PMC7705065_01
Series of brain MRI with contrast. (a,b) In March 2012, curvilinear and punctate enhancements of pontine, cerebellum, and basal ganglion were demonstrated. (c,d) In June 2012, enhancement of pontine, cerebellum, and basal ganglion improved after treatment with steroids. (e,f) In June 2015, novel dot-like and liner enhancement in pontine, semiovale center, and juxtacortical area were shown. (g,h) In September 2015, enhancement in pontine, semiovale center, and juxtacortical area improved again after treatment with steroids. (i-l) In September 2019, novel dot-like and liner enhancement in both hemispheres of the cerebellum, semiovale center, basal ganglion, and thalamus were demonstrated.
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Her initial brain magnetic resonance imaging (MRI) after 1 month revealed multiple areas of curvilinear and punctate enhancement of pontine, cerebellum, and basal ganglion (Figures 1a,b). She had rapid clinical and radiological improvements (Figures 1c,d). During the regular follow-up at the outpatient clinic in the past 7 years, the patient's symptom and/or brain MRI enhancement deteriorated every time the oral prednisone tapered near the dose of 20 mg per day, although immunosuppressive agent such as azathioprine was tried (Table 1, Figures 1e,f). However, she obtained relief soon after high-dose IVMP or oral prednisone was added on (Figures 1g,h). September 2019, and it showed novel dot-like and liner enhancement in both hemispheres of the cerebellum, semiovale center, basal ganglion, and thalamus (Figures 1i-l). She refused repeated treatment of steroids then.
GFAPalpha-IgG test results by GFAP-transfected HEK293 cell-based immunofluorescence assay. (a,c,e,g) HEK293 cells expressing green fluorescent protein (GFP)-tagged GFAP (green). (b,d,f,h) HEK293 cells immunostained with human IgG (red if positive): (b) Positive control with human anti-GFAPalpha IgG, (d) negative with healthy control, (f) positive result of serum (titer at 1:320), (h) positive result of cerebrospinal fluid (CSF) (titer at 1:32). Scale bar = 50 um.
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Further antibody analysis by antigen-transfected HEK293 cell-based assay (Supplementary Material 1) showed positive GFAPalpha antibodies with serum titer at 1:320 and CSF titer at 1:32 (Figure 2).