title: The neuropsychiatry and neuropsychology of lipoid proteinosis.
abstract: 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.
authors.affiliation: Department of Psychiatry, University of Cape Town, Cape Town, South Africa. helena.thornton@uct.ac.za
authors.forename: Helena B
authors.initials: HB
authors.lastname: Thornton
authors.forename: Daan
authors.initials: D
authors.lastname: Nel
authors.forename: Dorothy
authors.initials: D
authors.lastname: Thornton
authors.forename: Jack
authors.initials: J
authors.lastname: van Honk
authors.forename: Gus A
authors.initials: GA
authors.lastname: Baker
authors.forename: Dan J
authors.initials: DJ
authors.lastname: Stein
mesh_terms: D000293:Adolescent; D000328:Adult; D000704:Analysis of Variance; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D015994:Incidence; D008065:Lipoid Proteinosis of Urbach and Wiethe / Q000150:complications* / Q000523:psychology*; D008297:Male; D001523:Mental Disorders / Q000209:etiology*; D008875:Middle Aged; D009484:Neuropsychology; D011570:Psychiatry; D013019:South Africa