OBJECTIVE: To develop a diagnostic decision tree for the evaluation of rapidly progressive dementias (RPDS), including convening a consensus panel of national experts.
BACKGROUND: The RPDs are a group of heterogeneous disorders including autoimmune encephalopathies and prion diseases that often are not readily diagnosed. Delays in diagnosis may occur in part because of diagnostic complexity or in some cases because of a lack of practice parameters. Diagnostic delay can lead to increased morbidity and mortality, especially for antibody-mediated and other potentially reversible dementias. Standardized decision support tools based on consensus guidelines should enable a broader range of clinicians to identify, evaluate, appropriately treat and/or refer RPD patients.
DESIGN/METHODS: RPD was defined as the rapid onset of dementia over a period of less than two years, typically less than six months. A panel of national dementia experts was convened to review elements of the RPD decision tree. MRI parameters, including optimized sequences for prion and autoimmune disorders, were established for 1.5 and 3T scanners for the evaluation of RPD.
RESULTS: An algorithm to guide clinical decisions was constructed based on literature review utilizing clinical signs and symptoms, a standardized MRI protocol, laboratory and other testing. Review of the algorithm by an expert consensus panel led to additional modifications, including a delirium assessment. Educational materials were developed by dementia healthcare professionals to guide clinical management.
CONCLUSIONS: This algorithm, including a diagnostic decision tree, should facilitate the diagnosis of RPDs. This diagnostic pathway should lessen morbidity and reduce the cost of medical care for patients with RPD by standardizing evaluations and empowering clinicians to aid in the early identification of reversible forms of dementia. Integration into an EMR/IT-based dementia care pathway should further improve the utility of this algorithm.
Study supported by an NIH R01 AG031189 grant and support from Quest Diagnostics, Inc. Disclosure: Dr. Gelfand has received personal compensation for activities as a medical-legal consultant. Dr. Fredericks has nothing to disclose. Dr. Rabinowitz has nothing to disclose. Dr. Rankin has research support from Quest Diagnostics. Dr. Hess has nothing to disclose. Dr. Paoletti has nothing to disclose. Dr. Higgins has received personal compensation for activities with Quest Diagnostics. Dr. Williams has received personal compensation for activities with Quest Diagnostics as an employee. Dr. Miller has nothing to disclose. Dr. Rosen has nothing to disclose. Dr. Geschwind has received personal compensation for activities with Best Doctors, Advanced Medical, Guidepoint Global, Gerson-Lehrman Group, and Quest Diagnostics. Dr. Geschwind has received research support from NIH/NIA, the Tau Consortium, Michael J. H