Genetic Analysis for Two Italian Siblings with Usher Syndrome and Schizophrenia

Usher syndrome is a group of autosomal recessive genetic disorders characterized by deafness, retinitis pigmentosa, and sometimes vestibular areflexia. The relationship between Usher syndrome and mental disorders, most commonly a “schizophrenia-like” psychosis, is sometimes described in the literatu...

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Main Authors: Daniela Domanico, Serena Fragiotta, Paolo Trabucco, Marcella Nebbioso, Enzo Maria Vingolo
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Case Reports in Ophthalmological Medicine
Online Access:http://dx.doi.org/10.1155/2012/380863
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author Daniela Domanico
Serena Fragiotta
Paolo Trabucco
Marcella Nebbioso
Enzo Maria Vingolo
author_facet Daniela Domanico
Serena Fragiotta
Paolo Trabucco
Marcella Nebbioso
Enzo Maria Vingolo
author_sort Daniela Domanico
collection DOAJ
description Usher syndrome is a group of autosomal recessive genetic disorders characterized by deafness, retinitis pigmentosa, and sometimes vestibular areflexia. The relationship between Usher syndrome and mental disorders, most commonly a “schizophrenia-like” psychosis, is sometimes described in the literature. The etiology of psychiatric expression of Usher syndrome is still unclear. We reported a case of two natural siblings with congenital hypoacusis, retinitis pigmentosa, and psychiatric symptoms. Clinical features and genetic analysis were also reported. We analyzed possible causes to explain the high prevalence of psychiatric manifestations in Usher syndrome: genetic factors, brain damage, and “stress-related” hypothesis.
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institution Kabale University
issn 2090-6722
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language English
publishDate 2012-01-01
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series Case Reports in Ophthalmological Medicine
spelling doaj-art-6d3c7ac2e20242d5bc15881d978fdfba2025-08-20T03:33:51ZengWileyCase Reports in Ophthalmological Medicine2090-67222090-67302012-01-01201210.1155/2012/380863380863Genetic Analysis for Two Italian Siblings with Usher Syndrome and SchizophreniaDaniela Domanico0Serena Fragiotta1Paolo Trabucco2Marcella Nebbioso3Enzo Maria Vingolo4Department of Ophthalmology, “S. M. Goretti” Hospital, Sapienza University of Rome, Via G. Reni, 04100 Latina, ItalyDepartment of Ophthalmology, “A. Fiorini” Hospital, Sapienza University of Rome, Polo Pontino, Via Firenze, 04019 Terracina, ItalyDepartment of Ophthalmology, “A. Fiorini” Hospital, Sapienza University of Rome, Polo Pontino, Via Firenze, 04019 Terracina, ItalyDepartment of Sense Organs, Centre of Ocular Electrophysiology, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, ItalyDepartment of Ophthalmology, “A. Fiorini” Hospital, Sapienza University of Rome, Polo Pontino, Via Firenze, 04019 Terracina, ItalyUsher syndrome is a group of autosomal recessive genetic disorders characterized by deafness, retinitis pigmentosa, and sometimes vestibular areflexia. The relationship between Usher syndrome and mental disorders, most commonly a “schizophrenia-like” psychosis, is sometimes described in the literature. The etiology of psychiatric expression of Usher syndrome is still unclear. We reported a case of two natural siblings with congenital hypoacusis, retinitis pigmentosa, and psychiatric symptoms. Clinical features and genetic analysis were also reported. We analyzed possible causes to explain the high prevalence of psychiatric manifestations in Usher syndrome: genetic factors, brain damage, and “stress-related” hypothesis.http://dx.doi.org/10.1155/2012/380863
spellingShingle Daniela Domanico
Serena Fragiotta
Paolo Trabucco
Marcella Nebbioso
Enzo Maria Vingolo
Genetic Analysis for Two Italian Siblings with Usher Syndrome and Schizophrenia
Case Reports in Ophthalmological Medicine
title Genetic Analysis for Two Italian Siblings with Usher Syndrome and Schizophrenia
title_full Genetic Analysis for Two Italian Siblings with Usher Syndrome and Schizophrenia
title_fullStr Genetic Analysis for Two Italian Siblings with Usher Syndrome and Schizophrenia
title_full_unstemmed Genetic Analysis for Two Italian Siblings with Usher Syndrome and Schizophrenia
title_short Genetic Analysis for Two Italian Siblings with Usher Syndrome and Schizophrenia
title_sort genetic analysis for two italian siblings with usher syndrome and schizophrenia
url http://dx.doi.org/10.1155/2012/380863
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