Table 2.
Comparison of clinical characteristics in AQP4-ab-positive and MOG-ab-positive children.
AQP4-ab (+) (n = 8) | MOG-ab (+) (n = 64) | P value | |
---|---|---|---|
Phenotype at onset, n (%) | |||
ADEM | 1 (12.5) | 34 (53.1) | 0.073 |
Encephalitis (other than ADEM) | 0 (0) | 13 (20.3) | 0.357 |
ON | 3 (37.5) | 14 (21.9) | 0.589 |
Myelitis | 4 (50) | 2 (3.1) | 0.001 |
ON with Myelitis | 0 (0) | 1 (1.6) | 1.000 |
First attack symptoms, n (%) | |||
Seizure | 1 (12.5) | 13 (20.3) | 0.958 |
Encephalopathy | 2 (25) | 36 (56.3) | 0.196 |
Intractable nausea/ vomiting/ hiccups (APS) | 4 (50) | 24 (37.5) | 0.765 |
Brainstem syndrome (excluding APS) | 1 (12.5) | 16 (25) | 0.731 |
Cerebellar symptoms | 2 (25) | 16 (25) | 1.0 |
Pyramidal signs | 7 (87.5) | 28 (43.8) | 0.05 |
Bladder/bowel | 6 (75) | 17 (26.6) | 0.018 |
Motor | 8 (100) | 34 (53.1) | 0.031 |
Sensory | 6 (75) | 10 (15) | 0.001 |
Vision | 6 (75) | 29 (45.3) | 0.227 |
CSF findingsa, n (%) | |||
CSF pleocytosis (>5 cells/mm3) | 2/5 (40) | 26/62 (41.9) | 1.0 |
CSF protein elevation (>40 mg/dL) | 2/5 (40) | 27/62 (43.5) | 1.0 |
Relapse, n (%) | |||
ON only | 1/5 (20) | 1/19 (5.3) | 0.430 |
TM only | 3/5 (60) | 1/19 (5.3) | 0.018 |
ON or TM with other | 1/5 (20) | 8/19 (42.1) | 0.520 |
Other | 0/5 (0) | 9/19 (47.4) | 0.105 |
Clinical recovery, n (%) | 0.032 | ||
Complete | 3/7 (42.9) | 48/63 (76.2) | |
Minor residuals | 0/7 (0) | 6/63 (9.5) | |
Severe residuals | 4/7 (57.1) | 9/51 (14.3) | |
EDSS score at onset, median (IQR) | 4 (3.25, 5) | 3 (1.38, 4) | 0.071 |
EDSS score at 1 y, median (IQR) | 3.5 (3.125, 3.875) | 0 (0, 0) | 0.002 |
EDSS score at 2 y, median (IQR) | 3 (0,4) | 0 (0, 0.5) | 0.103 |
EDSS score at FU, median (IQR) | 3 (0,3) | 0 (0, 0) | 0.062 |
Acute-phase therapy (IVMP/PLEX/IVIG) | 7/8 (87.5) | 62/64 (96.9) | 0.301 |
Maintenance therapy at FU, n (%) | 7/7 (100) | 25/63 (39.7) | 0.008 |
APS, area postrema syndrome; Ab, antibody; ADEM, acute disseminated encephalomyelitis; AQP4, aquaporin−4; CSF, cerebrospinal fluid; EDSS, Expanded Disability Status Scale; FU, follow-up; IQR, interquartile range; IVIG, intravenous immunoglobulins; IVMP, intravenous methylprednisolone; MOG, myelin oligodendrocyte glycoprotein; ON, optic neuritis; PLEX, plasma exchange; TM, transverse myelitis; y, year(s).
Only lumbar puncture performed within one month from onset of symptoms are shown in bold.