National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Frontotemporal Degeneration is not the name you expected.
Frontotemporal degeneration is a group of varied disorders that are characterized by neurodegenerative changes that affect the brain. The clinical presentation of frontotemporal degeneration is diverse. Affected individuals can experience gradual changes in their behavior and personality, and may have difficulties in thinking and communicating effectively. The progression and the specific symptoms that develop can vary from one person to another. Generally, the clinical symptoms of these disorders can be broadly grouped into three categories which display changes in behavior, language and/or motor function. Frontotemporal degeneration is caused by progressive damage and loss of nerve cells in the frontal and temporal lobes of the brain. In most people, this is accompanied by a buildup of one or the other of two proteins, tau or TDP-43. In FTD these proteins are misfolded (misshapen) which leads to their inappropriate buildup within brain cells and so interfering with or disrupting the normal function of these cells. The FTD clinical subtypes can also be classified as ‘tauopathies' or TDP43-opathies, depending on which misfolded protein accumulates in the brain. In about 10% of cases, a third protein, FUS, accumulates instead of tau or TDP43. The accumulation of tau protein or TDP-43 protein can also be observed in other neurological disorders.
Although once used more broadly, the term Pick's disease (after Dr. Arnold Pick, who first described it in 1892) is now reserved for a specific subtype of frontotemporal degeneration associated with a kind of abnormal tau protein known as 3R tau. In Pick's disease, the clumps of 3R tau are found in distinctive round, silver-staining inclusions known as Pick bodies.
There are many different terms and names that have been used to describe frontotemporal degeneration (FTD), also referred to as frontotemporal dementia. These names can be very confusing. Frontotemporal lobar degeneration is a general term that describes a group of disorders based on their neuropathology that cause damage and dysfunction of the frontal and temporal lobes of the brain. This is reflected in the spectrum of clinical subtypes listed above, which encompass behavioral, language and/or motor predominant decline in the ability to function normally. A clinical subtype refers to the symptoms that occur earliest and most prominently in a person.
Association for Frontotemporal Degeneration (AFTD)
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Genetic and Rare Diseases (GARD) Information Center
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- Fax: (301)251-4911
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NIH/National Institute of Neurological Disorders and Stroke
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- Tel: (800)352-9424
- Website: http://www.ninds.nih.gov/
NIH/National Institute on Aging
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- Website: https://www.nia.nih.gov/
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
Last Updated: 6/13/2016
Copyright 2016 National Organization for Rare Disorders, Inc.
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