AJDRAJNR - American Journal of Neuroradiology

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Neuropathology for the Neuroradiologist: Rosenthal Fibers

F.J. Wippold, IIa,b,c, A. Perryd,e and J. Lennerzd,e

a From the Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis, Mo
b Department of Radiology, Barnes-Jewish Hospital South, St. Louis, Mo
c Department of Radiology/Nuclear Medicine, Uniformed Services University of the Health Sciences, Bethesda, Md
d F. Edward Hébert School of Medicine, Uniformed Services University of the Health Sciences, Bethesda, Md
e Neuropathology Division, Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, Mo


Figure 1
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Fig 1. Photomicrograph from a pilocytic astrocytoma showing brightly staining red Rosenthal fibers (arrowheads). (Hematoxylin-eosin [H&E]; original magnification, 1000x).


Figure 2
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Fig 2. Drawing of the various classes and structures of intermediate filament proteins. The central rod is composed of 4 {alpha}-helical sub-domains separated by nonhelical linkers. The central rods are remarkably similar among the classes, whereas the heads and tails demonstrate considerable variability and partly contribute to tissue specificity. Most of the described mutations in Alexander disease occur in the 1A and 2A subdomains of the rod. Modified from ref. 22. With permission.


Figure 3
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Fig 3. Alexander disease.

A, T2-weighted MR image demonstrates the marked hyperintensity in the cerebral white matter of a small child. The white matter of the frontal lobes is often initially involved with posterior progression as the disease advances. Cystic changes may also eventually develop. As in this case, the caudate heads, anterior putamina, and thalami are often involved, a hypointense periventricular rim on T2-weighted images frequently occurs (arrowheads), and the cavum septi pellucidi may be expanded (case courtesy of A. James Barkovich).

B, Photomicrograph from a different patient showing extensive brightly staining red Rosenthal fibers in a predominantly subpial distribution (Hematoxylin-eosin [H&E]; original magnification, 100x).

C, Photomicrograph from a different patient showing extensive brightly staining red Rosenthal fibers in a perivascular distribution (H&E; original magnification, 1000x).