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Amyloid imaging in distinguishing atypical prion disease from Alzheimer disease

From the Memory and Aging Center (A.L.B., G.D.R., M.D.G., W.J., B.L.M.), Department of Neurology, University of California, San Francisco; Department of Molecular and Medical Pharmacology (V.K., S.-C.H., J.R.B.) and Center on Aging, Semel Institute for Neuroscience and Human Behavior (G.W.S.), David Geffen School of Medicine, University of California, Los Angeles; Gertrude H. Sergievsky Center at Columbia University (J.G.), College of Physicians and Surgeons, New York, NY; Helen Wills Neuroscience Institute (A.J.F., W.J.), University of California, Berkeley; Lawrence Berkeley Laboratory (S.L.B., J.P.O., W.J.), CA; and Department of Neurology (H.C.), University of Southern California.

Address correspondence and reprint requests to Dr Boxer, Memory and Aging Center, Department of Neurology, University of California, San Francisco, Box 1207, San Francisco, CA 94143-1207

Objective: To compare the in vivo uptake of two amyloid-binding PET agents, PIB and FDDNP, in human subjects with a prion protein (PrP) gene (PRNP) mutation that produces a clinical syndrome similar to Alzheimer disease (AD).

Background: Amyloid imaging with specific PET ligands offers great promise for early detection and differential diagnosis of AD. Genetic forms of prion disease can present with clinical features that resemble AD, and at autopsy may show deposition of mutant PrP-amyloid. FDDNP binds to PrP-amyloid in postmortem human specimens, but has not been reported in vivo in prion disease. The ability of PIB to bind PrP-amyloid is not known.

Methods: Two brothers with a 6 octapeptide repeat insertion mutation (6-OPRI) in the PRNP gene underwent clinical, structural MRI, and FDG-PET evaluations. One brother received a PIB-PET evaluation, while the other received an FDDNP-PET scan. PET results were compared with five normal subjects and five individuals with AD scanned with either agent.

Results: PIB uptake was similar to controls in one brother, while FDDNP uptake was intermediate between AD and controls in the other brother.

Conclusions: Different amyloid-binding agents may have differential sensitivity to prion-related brain pathology. A combination of amyloid imaging agents may be useful in the diagnosis of early-onset dementia.

aboxer{at}memory.ucsf.edu

Supplemental data at www.neurology.org

Supported by The John Douglas French Foundation (A.L.B., M.D.G.), K23 NS48855 (A.L.B.), K23 AG021989 (M.D.G.), P50 AG-03-006-01 (B.L.M.), P01 AG019724 (B.L.M.), The Larry Hillblom Foundation (B.L.M.), NINDS N01 NS02328 and M01 RR00079, the UCSF General Clinical Research Center, and the US Department of Energy under contracts DE-AC03-76SF00098 (W.J.) and DE-FC02-02ER63420 (J.R.B.).

Disclosure: Drs. Small, Huang, and Barrio are co-inventors of a UCLA patent on FDDNP technology that has been licensed to Siemens. Drs. Barrio and Small have been paid to give expert testimony related to FDDNP (<$10,000/year).

Received August 3, 2006. Accepted in final form February 23, 2007.