Neuropsychological Performance in Hallervorden-Spatz Syndrome: A Report of Two Cases

Hallervorden-Spatz disease is a rare progressive disorder that is characterized neuropathologically by iron deposition in the globus pallidus and zona reticularis of the substantia nigara. Neuropsychological findings are reported in two patients whose diagnoses of probable Hallervorden-Spatz disease are based upon typical clinical course in conjunction with magnetic resonance imaging abnormalities restricted to the region of the globus pallidus. Neuropsychological deficits included slowed cognitive processing speed, motor sequencing difficulty, constructional dyspraxia, and impaired recent memory functions. This constellation of behavioral deficits can be attributed primarily to dysfunction of the frontal lobe, basal ganglia, or disruption of their functional interconnections. However, as with other "subcorti-Cal" dementias, additional deficits in language and visuospatial processing were observed.