PMC6407169_01
A, Magnetic resonance imaging (MRI) of the brain suggested altered signal intensities, hyperintense on T2 in subcortical and deep white matter over parieto-occipital and bilateral cerebellar region. B, The MRI of the brain suggested white matter signal changes in bilateral cerebellar hemispheres, cerebellar peduncles, pons, and midbrain. Similar changes in left thalamus, adjacent posterior limb of internal capsule, parahippocampal gyrus, and parietal regions on both sides. Small changes in the left frontoparietal subcortical white matter. C, Significant decrease in hyperintensities as compared to (C), but not totally cleared. D, Reappearance of lesions and patchy involvement of pons, midbrain, cerebellar peduncles, and cerebellum.
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Magnetic resonance imaging of the brain (Figure 1A) suggested altered signal intensities, hyperintense on T2 in subcortical and deep white matter over parieto-occipital and bilateral cerebellar regions. Magnetic resonance imaging of the brain (Figure 1B) suggested white matter signal changes in bilateral cerebellar hemispheres, cerebellar peduncles, pons, and midbrain. Follow-up MRI brain done 3 months after the episode showed significant improvement but not total clearing of lesions (Figure 1C). Repeat MRI brain (Figure 1D) showed reappearance of lesions and patchy involvement of pons, midbrain, cerebellar peduncles, and cerebellum.
Histopathology representative photomicrographs show inflammatory cell infiltrate involving the leptomeningeal surface (A: x40, C: x100; HE) with secondary infiltration into the parenchyma along the thin-walled capillary-sized blood vessels (B: x40; D: x100; HE). HE, Hematoxylin and Eosin Stain.
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Here, a review of biopsy was done (Figure 2) and malignancy was ruled out and then referred to us. Histologically, fragments of cerebellar folia showed dense band-like lymphoplasmacytic cell-rich leptomeningeal infiltrate (Figure 2A and C), extending into the subjacent parenchyma along the blood vessels with foci of perivascular aggregation (Figure 4B and D). The gradient of the inflammatory infiltrate was decreasing from the pial surface to the white matter parenchyma (Figure 2A and B).
A, It suggested punctate T2 hyperintense lesions in pons, bilateral cerebellar hemispheres, brachium pontis, ventral medulla, midbrain extending cranially into thalamus, and posterior limbs of internal capsule. Extensive confluent lesions were also present posteriorly in parieto-occipital region and focal anteriorly in frontal lobes with few showing nodular enhancement with involvement of corpus callosum. B, Considerable improvement as compared to (A).
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Repeat MRI brain with contrast (Figure 3A) suggested punctate T2 hyperintense lesions in pons, bilateral cerebellar hemispheres, brachium pontis, ventral medulla, midbrain extending cranially into thalamus, and posterior limbs of internal capsule. His repeat MRI of the brain on tapering steroids and methotrexate shows considerable improvement (Figure 3B).
Immunohistochemical evaluation representative photomicrographs of immunohistochemical evaluation, which shows CD3 (A: x40; B: x100) and CD7-positive (C: x100) T-lymphoid cells admixed with CD163-positive (D: x100) histiocytes in the leptomeningeal space with involvement of the subjacent parenchyma. The T-lymphoid cells show marked preponderance for CD4 (F: x100) over CD8 (G: x100).
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Histologically, fragments of cerebellar folia showed dense band-like lymphoplasmacytic cell-rich leptomeningeal infiltrate (Figure 2A and C), extending into the subjacent parenchyma along the blood vessels with foci of perivascular aggregation (Figure 4B and D). T-lymphoid cells were the predominant cellular component with conspicuous admixture by CD163-positive histiocytes (Figure 4D). The T-lymphoid cells showed marked CD4 preponderance over CD8 (Figure 4E and F), unlike the post/viral-related lymphocytic infiltrate, which is CD8 preponderant.
Electropherograms representing T-cell receptor (TCR)-gamma (A and B) and TCR-beta (C, E, G and D, F, H representing 3 tubes) in duplicate concentrations (the electrograms on the left, ie, A, C, E, G representing 50 mug of sample DNA; while those on right, ie, B, D, F, H represent 100 mug of sample DNA), do not reveal any presence of clonal peak, suggesting absence of clonal T-lymphoid cells.
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Molecular evaluation for T-cell receptor gene rearrangement, evaluated by capillary electrophoresis (Figure 5), did not reveal presence of any clonal T-lymphoid cells, excluding the possibility of T-cell lymphoma.