PMC7434539_01
Eyelid swelling (A, arrow), blepharoptosis, and restricted ocular movement at lateral and inferior gaze in the left eye (B, arrows) in the present case.
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A neurological examination revealed lateral and inferior gaze palsy, eyelid swelling, blepharoptosis, and proptosis in the left eye (Fig. 1).
Cerebral short tau inversion recovery magnetic resonance imaging of the present case showed a high-intensity area in the left orbital periosteum (A and B, arrows). This lesion was enhanced after gadolinium injection (C and D, arrows).
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Cerebral short tau inversion recovery magnetic resonance imaging (MRI) revealed a high-intensity area in the left orbital periosteum (Fig. 2A, B). This lesion was enhanced after gadolinium injection (Fig. 2C, D). 18F-fluorodeoxyglucose positron emission tomography revealed no increased uptake in the left orbital or other body areas.
Microscopic findings of the biopsy specimen revealed nonspecific lymphocytic infiltration with fibrosis (Hematoxylin and Eosin staining) (A). The proportion of IgG4-positive cells among background cells was estimated to be 1.0%, and the IgG4-positive plasma cell count was 2 cells per high-power field (B). The proportion of IgG-positive cells among background cells was estimated to be 40.0% (C). The proportion of IgG4-positive cells among IgG-positive cells was estimated to be 2.5% (B and C). A lymph node tissue specimen from a control subject was used as a positive control (D). Graphs of the proportions of IgG4-positive or IgG-positive cells among background cells, and IgG4-positive cells among IgG-positive cells are shown (E).
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A needle biopsy was performed, and Hematoxylin and Eosin staining revealed nonspecific lymphocytic infiltration with fibrosis (Fig. 3A). The proportion of IgG4-positive cells among background cells was estimated to be 1.0%, while 2 IgG4-positive plasma cells were observed per high-power field (Fig. 3B). The proportion of IgG-positive cells among background cells was estimated to be 40.0% (Fig. 3C). The proportion of IgG4-positive cells among IgG-positive cells was estimated to be 2.5% (Fig. 3B, C). A lymph node tissue specimen from a control subject was used as a positive control (Fig. 3D). IgG-positive cells to the background cells, and of IgG4-positive cells to IgG-positive cells are shown (Fig. 3E). IgG4-related disease was excluded, and the patient was finally diagnosed with IOI. Intravenous methylprednisolone pulse therapy (1,000 mg, for 3 consecutive days) was administered followed by oral prednisolone with gradual tapering.
The clinical course of the present case is shown. Intravenous methylprednisolone pulse therapy (1,000 mg, for 3 consecutive days) was administered followed by oral prednisolone with gradual tapering. The restriction of ocular movement (A, arrow) totally recovered within 3 weeks (B, arrow) with an improvement of the erythrocyte sedimentation rate. The gadolinium-enhanced lesion in the left orbit (C, arrow) disappeared on magnetic resonance imaging at 3 weeks after treatment (D, arrow). mPSL: methylprednisolone, PSL: prednisolone, MRI: magnetic resonance imaging
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His ocular symptoms showed a complete improvement within 3 weeks, and the gadolinium-enhanced lesion in the left orbit disappeared on MRI at 3 weeks after the initiation of prednisolone treatment (Fig. 4).