Rare Pediatrics News
Disease Profile
Lewy body dementia
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.
#N/A
Age of onset
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ICD-10
#N/A
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)
Lewy body disease; DLB; Diffuse Lewy body disease;
Summary
Lewy body
Symptoms
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
Medical Terms | Other Names |
Learn More:
HPO ID
|
---|---|---|
Percent of people who have these symptoms is not available through HPO | ||
0000006 | ||
Delusions | 0000746 | |
Dementia, progressive
Progressive dementia
[ more ] |
0000726 | |
Fluctuations in consciousness | 0007159 | |
Lewy bodies | 0100315 | |
Parkinsonism | 0001300 | |
Visual hallucinations | 0002367 |
Cause
Although Lewy body dementia usually occurs sporadically, more than one family member can rarely be affected. Studies of these families suggest that there may be a genetic component to the condition in some cases. For example, copy number variants or changes (
Diagnosis
Making a diagnosis for a genetic or rare disease can often be challenging. Healthcare professionals typically look at a person’s medical history, symptoms, physical exam, and laboratory test results in order to make a diagnosis. The following resources provide information relating to diagnosis and testing for this condition. If you have questions about getting a diagnosis, you should contact a healthcare professional.
Testing Resources
- The Genetic Testing Registry (GTR) provides information about the genetic tests for this condition. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.
Treatment
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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Lewy Body Dementia Association
912 Killian Hill Road SW, Suite 202C
Atlanta, GA 30047
Toll-free: 800-539-9767
Telephone: 404-935-6444
Fax: 480-422-5434
E-mail: [email protected]
Website: https://www.lbda.org/ -
Lewy Body Dementia Resource Center (LBDRC)
750 West Broadway Suite 2R
Long Beach, NY 11561
Telephone: 516-218-2026
E-mail: [email protected]
Website: https://lewybodyresourcecenter.org/
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
- Mayo Clinic has an information page on Lewy body dementia.
- MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
- 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 Institute on Aging (NIA) leads a national program of research on the biomedical, social, and behavioral aspects of the aging process; the prevention of age-related diseases and disabilities; and the promotion of a better quality of life for all older Americans. Click on the link to view information on this topic.
In-Depth Information
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
- 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 Lewy body dementia. Click on the link to view a sample search on this topic.
References
- Dementia With Lewy Bodies Information Page. National Institute of Neurological Disorders and Stroke (NINDS). November 2015; https://www.ninds.nih.gov/Disorders/All-Disorders/Dementia-Lewy-Bodies-Information-Page.
- Crystal HA. Dementia With Lewy Bodies. Medscape Reference. April 2014; https://emedicine.medscape.com/article/1135041-overview#a4.
- Lewy Body Disease. MedlinePlus. October 2015; https://www.nlm.nih.gov/medlineplus/lewybodydisease.html.
- Lewy Body Dementia: Information for Patients, Families, and Professionals. National Institute on Aging. September 2015; https://www.nia.nih.gov/alzheimers/publication/lewy-body-dementia/introduction.
- DEMENTIA, LEWY BODY. OMIM. December 2012; https://www.omim.org/entry/127750.
- Farlow MR. Epidemiology, pathology, and pathogenesis of dementia with Lewy bodies. UpToDate. January 2015; https://www.uptodate.com/contents/epidemiology-pathology-and-pathogenesis-of-dementia-with-lewy-bodies.
- Dementia with Lewy body. Orphanet. December 2012; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=1648.
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