What is the prognosis of frontotemporal dementia (FTD)?

Updated: Jun 14, 2018
  • Author: Howard S Kirshner, MD; Chief Editor: Jasvinder Chawla, MD, MBA  more...
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Answer

Answer

Frontotemporal lobe dementia (FTD), like all dementing illnesses, shortens life expectancy. The exact influence on mortality is unknown, and the rate of disease progression is variable.

Among patients younger than 65 years, FTD has a similar or greater prevalence, as compared with Alzheimer disease. Among patients in their 70s and older, the prevalence of Alzheimer disease far exceeds that of FTD. The average age of onset for FTD, as reported by Westbury and Bub, [49] is younger than that of Alzheimer disease.

The rate of progression from focal presentation to a more generalized dementia varies. The literature contains many cases of slowly progressive language dysfunction developing over a period of as long as 10-12 years, without obvious deterioration of other cognitive functions that would justify a diagnosis of dementia. Other patients progress to dementia within a few years.

One area of controversy in PPA concerns whether a generalized dementia eventually develops in all patients with PPA. The incidence of dementia in patients with PPA is unknown, but it likely approaches 50% over several years.

In the subset of cases of patients with FTD who develop motor neuron disease, the mortality rate is higher than for other FTD patients. Swallowing difficulty and aspiration pneumonia are especially common in this subgroup, but even patients with primary progressive aphasia (PPA) can develop dysphagia late in the course of the illness.


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