Hallervorden-Spatz syndrome: clinical and magnetic resonance imaging correlations.
Two women with a presumptive diagnosis of Hallervorden-Spatz syndrome had a combination of dystonia and parkinsonism. One had retinitis pigmentosa. Neuropsychological testing revealed decreased verbal fluency and visuoconstructional and motor deficits. Magnetic resonance imaging performed with a high-field-strength unit (1.5 Tesla) showed striking abnormalities in the globus pallidus bilaterally ("eye-of-the-tiger" sign). Magnetic resonance imaging may prove useful in the diagnosis of Hallervorden-Spatz syndrome.
Sethi
K D
KD
Department of Neurology, Medical College of Georgia, Augusta 30912.
Adams
R J
RJ
Loring
D W
DW
el Gammal
T
T
D000328:Adult; D001480:Basal Ganglia Diseases / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009483:Neuropsychological Tests; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000175:diagnosis / Q000473:pathology* / Q000523:psychology