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Clin Neurol Neurosurg. 1992;94:S57-60. doi: 10.1016/0303-8467(92)90023-v.

Follow-up magnetic resonance imaging in Hallervorden-Spatz disease.

Clinical neurology and neurosurgery

O F Brouwer, P M Laboyrie, A C Peters, G J Vielvoye

Affiliations

  1. Department of Neurology, University Hospital, Leiden, The Netherlands.

PMID: 1320520 DOI: 10.1016/0303-8467(92)90023-v

Abstract

Bilateral high signal emitting areas in the globus pallidus surrounded by low signal emitting areas have been described as a typical MRI finding in Hallervorden-Spatz disease (HSD). We made a diagnosis of HSD in an 11-year-old girl with progressive dystonia of 4 years duration who showed these typical MRI abnormalities. An initial MRI at the age of 9 was normal. Pathological confirmation of these typical MRI findings has not yet been described, but earlier reports as well as our case suggest that MRI may be helpful in making a clinical diagnosis of HSD. This case further shows that MRI may be normal in an early stage of the disease.

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