The neuropsychiatry and neuropsychology of lipoid proteinosis

Helena B. Thornton, Daan Nel, Dorothy Thornton, Jack Van Honk, Gus A. Baker, Dan J. Stein

Research output: Contribution to journalArticlepeer-review

55 Scopus citations


Lipoid proteinosis is a rare hereditary disease which often results in bilateral calcifications in the medial temporal region. Thirty-four adults living with lipoid proteinosis (>10% of the world population) were extensively assessed with standardized neuropsychiatric and neuropsychological measures. Of these, 27 patients representing a homogenous group living in the Northern Cape were matched with 47 controls. Subjects with lipoid proteinosis had a high incidence of neuropsychiatric disorders and performed poorly on facial recognition of positive and negative emotions and on many neuropsychological measures. These findings are consistent with involvement of the medial temporal areas in cognitive and emotive processing.

Original languageEnglish
Pages (from-to)86-92
Number of pages7
JournalJournal of Neuropsychiatry and Clinical Neurosciences
Issue number1
StatePublished - 2008
Externally publishedYes


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