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Histopathologic correlates of radial stripes on MR images in lysosomal storage disorders
J Patrick van der Voorn1, Petra J W Pouwels, Wout Kamphorst
1Department of Child Neurology, VU University Medical Center, Amsterdam, The Netherlands.
Background And Purpose:
Radially oriented hypointense stripes in hyperintense cerebral white matter are recognized on T2-weighted images of certain lysosomal storage disorders. We compared in vivo and postmortem MR imaging with histopathologic findings in three patients with metachromatic leukodystrophy (MLD), globoid cell leukodystrophy (GLD), and infantile GM1 gangliosidosis (GM1) to understand this characteristic MR imaging pattern.
Methods:
The in vivo MR imaging protocol comprised T1- and T2-weighted spin-echo and fluid-attenuated inversion recovery imaging. Postmortem MR imaging, including coronal 1-cm-thick brain sections, was performed after at least 5 weeks of formalin fixation and included T2-weighted spin-echo images and 3D fluid-attenuated inversion recovery images with high spatial resolution. Afterward, the sections were embedded in paraffin, whole-mount sections were made, and neuropathologic stains were applied.
Results:
Similar imaging features were found in the three patients on in vivo and postmortem images, with more prominent stripes in GLD and MLD than in GM1. Neuropathologic examination revealed that the stripes were related to relative sparing of myelin in the perivenular regions in GM1 and MLD, but lipid-containing glial cells were also present in these areas in MLD. Perivenular clusters of globoid cells containing lipid material in absence of any myelin corresponded to the stripes in GLD.
Conclusion:
Results of our postmortem study showed that radial stripes of white matter on MR images represented relative myelin sparing in some lysosomal storage disorders, but they may also represent lipid storage.
Insights
Radial white matter stripes in lysosomal storage disorders on MRI represent myelin sparing or lipid storage. This study compared in vivo and postmortem imaging with histopathology in metachromatic leukodystrophy, globoid cell leukodystrophy, and GM1 gangliosidosis.
Area of Science:
- Neuroimaging
- Neuropathology
- Lysosomal Storage Disorders
Background:
- T2-weighted MRI can reveal radially oriented hypointense stripes in cerebral white matter in certain lysosomal storage disorders.
- These characteristic imaging patterns necessitate further investigation to understand their underlying pathology.
Purpose of the Study:
- To compare in vivo and postmortem MR imaging findings with histopathologic results.
- To elucidate the nature of radial white matter stripes in metachromatic leukodystrophy (MLD), globoid cell leukodystrophy (GLD), and infantile GM1 gangliosidosis (GM1).
Main Methods:
- Utilized T1- and T2-weighted spin-echo and fluid-attenuated inversion recovery (FLAIR) MR imaging protocols in vivo.
- Conducted high-resolution postmortem MR imaging on formalin-fixed brain sections, followed by neuropathologic staining and analysis.
Main Results:
- Consistent imaging features were observed across in vivo and postmortem studies, with more pronounced stripes in GLD and MLD compared to GM1.
- Histopathology revealed stripes related to relative myelin sparing in perivenular regions (GM1, MLD) and lipid-laden glial cells (MLD).
- In GLD, stripes corresponded to perivenular globoid cell clusters containing lipids without myelin.
Conclusions:
- Postmortem analysis indicates that radial white matter stripes on MR images can represent relative myelin sparing in lysosomal storage disorders.
- These stripes may also reflect lipid accumulation within the affected white matter regions.
