{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T19:02:39Z","timestamp":1740164559225,"version":"3.37.3"},"reference-count":9,"publisher":"Wiley","license":[{"start":{"date-parts":[[2014,1,1]],"date-time":"2014-01-01T00:00:00Z","timestamp":1388534400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Case Reports in Psychiatry"],"published-print":{"date-parts":[[2014]]},"abstract":"<jats:p>Neurodegeneration with brain iron accumulation (NBIA) is a collective term to indicate a group of neurodegenerative diseases presenting accumulation of iron in the basal ganglia. These disorders can result in progressive dystonia, spasticity, parkinsonism, neuropsychiatric abnormalities, and optic atrophy or retinal degeneration. Onset age ranges from infancy to late adulthood and the rate of progression is very variable. So far, the genetic bases of nine types of NBIA have been identified, pantothenate-kinase-associated neurodegeneration (PKAN) being the most frequent type. The brain MRI \u201ceye-of-the-tiger\u201d sign, T2-weighted hypointense signal in the<jats:italic>globus pallidus<\/jats:italic>with a central region of hyperintensity, has been considered virtually pathognomonic for PKAN but recently several reports have denied this. A significant percentage of individuals with clinical and radiographic evidence of NBIA do not have an alternate diagnosis or mutation of one of the nine known NBIA-associated genes (idiopathic NBIA). Here we present an adult-onset case of \u201cundiagnosed\u201d NBIA with the brain MRI \u201ceye-of-the-tiger\u201d sign, and with psychotic symptoms which were successfully treated with antipsychotic and mood stabilizer medications. Here, the term \u201cundiagnosed\u201d is used because the patient has not been screened for all known NBIA genes, but only for two of them.<\/jats:p>","DOI":"10.1155\/2014\/742042","type":"journal-article","created":{"date-parts":[[2014,5,20]],"date-time":"2014-05-20T17:17:12Z","timestamp":1400606232000},"page":"1-3","source":"Crossref","is-referenced-by-count":2,"title":["Adult-Onset Case of Undiagnosed Neurodegeneration with Brain Iron Accumulation with Psychotic Symptoms"],"prefix":"10.1155","volume":"2014","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5976-6738","authenticated-orcid":true,"given":"Luigi","family":"Attademo","sequence":"first","affiliation":[{"name":"School of Psychiatry, University of Perugia, 06156 Perugia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Enrico","family":"Paolini","sequence":"additional","affiliation":[{"name":"School of Psychiatry, University of Perugia, 06156 Perugia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Bernardini","sequence":"additional","affiliation":[{"name":"School of Psychiatry, University of Perugia, 06156 Perugia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roberto","family":"Quartesan","sequence":"additional","affiliation":[{"name":"School of Psychiatry, University of Perugia, 06156 Perugia, Italy"},{"name":"Division of Psychiatry, Clinical Psychology and Psychiatric Rehabilitation, Department of Clinical and Experimental Medicine, Santa Maria della Misericordia Hospital, University of Perugia, 06156 Perugia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrizia","family":"Moretti","sequence":"additional","affiliation":[{"name":"School of Psychiatry, University of Perugia, 06156 Perugia, Italy"},{"name":"Division of Psychiatry, Clinical Psychology and Psychiatric Rehabilitation, Department of Clinical and Experimental Medicine, Santa Maria della Misericordia Hospital, University of Perugia, 06156 Perugia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","reference":[{"first-page":"1993","volume-title":"Neurodegeneration with Brain Iron Accumulation disorders overview","year":"2013","key":"1"},{"key":"2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa020817"},{"issue":"1","key":"3","first-page":"59","volume":"11","year":"2013","journal-title":"Current Neuropharmacology"},{"issue":"4","key":"4","first-page":"260","volume":"39","year":"2011","journal-title":"Actas Espa\u00f1olas de Psiquiatr\u00eda"},{"key":"5","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/860201"},{"issue":"4","key":"6","first-page":"286","volume":"43","year":"2005","journal-title":"Folia Neuropathologica"},{"issue":"1","key":"7","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1002\/brb3.8","volume":"1","year":"2011","journal-title":"Brain and Behavior"},{"key":"8","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.63.2.292"},{"key":"9","doi-asserted-by":"publisher","DOI":"10.4306\/pi.2009.6.3.226"}],"container-title":["Case Reports in Psychiatry"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/downloads.hindawi.com\/journals\/crips\/2014\/742042.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/crips\/2014\/742042.xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/crips\/2014\/742042.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2017,6,22]],"date-time":"2017-06-22T09:31:42Z","timestamp":1498123902000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.hindawi.com\/journals\/crips\/2014\/742042\/"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014]]},"references-count":9,"alternative-id":["742042","742042"],"URL":"https:\/\/doi.org\/10.1155\/2014\/742042","relation":{},"ISSN":["2090-682X","2090-6838"],"issn-type":[{"type":"print","value":"2090-682X"},{"type":"electronic","value":"2090-6838"}],"subject":[],"published":{"date-parts":[[2014]]}}}