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Clinical and Neuroimaging Aspects of Familial Frontotemporal Lobar Degeneration Associated with MAPT and GRN Mutations

Advances in Experimental Medicine and Biology, ISSN: 2214-8019, Vol: 1281, Page: 77-92
2021
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Clinical and Neuroimaging Aspects of Familial Frontotemporal Lobar Degeneration Associated with MAPT and GRN Mutations.

Adv Exp Med Biol. 2021;1281:77-92. Authors: Boeve BF, Rosen H PubMed: 33433870 Submit Comment

Book Chapter Description

Numerous kindreds with familial frontotemporal lobar degeneration have been linked to mutations in microtubule-associated protein tau (MAPT) or progranulin (GRN) genes. While there are many similarities in the clinical manifestations and associated neuroimaging findings, there are also distinct differences. In this review, we compare and contrast the demographic/inheritance characteristics, histopathology, pathophysiology, clinical aspects, and key neuroimaging findings between those with MAPT and GRN mutations.

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