Aprosodic speech with insular hyperintensities and 4R Tau pathology on autopsy

Abstract

We describe a 46-year-old woman who presented with a 2-year history of aprosodic speech together with apathy and disinhibition. Brain magnetic resonance imaging showed subcortical hyperintensities over both insular regions that later extended to both frontal and temporal cortices. The post-mortem exam showed a massive tau protein deposition throughout the brain. No mutation in the gene MAPT was detected. This case illustrates an atypical clinical-radiological presentation of a frontotemporal dementia with an unusual speech and abnormal signal of both insulae. Furthermore, it reinforces the crucial role of the insula in the development of symptoms in frontotemporal dementia.

Familial Behavioral Variant Frontotemporal Dementia Associated With Astrocyte-Predominant Tauopathy

“…Her parents were first-degree cousins. No mutations in the MAPT gene were detected at that time (19). …”