Table 2.
Diagnostic testing and treatment of GFAP-IgG patients.
| Characteristic | Value | Pediatric patients | Adult patients | P |
|---|---|---|---|---|
| CSF analysis at onset, n/total (%) | ||||
| Pleocytosis (> 5 cells/mm3) | 46/58(79.3%) | 15/18(83.3%) | 31/40(77.5%) | 0.875 |
| Elevated protein level (> 0.5 g/L) | 35/58 (60.3%) | 6/18(33.3%) | 29/40(72.5%) | 0.005 |
| Hypoglycorrhachia (<2.5 mmol/L) | 12/58 (20.7%) | 2/18(11.1%) | 10/40(25%) | 0.391 |
| CSF Oligoclonal Bands (type 2) | 22/54(40.7%) | 6/14(42.9%) | 16/40(40.0%) | 0.851 |
| Overlapping antibody n/total (%) | ||||
| AQP4-IgG | 7/59(11.9%) | 2/18(11.1%) | 5/41(12.2%) | |
| MOG-IgG | 5/59(8.5%) | 3/18(16.7%) | 2/41(4.9%) | |
| NMDAR-IgG | 3/59(5.1%) | 1/18(5.6%) | 2/41(4.9%) | |
| Others* | 3/59(5.1%) | 0/18 | 3/41(7.3%) | |
| MRI, n/total (%) | ||||
| MRI (brain) abnormalities | 43/59(72.9%) | 16/18(88.9%) | 27/41(65.9%) | 0.130 |
| Gadolinium enhanced lesion (brain) | 23/40(57.5%) | 5/7(71.4%) | 18/33(54.5%) | 0.689 |
| Leptomeninges enhancement | 11/40(27.5%) | 2/7(28.6%) | 9/33(27.3%) | 1 |
| Perivascular-radial enhancement | 4/40(10.0%) | 0 | 4/33(12.1%) | 1 |
| Lesion location | ||||
| Juxtacortical | 22/59(37.3%) | 8/18(44.4%) | 14/41(34.1%) | 0.451 |
| Periventricular | 9/59(15.3%) | 2/18(11.1%) | 7/41(17.1%) | 0.847 |
| Corpus callosum | 9/59(15.3%) | 4/18(22.2%) | 5/41 (12.2%) | 0.553 |
| Basal ganglia | 13/59(22.0%) | 4/18(22.2%) | 9/41 (22.0%) | 1 |
| Thalamus | 14/59(23.7%) | 6/18(33.3%) | 8/41 (19.5%) | 0.414 |
| Brachium pontis | 4/59(6.8%) | 1/18(5.6%) | 3/41(7.3%) | 1 |
| Brainstem tegmentum | 16/59(27.1%) | 3/18(33.3%) | 10/41(24.4%) | 0.751 |
| Cerebellar hemispheres | 5/59(8.5%) | 2/18 (11.1%) | 3/41(7.3%) | 1 |
| MRI (spinal cord) abnormalities | 35/50(70.0%) | 13/16(81.3%) | 22/34(64.7%) | 0.390 |
| Gadolinium enhanced lesion (spinal cord) | 13/21(61.9%) | 3/3(100%) | 10/18(55.6%) | 0.409 |
| LETM | 17/50(34.0%) | 8/16(50.0%) | 9/34(26.5%) | 0.101 |
| Cervical cord | 26/50 (52.0%) | 10/16(62.5%) | 16/34(47.1%) | 0.308 |
| Thoracic cord | 24/50(48.%) | 10/16(62.5%) | 14/34(41.2%) | 0.159 |
| Medullary cone | 2/50(4.0%) | 2/16(12.5%) | 0/34 | 0.098 |
| Acute phase treatment n/total (%) | ||||
| IVMP alone | 26/59(44.1%) | 5/18(27.8%) | 21/41(51.2%) | |
| IVMP+IVIG | 22/59(37.3%) | 11/18(61.1%) | 11/41(26.8%) | |
| Others^ | 7/59(11.9%) | 2/18(11.1%) | 5/41(12.2%) | |
| No immunotherapy | 4/59(6.8%) | 0 | 4/41(9.8%) | |
| Maintenance therapy n/total (%) | ||||
| Glucocorticoids alone | 40/59(67.8%) | 15/18(83.3%) | 25/41(61.0%) | |
| Mycophenolate mofetil with or without glucocorticoids | 9/59(15.3%) | 1/18(5.6%) | 8/41(19.5%) | |
| Others # | 2/59(3.4%) | 1/18(5.6%) | 1/41(2.4%) | |
*: GAD65-IgG, Yo-IgG, GlyR-IgG
^:IVMP+RTX,n = 2;IVMP+PE+IVIG,n = 2;IVMP+IVIG+RTX,n = 1;IVMP+PE,n = 1;IVIG+EIA,n = 1.
#: tacrolimus in one adult patients, azathioprine in one pediatric patient
CSF, cerebrospinal fluid; AQP4, Aquaporin 4; MOG, Myelin oligodendrocyte glycoprotein; NMDAR, N-methyl-D-aspartate receptor; MRI, magnetic resonance imaging; LETM, longitudinally extensive transverse myelitis; IVIG, intravenous immunoglobulin; IVMP, intravenous methylprednisolone; RTX, rituximab; PE, plasma exchange; EIA, extracorporeal immunoadsorption.