The neuropsychiatry and neuropsychology of lipoid proteinosis.

Full 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.

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Author information

Author/s: Thornton, Helena B (HB); Nel, Daan (D); Thornton, Dorothy (D); van Honk, Jack (J); Baker, Gus A (GA); Stein, Dan J (DJ);

Affiliation: Department of Psychiatry, University of Cape Town, Cape Town, South Africa. helena.thornton(-atsign-)uct.ac.za

Journal and publication information

Publication Type: Journal Article; Research Support, Non-U.S. Gov't

Journal: The Journal of neuropsychiatry and clinical neurosciences (J Neuropsychiatry Clin Neurosci), published in United States. (Language: eng)

Reference: 2008-; vol 20 (issue 1) : pp 86-92

Dates: Created 2008/02/28; Completed 2008/07/02;

PMID: 18305289, status: MEDLINE (last retrieval date: 11/6/2008)

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MeSH headings (categories)

This article was linked to the MESH Headings shown below.

Lipoid Proteinosis of Urbach and Wiethe - complications; psychology
Male
Mental Disorders - etiology
Middle Aged
Neuropsychology
Psychiatry
South Africa

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