Rare Gastroenterology News

Disease Profile

Paraneoplastic Neurologic Disorders

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

PND

Categories

Hereditary Cancer Syndromes

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]

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

    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

        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

          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

              References

              1. 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.
              2. Josep Dalmau, Myrna R Rosenfeld. Overview of paraneoplastic syndromes of the nervous system. UpToDate. October 5, 2015; https://www.uptodate.com/contents/overview-of-paraneoplastic-syndromes-of-the-nervous-system.
              3. 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/.
              4. 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.