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Parashari UC, Aga P, Parihar A, et al. Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration. Indian J Radiol Imaging. 2010;20(3):188-91doi: 10.4103/0971-3026.69353.
Parashari, U. C., Aga, P., Parihar, A., Singh, R., & Joshi, V. (2010). Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration. The Indian journal of radiology & imaging, 20(3), 188-91. https://doi.org/10.4103/0971-3026.69353
Parashari, Umesh C, et al. "Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration." The Indian journal of radiology & imaging vol. 20,3 (2010): 188-91. doi: https://doi.org/10.4103/0971-3026.69353
Parashari UC, Aga P, Parihar A, Singh R, Joshi V. Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration. Indian J Radiol Imaging. 2010 Aug;20(3):188-91. doi: 10.4103/0971-3026.69353. PMID: 21042441; PMCID: PMC2963751.
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Indian J Radiol Imaging. 2010 Aug;20(3):188-91. doi: 10.4103/0971-3026.69353.

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

The Indian journal of radiology & imaging

Umesh C Parashari, Pallavi Aga, Anit Parihar, Ragini Singh, Vindhya Joshi

Affiliations

  1. Department of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, India.

PMID: 21042441 PMCID: PMC2963751 DOI: 10.4103/0971-3026.69353

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; Hallervorden–Spatz disease; PKAN; dystonia; iron deposition; magnetic resonance spectroscopy

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