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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.
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Sethi et al. (1988) studied this question.
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