This is a novel finding suggesting that BPAN may be considered as a tauopathy and further strengthening the link between tauopathies and other forms of NBIA such as PKAN, PLAN and MPAN in which a degree of neurofibrillary tangle pathology is a feature in some cases although extensive tau pathology is more often associated with PLAN [2, 10–13]. This evidence concerns the gene MAPT and neurodegeneration with brain iron accumulation 5.