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Disease Profile
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids
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.
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Age of onset
Adult
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.
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.
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.
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.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
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.
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.
Not applicable
Other names (AKA)
CLIPPERS
Categories
Nervous System Diseases
Summary
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a treatable inflammatory disease of the
Symptoms usually develop over weeks or months, and only rarely over days.[1] The most common symptoms of CLIPPERS include uncoordinated walking (gait
The underlying cause of CLIPPERS currently is unclear.[1] There is no one laboratory or
Corticosteroids may first be taken intravenously (by IV) for several days, and then orally. Most people with CLIPPERS recover significantly within weeks of starting corticosteroids. However, depending on symptom severity and how long the disease was present before treatment, some people will not recover completely. Prolonged treatment with corticosteroids or other immunosuppressant medications (for at least one year) usually is necessary to prevent symptoms from recurring or worsening again.[1]
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
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Brain & Spine Foundation
LG01, Lincoln House
Kennington Park
1-3 Brixton Road
London, SW9 6DE United Kingdom
Telephone: 0808 808 1000
E-mail: [email protected]
Website: https://www.brainandspine.org.uk/ -
Encephalitis Society
32 Castlegate
Malton, North Yorkshire YO17 7DT
United Kingdom
Telephone: +44 (0)1653 699599
E-mail: [email protected]
Website: https://www.encephalitis.info/
Social media: Twitter: @encephalitis Facebook: /encephalitissociety -
National Ataxia Foundation
600 Highway 169 South
Suite 1725
Minneapolis, MN 55426
Telephone: +1-763-553-0020
Fax: +1-763-553-0167
E-mail: [email protected]
Website: https://ataxia.org/
Social Networking Websites
- Living with CLIPPERS is a blog about one person's experience of living with this condition.
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.
In-Depth Information
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Click on the link to view a sample search on this topic.
Selected Full-Text Journal Articles
- Pittock SJ, Debruyne J, Krecke KN, Giannini C, van den Ameele J, De Herdt V, et al. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. 2010 Sep;133(9):2626-34.
References
- Zalewski NL, Tobin WO. CLIPPERS. Curr Neurol Neurosci Rep. September, 2017; 17(9):65. https://www.ncbi.nlm.nih.gov/pubmed/28726127.
- Tobin WO, Guo Y, Krecke KN et al. Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. September, 2017; 140(9):2415-2425. https://academic.oup.com/brain/article-abstract/140/9/2415/4096699?redirectedFrom=fulltext.
- Pittock SJ, Debruyne J, Krecke KN, Giannini C, van den Ameele J, De Herdt V, et al. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. Sept 2010; 133(9):2626-34. https://brain.oxfordjournals.org/content/133/9/2626.long.