Histopathologic Correlates of Radial Stripes on MR Images in Lysosomal Storage Disorders
J. Patrick van der Voorna,b,
Petra J. W. Pouwelsc,
Wout Kamphorstb,
James M. Powersf,
Martin Lammense,
Frederik Barkhofd and
Marjo S. van der Knaapa
a Department of Child Neurology, VU University Medical Center, Amsterdam
b Department of Pathology, VU University Medical Center, Amsterdam
c Department of Physics and Medical Technology, VU University Medical Center, Amsterdam
d Department of Radiology, VU University Medical Center, Amsterdam
e the Departments of Pathology and Neurology, University Medical Center Nijmegen, the Netherlands
f the Departments of Pathology and Neurology, University of Rochester School of Medicine and Dentistry, NY
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FIG 1. Patient 1, who had GM1.
A and B, In vivo transverse T2-weighted image (A) and postmortem coronal 3D FLAIR image (B) show a diffuse and symmetric increase in signal intensity in the cerebral white matter, with radial stripes of low signal intensity (arrows).
C and D, Photomicrograph (hematoxylin and eosin, original magnification x1.5) of a matched histopathologic section (C) and magnified view (Luxol fast blue, original magnification x75) of the small box in C (D) reveal perivenular areas of relatively normal density of myelin sheaths (blue in D) in the hypomyelinated central white matter (top left and bottom right in D); these areas corresponding to the hypointense stripes on MR imaging.
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FIG 2. Patient 2, who had MLD.
A and B, In vivo transverse T2-weighted image (A) and postmortem coronal 3D FLAIR image (B) show a hyperintense centrum semiovale with radially oriented stripes of hypointense signal intensity (arrows).
C, Photomicrograph (Luxol fast blue, original magnification x1) demonstrates a profound absence of myelin throughout the centrum semiovale with some sparing of the areas of subcortical U fibers.
D and E, Magnified views of hematoxylin and eosin (D, original magnification x37.5) and Luxol fast blue (E, original magnification x75) stains of the small box in C show that the hypointense linear bands correspond with numerous eosinophilic granule containing glial cells and macrophages (arrowheads) around venules (v) and myelin (blue stripes in E).
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FIG 3. Patient 3, who had GLD.
A and B, In vivo transverse T2-weighted image (A) and postmortem coronal 3D FLAIR image (B) show an atrophic brain with hyperintense white matter and linear areas of hypointense signal intensity, extending from the cerebral cortex to the ventricular wall (arrow).
C and D, Photomicrograph of hematoxylin and eosin stain (C, original magnification, x1.5) and magnified view of CD68 stain (D, original magnification x37.5) of the small box in C show perivascular clustering of multinucleated globoid cells (arrowheads, v = venule).
E, MBP stain (original magnification x75) shows almost complete absence of myelin sheaths (arrow).
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