Table 1.
Clinical features, auxiliary examinations, treatment strategies, prognosis in GFAP astrocytopathy patients with seizures.
| Patient no. | Sex | Age at onset | Clinical symptoms besides epilepsy | CSF white blood cell count,/L; protein, g/L | Serum antibody titer | CSF antibody titer | MRI findings | Immunomodulatory therapy | ICU admission | mRS at admission/discharge | Response to therapy |
|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | Female | 7y5 m | Fever, headache, disorders of consciousness, bowel or micturition disorder, dyskinesia | wbc:240; p:1.56 | GFAP-IgG (0 → 1:100) | GFAP-IgG (0 → 1:32) | Brain: T2-hyperintense lesions in frontal, temporal, parietal, occipital, basal ganglia, thalamus, periventricular, hippocampus, corpus callosum, brain stem Spine: lesions in T1-L1 (Lesion disappeared after 6 months) |
IVIG, IVMP | Yes | 5/3 | Symptoms improved |
| 2 | Male | 7y1 m | Only epilepsy | wbc:6; p:0.19 | GFAP-IgG (1:50) | GFAP-IgG (1:1) | Brain: T2-hyperintense lesions in right cerebellum Spine: normal |
IVMP | No | 1/0 | Symptoms disappeared |
| 3 | Male | 11y | Memory disorder | wbc:28; p:0.21 | NMDAR-IgG (1:10) MOG-IgG (1:100) |
GFAP-IgG(1:32) NMDAR-IgG(1:1) MOG-IgG(1:100) |
Brain: T2-hyperintense lesions in frontal, temporal, parietal, occipital, optic nerve Spine: normal (Lesion disappeared after 2 months) |
IVIG, IVMP | No | 3/0 | Symptoms disappeared |
| 4 | Male | 1y4 m | Fever, disorders of consciousness, dyskinesia | wbc:50; p:0.6 | Antibody (−) | GFAP-IgG (1:32) | Brain: T2-hyperintense lesions in frontal, temporal, parietal, occipital, cerebellum, corpus callosum, brain stem Spine: NA |
IVMP | Yes | 5/5 | Dead |
| 5 | Female | 5y8m | Fever, headache, psychiatric symptoms | wbc:0; p:0.191 | GFAP-IgG (1:320) | GFAP-IgG (1:32) | Brain: T2-hyperintense lesions in right frontal, left periventricular Spine: normal |
IVIG, IVMP | Yes | 4/1 | Symptoms improved |
| 6 | Male | 3y9m | Fever, disorders of consciousness, peripheral facial palsy, impaired hearing, dyskinesia | wbc:400; p:0.63 | GFAP-IgG (1:32) | GFAP-IgG (1:100) | Brain: T2-hyperintense lesions in frontal, temporal, parietal, occipital, basal ganglia, thalamus, periventricular, corpus callosum Spine: normal |
IVMP | Yes | 5/3 | Symptoms improved |
| 7 | Female | 1y10m | Fever, disorders of consciousness | wbc:233; p:0.46 | GFAP-IgG (1:32) | GFAP-IgG (1:32) | Brain: T2-hyperintense lesions in thalamus, brain stem, cerebellum, left frontal Spine: lesions in C3–C6 (lesion disappeared after 2 months) |
IVIG, IVMP | No | 3/0 | Symptoms disappeared |
| 8 | Male | 6y6m | Fever | wbc:26; p:0.189 | GFAP-IgG (1:32) | GFAP-IgG (1:32) | Brain: normal Spine: normal |
IVMP | No | 3/0 | Symptoms disappeared |
| 9 | Male | 10y9m | Fever, headache, disorders of consciousness, bowel or micturition disorder, dyskinesia, automatic nervous disorder, peripheral motor or sensory nerve damage | wbc:382; p:0.84 | Antibody (−) | GFAP-IgG (1:32) | Brain: T2-hyperintense lesions in basal ganglia, caudate nucleus, thalamus, brain stem, cerebellum Spine: extensive lesions |
IVIG, IVMP, PLEX | Yes | 5/4 | Symptoms improved |
| 10 | Female | 9y3m | disorders of consciousness, psychiatric symptoms, sleep disorder, involuntary movements | wbc:120; p:0.21 | GFAP-IgG (1:32) NMDAR-IgG (1:10) |
GFAP-IgG (1:32) NMDAR-IgG (1:30) |
Brain: normal Spine: NA |
IVIG, IVMP | No | 5/4 | Symptoms improved |
| 11 | Male | 10y6m | Fever, headache, disorders of consciousness, psychiatric symptoms, involuntary movements | wbc:8; p:0.19 | GFAP-IgG (1:100) | GFAP-IgG (1:32) | Brain: T2-hyperintense lesions in parietal, occipital, corpus callosum Spine: NA (lesion disappeared after 2 years and 3 months) |
IVIG, IVMP | Yes | 5/2 | Symptoms disappeared |
| 12 | Male | 1y8m | Fever, disorders of consciousness, dyskinesia, peripheral motor or sensory nerve damage | wbc:80; p:0.53 | GFAP-IgG (1:10) | GFAP-IgG(1:1) | Brain: T2-hyperintense lesions in frontal, temporal, parietal, occipital, thalamus Spine: normal |
IVIG, IVMP | Yes | 5/4 | Symptoms improved |
| 13 | Male | 7y6m | Fever, disorders of consciousness, dyskinesia, psychiatric symptoms, involuntary movements | wbc:2; p:0.05 | GFAP-IgG (1:32) | GFAP-IgG(1:32) | Brain: T2-hyperintense lesions in frontal, temporal, parietal, occipital, periventricular Spine: normal |
IVMP | Yes | 5/5 | Symptoms improved |
| 14 | Female | 5y9m | Fever, disorders of consciousness, psychiatric symptoms, involuntary movements, bulbar palsy | wbc:14; p:0.1 | GFAP-IgG (1:320) | GFAP-IgG (1:1) | Brain: T2-hyperintense lesions in frontal, parietal, occipital Spine: NA |
IVIG, IVMP | Yes | 5/3 | Symptoms improved |