case_images: PMC11169638_01
case_images: Case 1 cerebral CT demonstrated multiple bilateral frontal and parietal lobe lesions with low density, without evidence of intracranial bleeding.
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case_images: F1
case_images: Cerebral computed tomography (CT) scan showed multiple bilateral frontal and parietal lobe lesions with low density, without evidence of intracranial bleeding (Figure 1).
case_images: (A) Cerebral MRI demonstrated extensive bilateral T2 FLAIR/DWI hyperintense and T1 hypointense lesions, without ADC value reduction. (B) Significant reduction in abnormal signals compared with A. (C) Complete resolution of abnormal signals. FLAIR, fluid attenuated inversion recovery; DWI, diffusion weighted imaging; ADC, apparent diffusion coefficient.
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case_images: F2
case_images: Cerebral magnetic resonance imaging (MRI) revealed extensive T2 hyperintense and T1 hypointense bilateral lesions (Figure 2A). Cerebral MRI on hospitalization day 50 revealed significantly reduced abnormal signals (Figure 2B) and repeated EEG also showed brainwave activity improvement. Her NXP2 antibody titer IgG had decreased to 20 RU/ml and her cerebral MRI remained stable throughout follow-up, similar to the results shown in Figure 2C.
case_images: Case 1 clinical course.
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case_images: F3
case_images: As shown in Figure 3, the patient was started on intravenous methylprednisolone (IVMP, 1.6 mg/kg/day).
case_images: PMC11169638_02
case_images: (A) Periorbital edema before treatment and (B) after one year of treatment.
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case_images: F4
case_images: PICU. Physical examination revealed significant skin and periorbital edema (Figure 4A). IVIG at a dose of 2 g/kg. In March 2024 (20 months into the disease course), the steroid dosage had been decreased to 2.5 mg/day and assessments showed a CMAS score of 52, complete resolution of the rash (Figure 4B), no neurological symptoms, and normal muscle enzyme levels.
case_images: (A) Contrast-enhanced cerebral MRI showing abnormal signals in the left parieto-occipital lobe and thalamus on hospitalization day 2. (B) Cerebral MRI revealed less pronounced abnormal signals in the left parieto-occipital lobe and thalamus on hospitalization day 28. (C) No significant abnormal signals in the brain parenchyma were observed on hospitalization day 83.
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case_images: F5
case_images: Cerebral CT showed blurred gray-white matter differentiation, and contrast-enhanced cerebral MRI (Figure 5A) revealed abnormal signals in the left parieto-occipital lobe and thalamus. Cerebral MRA and MRV were negative. Cerebral MRI on hospitalization day 28 (Figure 5B) indicated less pronounced abnormalities, and cerebral MRI on hospitalization day 83 (Figure 5C) revealed no significant abnormalities.
case_images: Case 2 clinical course.
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case_images: PMC11169638_02_fped-12-1409950-g006.jpg
case_images: F6
case_images: As shown in Figure 6, four PEs were performed, including two sessions of double filtration plasmapheresis.