Clinical and Pathological Continuum of Multisystem TDP-43 Proteinopathies

Objective: To determine the extent of transactivation response DNA-binding protein with a molecular weight of 43 kDa (TDP-43) pathology in the central nervous system of patients with clinically and autopsyconfirmed diagnoses of frontotemporal lobar degeneration with and without motor neuron disease and amyotrophic lateral sclerosis with and without cognitive impairment. Design: Performance of immunohistochemical whole– central nervous system scans for evidence of pathological TDP-43 and retrospective clinical medical record review.