Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration

We report a case of a 13-year-old girl with Hallervorden-Spatz disease (HSD) or pantothenate kinase-2 associated neurodegeneration (PKAN). HSD is a rare neurodegenerative disorder, which is characterized by a rapidly progressive extrapyramidal syndrome, dementia with optic atrophy, and retinal degen...

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Autores principales: Parashari, Umesh C, Aga, Pallavi, Parihar, Anit, Singh, Ragini, Joshi, Vindhya
Formato: Texto
Lenguaje:English
Publicado: Medknow Publications 2010
Materias:
Neuroradiology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2963751/
https://www.ncbi.nlm.nih.gov/pubmed/21042441
http://dx.doi.org/10.4103/0971-3026.69353
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author Parashari, Umesh C
Aga, Pallavi
Parihar, Anit
Singh, Ragini
Joshi, Vindhya
author_facet Parashari, Umesh C
Aga, Pallavi
Parihar, Anit
Singh, Ragini
Joshi, Vindhya
author_sort Parashari, Umesh C
collection PubMed
description We report a case of a 13-year-old girl with Hallervorden-Spatz disease (HSD) or pantothenate kinase-2 associated neurodegeneration (PKAN). HSD is a rare neurodegenerative disorder, which is characterized by a rapidly progressive extrapyramidal syndrome, dementia with optic atrophy, and retinal degeneration. It is associated with accumulation of cysteine-iron complex in the globus pallidi and substantia nigra. The MRI “eye of the tiger” sign is the characteristic. MRI spectroscopy is also characteristic. It shows markedly decreased NAA/Cr values in the globus pallidi and substantia nigra with increased mI/Cr values that suggest of gliosis.
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spelling pubmed-29637512010-11-01 Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration Parashari, Umesh C Aga, Pallavi Parihar, Anit Singh, Ragini Joshi, Vindhya Indian J Radiol Imaging Neuroradiology We report a case of a 13-year-old girl with Hallervorden-Spatz disease (HSD) or pantothenate kinase-2 associated neurodegeneration (PKAN). HSD is a rare neurodegenerative disorder, which is characterized by a rapidly progressive extrapyramidal syndrome, dementia with optic atrophy, and retinal degeneration. It is associated with accumulation of cysteine-iron complex in the globus pallidi and substantia nigra. The MRI “eye of the tiger” sign is the characteristic. MRI spectroscopy is also characteristic. It shows markedly decreased NAA/Cr values in the globus pallidi and substantia nigra with increased mI/Cr values that suggest of gliosis. Medknow Publications 2010-08 /pmc/articles/PMC2963751/ /pubmed/21042441 http://dx.doi.org/10.4103/0971-3026.69353 Text en © Indian Journal of Radiology and Imaging http://creativecommons.org/licenses/by/2.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Neuroradiology
Parashari, Umesh C
Aga, Pallavi
Parihar, Anit
Singh, Ragini
Joshi, Vindhya
Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration
title Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration
title_full Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration
title_fullStr Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration
title_full_unstemmed Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration
title_short Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration
title_sort case report: mr spectroscopy in pantothenate kinase-2 associated neurodegeneration
topic Neuroradiology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2963751/
https://www.ncbi.nlm.nih.gov/pubmed/21042441
http://dx.doi.org/10.4103/0971-3026.69353
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