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Causes

By Mayo Clinic staff

A variety of mutations on several different genes have been linked to specific subtypes of frontotemporal dementia. But more than half the people who develop frontotemporal dementia have no family history of dementia.

In some cases of frontotemporal dementia, the affected parts of the brain contain microscopic Pick bodies — abnormal protein-filled structures that develop within brain cells. Frontotemporal dementia was once known as Pick's disease, but now that terminology is reserved for the subtype that actually features these abnormal structures.

References
  1. Ropper AH, et al. Degenerative diseases of the nervous system. In: Ropper AH, et al. Adams and Victor's Principles of Neurology. 9th ed. New York, N.Y.: The McGraw-Hill Companies; 2009. http://www.accessmedicine.com/content.aspx?aID=3639100&searchStr=frontotemporal+dementia. Accessed Nov. 7, 2010.
  2. Simon RP, et al. Disorders of cognitive function. In: Simon RP, et al. Clinical Neurology. 7th ed. New York, N.Y.: The McGraw-Hill Companies; 2009. http://www.accessmedicine.com/content.aspx?aID=5145195&searchStr=frontotemporal+dementia. Accessed Nov. 7, 2010.
  3. Randall C. Frontotemporal dementia: Clinical features and diagnosis. http://www.uptodate.com/home/index.html. Accessed Nov. 7, 2010.
  4. NINDS Frontotemporal dementia information page. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/picks/picks.htm. Accessed Nov. 7, 2010.
  5. Seelaar H, et al. Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review. Journal of Neurology, Neurosurgery and Psychiatry. In press. Accessed Nov. 7, 2010.
  6. Kirshner HS. Frontotemporal dementia and primary progressive aphasia: An update. Current Neurology and Neuroscience Reports. 2010;10:504.
  7. Bird TD, et al. Dementia. In: Fauci AS, et al. Harrison's Principles of Internal Medicine. 17th ed. New York, N.Y.: The McGraw-Hill Companies. 2008. http://www.accessmedicine.com/content.aspx?aID=2904115&searchStr=frontotemporal+dementia. Accessed Nov. 7, 2010.
  8. Shadlen M, et al. Evaluation of cognitive impairment and dementia. http://www.uptodate.com/home/index.html. Accessed Nov. 7, 2010.
  9. Neurological diagnostic tests and procedures. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/misc/diagnostic_tests.htm. Accessed Nov. 7, 2010.
  10. Press D, et al. Treatment of behavioral symptoms related to dementia. http://www.uptodate.com/home/index.html. Accessed Nov. 7, 2010.
  11. Knopman DS (expert opinion), Mayo Clinic, Rochester, Minn. Nov. 14, 2010.
DS00874 Oct. 19, 2011

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