Paraneoplastic Neuropathies

Srikanth Muppidi, MD; Steven Vernino, MD, PhD, FAAN Peripheral Nervous System Disorders p. 1359-1372 October 2014, Vol.20, No.5 doi: 10.1212/
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Purpose of Review: This article provides an approach to the recognition and management of paraneoplastic neuropathies.

Recent Findings: Paraneoplastic neuropathies may have unique phenotypic presentations, such as sensory neuronopathy, autonomic enteric neuropathy, demyelinating neuropathy, and, rarely, motor neuropathy. Paraneoplastic sensorimotor neuropathy, on the other hand, may be indistinguishable from other common types of axonal polyneuropathy. Certain patterns of neuropathies are commonly seen with different types of cancers, but this relationship is not exclusive and not all patients whose pattern of neuropathy suggests a paraneoplastic disorder have an underlying cancer. In addition to definitive therapy for malignancy, immunomodulatory therapy, such as corticosteroids, IV immunoglobulin (IVIg), or immunosuppressants, may benefit some patients, but there are very few published treatment data for paraneoplastic neuropathies.

Summary: Prompt recognition of paraneoplastic neuropathies may lead to identification and treatment of an occult cancer. Treatment can potentially arrest the progression of neuropathy.

Address correspondence to Dr Srikanth Muppidi, Department of Neurology and Neurological Sciences, Stanford University Medical Center, 300 Pasteur Drive, Stanford, CA 94305, [email protected].

Relationship Disclosure: Dr Muppidi reports no disclosure. Dr Vernino served on the advisory board of Chelsea Therapeutics and has received honorarium for speaking engagements from the American Academy of Neurology and the Texas Neurological Society. Dr Vernino’s institution receives laboratory support from Athena Diagnostics, Inc.

Unlabeled Use of Products/Investigational Use Disclosure: Drs Muppidi and Vernino discuss the unlabeled use of rituximab in the treatment of paraneoplastic neuropathies.

© 2014 American Academy of Neurology