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CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2010; 20(03): 188-191
DOI: 10.4103/0971-3026.69353
Neuroradiology

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

Umesh C Parashari
Department of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, India
,
Pallavi Aga
Department of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, India
,
Anit Parihar
Department of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, India
,
Ragini Singh
Department of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, India
,
Vindhya Joshi
Department of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, India
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Abstract

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.

Keywords

Basal ganglia - dystonia - Hallervorden–Spatz disease - iron deposition - magnetic resonance spectroscopy - PKAN


Publication History

Article published online:
02 August 2021

© 2010. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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