PMC7530192_01
Imaging studies and clinical courses. (A) FLAIR MRI scan at initial presentation. (B) Contrast enhanced T1 weighted MRI. (C) FLAIR MRI 2 weeks after the start of treatment with regular IVIG administration. (D) Diffusion weighted MRI at the onset of left facial paralysis and left hemiplegia. (E) MR angiography showing a severe stenosis in the right MCA. (F) CT scan at 11 months after the start of IVIG. (G) FLAIR MRI immediately after the onset of the first generalized convulsion, revealing high signal intensity and swelling in the left occipital legion. (H) FLAIR MRI 6 weeks after convulsion. (I) Clinical course of the patient. The closed circles and open squares depict the number of mononuclear cells and the level of protein concentration in the CSF, respectively.
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F1
The FLAIR images of the MRI brain scan demonstrated a large area with increased signal intensity involving the basal ganglia, hypothalamus, midbrain, and pons, consistent with inflammatory changes (Figure 1A). Contrast-enhanced T1-weighted MRI showed multiple nodular lesions with ring enhancement located in the area (Figure 1B). At this time, the maternal grandmother of the index patient stated that two of her older sons (the patient's uncles) had been diagnosed with hypogammaglobulinemia and died during childhood or adolescence (Supplementary Figure 1). The MRI images rapidly ameliorated (Figure 1C), while repeated lumbar puncture continued to show pleocytosis (Figure 1I). At 19 months of age, after an uneventful 4 months with a total of 7 doses of IVIG, he presented with mild fever, left facial paralysis, and left hemiplegia (Figure 1I). MRI scan revealed a large infarct in the territory of medial lenticulostriate branches of the right middle cerebral artery (MCA) (Figure 1D). MR angiography showed a severe stenosis in the right MCA and in the right anterior cerebral artery (Figure 1E). CT revealed calcification of the nodules (Figure 1F). The FLAIR MRI images of the brain revealed a new lesion of high signal intensity and swelling in the left occipital legion (Figures 1G,H), suggesting the ischemic change in the brain might have been caused by vasculitis due to VZV infection.
Immunophenotyping of lymphocytes in the CSF and peripheral blood and biopsy specimen of the patient collected at the installation of the Ommaya reservoir. (A,B) Dot plot analysis of the CSF and peripheral lymphocytes are shown. The dot-plot in the top of each row was used to gate a population of interest. (C-G) The biopsied leptomeninges show fibrous thickening and infiltration of mononuclear cells. (C) Hematoxylin eosin staining. (D-G) The cells infiltrating in the leptomeninges (D,E) and perivascular areas (F,G arrows) are positive for several T-cell markers, including CD3 (D), CD8 (E,F), and CD4 (G). Bar: (C) 100 mum, (D-G) 25 mum.
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As pleocytosis continued to be present, we then examined mononuclear cells in the CSF collected by lumbar puncture (Figures 2A,B). The pathological findings of the specimen included fibrous thickening with infiltration of CD4+ and CD8+ T cells (Figures 2C-G).