Generalized freezing in Hallervorden-Spatz syndrome: case report

We report the case of a female patient who was exhibiting slowly progressive, severe, generalized freezing of voluntary movement and disequilibrium. Brain magnetic resonance imaging showed a very low signal intensity in both pallidal nuclei, with a high signal intensity in the central portion, the so called 'eye-of-the-tiger' sign, Despite the unusual clinical features, we believe that this is a case of Hallervorden-Spatz syndrome, although without neuropathological examination we were unable to confirm the diagnosis with certainty, This case further demonstrates the complex role of the pallidum in voluntary movement, Eur J Neurol 6:509-513 (C) 1999 Lippincott Williams & Wilkins.