PMC9554464_01
Magnetic resonance imaging of the brain before (A) and after (B) treatment. Yellow arrows indicate white matter lesions.
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F1
Brain MRI revealed multiple coin-like white matter lesions and one mesencephalon lesion (Figure 1A). A repeated brain MRI revealed shrinkage of the intracranial lesions, and the lab tests showed a rapid drop in serum levels of IgG and IgG4 (Figures 1B, 3).
Sagittal T2-weighted magnetic resonance imaging of the upper (A) and lower (B) spinal cord. Yellow arrows indicate white matter lesions.
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F2
Sagittal T2-weighted imaging of the brainstem and the spinal cord showed an enhanced lesion in the cerebral peduncle (Figure 2A) and in the spinal cord at the level of thoracic 12 (Figure 2B).
The disease course, treatments, and the changes of serum IgG and IgG4 of the patient.
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F3
Serum IgG was 70.7 g/L (normal range 8.0-15.5 g/L), IgG4 was 28.5 g/L, and IgE was 4,810 mg/L (normal range 5-150 mg/L), while IgA and IgM were within the normal range (Figure 3). A repeated brain MRI revealed shrinkage of the intracranial lesions, and the lab tests showed a rapid drop in serum levels of IgG and IgG4 (Figures 1B, 3).
Histological examination of the biopsy of inguinal lymph node (A) H&E staining; (B) immunohistochemistry for CD138; (C) immunohistochemistry for IgG4; 200x).
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F4
The histopathological evaluation of the biopsy specimen of the inguinal lymph node revealed mixed inflammation containing predominantly plasma cells (Figures 4A-C). Immunostaining showed an increased number of IgG4+ plasma cells (>200/HPF) and an elevated ratio of IgG4+ cells to CD138+ plasma cells (~80%) (Figures 4B,C).