Yonsei Med J.  2006 Dec;47(6):873-876. 10.3349/ymj.2006.47.6.873.

MR Findings of Fulminent Leukoencephalopathy in EBV-Associated Hemophagocytic Syndrome

• Kim J
• Lee SK
• Joo SH
• Kim DI
• Kim HD

• KMID: 1777179
• DOI: http://doi.org/10.3349/ymj.2006.47.6.873

Abstract

Various manifestations of brain involvement for patients with virus-associated hemophagocytic syndrome have been reported. Here, we report on the sequential magnetic resonance (MR) findings of acute demyelination of the entire brain with subsequent brain atrophy in a follow-up study of a 25-month- old boy who was admitted with fever and then diagnosed with infectious mononucleosis and EBV-associated hemophagocytic syndrome. We also review other conditions that should be included in the differential diagnosis of this disease.

Keyword

Epstein-Barr virus infections; histiocytosis; non- Langerhans-cell; demyelinating disease; magnetic resonance imaging

MeSH Terms

Male
*Magnetic Resonance Imaging
Lymphohistiocytosis, Hemophagocytic/etiology/*pathology/virology
Humans
Epstein-Barr Virus Infections/complications/*pathology
Diagnosis, Differential
Demyelinating Diseases/complications/*pathology/virology
Child, Preschool
Brain Diseases/complications/*pathology/virology

Figure

• 

  Fig. 1 Marrow biopsy section of the patient with EBV-associated hemophagocytic syndrome. (A) There are several macrophages with phagocytosis of erythrocytes and lymphocytes (arrows) on hematoxylin and eosin stains (× 400). (B) Immunohistochemical staining with panmacrophage marker CD68 shows abundant macrophages (× 400).

• 

  Fig. 2 (A-C) Axial FLAIR images of the brain show high signal intensities of the entire white matter in both hemispheres, including the corpus callosum, internal capsule and middle cerebellar peduncle, suggesting a fulminent demyelinating process. The preservation of white matter volume without a mass effect and a relative sparing of the subcortical U-fibers are also noted.

• 

  Fig. 3 Follow-up MR findings after two months with chemotherapy. (A-C) Axial FLAIR images of the brain show partial resolution of high signal intensities of the entire white matter and the progression of atrophy at the previously-involved site.

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