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From the Departments of Neurology, Divisions of Movement Disorders (K.A.J., B.F.B.), Behavioral Neurology (K.A.J., R.C.P., D.S.K., B.F.B.), and Radiology Research (J.L.W.), Speech Pathology (J.R.D.), and Laboratory Medicine and Pathology (J.E.P.), Mayo Clinic Rochester, MN; and Department of Neuroscience and Pathology (D.W.D.), Mayo Clinic, Jacksonville, FL.
Address correspondence and reprint requests to Dr. Keith A. Josephs, Department of Neurology, Divisions of Movement Disorders & Behavioral Neurology, Mayo Clinic, Rochester, MN 55905; e-mail: josephs.keith{at}mayo.edu
Objective: To examine the relationship between early clinical features, pathologies, and biochemistry of the frontotemporal lobar degenerations (FTLDs), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD).
Methods: The authors conducted pathologic reexamination with the most recent immunohistochemistry of all cases diagnosed with FTLD, PSP, and CBD between 1970 and 2004. The authors also reviewed the early clinical features for clinical diagnosis and application of published research criteria.
Results: Of 127 cases analyzed, 57 had a pathologic diagnosis of FTLD, 49 PSP, and 21 CBD. Of these, 38 were clinically reclassified as frontal variant frontotemporal dementia (FTD), 13 as progressive non-fluent aphasia (PNFA), 21 as CBD-like, 33 as PSP-like, and 13 with frontotemporal dementia with coexisting motor neuron disease (FTD-MND). The authors were unable to classify nine cases. All cases of FTD-MND were tau-negative and had pathologic evidence of motor neuron degeneration. All cases classified as PSP-like or CBD-like had tau-positive pathology. Of the 13 cases with PNFA, PSP and CBD accounted for almost 70% of the cases, while FTD was almost equally divided between tau-positive and tau-negative diseases.
Conclusion: Frontotemporal lobar degeneration, corticobasal degeneration (CBD), and progressive supranuclear palsy (PSP) have overlapping clinical features. The prediction of tau-positive pathology from a CBD or PSP-like presentation is good, while the frontotemporal dementia (FTD)-motor neuron disease syndrome almost certainly predicts motor neuron degeneration. Surprisingly, PSP and CBD accounted for most cases classified as progressive non-fluent aphasia. Frontal variant FTD is an unpredictable disease in terms of its biochemistry.
Editorial, see page 8
See also page 102
Supported by grants P50 AG16574, UO1 AG06786, RO1 AG23195, and PO1 AG7216 from the National Institute on Aging, Bethesda, MD; and by the Robert H. and Clarice Smith and Abigail Van Buren AD Research Program of the Mayo Foundation.
Disclosure: The authors report no conflicts of interest.
Received April 20, 2005. Accepted in final form September 21, 2005.
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