PMC8176955_01
Clinical signs, treatment course, peripheral blood CD20+ B cells, and cerebral spinal fluid analysis, including anti-NMDAR antibody titres and neopterin, over time. mPSL, methylprednisolone; PSL, prednisolone; IVIg, intravenous immunoglobulin; CSF, cerebral spinal fluid; NMDA, N-methyl-D-aspartate; GluRs, glutamate receptors; ELISA, enzyme-linked immunosorbent assays; GluN2B-NT2, N-terminal of GluN2B; OD, optical density; GluN2B-CT, C-terminal of GluN2B; GluN1-NT, N-terminal of GluN1; SD, standard deviation.
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PMC8176955_02_fneur-12-679164-g0001.jpg
F1
The neopterin level in the CSF, which acts as a non-specific biomarker of central nervous system inflammation, was markedly elevated (normal range for 1-12-month-old infants: 10.3-34.6 nmol/L; Figure 1). After the third mPSL pulse therapy and a single dose of intravenous Ig (1 g/kg) were administered without significant effects on the clinical course, the results of anti-NMDAR antibody titres using a cell-based assay and antibodies to NMDA-type glutamate receptors (GluRs) examined using conventional enzyme-linked immunosorbent assays in CSF at 4 months of age were positive (Figure 1). National Glycohaemoglobin Standardisation Program) at 7 months of age. Neopterin in the CSF was reduced, while antibodies to NMDA-type GluRs titres in the CSF further increased at 8 months of age (Figure 1).
Brain magnetic resonance imaging (MRI) over time. MRI at 3 months old showed white matter lesions with high intensity on apparent diffusion coefficient maps (A) and T2 images (D), and low intensity on diffusion-weighted (DW) images (B) and T1-weighted images (C) within the right frontal and parietal lobes and both occipital lobes, suggesting demyelination. MRI at 5 months old revealed a significant decrease in white matter volume with slight improvements in abnormal lesions within the right frontal, left parietal, and left occipital lobes. No remarkable changes were observed in lesions within the right occipital lobe with high intensity on apparent diffusion coefficient maps (E) and T2-weighted images (H), and low intensity on DW images (F) and T1-weighted images (G).
fneur-12-679164-g0002.jpg
PMC8176955_02_fneur-12-679164-g0002.jpg
F2
MRI at 5 months of age revealed a significant decrease in the volume of white matter (Figures 2E-H).
PMC8176955_02
Clinical signs, treatment course, peripheral blood CD20+ B cells, and cerebral spinal fluid analysis, including anti-NMDAR antibody titres and neopterin, over time. mPSL, methylprednisolone; PSL, prednisolone; IVIg, intravenous immunoglobulin; CSF, cerebral spinal fluid; NMDA, N-methyl-D-aspartate; GluRs, glutamate receptors; ELISA, enzyme-linked immunosorbent assays; GluN2B-NT2, N-terminal of GluN2B; OD, optical density; GluN2B-CT, C-terminal of GluN2B; GluN1-NT, N-terminal of GluN1; SD, standard deviation.
fneur-12-679164-g0001.jpg
PMC8176955_02_fneur-12-679164-g0001.jpg
F1
The neopterin level in the CSF, which acts as a non-specific biomarker of central nervous system inflammation, was markedly elevated (normal range for 1-12-month-old infants: 10.3-34.6 nmol/L; Figure 1). After the third mPSL pulse therapy and a single dose of intravenous Ig (1 g/kg) were administered without significant effects on the clinical course, the results of anti-NMDAR antibody titres using a cell-based assay and antibodies to NMDA-type glutamate receptors (GluRs) examined using conventional enzyme-linked immunosorbent assays in CSF at 4 months of age were positive (Figure 1). National Glycohaemoglobin Standardisation Program) at 7 months of age. Neopterin in the CSF was reduced, while antibodies to NMDA-type GluRs titres in the CSF further increased at 8 months of age (Figure 1).
