Hallervorden-Spatz disease with bilateral involvement of globus pallidus and substantia nigra : MR demonstration

Hallervorden-Spatz disease presents with relentless mental and physical deterioration. Pathologically there is abnormal iron deposition in the globi pallidi, substantia nigra, and red nuclei. A spectrum of abnormal basal ganglia signal intensities have been described on MRI. A pediatric case with low signal intensity in the globi pallidi and substantia nigra is presented