PMC9744753_01
Electroencephalogram. The EEG showed (A) spike wave (thick solid rectangle) occurred in sphenoid bone and (B) slow waves (thick solid rectangle) interspersed with low amplitude of brain electrical activity (fine dotted rectangle) at regular intervals of 6-10 s.
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F1
Spike waves occurred in the sphenoid bone (Figure 1).
Blood and cerebrospinal fluid TBA screening. The TBA method was applied to incubate the patient's cerebrospinal fluid specimen with sections of cerebellar, hippocampal, cortical, and brainstem tissues from rats. Compared with the negative control (B), the patient's cerebrospinal fluid (A) was present antibodies that might have the immune reactions with the rat brainstem tissues. The image shows specific fluorescent signals, indicating that there are positive cell membrane antibodies in brainstem neurons, whereas the patient's cerebrospinal fluid sample showed no fluorescence with rat cerebellar, hippocampal, and cortical tissue sections (data not shown).
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F2
Blood and cerebrospinal fluid tissue-based assay (TBA) screening showed weakly positive antibodies against cell membranes in the brainstem (Figure 2).
Blood and cerebrospinal fluid CBA screening. The CBA method was applied to incubate the patient's cerebrospinal fluid and serum (both diluted 1:100) with human embryonic kidney cell 293T (HEK293T) loaded with GlyR plasmids labeled with green fluorescent protein (GFP). The green fluorescence represents HEK293T cells containing the target antigen, the red fluorescence represents the fluorescent antibody in the specimen, and the positive means that the red fluorescence coincides with a specific part of the green fluorescent cell. Compared with the negative control (C), there are autoantibodies that bind to GlyR expressed on the surface of HEK293T cells in the patient's cerebrospinal fluid (A) and serum (B).
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F3
SOX1, titin, Zic4, GAD65, and Tr), and demyelinating antibodies (AQP4, MOG, MAP, and GFAP). Serum and cerebrospinal fluid cell-based assay (CBA) screening for autoimmune encephalitis antibodies showed that IgG antibodies against glycine receptor (GlyR) was positive at 1:100 in both serum and cerebrospinal fluid (Figure 3), while the remaining proteins or receptor antibodies (NMDAR, LGI1, CASPR2, GABABR, AMPAR1, AMPAR2, IgLON5, DPPX, GAD65, and mGluR5, D2R) were all negative.
A schematic showing the timeline of the patient's conditions.
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F4
Putting the patient's clinical symptoms and auxiliary test results together (Figure 4), a diagnosis of anti-GlyR antibody-positive PERM induced by varicella-zoster virus was made.