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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| Format: | Article |
| Language: | English |
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Thieme Medical and Scientific Publishers Pvt. Ltd.
2010-07-01
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| Series: | Indian Journal of Radiology and Imaging |
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| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.4103/0971-3026.69353 |
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| author | Umesh C Parashari Pallavi Aga Anit Parihar Ragini Singh Vindhya Joshi |
| author_facet | Umesh C Parashari Pallavi Aga Anit Parihar Ragini Singh Vindhya Joshi |
| author_sort | Umesh C Parashari |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-17T21:03:23Z |
| format | Article |
| id | doaj.art-60de28d060db4d04904876f1687fdf04 |
| institution | Directory Open Access Journal |
| issn | 0971-3026 1998-3808 |
| language | English |
| last_indexed | 2024-12-17T21:03:23Z |
| publishDate | 2010-07-01 |
| publisher | Thieme Medical and Scientific Publishers Pvt. Ltd. |
| record_format | Article |
| series | Indian Journal of Radiology and Imaging |
| spelling | doaj.art-60de28d060db4d04904876f1687fdf042022-12-21T21:32:40ZengThieme Medical and Scientific Publishers Pvt. Ltd.Indian Journal of Radiology and Imaging0971-30261998-38082010-07-01200318819110.4103/0971-3026.69353Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegenerationUmesh C Parashari0Pallavi Aga1Anit Parihar2Ragini Singh3Vindhya Joshi4Department of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, IndiaDepartment of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, IndiaDepartment of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, IndiaDepartment of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, IndiaDepartment of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, IndiaWe 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.http://www.thieme-connect.de/DOI/DOI?10.4103/0971-3026.69353basal gangliadystoniahallervorden–spatz diseaseiron depositionmagnetic resonance spectroscopypkan |
| spellingShingle | Umesh C Parashari Pallavi Aga Anit Parihar Ragini Singh Vindhya Joshi Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration Indian Journal of Radiology and Imaging basal ganglia dystonia hallervorden–spatz disease iron deposition magnetic resonance spectroscopy pkan |
| 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 | basal ganglia dystonia hallervorden–spatz disease iron deposition magnetic resonance spectroscopy pkan |
| url | http://www.thieme-connect.de/DOI/DOI?10.4103/0971-3026.69353 |
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