspect a paraneoplastic neurologic syndrome, the diagnostic utility and pitfalls of neural autoantibody testing, how to best detect the underlying tumor, and the treatment approach that involves combinations of antineoplastic treatments, immunosuppressants, and supportive/symptomatic treatments.

Address correspondence to Dr Eoin P. Flanagan, Mayo Clinic, Department of Neurology, 200 First St SW, Rochester, MN 55905, [email protected].

RELATIONSHIP DISCLOSURE: Dr Flanagan has received research/grant support from MedImmune/Viela Bio.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Flanagan discusses the unlabeled/investigational use of azathioprine, corticosteroids, cyclophosphamide, IV immunoglobulin, methotrexate, mycophenolate mofetil, plasma exchange, rituximab, and tocilizumab for the treatment of paraneoplastic disorders of the nervous system.

© 2020 American Academy of Neurology.