[Nuclear magnetic resonance tomography and differential diagnosis of Hallervorden-Spatz disease]

Abstract

The case of a 15 year old male patient with progressive dystonia, dysarthria and dementia is presented. No diagnosis could be found on clinical grounds. Low field MRI (0.23 T) demonstrated bilateral necrosis in the globus pallidus of clinically unknown etiology. Only high field imaging (2.0 T) revealed a pattern of lesions which is supposed to be specific for Hallervorden-Spatz disease (HSD): bilateral symmetrical decreased signal intensity in the globus pallidus secondary to the T2* effect of paramagnetic substances, principally iron. The neuroradiological differential diagnosis and pathognomonic signs of HSD are discussed.