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Psychotic manifestation in a patient with Wolfram syndrome (DIDMOAD)

Musa, Ramli and NNA, Fatnoon (2014) Psychotic manifestation in a patient with Wolfram syndrome (DIDMOAD). Malaysian Journal of Psychiatry, 23 (1). ISSN 0128-8628

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Wolfram syndrome (WFS) is a rare neurodegerative disorder which is characterized by presentation of diabetes insipidus, juvenile diabetes mellitus, optic atrophy and deafness. We describe a case of WFS with presentation of psychosis. A 17-year-old female presented with psychiatric manifestations, namely inappropriate behaviour and second person auditory hallucination since the age of 16 years. The patient was diagnosed with type 1 diabetes mellitus at the age of 10 years old and subsequently progressive hearing and visual loss a year later. Her ophthalmic evaluation revealed total blindness due to optic atrophy. However she did not have renal dysfunction and diabetes insipidus which are also features of the syndrome. There is scarce literature to describe on psychiatric presentation in WFS. In the past,the psychiatric manifestation which was reported most of times was mood and suicidal behaviour. Hardly any article reported about psychosis (hallucination). We believe, her psychiatric manifestations were related to sensory deprivation due to blindness and deafness caused by the progression of WFS.

Item Type: Article (Journal)
Additional Information: 5818/35602
Uncontrolled Keywords: Wolfram Syndrome, Diabetes Mellitus, Optic Atrophy, Psychiatric Manifestation
Subjects: R Medicine > RC Internal medicine > RC321 Neuroscience. Biological psychiatry. Neuropsychiatry
Kulliyyahs/Centres/Divisions/Institutes (Can select more than one option. Press CONTROL button): Kulliyyah of Medicine > Department of Psychiatry
Depositing User: Professor Ramli Musa
Date Deposited: 20 Feb 2014 12:02
Last Modified: 20 Feb 2014 12:02
URI: http://irep.iium.edu.my/id/eprint/35602

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