11 C-PiB PET A Bri Imaging in Worster-Drought Syndrome (Familial British Dementia): A Case Report

Abstract

Brain amyloid imaging is becoming an essential tool for the pre-mortem evaluation of Alzheimer's disease (AD). This study explores the pattern of ¹¹C-PiB retention in a subject with Worster-Drought syndrome (WDS). A 55 year-old male carrier of the WDS gene mutation with mild signs of ataxia and subtle cognitive impairment underwent MRI and ¹¹C-PiB-PET studies. Brain PiB regional distribution was compared to those from cohorts of healthy controls and AD patients. While no significant cortical ¹¹C-PiB retention was present, a high degree of cerebellar ¹¹C-PiB retention was observed in a genetically confirmed carrier of the WDS gene. We speculate that the sparsity of A_Bri plaques in the neocortex together with its high deposition in the cerebellum, might explain the observed pattern of ¹¹C-PiB retention.