The patient's father had presented with a PSP-like clinical phenotype, whereas pathologic re-assessment of his brain tissue confirmed a tauopathy felt to be more consistent with CBD and involving extensive brainstem TDP-43 pathology, including the substantia nigra, tectum, and oculomotor nuclei of the midbrain, the basis pontis, and medullary olives. Here, TARDBP is linked to tauopathy.