Brain bank series finds 47% clinical-pathological concordance in primary tauopathiesBrain bank study finds clinical diagnosis often differs from autopsy findings in tau diseases

Frontiers in Medicine Published April 2, 2026 DOI ↗ Editorial oversight: Dr. Ji-eun Park, MD · Brain, Mind & Pain

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Key Takeaway

Note the low clinical-pathological concordance and marked heterogeneity in primary tauopathies from a small brain bank series.

This systematic review analyzed the clinicopathological features of primary tauopathies in a series of 15 brain donation cases from a single national brain bank (Neurological Foundation Human Brain Bank) collected between 1994 and 2022. The study aimed to identify cases and analyze their features, with no specific intervention or comparator reported. The identified tauopathies included argyrophilic grain disease, primary age-related tauopathy, corticobasal degeneration, genetic frontotemporal dementia, and progressive supranuclear palsy.

The main findings show a mean age of onset of 69 years and an average survival of 6 years. A key result was that only 47% of cases had concordance between the clinical diagnosis made during life and the final post-mortem neuropathological diagnosis. The neuropathological findings revealed marked diversity in tau pathology and regional vulnerability, with most cases demonstrating both neuronal and glial tau pathology.

Safety and tolerability data were not reported. The study's key limitations, as noted, include its nature as a single brain bank series, retrospective analysis, and small sample size of only 15 cases. These factors significantly limit the generalizability of the findings.

In practice, this work highlights the complexity and heterogeneity of primary tauopathies and reinforces the critical role of post-mortem neuropathological examination for definitive diagnosis. The low clinical-pathological concordance rate suggests that antemortem diagnosis remains challenging. Clinicians should interpret these findings cautiously due to the observational nature and limited scope of the evidence.

Researchers looked back at 15 brain donations to a New Zealand brain bank over nearly 30 years to learn more about primary tauopathies. These are a group of brain diseases, like corticobasal degeneration and progressive supranuclear palsy, where a protein called tau builds up abnormally in brain cells. The study aimed to understand the relationship between what doctors thought was wrong during a person's life and what was actually found by examining the brain after death.

The main finding was that the clinical diagnosis made while the person was alive matched the definitive brain autopsy diagnosis only 47% of the time. This means in more than half of these 15 cases, the true disease was different from what was suspected. The study also showed these diseases are very complex, with different patterns of tau buildup in different brain areas from person to person.

This research is important because it shows how difficult it can be to accurately diagnose these specific brain diseases based on symptoms alone. However, readers should know this study was very small, looking at only 15 cases from one location. The results are a careful look at a complex problem, not a final answer. They remind us that definitive diagnosis for these conditions often requires a brain autopsy, and more research with many more people is needed.

What this means for you:

A small brain bank study found symptoms alone often don't match the final brain disease diagnosis, highlighting diagnostic complexity.

Study Details

Study typeSystematic review

EvidenceLevel 1

PublishedMar 2026

View Original Abstract ↓

IntroductionThis study presents the first comprehensive clinicopathological analysis of primary tauopathies from the Neurological Foundation Human Brain Bank (NFHuBB). Primary tauopathies are a heterogeneous group of neurodegenerative disorders, defined by abnormal tau protein deposition as the core pathological feature. They are further defined by the absence of an additional pathological driver. Historically, they were often accepted into brain banks as Alzheimer’s, Parkinson’s, or dementia cases.MethodsHere, we retrospectively examined all brain donations to the NFHuBB from 1994 to 2022 to identify cases of primary tauopathy, excluding those with known secondary causes such as repetitive head trauma. This is a national-level, single brain bank series. Detailed clinical records, demographic data, and post-mortem pathological investigations, including immunohistochemistry, were analysed to establish clinical and pathological diagnoses according to current criteria.ResultsFifteen cases were identified, spanning argyrophilic grain disease, primary age-related tauopathy, corticobasal degeneration, genetic frontotemporal dementia, and progressive supranuclear palsy. The mean age of onset was 69 years, with average survival of 6 years. Notably, only 47% of cases showed concordance between clinical and pathological diagnosis, underscoring diagnostic challenges. Neuropathological review revealed marked diversity in tau pathology and regional vulnerability, with most cases demonstrating both neuronal and glial tau pathology.DiscussionThis case series highlights the complexity and heterogeneity of primary tauopathies and the critical role of post-mortem neuropathological examination for diagnosis. By making fixed and frozen tissue, and clinical data available for national and international dissemination, this study provides a valuable resource to advance global research into the pathogenesis and classification of tauopathies. Furthermore, this work highlights the important role of brain banking in the study of rare diseases.

Brain bank series finds 47% clinical-pathological concordance in primary tauopathiesBrain bank study finds clinical diagnosis often differs from autopsy findings in tau diseases

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Brain bank series finds 47% clinical-pathological concordance in primary tauopathiesBrain bank study finds clinical diagnosis often differs from autopsy findings in tau diseases

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