PMC5491822_01
Serial MRI findings on FLAIR images. A: On admission, a large left frontal white matter lesion was seen. B: On brain biopsy, the left white matter lesion was enlarged, involving the parietal lobe. C: At 18 months after diagnosis, the size of the white matter lesion was found to have markedly decreased in association with slight atrophy of the left frontal lobe.
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On brain MRI, a large white matter lesion was seen in the left frontal lobe, with small dot-like lesions in both occipital lobes (Fig. 1A). DNA test for JC virus in CSF, as described previously, did not support this diagnosis. Despite reducing the daily dose of prednisolone from 27.5 to 22.5 mg/day, her neurological condition deteriorated rapidly with the expansion of the white matter lesions (Fig. 1B), leading to a bed-ridden state. She was discharged from our hospital, and at 18 months after the diagnosis MRI showed a marked decrease in the size of the left frontal lesion (Fig. 1C).
Brain biopsy findings. A: Hematoxylin and Eosin staining showing the presence of abnormal oligodendrocytes with enlarged nuclei (indicated by arrows). Original magnification x400. The insert shows astrocytes with nuclear atypia (indicated by arrows). Original magnification x300. B: Immunohistochemistry with an anti-VP1 antibody showing a positive reaction for nuclei in some glial cells. Original magnification x400. The insert shows a positive reaction for agnoprotein in the cytoplasm of the affected glial cells. Original magnification x300.
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The biopsied small piece of the left frontal lobe showed an abnormal histopathology, including the presence of oligodendrocytes with enlarged nuclei and atypical astrocytes (Fig. 2A), and immunohistochemistry revealed positive reactivity for JC virus-related antigens on these abnormal glial cells (Fig. 2B).