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.
Copyright 1999 Lippincott Williams & Wilkins