case_images: PMC10616785_01
case_images: Timeline depicting visual acuity change, immunotherapy, and serum AQP4 IgG. VA, visual acuity; AQP4, aquaporin-4; IA, immunoadsorption; IVIG, intravenous immunoglobulin; IVMP, intravenous methylprednisolone; MMF, mycophenolate mofetil; RTX, rituximab.
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case_images: PMC10616785_01_fimmu-14-1257955-g001.jpg
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case_images: Snellen visual acuity (VA) test showed that the right eye was blind without any light perception, and VA on the left was 1.5 ( Figure 1 ). After treatment of IA, VA on the left was improved to 0.6, and on the right it was improved to "hand movement" from 70 cm ( Figure 1 ). One month after IA in July 2020, the patient's VA on the right was reduced to only light perception ( Figure 1 ). Two months after RTX treatment in October 2020, the patient relapsed with right eye blindness without light perception under prednisone treatment, tailed to 12.5 mg/day ( Figure 1 ). Six months after MMF treatment in April 2021 (around 1 month after prednisone withdrawal), the patient relapsed with VA on the right reduced to light perception ( Figure 1 ) and MRI showed new T2-hyperintense lesions in the bilateral optic nerve with gadolinium enhancement in T1-weighted gadolinium-enhanced image ( Figures 3A, B ). Eight months later, in December 2021, the patient relapsed with VA on the left reduced to "counting fingers" from 20 cm ( Figure 1 ). One month later, in January 2022, the patient relapsed with the VA on the right reduced to blindness without light perception, and on the left reduced to 0.05 under the blood CD19+ B-cell depletion ( Figure 1 ). VA on the right was light perception, and on the left was "counting fingers" from 60 cm ( Figure 1 ).
case_images: MRI at onset and follow-up. Axial cranial T2-weighted images showing T2-hyperintense lesions in pons (A), midbrain (B), posterior limb of internal capsule (C), and subcortical white matter of the frontal and parietal lobe (D). Axial orbital T2-weighted image showing longitudinally extensive T2-hyperintense lesions in the bilateral optic nerve (E) with gadolinium enhancement in the T1-weighted gadolinium-enhanced image (F). Axial cranial T2-weighted images showing new T2-hyperintense lesions in subcortical and deep white matter of the frontal, parietal, and occipital lobes after treatment with IVMP and IVIG at onset attack (G). Axial orbital T2-weighted image showing that lesions in the left optic nerve disappeared, whereas in the right optic nerve they became smaller without gadolinium enhancement in T1-weighted gadolinium-enhanced image at follow-up after immunosorbent therapy (H, I). Sagittal spinal T2 weighted image showing T2-hyperintense lesions at onset (J) and resolution of lesions at follow-up after immunosorbent therapy (K).
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case_images: T2-hyperintense lesions in the pons, midbrain, posterior limb of the internal capsule, and subcortical white matter of the frontal and parietal lobe without gadolinium enhancement ( Figures 2A-D ). T2-hyperintense lesion with gadolinium enhancement in T1-weighted gadolinium-enhanced imaging in the bilateral optic nerves ( Figures 2E, F ). The spinal Figure 2J ). The patient was treated with two courses of IVMP (15 mg/kg/day for 3 days tailed to oral prednisone 40 mg/day) in combination with IVIG (total 2 g/kg split over 3 days). Moreover, there was no improvement in visual impairment. Furthermore, VA on the left was reduced to 0.25, together with new T2-hyperintense lesions found in the subcortical and deep white matter of the frontal, parietal, and occipital lobes and bilateral thalamus ( Figure 2G ). Then, protein A IA was started. MRI showed that T2-hyperintense lesions in the spinal cord and left optic nerve disappeared, in the right optic nerve it became smaller, and in the brain it was similar to previous results ( Figures 2H, I, K ).
case_images: MRI at relapse and follow-up. Axial orbital T2-weighted image showing lesions in bilateral optic nerve with gadolinium enhancement in the T1-weighted gadolinium-enhanced image at relapse in April 2021 (A, B). Axial orbital T2-weighted image showing lesions in the left optic nerve with gadolinium enhancement in the T1-weighted gadolinium-enhanced image at relapse in December 2021 (C, D). Axial orbital T2-weighted image showing that lesions disappeared in the bilateral optic nerve at the last follow-up in April 2023 (E, F).
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case_images: PMC10616785_01_fimmu-14-1257955-g003.jpg
case_images: f3
case_images: Six months after MMF treatment in April 2021 (around 1 month after prednisone withdrawal), the patient relapsed with VA on the right reduced to light perception ( Figure 1 ) and MRI showed new T2-hyperintense lesions in the bilateral optic nerve with gadolinium enhancement in T1-weighted gadolinium-enhanced image ( Figures 3A, B ). T2-hyperintense lesions in the left optic nerve with gadolinium enhancement in the T1-weighted gadolinium-enhanced image ( Figures 3C, D ). The patient received acyclovir treatment, IVMP treatment (20 mg/kg/day for 3 days tailed to oral prednisone 40 mg/day), and RTX treatment. No new lesions were found in the brain and orbital MRI ( Figures 3E, F ).