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

David W. Loring, Kapil D. Sethi, Gregory P. Lee, Kimford J. Meador

Research output: Contribution to journalArticlepeer-review

4 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)191-199
Number of pages9
Issue number3
StatePublished - Jul 1990
Externally publishedYes


  • Hallervorden-Spatz
  • dementia
  • movement disorders
  • subcortical dementia

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology


Dive into the research topics of 'Neuropsychological Performance in Hallervorden-Spatz Syndrome: A Report of Two Cases'. Together they form a unique fingerprint.

Cite this