Table 1.
Clinical features, auxiliary examinations, diagnosis and treatment strategies, prognosis in pediatric patients positive for GFAP-IgG.
| Patient no. Sex/age of onset | Summary clinical | MRI | EEG | Serum antibody | CSF abnormality | Therapy | Response To therapy | mRS at onset/last | Once lived in ICU |
|---|---|---|---|---|---|---|---|---|---|
| 1.M/11.75 | vertigo | T2-hyperintense lesions in white matter around posterior horn of bilateral lateral ventricles |
normal | GFAP-IgG(1:32) | Wbc:3 P:0.18 Antibody (-) |
Prednisone | no change of lesions | 1/0 | No |
| 2.F/1.67 | Fever, dyskinesia | Space occupying lesions in suprasellar region Combined supratentorial hydrocephalus | normal | GFAP-IgG(1:100) | WBC:1 P:0.26 Antibody:NA |
Surgery + biopsy | Brain glioma, death | 4/5 | No |
| 3.M/2.75 | epilepsy(Focal attack) | T2-hyperintense lesions in midbrain, cerebral foot, dorsal thalamus, basal ganglia and temporal lobe, | Slow background,left temporal sharp wave | GFAP-IgG(1:100) | WBC:8 P:0.07 Antibody (-) |
IVIG,IVMP,AEDS | Brain glioma, aggravated | 3/5 | No |
| 4.F/6.33 | vertigo | T2-hyperintense lesions in bilateral subcortical white matter of frontal, temporal,parietal lobes | NA | GFAP-IgG(1:100) | WBC:2 P:0.11 Antibody (-) |
IVIG,IVMP | no change of lesions | 2/0 | No |
| 5.M/12.41 | epilepsy | T2-hyperintense lesions in parasylvian cistern of right parietal lobe, left frontal lobe | right parietal, left frontal sharp wave |
GFAP-IgG(1:32) | WBC:3 P:0.412 Antibody (-) |
IVIG,IVMP | no change of lesions | 2/0 | No |
| 6.F/4 | psychosis | T2-hyperintense lesions and enhancement in frontal,parietal lobe, thalamus, midbrain | Slow background | GFAP-IgG(1:32) | WBC:8 P:0.25 Antibody (-) |
IVIG,IVMP | Symptoms disappeared, Lesion reduced |
1/0 | No |
| 7.M/11 | Limb numbness, Dyskinesia,ataxia |
SC:C2-4 | Normal | GFAP-IgG(1:32) | WBC:4 P:0.14 Antibody (-) |
IVIG,IVMP | Symptoms improved, Lesion reduced |
4/1 | No |
| 8.M/2.17 | epilepsy(Focal attack) | T2-hyperintense lesions in left temporal,occipital lobe | Slow background,left temporal,occipital sharp wave | GFAP-IgG(1:32) after 6 month:GFAP-IgG(1:100) |
WBC:55 P:0.13 Antibody (-) |
IVIG,IVMP,AEDS | Seizure frequency decreased, cognitive impaired. Lesion reduced |
2/0 | No |
| 9.M/2 | ataxia, Relapse 2 times |
T2-hyperintense lesions in white matter around left lateral ventricle | Slow background | GFAP-IgG(1:32) | WBC:32 P:0.19 Antibody :NA |
IVIG,IVMP | Retroperitonea-l ganglioma. Lesion reduced |
4/1 | No |
| 10.M/10.25 | Headache,right optic neuritis, Relapse 1 times |
T2-hyperintense lesions and enhancement of right optic nerve | normal | GFAP-IgG(1:32) | WBC:0 P:0.25 Antibody (-) |
IVMP | Symptoms improved, Lesion reduced |
3/0 | No |
| 11.M/6 | Fever, epilepsy, psychosis, hypersomnia |
T2-hyperintense lesions in bilateral thalamus,frontal, temporal, parietal lobes. | Slow background | GFAP-IgG(1:32) | WBC:2 P:0.18 Antibody :NA |
IVMP | Symptoms disappeared, Lesion disappeared |
3/0 | Yes |
| 12.M/10.41 | Fever, headache, psychosis, hypersomnia, epilepsy, dyskinesia |
T2-hyperintense lesions in parietal lobe, occipital lobe, corpus callosum | Slow background,occipital and posterior temporal electric attack | GFAP-IgG(1:100) | WBC:5 P:0.18 Antibody (-) |
IVIG,IVMP,AEDS | Symptoms disappeared, Lesion reduced |
5/1 | Yes |
