부산대학교 도서관

ObjectiveaaTo determine the volume changes in gray and white matter during a long-term follow-up in patients suffering frompantothenate kinase-associated neurodegeneration (PKAN). MethodsaaMagnetic resonance imaging was repeated in 13 patients and 14 age-matched controls after a mean interval of morethan 7 years. T1-weighted sequences were evaluated by fully automated atlas-based volumetry, compared between groups andcorrelated with disease progression. ResultsaaThe patients did not show generalized cerebral atrophy but did show a significantly faster volume reduction in theglobus pallidus during follow-up (between -0.96% and -1.02% per year, p < 0.05 adjusted for false discovery rate) than controls,which was significantly related to the progression in their dystonia scores (p = 0.032). ConclusionaaThe volume loss in the globus pallidus over time—together with the accumulation of iron known as the “tiger’seye”—supports the pathophysiologic concept of this nucleus as a center of inhibition and its severe malfunction in PKAN.