Amyloid-negative pathology underlying primary progressive prosopagnosia usually affects the tip of the right temporal lobe first.1,2 However, a 69-year-old right-handed man, with amyloid-negative PET imaging, had severe progressive prosopagnosia associated with changes in the right posterior temporo-occipital cortex, including the fusiform face area, but sparing the temporal lobe tip. Brain regions affected were documented by cortical thinning on MRI and by abnormal metabolism, tau, and inflammation on PET (figure). By age 71, he had developed signs of the clinical corticobasal syndrome (CBD), as manifested by apraxia, motor impersistence, rigidity, and a supranuclear eye movement disorder. CBD was heralded by right premotor involvement in his earlier cortical thickness study (figure).
Greatest cortical thinning (top, yellow) corresponded to lowest metabolism and highest tau and inflammation uptake (arrows). standardized uptake value ratios (SUVR) were obtained by referencing to the cerebellar gray matter. 11C-PBR28 parametric images of total distribution volume (VT) were calculated using the Logan plot and a metabolite-corrected arterial input function.
Study funding
The study was funded by the Chao, Graham, Harrison, and Nantz Funds from the Houston Methodist Foundation; Avid Radiopharmaceuticals provided the 18F-AV-1451 precursor free of charge.
Disclosure
B. Pascual reports no disclosures relevant to the manuscript. J. Masdeu is a consultant for General Electric Healthcare and has received research support from Avid Radiopharmaceuticals. Q. Funk, P. Zanotti-Fregonara, M. Shyer, and E. Rockers report no disclosures relevant to the manuscript. P. Schulz has received research support from Avid Radiopharmaceuticals. Go to Neurology.org/N for full disclosures.
Acknowledgment
Avid Radiopharmaceuticals provided the precursor for 18flortaucipir.
Appendix Authors
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
↵* These authors contributed equally to this work.
- © 2020 American Academy of Neurology
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