# Accuracy of clinical diagnosis of behavioral variant frontotemporal dementia: A systematic review and meta‐analysis

**Authors:** Daniele Urso, Stefano Giannoni‐Luza, Silvio Ionta, Stefania Mondello, Giancarlo Logroscino

PMC · DOI: 10.1002/dad2.70266 · Alzheimer's & Dementia : Diagnosis, Assessment & Disease Monitoring · 2026-02-28

## TL;DR

This study reviews how well doctors can diagnose a type of dementia called bvFTD using different criteria over time.

## Contribution

The study shows that diagnostic sensitivity for bvFTD has improved over time, but at the cost of reduced specificity.

## Key findings

- Sensitivity of bvFTD diagnosis increased from 70% to 90% over time.
- Psychiatric and Alzheimer's disease were the most common misdiagnoses.
- Pathology-based diagnoses had higher sensitivity than clinical follow-up.

## Abstract

Accurate diagnosis of behavioral variant frontotemporal dementia (bvFTD) remains difficult due to overlapping symptoms with other conditions. We aimed to evaluate the diagnostic accuracy of bvFTD clinical criteria and whether it has improved over time.

We systematically reviewed studies assessing the Lund‐Manchester (1994), Neary (1998), and Rascovsky (2011) criteria, using pathology or follow‐up diagnosis as reference standards. A Bayesian bivariate model was used to estimate pooled sensitivity and specificity.

Nine studies (1130 participants) were included. Sensitivity increased from 0.70 (95% confidence interval [CI], 0.41–0.89; Lund‐Manchester) to 0.80 (95% CI, 0.48–0.95; Neary) and 0.90 (95% CI, 0.81–0.95; Rascovsky, probable diagnosis), at the cost of a decreased specificity (0.94, 0.95, and 0.85, respectively).

The diagnostic sensitivity of bvFTD criteria has improved over time, though specificity has declined. Ongoing refinement and biomarker development are needed to enhance diagnostic accuracy, especially in early disease stages.

Sensitivity of behavioral variant frontotemporal dementia (bvFTD) clinical criteria improved from 70% to 90% over time.Psychiatric and Alzheimer's disease misdiagnoses were most frequent sources of error.Pathology‐based diagnoses yielded higher sensitivity than clinical follow‐up.Updated criteria enhance bvFTD detection, but specificity needs refinement.

Sensitivity of behavioral variant frontotemporal dementia (bvFTD) clinical criteria improved from 70% to 90% over time.

Psychiatric and Alzheimer's disease misdiagnoses were most frequent sources of error.

Pathology‐based diagnoses yielded higher sensitivity than clinical follow‐up.

Updated criteria enhance bvFTD detection, but specificity needs refinement.

## Linked entities

- **Diseases:** Alzheimer's disease (MONDO:0004975)

## Full-text entities

- **Genes:** TARDBP (TAR DNA binding protein) [NCBI Gene 23435] {aka ALS10, TDP-43}, HMBS (hydroxymethylbilane synthase) [NCBI Gene 3145] {aka ENCEP, LENCEP, PBG-D, PBGD, PORC, UPS}
- **Diseases:** FTD (MESH:D057180), cognitive decline (MESH:D003072), TDP (MESH:D016171), dementia (MESH:D003704), Psychiatric (MESH:D001523), FTLD (MESH:D057174), AD (MESH:D000544), Huntington's disease (MESH:D006816), Lewy body dementia (MESH:D020961), proteinopathies (MESH:D057165), neurodegeneration (MESH:D019636), RESEARCH (MESH:D014947)
- **Chemicals:** HMPAO (-), DTA (MESH:C042899)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12949661/full.md

## References

41 references — full list in the complete paper: https://tomesphere.com/paper/PMC12949661/full.md

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Source: https://tomesphere.com/paper/PMC12949661