Paraneoplastic Neurologic Disorders

Title

Other Names:

PND

Categories:

Hereditary Cancer Syndromes

This disease is grouped under:

Melanoma-associated retinopathy

Summary Summary

Paraneoplastic neurologic disorders are a group of rare degenerative conditions that are triggered by a person's immune system response to a cancerous tumor. Researchers believe these disorders occur when cancer-fighting antibodies or white blood cells known as T cells mistakenly attack normal cells in the nervous system. Paraneoplastic neurologic disorders typically develop after mid-adulthood and are most common in people with lung, ovarian, lymphatic, or breast cancer. Symptoms generally develop over a period of days to weeks and usually occur prior to tumor detection. 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. Paraneoplastic neurologic disorders 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.^[1]^[2] Treatment involves treatment of the underlying tumor, immunotherapy, and supportive therapy.^[3]

Last updated: 6/9/2016

Prognosis Prognosis

The prognosis for people with a paraneoplastic neurologic disorder depends on the type of paraneoplastic disorder and the type of cancer with which they are diagnosed.^[1] There are no cures for paraneoplastic disorders and resulting progressive neurological damage. Prompt treatment of the underlying tumor and immunotherapy may be beneficial in stabilizing a person's neurologic symptoms.^[4]^[2] Less often, treatment of the tumor improves the neurologic symptoms.^[2]

Last updated: 6/9/2016

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Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

International Autoimmune Encephalitis Society
Telephone: 352-527-2470
E-mail: IAES@autoimmune-encephalitis.org
Website: https://autoimmune-encephalitis.org/

Social Networking Websites

The International Autoimmune Encephalitis Society Facebook group offers educational support through this forum.
RareConnect has an online community for patients and families with this condition so they can connect with others and share their experiences living with a rare disease. The project is a joint collaboration between EURORDIS (European Rare Disease Organisation) and NORD (National Organization for Rare Disorders).

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
Eastpointe, MI 48021
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/
American Cancer Society
250 Williams Street NW
Atlanta, GA 30329
Toll-free: 1-800-227-2345
Website: https://www.cancer.org

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Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

The International Autoimmune Encephalitis Society offers patients and families detailed information on diagnosis and treatment.
The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.

Selected Full-Text Journal Articles

Binks SNM, Klein CJ, Waters P, Pittock SJ, Irani SR. LGI1, CASPR2 and related antibodies: a molecular evolution of the phenotypes. J Neurol Neurosurg Psychiatry. 2018 May;89(5):526-534.
McKeonA. Paraneoplastic and other autoimmune disorders of the central nervous system. Neurohospitalist. 2013 Apr; 3(2):53-64.
Jammoul A, Li Y, Rae-Grant A. Autoantibody-mediated encephalitis: Not just paraneoplastic, not just limbic, and not untreatable. Cleveland Clinic Journal of Medicine. 2016 January;83(1):43-53.

GARD Answers GARD Answers

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If the symptoms of paraneoplastic neurologic disorders are caused by cancer, why do the symptoms appear before cancer is diagnosed? What is the prognosis? See answer

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References References

NINDS Paraneoplastic Syndromes Information Page. National Institute of Neurological Disorders and Stroke Website. March 12, 2009; https://www.ninds.nih.gov/Disorders/All-Disorders/Paraneoplastic-Syndromes-Information-Page. Accessed 6/9/2016.
Josep Dalmau, Myrna R Rosenfeld. Overview of paraneoplastic syndromes of the nervous system. UpToDate. October 5, 2015; http://www.uptodate.com/contents/overview-of-paraneoplastic-syndromes-of-the-nervous-system.
Sudheeran Kannoth. Paraneoplastic neurologic syndrome: A practical approach. Annals of Indian Academy of Neurology. Jan-Mar 2012; 15(1):6-12. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3299076/.
Dalmau J, Rosenfeld M. Paraneoplastic neurologic syndromes. In: Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKenna WG. Abeloff's Clinical Oncology, 4th ed. Philadelphia: Churchill Livingstone; 2008; 767-778.