American Journal of Neuroradiology, Vol 14, Issue 1 155-162, Copyright © 1993 by American Society of Neuroradiology
ARTICLES |
Hallervorden-Spatz disease: MR and pathologic findings
M Savoiardo, WC Halliday, N Nardocci, L Strada, L D'Incerti, L Angelini, V Rumi and JD Tesoro-Tess
Department of Neuroradiology, Istituto Nazionale Neurologico C. Besta, Milano, Italy.
PURPOSE: To compare the MR findings of eight cases with clinical diagnosis of Hallervorden-Spatz disease (HSD) with the pathologic findings of two other cases of HSD. MATERIALS AND METHODS: The eight imaged cases were studied with 0.5-T (seven cases) and/or 1.5-T (five cases) units. Six patients also had CT scans. The two other cases with proven HSD had detailed histologic evaluation. RESULTS: The 1.5-T findings showed abnormalities confined to the pallidum, which presented a diffuse low signal intensity in T2-weighted images, and an anteromedial area of high signal intensity (eye-of-the-tiger sign). In 0.5-T studies, low signal intensity was less evident and poorly detectable in spin echo, but gradient-echo images could enhance its demonstration; the area of high signal intensity was always well demonstrated. In three cases (three with 1.5 T, one with 0.5 T) a central spot of low signal intensity was seen in this area. The pathologic cases, in addition to neuroaxonal swellings and iron deposits, exhibited areas of "loose" tissue with vacuolization and lesser amounts of iron in the anteromedial part of the pallidum, in a location corresponding to the area of high signal intensity of the imaged cases. CONCLUSION: Comparison of MR findings with the pathologic studies demonstrates that the low signal intensity in T2-weighted images at 1.5 T corresponds to iron deposits in a dense tissue, and that the high signal intensity of the eye-of-the-tiger sign corresponds to an area of loose tissue with vacuolization. No correlation was found in the two pathologic cases for the central spot of low signal intensity.
This article has been cited by other articles:
 |
|
 |
|
  M. Koyama and A. Yagishita Pantothenate Kinase-Associated Neurodegeneration with Increased Lentiform Nuclei Cerebral Blood Flow AJNR Am. J. Neuroradiol., January 1, 2006; 27(1): 212 - 213. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. T. Pellecchia, E. M. Valente, L. Cif, S. Salvi, A. Albanese, V. Scarano, U. Bonuccelli, A. R. Bentivoglio, A. D'Amico, C. Marelli, et al. The diverse phenotype and genotype of pantothenate kinase-associated neurodegeneration Neurology, May 24, 2005; 64(10): 1810 - 1812. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Grisoli, A. Piperno, L. Chiapparini, R. Mariani, and M. Savoiardo MR Imaging of Cerebral Cortical Involvement in Aceruloplasminemia AJNR Am. J. Neuroradiol., March 1, 2005; 26(3): 657 - 661. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. N. Sener Pantothenate Kinase-Associated Neurodegeneration: MR Imaging, Proton MR Spectroscopy, and Diffusion MR Imaging Findings AJNR Am. J. Neuroradiol., September 1, 2003; 24(8): 1690 - 1693. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. P. Guillerman The Eye-of-the-Tiger Sign Radiology, December 1, 2000; 217(3): 895 - 896. [Full Text]
|
|
|
|
 |
|
 J. E. Galvin, B. Giasson, H. I. Hurtig, V. M.-Y. Lee, and J. Q. Trojanowski Neurodegeneration with Brain Iron Accumulation, Type 1 Is Characterized by {alpha}-, {beta}-, and {gamma}-Synuclein Neuropathology Am. J. Pathol., August 1, 2000; 157(2): 361 - 368. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. I. Grossman BRAIN IMAGING AJNR Am. J. Neuroradiol., January 1, 2000; 21(1): 9 - 18. [Full Text] [PDF]
|
|
|
|
|
|
N. Nardocci, G. Zorzi, L. Farina, S. Binelli, W. Scaioli, C. Ciano, L. Verga, L. Angelini, M. Savoiardo, and O. Bugiani Infantile neuroaxonal dystrophy: Clinical spectrum and diagnostic criteria Neurology, April 1, 1999; 52(7): 1472 - 1472. [Abstract] [Full Text]
|
|