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FRONTOTEMPORAL DEMENTIA

About FTD

Frontotemporal dementia (FTD) is a devastating form of early-onset dementia and is characterized by a rapid decline in executive function, behavior and/or language,¹ and typically leads to death within seven to 13 years of symptom onset and three to 10 years from diagnosis.²˒³ People with FTD experience severe personality changes, behavioral disturbance, loss of language, apathy, and reduced mobility.

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FTD is a leading cause of dementia in people under the age of 65, with an estimated prevalence at any one time of up to 4.6 cases per 1,000 of the population.
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FTD typically strikes younger than Alzheimer’s disease and the majority of FTD cases occur between 45 and 68 years of age.⁷˒⁸ Given the early onset, FTD has a substantially greater impact on work, family, and finances than Alzheimer’s disease.

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FTD can be separated into familial and sporadic FTD. Sporadic FTD is more common and occurs when only one person in a family has FTD. Whilst some familial FTD has no known cause, a strong family history is found in about one-third of cases. The majority of inherited FTD is accounted for by autosomal dominant disease-causing mutations in three genes, including the GRN (progranulin) gene.¹⁰

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FTD often presents insidiously and progresses over time, and can be diagnostically challenging. As disease education, genetics knowledge, access to genetic testing and research and treatment options grow, the numbers of diagnosed individuals, as well as those identified to be at-risk, are expected to increase.

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Approximately 11,000 people in the U.S. and EU5 are living with FTD-GRN, and it’s estimated that 2,000 new cases are confirmed annually.¹˒¹¹

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Currently there are no disease modifying treatments available for people with FTD.

REFERENCES

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  5. Hendriks S et al, JAMA Neurol. 2021;78 (9): 1080-1090;
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  7. Moore KM et al. Lancet Neurol. 19(2):145–156;
  8. Kansal K et al. Dement Geriatr Cogn Disord. 2016;41:109–122;
  9. Galvin JE et al. Neurology. 89(20):2049–2056;
  10. Greaves and Rohrer J Neurol. 2019; 266(8): 2075–2086.
  11. Kuang, L., et. al. Frontotemporal dementia non-sense mutation of progranulin rescued by aminoglycosides. Human Molecular Genetics 2020;29(4):624-634;
  12. Chiò A et al. Amyotroph Lateral Scler. 2009;10(5–6):310–23;
  13. Masrori P and Van Damme P. Eur J Neurol. 2020;27(10):1918–29;
  14. Salmon K et al. Brain. 2022;145(4):1207–10;
  15. Longinetti E, Fang F. Epidemiology of amyotrophic lateral sclerosis: an update of recent literature. Curr Opin Neurol. 2019;32(5):771-776;
  16. Ingre C, Roos PM, Piehl F, Kamel F, Fang F. Risk factors for amyotrophic lateral sclerosis. Clin Epidemiol. 2015;7:181-193;
  17. Schultz J. Am J Manag Care. 2018;24(15 Suppl):S327–335;
  18. Hergesheimer R et al. Expert Opin Pharmacother. 2020;21(9):1103–10.

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