Antemortem radiologic and histopathologic presentation of Marchiafava-Bignami disease

Figures

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  Figure 1.. Imaging features of antemortem case 1. (A-C) Sagittal MRI prior to biopsy shows the lesion, which occupies the full thickness of the posterior body and isthmus of the corpus callosum, “points” toward the center of the corpus callosum anteriorly, and is sharply marginated vertically posteriorly. On day 8 of admission, the callosal lesion shows low signal intensity (SI) on the T1-weighted (T1W) image (A), punctate and patchy enhancement on the Gadolinium-enhanced T1W image (B), and high SI on the T2-weighted (T2W) image (C). (D) On day 25 of admission, sagittal reformatted CT scan documents the site of the biopsy at the mid-body of the corpus callosum (arrowhead), air within the biopsy cavity, within the ventricle, and within the subarachnoid cisterns. (E-H) On day 8 of admission, axial MRIs show asymmetry, slightly greater extent, and greater SI of the lesion on the right side. There is high SI on the diffusion-weighted image (E), low SI on the corresponding apparent diffusion coefficient (ADC) map (F), high SI on the T2W FLAIR (fluid-attenuated inversion recovery) series (G), and “horizontal” linear enhancement oriented parallel to the course of the crossing callosal fibers on…

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  Figure 2.. Biopsy features of antemortem case 1. (A, B) H&E staining demonstrates macrophages and perivascular lymphocytic inflammation. (C, D) LFB/H&E stains demonstrate loss of myelin in a fragment of white matter and macrophages with myelinophagia. (E, F) CD68 immunohistochemistry demonstrates abundant macrophages. (G, H) Neurofilament immunohistochemistry demonstrates scattered residual axons between sheets of macrophages. Scale bars: A, C, E, G = 1 mm; B, D, F, H = 100 µm. Abbreviations: H&F, hematoxylin and eosin; LFB, Luxol fast blue.

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  Figure 3.. Imaging features of antemortem case 2. (A-H) Serial pairs of sagittal (A-D) and axial diffusion-weighted images (DWI) MRI (E-H) at 4 time points in the patient’s hospital course. Noncontrast MRI at admission (A) shows patchy involvement of the whole corpus callosum without significant callosal thickening and with very limited patchy high signal intensity on DWI (E). Gadolinium-enhanced sagittal MRI (B) and axial DWI (F) 4½ weeks after admission show prominent punctate and slightly patchy enhancement within crossing callosal fibers, and much greater, nearly symmetrical increased SI on DWI image (F), with a thickened-appearing callosum. Gadolinium-enhanced sagittal MRI (C) and axial DWI (G) 8½ weeks after admission show reduced thickness of the corpus callosum, similar punctate and patchy callosal enhancement, and substantial interval increase in ventricular size. Gadolinium-enhanced sagittal MRI (D) and axial DWI (H) 20 weeks following admission show multifocal cavitation of the center of the corpus callosum with little to no central contrast enhancement. The axial DWI image shows near disappearance of the diffusion signal, reduced to scattered small patches of faint signal…

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  Figure 4.. Extracallosal radiologic features of antemortem case 2, 4½ weeks after admission. (A-D) Contrast-enhanced axial T2 FLAIR MR demonstrate increased signal intensity in the centrum semiovale (A), basis pontis and middle cerebellar peduncles (B), and in the ventral striatum and uncus (C), confirmed with diffusion-weighted images (DWI) imaging (D).

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  Figure 5.. Biopsy features of antemortem case 2. (A) H&E staining demonstrates scattered foamy macrophages in a background of white matter with reactive gliosis (inset=foamy macrophages identified on intraoperative smear preparation). (B) LFB/H&E staining demonstrates loss of myelin in a fragment of white matter and macrophages with phagocytosed myelin. (C) CD68 immunohistochemistry demonstrates abundant macrophages. (D) Neurofilament immunohistochemistry demonstrates relative preservation of axons between macrophages with rare axonal spheroids. Scale bars: 100 µm. Abbreviations: H&F, hematoxylin and eosin; LFB, Luxol fast blue.

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  Figure 6.. Representative autopsy features of a demyelinating lesion in the corpus callosum of a 73-year-old man with severe malnutrition, type 2 diabetes mellitus, and end-stage renal disease. (A, B) H&E stain (A) and gross examination (B) demonstrate pallor and cystic changes in the corpus callosum. (C, D) LFB/H&E stains demonstrate loss of myelin with abundant macrophages with phagocytosed myelin. (E, F) CD68 immunohistochemistry demonstrates abundant macrophages. (G, H) Neurofilament immunohistochemistry demonstrates a degree of retained axons between macrophages. Scale bars: A, B, C, E, G = 1 mm; D, F, H = 100 µm. Abbreviations: H&F, hematoxylin and eosin; LFB, Luxol fast blue.