Generalized freezing in Hallervorden-Spatz syndrome: case report

We report the case of a female patient who was exhibiting slowly progressiv e, severe, generalized freezing of voluntary movement and disequilibrium. B rain magnetic resonance imaging showed a very low signal intensity in both pallidal nuclei, with a high signal intensity in the central portion, the s o called 'eye-of-the-tiger' sign, Despite the unusual clinical features, we believe that this is a case of Hallervorden-Spatz syndrome, although witho ut neuropathological examination we were unable to confirm the diagnosis wi th certainty, This case further demonstrates the complex role of the pallid um in voluntary movement, Eur J Neurol 6:509-513 (C) 1999 Lippincott Willia ms & Wilkins.