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

Parashari UC, Aga P, Parihar A, Singh R, Joshi V - Indian J Radiol Imaging (2010)

Bottom Line: 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.It shows markedly decreased NAA/Cr values in the globus pallidi and substantia nigra with increased mI/Cr values that suggest of gliosis.

View Article: PubMed Central - PubMed

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

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.

No MeSH data available.


Related in: MedlinePlus

Axial FLAIR MRI image shows hypointense signals (arrows) within both globus pallidi
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Figure 0002: Axial FLAIR MRI image shows hypointense signals (arrows) within both globus pallidi

Mentions: MRI examination was performed on a 1.5T scanner (GE Medical Systems, Milwaukee, USA). MRI showed marked hypointensity within both globus pallidi, with a small area of central hyperintensity (eye-of-the-tiger sign) on T2W images [Figure 1AB]. Similar hypointense signals were also seen on the FLAIR images [Figure 2]. The marked hypointensity in the globus pallidi was better appreciated on T2W gradient-recalled-echo (GRE) images due to the susceptibility effect, suggesting iron deposition [Figure 3A]. Similar T2-hypointense lesions were seen in the substantia nigra [Figure 3B]. Mild thinning of the corpus callosum was noted in the region of the body. Based on the clinical assessment and the typical MRI findings, we arrived at the diagnosis of PKAN.


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

Parashari UC, Aga P, Parihar A, Singh R, Joshi V - Indian J Radiol Imaging (2010)

Axial FLAIR MRI image shows hypointense signals (arrows) within both globus pallidi
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2963751&req=5

Figure 0002: Axial FLAIR MRI image shows hypointense signals (arrows) within both globus pallidi
Mentions: MRI examination was performed on a 1.5T scanner (GE Medical Systems, Milwaukee, USA). MRI showed marked hypointensity within both globus pallidi, with a small area of central hyperintensity (eye-of-the-tiger sign) on T2W images [Figure 1AB]. Similar hypointense signals were also seen on the FLAIR images [Figure 2]. The marked hypointensity in the globus pallidi was better appreciated on T2W gradient-recalled-echo (GRE) images due to the susceptibility effect, suggesting iron deposition [Figure 3A]. Similar T2-hypointense lesions were seen in the substantia nigra [Figure 3B]. Mild thinning of the corpus callosum was noted in the region of the body. Based on the clinical assessment and the typical MRI findings, we arrived at the diagnosis of PKAN.

Bottom Line: 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.It shows markedly decreased NAA/Cr values in the globus pallidi and substantia nigra with increased mI/Cr values that suggest of gliosis.

View Article: PubMed Central - PubMed

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

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.

No MeSH data available.


Related in: MedlinePlus