Diagnostic Evaluation and Monitoring of Patients With Posterior Cortical Atrophy

Abstract

Posterior cortical atrophy (PCA) is a progressive neurocognitive syndrome, most commonly associated with the loss of complex visuospatial functions. Diagnosis is challenging, and international consensus classification and nomenclature for PCA subtypes have only recently been reached. Presently, no established treatments exist. Efforts to develop treatments are hampered by the lack of standardized methods to monitor illness progression. Although measures developed from work with Alzheimer’s disease and other dementias provide a foundation for diagnosing and monitoring progression, PCA presents unique challenges for clinicians counseling patients and families on clinical status and prognosis, and experts designing clinical trials of interventions. Here, we review issues facing PCA clinical research and care, summarize our approach to diagnosis and monitoring of disease progression, and outline ideas for developing tools for these purposes.

Keywords:

• Alzheimer’s disease
• biomarkers
• cerebral cortex
• clinical trials
• MRI
• neuropsychology
• positron emission tomography
• posterior cortical atrophy

Acknowledgments

The authors express special appreciation to the participants in our PCA program and their families for their valuable contributions.

Financial & competing interests disclosure

This work was supported by grants from the NIA R21-AG051987, P50-AG005134 and R01-DC014296 and philanthropic funding to the MGH FTD Unit including the Mooney Family Fund. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Notes

Reproduced with permission from [9].

PCA: Posterior cortical atrophy.