Neurodegeneration with Brain Iron Accumulation in an Eleven-Year-Old Jamaican Male

We present a case of an eleven-year-old boy presenting with progressive extrapyramidal signs and dementia. His imaging findings demonstrated the classic eye-of-the-tiger sign on T2W magnetic resonance imaging. He was diagnosed with pantothenate kinase-associated neurodegeneration (PKAN). This is a r...

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Main Authors: Peter Johnson, Roxanne Melbourne-Chambers, Nilesh Desai, Emma Greenaway
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Radiology
Online Access:http://dx.doi.org/10.1155/2014/858056
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author Peter Johnson
Roxanne Melbourne-Chambers
Nilesh Desai
Emma Greenaway
author_facet Peter Johnson
Roxanne Melbourne-Chambers
Nilesh Desai
Emma Greenaway
author_sort Peter Johnson
collection DOAJ
description We present a case of an eleven-year-old boy presenting with progressive extrapyramidal signs and dementia. His imaging findings demonstrated the classic eye-of-the-tiger sign on T2W magnetic resonance imaging. He was diagnosed with pantothenate kinase-associated neurodegeneration (PKAN). This is a rare autosomal recessive inborn error of coenzyme A metabolism, caused by mutations in PANK2. This is the first reported case of PKAN from the Caribbean.
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institution Kabale University
issn 2090-6862
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publishDate 2014-01-01
publisher Wiley
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series Case Reports in Radiology
spelling doaj-art-2e0545cd31b74656b3cbc3e02a71d9902025-08-20T03:55:07ZengWileyCase Reports in Radiology2090-68622090-68702014-01-01201410.1155/2014/858056858056Neurodegeneration with Brain Iron Accumulation in an Eleven-Year-Old Jamaican MalePeter Johnson0Roxanne Melbourne-Chambers1Nilesh Desai2Emma Greenaway3Department of Surgery, Radiology, Anaesthetics and Intensive Care, Faculty of Medical Sciences, University of the West Indies (Mona Campus), Kingston, JamaicaDepartment of Child and Adolescent Health, Faculty of Medical Sciences, University of the West Indies (Mona Campus), Kingston, JamaicaDepartment of Radiology and Imaging Sciences, Emory University School of Medicine, Emory University Hospital, BG21, 1364 Clifton Road, Atlanta, GA 30322, USADepartment of Child and Adolescent Health, Faculty of Medical Sciences, University of the West Indies (Mona Campus), Kingston, JamaicaWe present a case of an eleven-year-old boy presenting with progressive extrapyramidal signs and dementia. His imaging findings demonstrated the classic eye-of-the-tiger sign on T2W magnetic resonance imaging. He was diagnosed with pantothenate kinase-associated neurodegeneration (PKAN). This is a rare autosomal recessive inborn error of coenzyme A metabolism, caused by mutations in PANK2. This is the first reported case of PKAN from the Caribbean.http://dx.doi.org/10.1155/2014/858056
spellingShingle Peter Johnson
Roxanne Melbourne-Chambers
Nilesh Desai
Emma Greenaway
Neurodegeneration with Brain Iron Accumulation in an Eleven-Year-Old Jamaican Male
Case Reports in Radiology
title Neurodegeneration with Brain Iron Accumulation in an Eleven-Year-Old Jamaican Male
title_full Neurodegeneration with Brain Iron Accumulation in an Eleven-Year-Old Jamaican Male
title_fullStr Neurodegeneration with Brain Iron Accumulation in an Eleven-Year-Old Jamaican Male
title_full_unstemmed Neurodegeneration with Brain Iron Accumulation in an Eleven-Year-Old Jamaican Male
title_short Neurodegeneration with Brain Iron Accumulation in an Eleven-Year-Old Jamaican Male
title_sort neurodegeneration with brain iron accumulation in an eleven year old jamaican male
url http://dx.doi.org/10.1155/2014/858056
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AT emmagreenaway neurodegenerationwithbrainironaccumulationinanelevenyearoldjamaicanmale