Chapter 12 - Tauopathies

Summary

Tauopathies are associated with widely ranging patterns of structural and functional imaging abnormalities, demonstrating that the deposition of abnormal tau is not associated with one specific neuroimaging signature. The most common tauopathies include progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), Pick's disease (PiD), and those cases where tau deposition is associated with mutations in the microtubule-associated protein tau (MAPT) gene on chromosome 17. A number of different MRI techniques are now available that allow the interrogation of different structural and functional properties of the brain: structural MRI, diffusion tensor imaging (DTI), proton magnetic resonance spectroscopy (MRS), and resting-state functional MRI (fMRI). Patterns of atrophy observed in patients with mutations in MAPT are strikingly different from those observed in PSP and CBD. Finally, some of the rarer tauopathies are presented, including argyrophilic grain disease, globular glial tauopathy, Niemann-Pick type C, postencephalitic Parkinsonism (PEP), subacute sclerosing panencephalitis (SSPE), and tangle-dominant dementia.