Abstract
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.
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Anonymized data are available by request from the corresponding author.
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Acknowledgements
This study was funded by The Rehabilitation Research Fund of Social Welfare Corporation Nagoya City Rehabilitation Agency. We deeply appreciate the Japanese Society of Pathology for publishing “Annual of the Pathological Autopsy Cases in Japan” each year. We are especially grateful to Dr. Hidehiro Kabasawa, former director of Nagoya City Rehabilitation Center Hospital, to the late Dr. Michitaka Matsubara, former Director of Department of Planning and Research, Nagoya City Rehabilitation Center, and to the late Professor Tomoyuki Shirai, former President of Nagoya City Rehabilitation Center and Professor Emeritus of Nagoya City University, for their kind assistances.
Funding
This study was funded by The Rehabilitation Research Fund of Social Welfare Corporation Nagoya City Rehabilitation Agency.
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All authors have read the manuscript and approved this submission. Yoshihiko Horimoto: Research project (Conception, Organization, Execution), Recruitment of patients who met the inclusion criteria, Analyzing patient information, Statistical Analysis (Design, Execution, Review and Critique), Manuscript Preparation (Writing of the first draft, Review and Critique). Chikako Sato: Recruitment of patients who met the inclusion criteria, Manuscript Preparation (Review and Critique). Ayuko Suzuki: Recruitment of patients who met the inclusion criteria, Manuscript Preparation (Review and Critique). Aki Inagaki: Recruitment of patients who met the inclusion criteria, Manuscript Preparation (Review and Critique). Toshihisa Tajima: Recruitment of patients who met the inclusion criteria, Manuscript Preparation (Review and Critique). Hiroaki Hibino: Recruitment of patients who met the inclusion criteria, Manuscript Preparation (Review and Critique). Hiroshi Inagaki: Research project (Execution), Negotiations with the Japanese Society of Pathology, Manuscript Preparation (Review and Critique).
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This study was a retrospective review of published data; ethics committee approval was therefore not required. The study was conducted in accordance with the Declaration of Helsinki, 1975, and was authorized by the Japanese Society of Pathology.
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Horimoto, Y., Sato, C., Suzuki, A. et al. Frontotemporal lobar degeneration in the “Annual of the Pathological Autopsy Cases in Japan”. J Neurol (2024). https://doi.org/10.1007/s00415-024-12528-5
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DOI: https://doi.org/10.1007/s00415-024-12528-5