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

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

Research output: Contribution to journalArticle

4 Scopus citations

Abstract

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
JournalNeuropsychology
Volume4
Issue number3
DOIs
StatePublished - Jan 1 1990

Keywords

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

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology

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