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.
Porter-Grenn
L
L
Department of Diagnostic Radiology, Henry Ford Hospital, Detroit, MI 48202.
Silbergleit
R
R
Mehta
B A
BA
D002648:Child; D005260:Female; D005917:Globus Pallidus / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000175:diagnosis* / Q000473:pathology; D013378:Substantia Nigra / Q000473:pathology*