involvement of globus pallidus and midbrain nuclei in pantothenate kinaseassociated neurodegeneration measurement of t2 and t2 time purpose to quantify involvement of globus pallidus and two midbrain nuclei substantia nigra and red nucleus in pantothenate kinaseassociated neurodegeneration pkanmaterial and methods we performed t2 and t2 weighted imaging with calculation of the corresponding relaxation times on a subset of 5 patients from a larger group of 20 patients with pkan from the southwest part of the dominican republic examinations were carried out on a 3t scanner and included a multiecho spinecho as well as a multiecho gradient echo sequence results were compared to a control group of 19 volunteersresults t2 and t2 weighted sequences showed abnormal signal reduction in the globus pallidus of all patients on t2 weighted imaging abnormal signal in the substantia nigra could reliably be detected in 75 of cases but differentiation from normal was less reliable in t2 weighted scans correspondingly relaxation times differed from normal with very high significance p 00001 in the globus pallidus but with with less significance in the substantia nigra p 003 the red nucleus was not affectedconclusions signal reduction in the globus pallidus which probably is due to abnormal accumulation of iron is severe in pkan and can be differentiated from normal with high reliability the substantia nigra is affected to a lesser degree and the red nucleus is not involved the reason for this selective susceptibility of normally ironrich brain structures for pathological accumulation of iron remains speculative our quantitative results might be helpful to assess the value of an iron chelation approach to therapy