| 13.M/8.41 | Fever, headache | T2-hyperintense lesions in bilateral subcortical white matter of frontal,parietal lobes | Slow background | GFAP-IgG(1:100) | WBC:1 P:0.13 Antibody :NA |
IVMP | Symptoms disappeared, Lesion reduced |
2/0 | No |
| 14.F/8.08 | Headache, psychosis, Relapse 1 times |
T2-hyperintense lesions in bilateral thalamus,temporal lobes. | Slow background | GFAP-IgG(1:32) | 1.WBC:2 P:0.164 2.WBC:72 P:0.167 Antibody (-) |
IVIG,IVMP | Symptoms disappeared, Lesion reduced |
2/0 | No |
| 15.F/1.92 | Fever, headache,hypersomnia | T2-hyperintense lesions in bilateral thalamus, cerebral peduncle, dorsal pons, cerebellar dentate nucleus and left frontal lobe. SC:lesions and mild enhancement in C3-6 |
Slow background | GFAP-IgG(1:32) | Wbc.231 P.0.47 Antibody :NA |
IVIG,IVMP | Symptoms disappeared, Lesion disappeared |
2/0 | No |
| 16.M/10.58 | After mumps,fever, headache,vomit | T2-hyperintense lesions in corpus callosum | Slow background | GFAP-IgG(1:100) | wbc:550 P:0.34 Antibody (-) |
IVIG | Symptoms disappeared, Lesion disappeared |
1/0 | No |
| 17.M/2 | Fever, headache, epilepsy, psychosis, hypersomnia, Dyskinesia,Autonomic nerve dysfunction |
Demyelinating changes in frontal, parietal, occipital, temporal, cerebellum, brainstem, basal ganglia, thalamus and corpus callosum. Mild enhancement of basal ganglia lesions.Periventricular radial linear enhancement. |
Slow background,frontal, central, temporal spike wave | GFAP-IgG(1:10) | Wbc:55 P:0.29 Antibody (-) |
IVIG,IVMP | Symptoms improved, Lesion reduced, cognitive, Movement, language impaired. |
5/4 | Yes |
| 18.F/4.75 | right optic neuritis,right limb numbness, Relapse 2 times |
Lesions in right optic nerve | Normal | 1.MOG (1:100) GFAP( 1:32) 2.MOG (1:320) GFAP( 1:100) |
WBC:80 P:0.21 MOG-IgG (1:100) |
IVIG,IVMP,Rituximab | Symptoms improved, Lesion disappeared |
2/1 | No |
| 19.F/5.67 | dyskinesia | Enhancement of the anterior root of spinal cord below T 12 | Normal | GM1 IgM、GM2 IgM、GFAP-IgG(1:32) | WBC:1 p:0.94 Antibody :NA |
IVMP | Symptoms improved, Lesion disappeared |
4/3 | No |
| 20.M/10.67 | Optic neuritis,vertigo | Lesions in bilateral optic nerve, optic chiasm, optic tract | normal | MOG-IgG(1:10)、 GFAP-IgG(1:32) |
WBC:6 P:0.24 Antibody (-) |
IVMP | Symptoms improved, Lesion disappeared |
2/1 | No |
| 21、F/9 | Fever, headache, hypersomnia, Optic neuritis, dyskinesia,dysuria,Autonomic nerve dysfunction. Relapse 5 times |
T2-hyperintense lesions in brainstem,lateral ventricle, bilateral optic nerve, Sc:C3-6,T1-10 Enhancement around lateral ventricle |
Slow background | AQP4-IgG(1:32) 、GFAP-IgG(1:32) | WBC:40 P:0.23 AQP4-IgG(1:320) |
IVIG,IVMP,Azathioprine,Rituximab | recurrence | 5/3 | No |
| 22.M/1.58 | Fever, hypersomnia, ataxia, dyskinesia, tumor |
T2-hyperintense lesions in blateral midbrain, dorsal thalamus, basal ganglia, bilateral cerebral hemispheres, pons and medulla oblongata SC :Cervical spinal cord and below T8 |
NA | YO IgG、GFAP-IgG(1:100) | WBC:25; p:0.41 Antibody :NA |
IVIG,IVMP | Symptoms improved, Lesion disappeared |
4/1 | No |
| 23.M/13.17 | Fever, headache, hypersomnia, seizures, Optic neuritis, left limb dyskinesia,left peripheral facial paralysis,limited abduction of left eyeball, Relapse 1 time |
T2-hyperintense lesions in frontal, temporal, parietal, occipital and left optic nerves. strip enhancement in left optic nerves and partial sulcus of bilateral cerebral hemispheres. |
Slow background | 1.MOG-IgG(1:32) 、 2.GFAP-IgG(1:32) MOG-IgG(1:100) |
