A case of semantic variant primary progressive aphasia with severe insular atrophy

Abstract

Insular degeneration has been linked to symptoms of frontotemporal dementia (FTD). Presented in this case is a patient exhibiting semantic variant primary progressive aphasia, behavioral disturbance. Upon autopsy, he was found to have severe insular atrophy. In addition, selective serotonin reuptake inhibitors were ineffective in reducing symptoms of obsessive–compulsive behaviors or emotional blunting. This case suggests that Seeley et al.'s (2007, Alzheimer Disease & Associated Disorders, 21, S50) hypothesis that von Economo neurons and fork cell-rich brain regions, particularly in the insula, are targeted in additional subtypes of FTD beyond the behavioral variant.

Keywords:

• Frontotemporal dementia
• MRI
• PET
• SPECT
• Insula

Acknowledgments

The authors thank Mr. James Shin for assistance with formatting. Nicole Yin performed immunohistochemical studies and assisted with preparation of micrographs. This work was funded by NIA grant F32 AG022802 (TWC); the University of Toronto Dean's Fund for New Faculty (#457494 TWC); an endowment to the Sam and Ida Ross Memory Clinic (TWC); and a gift from the Moir Family (KL). HVV is supported in part by P50 AG 16570 and the Daljit S. and Elaine Sarkaria Chair in Diagnostic Medicine.