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Paraneoplastic syndromes affecting the spinal cord and dorsal root ganglia

Josep Dalmau, MD, PhD
Myrna R Rosenfeld, MD, PhD
Section Editor
Patrick Y Wen, MD
Deputy Editor
April F Eichler, MD, MPH


Paraneoplastic neurologic syndromes are a heterogeneous group of neurologic disorders associated with systemic cancer and caused by mechanisms other than metastases, metabolic and nutritional deficits, infections, coagulopathy, or side effects of cancer treatment. These syndromes may affect any part of the nervous system from cerebral cortex to neuromuscular junction and muscle, either damaging one area or multiple areas.

Paraneoplastic syndromes affecting the spinal cord will be reviewed here. An overview of paraneoplastic syndromes and other paraneoplastic disorders are discussed separately. (See "Overview of paraneoplastic syndromes of the nervous system" and "Paraneoplastic syndromes affecting peripheral nerve and muscle" and "Paraneoplastic visual syndromes" and "Paraneoplastic cerebellar degeneration" and "Paraneoplastic and autoimmune encephalitis".)


Paraneoplastic necrotizing myelopathy is a rare disorder that occurs in association with several carcinomas and lymphoma [1,2].

At presentation, symptoms usually involve the thoracic portion of the spinal cord, including ascending sensory deficits, sphincter dysfunction, and flaccid or spastic paraplegia, which may evolve to tetraplegia. Back and/or radicular pain are infrequent [1,2]. Symptoms progress over days or weeks and often terminate in respiratory failure and death. CSF examination reveals an elevated protein concentration, usually without pleocytosis. There are no biological markers of this disorder; as a result, a definitive diagnosis cannot be made premortem. The MRI may show contrast enhancement within the spinal cord.

In one report of a patient with paraneoplastic necrotizing myelopathy who responded to treatment with corticosteroids but eventually died of complications of metastatic esophageal cancer, the autopsy confirmed the presence of necrosis of the spinal cord and mild inflammatory infiltrates [3].

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Literature review current through: Oct 2017. | This topic last updated: Jan 19, 2016.
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