WBC:360 P:0.2 MOG-IgG(1:32) |
IVIG,IVMP+Rituximab | Symptoms improved, Lesion disappeared |
5/2 | No |
| 24.F/12.25 | Fever, psychosis, hypersomnia, seizures, |
T2-hyperintense lesions in right temporal, parietal,occipital and thalamus | Slow background | NMDAR-IgG(1:10) 、 GFAP-IgG(1:100) |
WBC:37 P:0.15 NAMDR-IgG(1:10) |
IVIG,IVMP | Symptoms disappeared, Lesion disappeared |
4/1 | No |
| 25.M/9.25 | psychosis, hypersomnia, seizures, |
T2-hyperintense lesions in white matter of right parietal lobe, bilateral basal ganglia, thalamus | NA | NMDAR-IgG(1:10)、GFAP-IgG(1:32) | WBC:120 P:0.21 NMDAR-IgG(1:30)、GFAP(1:32) |
IVIG,IVMP | Symptoms improved, Lesion reduced |
5/3 | No |
| 26.M/11 | epilepsy(Focal attack) | T2-hyperintense lesions in right part of cerebral cortex and right optic nerve. Gyrus like enhancement in partial sulcus of right frontal, temporal, parietal and occipital lobes. |
Slow background, right temporal Sharp wave |
NMDAR-IgG(1:10)、MOG-IgG(1:100) | WBC:28 P:0.21 NMDAR-IgG(1:1)、MOG-IgG(1:100)、GFAP-IgG(1:32) |
IVIG,IVMP | Symptoms disappeared, Lesion disappeared |
1/0 | No |
| 27.F/5.67 | headache, psychosis, hypersomnia, visual ghosting |
T2-hyperintense lesions in cerebellar parenchyma, brain stem, thalamus, some cerebral hemispheres,right optic nerve. Enhancement of hippocampus and optic nerve. SC:C2-C7、T8-T12. |
Slow background | MOG-IgG(1:10) | WBC:26 P:0.15 GFAP-IgG(1:32)、NMDAR-IgG(1:32) |
IVIG,IVMP | Symptoms disappeared, Lesion reduced |
3/0 | No |
| 28.M/3.08 | Fever, headache, hypersomnia, weakened knee reflex, ptosis, ataxia |
T2-hyperintense lesions and enhancement in bilateral basal ganglia, thalamus and brainstem, bilateral cerebellar hemispheres. Strip enhancement of bilateral temporal, parietal, occipital meninges |
Slow background | GFAP-IgG(1:32)、GM1-IGM | WBC:180 P:0.751 GFAP-IgG(1:32) |
IVIG,IVMP | Symptoms disappeared, Lesion reduced |
4/0 | No |
| 29.F/3.58 | Fever, headache | T2-hyperintense lesions in Bilateral cerebral hemispheres, around the fourth ventricle, cerebellum, brain stem white matter and sacrococcygeal meninges. SC :Sacrococcygeal meningeal enhancement |
Slow background | Antibody (-) | WBC:341 P:0.4 GFAP-IgG(1:32) |
IVIG,IVMP | Symptoms disappeared, Lesion reduced |
2/1 | No |
| 30.M/7 | Fever, nausea, vomiting, dizziness | T2-hyperintense lesions in white matter of Bilateral frontal parietal lobe, bilateral basal ganglia, thalamus and corpus callosum | Slow background | GFAP-IgG(1:32) | WBC:18 P:0.45 GFAP-IgG(1:320) |
IVIG,IVMP | Symptoms disappeared, Lesion reduced |
2/1 | Yes |
| 31.M/1.33 | Fever, seizures,hypersomnia,dyskinesia | T2-hyperintense lesions in Bilateral Cerebral lobe and cerebellum, brain stem and corpus callosum Meningeal enhancement, periventricular atrophy and right frontal subdural effusion |
Slow background,occipital sharp wave | Antibody (-) | WBC:50 P:0.60 GFAP-IgG(1:32) |
IVIG,IVMP,AEDS | Symptoms improved, Lesion reduced, cognitive, Movement, impaired. |
5/4 | Yes |
| 32.M/5.58 | Fever,headache,vomiting | Normal | normal | GFAP-IgG(1:32) | WBC:600 P:0.85 GFAP-IgG(1:32) |
IVIG | Symptoms disappeared | 1/1 | No |
| 33.F/7.41 | Fever, headache, hypersomnia, psychosis, seizures, dyskinesia,dysuria |
