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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

MRI spectroscopy performed with an ROI (region of interest) circle placed in the right globus pallidus (A). The spectra reveal a markedly decreased NAA peak (white arrowhead in B), with a decreased NAA/Cr and an increased myoinositol peak (thin white arrow in C) in the globus pallidi
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Figure 0004: MRI spectroscopy performed with an ROI (region of interest) circle placed in the right globus pallidus (A). The spectra reveal a markedly decreased NAA peak (white arrowhead in B), with a decreased NAA/Cr and an increased myoinositol peak (thin white arrow in C) in the globus pallidi

Mentions: To evaluate the neuroaxonal degeneration quantitatively, proton MRS was performed using multivoxel chemical-shift imaging with spin-echo, point resolved spectroscopy (PRESS). The imaging parameters were as follows: TR - 4000 ms; TE - 86.6 and 35 ms; slab thickness - 10 mm; voxel size - 20 × 20 × 20; number of acquisitions - 2, and scan time - 6.0 min. Automated software was used for the calibration of the spectrum. Spectra were taken with the ROI (region of interest) circle placed in the right globus pallidus [Figure 4A], which showed the following values; N-acetylaspartate(NAA)/creatine(Cr): 1.14 and myoinositol(mI)/Cr: 0.73. The study revealed a decreased NAA peak [Figure 3B] and a reduced NAA/Cr ratio [Figure 4B], suggesting neuroaxonal loss. An increased myoinositol peak (thin white arrow) and mI/Cr ratio were seen on MRS done at a TE of 35 ms, suggestive of glial proliferation [Figure 4C].


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)

MRI spectroscopy performed with an ROI (region of interest) circle placed in the right globus pallidus (A). The spectra reveal a markedly decreased NAA peak (white arrowhead in B), with a decreased NAA/Cr and an increased myoinositol peak (thin white arrow in C) in the globus pallidi
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 0004: MRI spectroscopy performed with an ROI (region of interest) circle placed in the right globus pallidus (A). The spectra reveal a markedly decreased NAA peak (white arrowhead in B), with a decreased NAA/Cr and an increased myoinositol peak (thin white arrow in C) in the globus pallidi
Mentions: To evaluate the neuroaxonal degeneration quantitatively, proton MRS was performed using multivoxel chemical-shift imaging with spin-echo, point resolved spectroscopy (PRESS). The imaging parameters were as follows: TR - 4000 ms; TE - 86.6 and 35 ms; slab thickness - 10 mm; voxel size - 20 × 20 × 20; number of acquisitions - 2, and scan time - 6.0 min. Automated software was used for the calibration of the spectrum. Spectra were taken with the ROI (region of interest) circle placed in the right globus pallidus [Figure 4A], which showed the following values; N-acetylaspartate(NAA)/creatine(Cr): 1.14 and myoinositol(mI)/Cr: 0.73. The study revealed a decreased NAA peak [Figure 3B] and a reduced NAA/Cr ratio [Figure 4B], suggesting neuroaxonal loss. An increased myoinositol peak (thin white arrow) and mI/Cr ratio were seen on MRS done at a TE of 35 ms, suggestive of glial proliferation [Figure 4C].

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