ironjustice

Original Research
Relaxation and susceptibility MRI characteristics in Hallervorden-
Spatz syndrome
Sunali Vinod Desai, MD 1 *, Parayil Shankaran Bindu, MD 2, Shivasankar
Ravishankar, MD 1, Peruvumba Narayan Jayakumar, MD 1, Pramod Kumar
Pal, MD 2
1Department of Neuroimaging and Interventional Radiology, National
Institute of Mental Health and Neurosciences, Bangalore, India
2Department of Neurology, National Institute of Mental Health and
Neurosciences, Bangalore, India

email: Sunali Vinod Desai (sunalidesai@yahoo.com)

*Correspondence to Sunali Vinod Desai, Department of Radiodiagnosis
and Imaging, Rasturba Medical College, MAHE, Udupi, Karnataka,
Manipal, India 576104

Keywords
Hallervorden-Spatz syndrome · MR imaging · SWI · BOLD · FLASH · proton
spectroscopy


Abstract

Purpose
To evaluate the imaging characteristics of the brain with respect to
relaxation and susceptibility in Hallervorden-Spatz syndrome (HSS), a
rare inherited neurodegenerative disorder (also referred to as
neurodegeneration with brain iron ac***ulation).

Materials and Methods
We reviewed 13 affected individuals who satisfied the inclusion
criteria for HSS. Clinically, the patients were divided into two
groups: early-childhood onset (age of onset before 10 years) and late-
childhood onset (age of onset after 10 years). MRI was performed on
1.5T MR equipment. The imaging protocol included spin-echo (SE) T1-
weighted (T1W), turbo spin-echo (TSE) T2W, and fluid attenuated
inversion recovery (FLAIR) sequences in all patients. Susceptibility-
weighted imaging (SWI) included a fast low-angle shot (FLASH) sequence
in 10 patients and a blood oxygen level-dependent (BOLD) sequence in
two patients.

Results
All of the patients showed hyperintensity on T1WI and hypointensity on
T2WI in the globus pallidi (GPs) bilaterally. Central or anteromedial
hyperintensity was found in all but one patient. FLASH showed
augmented hypointensity in 10 patients, and BOLD showed bilateral
striatonigral abnormal pigmentation in two patients. MR spectroscopy
(MRS) showed normal spectra in four patients, and a reduced NAA/Cho
ratio in two.

Conclusion
MRI showed prominent signal abnormalities in the GP bilaterally in
HSS. T1WI showed hyperintensity in all cases of HSS in addition to the
eye-of-the-tiger sign on T2WI. SWI, FLASH, and BOLD demonstrated
mineral deposition in the GP better than conventional imaging.
Involvement of the striatonigral pathways was demonstrated for the
first time on BOLD SWI. J. Magn. Reson. Imaging 2007. © 2007 Wiley-
Liss, Inc.

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Received: 2 April 2005; Accepted: 27 September 2006
Digital Object Identifier (DOI)

10.1002/jmri.20830 About DOI

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