The clinical features of two children of a family with rapidly progressive 
extrapyramidal-pyramidal-dementia complex have been described. Inheritance seems 
most likely to be autosomal recessive. Magnetic resonance imaging results of 
brain were negative. Even so, the authors argued in favor of a diagnosis of 
Hallervorden-Spatz disease because the cases fulfilled the clinical criteria for 
diagnosis of this disease. Apart from the negative magnetic resonance findings, 
the other unusual feature was the early development of levodopa-induced 
dyskinesia. Few conditions need to be considered in the differential diagnosis 
of a childhood-onset rapidly progressive extrapyramidal syndrome. Such 
conditions include Wilson's disease, Hallervorden-Spatz disease (HSD), juvenile 
form of Huntington's disease, juvenile neuronal ceroid lipofuscinosis, 
early-onset Machado-Joseph disease neuroacanthocytosis, storage disorders, and 
variant form of dopa-response dystonias (DRD). Rarer conditions are Leigh's 
disease, Lafora body disease, and dentato-rubro-pallido-luysian atrophy. HSD is 
a rare disorder characterized by progressive extrapyramidal dysfunction and 
dementia. Onset is most commonly in late childhood or early adolescence. The 
disease can be familial or sporadic. When familial, it is inherited recessively 
and has been linked to chromosome 20. Recently, a mutation in the pantothenate 
kinase (PANK2) gene on band 20pl3 has been described in patients with typical 
HSD. HSD produces typical magnetic resonance imaging (MRI) changes in brain, 
aiding in antemortem diagnosis. The typical finding is of bilaterally 
symmetrical hyperintense signal changes in the external segment of globus 
pallidus, with surrounding hypointensity on T(2)-weighted image. These imaging 
features are fairly diagnostic and have been termed the "eye-of-the tiger sign". 
The hyperintensity represents pathologic changes, including gliosis, 
demyelination, neuronal loss, and axonal swelling, and the surrounding 
hypointensity is caused by loss of signal secondary to iron deposition. 
Described herein are the clinical aspects of a family with autosomal recessive 
inheritance with rapidly progressive extrapyramidal-pyramidal-dementia complex 
but with negative brain MRI results. The diagnosis should be considered a 
variant form of HSD.