T2-hyperintense lesions and enhancement in bilateral frontal,parietal, occipital cortex and white matter, basal ganglia, thalamus and periphery of the fourth ventricle. Meningeal enhancement of basal cistern and diffuse enhancement of meninges. SC:lesion and enhancement in T1-L1,Strip enhancement of spinal meninges |
Slow background | GFAP-IgG(1:100) | WBC:240 P:1.56 GFAP-IgG(1:32) |
IVIG,IVMP | Symptoms disappeared, Lesion disappeared |
5/1 | Yes |
| 34.F/4.08 | Fever, hypersomnia, psychosis,Involuntary action |
T2-hyperintense lesions in Bilateral dorsal thalamus and basal ganglia, bilateral frontal parietal white matter and corpus callosum | NA | Antibody (-) | WBC:55 P:0.88 GFAP-IgG(1:32) |
IVIG,IVMP | Symptoms disappeared, Lesion disappeared |
4/1 | Yes |
| 35.M/1.67 | Fever,seizures,dyskinesia, coma,peripheral motor nerve damage | T2-hyperintense lesions in bilateral cerebral cortex, subcortical and thalamus | Slow background | GFAP-IgG(1:10) | WBC:16 P:0.53 GFAP-IgG(1:1) |
IVMP | Symptoms improved, Lesion reduced, cognitive, Movement, impaired. |
5/4 | Yes |
| 36.M/4.25 | Fever, headache, hypersomnia, psychosis, hyperhidrosis |
T2-hyperintense lesions in bilateral thalamus, frontal and temporal lobes. Leptomeningeal enhancement of cerebrum |
Slow background | GFAP-IgG(1:32) | WBC:420 P:1.235 GFAP-IgG(1:32) |
IVIG,IVMP | Symptoms disappeared, Lesion disappeared |
2/1 | No |
| 37.M/7.5 | Nephrotic syndrome was diagnosed for 5 years. Fever, psychosis, seizures,coma,dysuria |
T2-hyperintense lesions in bilateral cerebral cortex and subcortical white matter, bilateral paraventricular white matter. Linear and arc enhancement of bilateral cerebral sulcus and bilateral forehead |
Diffuse low voltage | GFAP-IgG(1:32) | WBC:2 P:0.05 GFAP-IgG(1:32) |
IVMP,ADES,Tacrolimus | Symptoms improved, Lesion reduced, cognitive, Movement, impaired,epilepsy |
5/4 | Yes |
| 38.M/3.75 | Fever, psychosis, seizures,coma,urinary retention,optic neuritis, | T2-hyperintense lesions in white matter of bilateral cerebral hemispheres, bilateral basal ganglia, thalamus and fourth ventricle. Periventricular radial linear enhancement. Leptomeningeal enhancement of bilateral occipito parietal lobes. |
Slow background,frontal sharp wave | GFAP-IgG(1:32) | WBC:400 P:0.63 GFAP-IgG(1:100) |
IVIG,IVMP | Symptoms disappeared, Lesion reduced, |
5/3 | Yes |
| 39.M/1.17 | Right peripheral facial paralysis | T2-hyperintense lesions in white matter around bilateral lateral ventricles | NA | GFAP-IgG(1:1000) | WBC:2 P:0.30 GFAP-IgG(1:100) |
IVIG | Symptoms improved, Lesion reduced |
2/1 | No |
| 40.M/10.75 | Fever, psychosis, seizures, coma, dyskinesia, hyponatremia, peripheral nerve injury | T2-hyperintense lesions and enhancement in bilateral basal ganglia, caudate nucleus, thalamus, brainstem and cerebellar dentate nucleus. SC:lesions and enhancement in Spinal cord (mainly cervical and thoracic cord). Strip enhancement of meninges in spine and meninges in basal cistern. |
Slow background | Antibody (-) | Wbc:382,P:0.84 GFAP-IgG(1:32) |
IVIG,IVMP,PLEX | Symptoms improved, Lesion reduced, cognitive, Movement, impaired. |
5/3 | Yes |
M, male, F, female; N, normal; NA, no available; AED, antiepileptic drug; CSF, cerebrospinal fluid; EEG, electroencephalography; MRI, magnetic resonance imaging; mRS, modified Rankin Scale. IVIG, intravenous immunoglobulin; IVMP, intravenous methylprednisolone; PLEX, Plasma exchange;AQP4, aquaporin-4; MOG, myelin oligodendrocyte glycoprotein; NMDAR, N-methyl-d-aspartate receptor; GM, monosialotetrahexosylgangliosid.