Brain magnetic resonance imaging (MRI) over time. MRI at 3 months old showed white matter lesions with high intensity on apparent diffusion coefficient maps (A) and T2 images (D), and low intensity on diffusion-weighted (DW) images (B) and T1-weighted images (C) within the right frontal and parietal lobes and both occipital lobes, suggesting demyelination. MRI at 5 months old revealed a significant decrease in white matter volume with slight improvements in abnormal lesions within the right frontal, left parietal, and left occipital lobes. No remarkable changes were observed in lesions within the right occipital lobe with high intensity on apparent diffusion coefficient maps (E) and T2-weighted images (H), and low intensity on DW images (F) and T1-weighted images (G).
fneur-12-679164-g0002.jpg
PMC8176955_02_fneur-12-679164-g0002.jpg
F2
MRI at 5 months of age revealed a significant decrease in the volume of white matter (Figures 2E-H).
Sleep electroencephalography (EEG) over time. (A) Sleep EEG at 6 months old. Interictal EEG showing hypsarrhythmia with multifocal spike- and sharp-wave activity and slow-wave activity exhibiting occasional interhemispheric synchrony and symmetry with recurrent episodes of generalised voltage attenuation. (B) Sleep EEG 2 weeks after the first rituximab administration at 7 months of age. Interictal EEG showing complete resolution of hypsarrhythmia and periodic generalised polyspikes, though sharp waves can occasionally be independently observed in the right and left hemispheres. (C) Sleep EEG when the patient became lethargic at 8 months of age. Interictal EEG showing a burst of 1.5 Hz slow spike-and-wave activity. (D) Sleep EEG at 12 months of age. EEG showing bursts of generalised fast activity (10-25 Hz) without clinical seizures.
fneur-12-679164-g0003.jpg
PMC8176955_04_fneur-12-679164-g0003.jpg
F3
Although she has not had any clinical seizures since 7 months of age, she showed severely delayed neurodevelopmental milestones (3 months old at her chronological age of 14 months) along with abnormal EEG findings (Figure 3D).
PMC8176955_03
Sleep electroencephalography (EEG) over time. (A) Sleep EEG at 6 months old. Interictal EEG showing hypsarrhythmia with multifocal spike- and sharp-wave activity and slow-wave activity exhibiting occasional interhemispheric synchrony and symmetry with recurrent episodes of generalised voltage attenuation. (B) Sleep EEG 2 weeks after the first rituximab administration at 7 months of age. Interictal EEG showing complete resolution of hypsarrhythmia and periodic generalised polyspikes, though sharp waves can occasionally be independently observed in the right and left hemispheres. (C) Sleep EEG when the patient became lethargic at 8 months of age. Interictal EEG showing a burst of 1.5 Hz slow spike-and-wave activity. (D) Sleep EEG at 12 months of age. EEG showing bursts of generalised fast activity (10-25 Hz) without clinical seizures.
fneur-12-679164-g0003.jpg
PMC8176955_04_fneur-12-679164-g0003.jpg
F3
Although she has not had any clinical seizures since 7 months of age, she showed severely delayed neurodevelopmental milestones (3 months old at her chronological age of 14 months) along with abnormal EEG findings (Figure 3D).
PMC8176955_04
Sleep electroencephalography (EEG) over time. (A) Sleep EEG at 6 months old. Interictal EEG showing hypsarrhythmia with multifocal spike- and sharp-wave activity and slow-wave activity exhibiting occasional interhemispheric synchrony and symmetry with recurrent episodes of generalised voltage attenuation. (B) Sleep EEG 2 weeks after the first rituximab administration at 7 months of age. Interictal EEG showing complete resolution of hypsarrhythmia and periodic generalised polyspikes, though sharp waves can occasionally be independently observed in the right and left hemispheres. (C) Sleep EEG when the patient became lethargic at 8 months of age. Interictal EEG showing a burst of 1.5 Hz slow spike-and-wave activity. (D) Sleep EEG at 12 months of age. EEG showing bursts of generalised fast activity (10-25 Hz) without clinical seizures.
fneur-12-679164-g0003.jpg
PMC8176955_04_fneur-12-679164-g0003.jpg
F3
Although she has not had any clinical seizures since 7 months of age, she showed severely delayed neurodevelopmental milestones (3 months old at her chronological age of 14 months) along with abnormal EEG findings (Figure 3D).