The neuropsychiatry and neuropsychology of Lipoid Proteinosis

dc.contributor.advisorStein, D. J.
dc.contributor.advisorBaker, G. A.
dc.contributor.authorThornton, H. B.en_ZA
dc.contributor.otherUniversity of Stellenbosch. Faculty of Health Sciences. Dept. of Psychiatry.
dc.descriptionThesis (PhD (Psychiatry))--University of Stellenbosch, 2006.
dc.description.abstractLipoid Proteinosis (LiP) is a rare hereditary disease, which often results in bilateral, symmetrical and circumscribed calcifications in the mesial temporal region (especially the amygdala). While several case studies have been published on individuals with this illness, there have been few systematic investigations of the neuropsychiatry and neuropsychology of a series of patients. Thirty-seven LiP patients were extensively assessed with standardized neuropsychiatric and neuropsychological measures. Of these, 27 patients from the Northern Cape in South Africa were matched (for age, gender, education, language, geographical area) with 53 controls. There was a high incidence of neuropsychiatric disorders in LiP (more than half of the subjects reported a history of depression or anxiety and 12% had a diagnosis of schizophrenia). Despite a wide variance, LiP subjects performed poorly on facial recognition for emotions and on most neuropsychological measures including intelligence, recall and executive functioning. These findings are consistent with involvement of the mesial temporal areas in mood, anxiety, and psychotic symptoms, and in the cognitive-affective processes. Future work aimed at delineating the associations between the clinical and neuropsychological findings reported here, for example, with brain-imaging techniques, is needed.en_ZA
dc.publisherStellenbosch : University of Stellenbosch
dc.subjectLipidoses -- South Africaen
dc.subjectDissertations -- Psychiatryen
dc.subjectTheses -- Psychiatryen
dc.titleThe neuropsychiatry and neuropsychology of Lipoid Proteinosisen_ZA
dc.rights.holderUniversity of Stellenbosch

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