Frontotemporal lobar degeneration in the "Annual of the Pathological Autopsy Cases in Japan".

Background: Good accuracy for the clinical diagnosis of frontotemporal lobar degeneration (FTLD) by specialists in an early onset dementia clinic has been reported.

Objective: To assess the diagnostic accuracy of FTLD in an entire population, without restrictions related to patient age or diagnosing physician.

Methods: Volumes of the "Annual of the Pathological Autopsy Cases in Japan," with reports of 130,105 autopsies throughout Japan from 2007 to 2016, were descriptively analyzed.

Results: There were 219 patients with clinical and/or pathological diagnoses of FTLD. The sensitivity and specificity were 24.5% and 76.9%, respectively. Age at death for pathologically confirmed patients was 76.3 ± 11.6 years (mean ± standard deviation). Overlooked patients died significantly older than patients with an accurate clinical diagnosis.

Conclusions: Clinical diagnoses of FTLD had low sensitivity. Furthermore, the age at death of pathologically confirmed patients suggests that FTLD affects a wide age range and is not restricted to presenile individuals.