NINDS Paraneoplastic Syndromes Information Page

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What are Paraneoplastic Syndromes?

Paraneoplastic syndromes are a group of rare degenerative disorders that are triggered by a person's immune system response to a neoplasm, or cancerous tumor. Neurologic paraneoplastic syndromes are believed to occur when cancer-fighting antibodies or white blood cells known as T cells mistakenly attack normal cells in the nervous system. These disorders typically affect middle-aged to older persons and are most common in persons with lung, ovarian, lymphatic, or breast cancer. Neurologic symptoms generally develop over a period of days to weeks and usually occur prior to tumor detection, which can complicate diagnosis. These symptoms may include difficulty in walking and/or swallowing, loss of muscle tone, loss of fine motor coordination, slurred speech, memory loss, vision problems, sleep disturbances, dementia, seizures, sensory loss in the limbs, and vertigo. Neurologic paraneoplastic syndromes include Lambert-Eaton myasthenic syndrome, stiff-person syndrome, encephalomyelitis (inflammation of the brain and spinal cord), myasthenia gravis, cerebellar degeneration, limbic and/or brainstem encephalitis, neuromyotonia, and opsoclonus (involving eye movement) and sensory neuropathy.

Is there any treatment?

The cancer is treated first, followed by efforts to decrease the autoimmune response - either through steroids such as cortisone or prednisone, high-dose intravenous immunoglobulin, or irradiation. Plasmapheresis, a process that cleanses antibodies from the blood, may ease symptoms in patients with paraneoplastic disorders that affect the peripheral nervous system. Speech and physical therapy may help patients regain some functions.

What is the prognosis?

There are no cures for paraneoplastic syndromes and resulting progressive neurological damage. Generally the presence of cancer determines the diagnosis.

What research is being done?

Research on neurologic paraneoplastic syndromes is aimed at enhancing scientific understanding of them and evaluating new therapeutic interventions. Researchers are seeking to learn what causes the autoimmune response in these disorders. Studies are directed toward developing assays that detect different types of antibodies. Scientists also hope to develop animal models for these diseases, which may be used to determine effective treatment strategies.

NIH Patient Recruitment for Paraneoplastic Syndromes Clinical Trials

Organizations

American Autoimmune Related Diseases Association
22100 Gratiot Avenue
Eastpointe, MI   48201-2227
aarda@aarda.org
http://www.aarda.org
Tel: 586-776-3900 800-598-4668
Fax: 586-776-3903

National Cancer Institute (NCI)
National Institutes of Health, DHHS
6116 Executive Boulevard, Ste. 3036A, MSC 8322
Bethesda, MD   20892-8322
cancergovstaff@mail.nih.gov
http://cancer.gov
Tel: 800-4-CANCER (422-6237) 800-332-8615 (TTY)

American Cancer Society
National Home Office
250 Williams Street, NW
Atlanta, GA   30303-1002
http://www.cancer.org
Tel: 800-ACS-2345 (227-2345)

National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT   06813-1968
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291



Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892



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Last updated February 14, 2007