Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration
Title: | Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration |
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Authors: | Umesh C Parashari, Pallavi Aga, Anit Parihar, Ragini Singh, Vindhya Joshi |
Source: | Indian Journal of Radiology and Imaging, Vol 20, Iss 03, Pp 188-191 (2010) |
Publisher Information: | Thieme Medical and Scientific Publishers Pvt. Ltd., 2010. |
Publication Year: | 2010 |
Collection: | LCC:Medical physics. Medical radiology. Nuclear medicine |
Subject Terms: | basal ganglia, dystonia, hallervorden–spatz disease, iron deposition, magnetic resonance spectroscopy, pkan, Medical physics. Medical radiology. Nuclear medicine, R895-920 |
More Details: | 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. |
Document Type: | article |
File Description: | electronic resource |
Language: | English |
ISSN: | 0971-3026 1998-3808 |
Relation: | https://doaj.org/toc/0971-3026; https://doaj.org/toc/1998-3808 |
DOI: | 10.4103/0971-3026.69353 |
Access URL: | https://doaj.org/article/60de28d060db4d04904876f1687fdf04 |
Accession Number: | edsdoj.60de28d060db4d04904876f1687fdf04 |
Database: | Directory of Open Access Journals |
ISSN: | 09713026 19983808 |
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DOI: | 10.4103/0971-3026.69353 |
Published in: | Indian Journal of Radiology and Imaging |
Language: | English |