Visual grasping in frontotemporal dementia and parkinsonism linked to chromosome 17 (microtubule-associated with protein tau): a comparison of N-Isopropyl-p-((123)I)-iodoamphetamine brain perfusion single photon emission computed tomography analysis with progressive supranuclear palsy.

摘要

Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) has been divided into two categories based on mutations in the gene encoding the microtubule-associated with protein tau (MAPT) and in progranulin. MAPT N279K mutation causes progressive supranuclear palsy (PSP)-like presentations. Visual grasping was first described as an environment-driven response where the gaze of a patient with PSP was attracted to an incidental object in the environment, such as a TV set, a camera, or a window. Once fixed, the patient showed an inability to release the gaze and shift to another object. However, to the best of our knowledge, since the first report, there have been no subsequent reports on environment-driven response in PSP. Here, we describe 3 patients with FTDP-17 carrying the MAPT N279K mutation who developed prominent visual grasping. To better understand the pathophys-iological mechanism of this symptom, we compared regional cerebral blood flow (rCBF) in patients with N279K-1 mutation to sporadic PSP patients without visual grasping.