With the emergence of multiple post-mortem case studies, CBS is now known to have heterogeneous neuropathological underpinnings including AD, Pick's disease, FTLD with transactivation response DNA binding protein 43 kDa (TDP-43) inclusions, and FTLD with ubiquitin-immunoreactive inclusions negative for TDP-43 (3, 7–9) as well as cerebrovascular pathology (3, 9, 10). Here, CBS is linked to Alzheimer disease.