Table 1.
Demographics, clinical features, main therapy, and long-term follow-up outcomes of Cases with MOG-antibody-associated disorders.
| Age/Sex | Prodromal symptoms | Neurological symptoms and signs | MOG-Ab in CSF/ Serum |
CSF*,† analysis | EEG | MRI | Acute therapy | Maintenance therapy‡,§ | Relapses | Long-term follow-up outcomes | VEP or OCT | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Case 1 | 52 Y/F | Headaches | Limb weakness, unsteady gait, positive Romberg sign, dysarthria, dysphagia | 1:10/1:10; Serum MOG-Ab 1:32 in the relapse |
CSF protein 0.61 g/L | Mild abnormality | Hyperintense signal on T2WI/FLAIR/ADC and hypointense signal on T1WI in the brainstem, left cerebellum, right temporal lobe, corpus callosum; the hyperintense MRI signal weakened 15 d later. optic nerve MRI: (−) | MPPT and high-dose IVIG | Low-dose MP and AZA for 30 Ms | 4 Ms after discharge | Symptoms were relieved at 30 Ms | None |
| Case 2 | 48 Y/M | Fever | Acute cognitive impairment, slow response, slight neck rigidity, increased muscle tone in the extremities | Negative/1:10 | No abnormality | Roughly normal EEG | Initial MRI showed hyperintense signal on T2WI/FLAIR and hypointense signal on T1WI in the brainstem, hypothalamus, and hippocampus. One mo later, the hyperintense MRI FLAIR signal in the brainstem disappeared | MPPT and high-dose IVIG | MP 48 mg/d, tapered off to 16 mg/d, maintained for 1 Y | None | Cognitive impairment, slow response, and needs assistance with activities of daily living at 25 Ms | None |
| Case 3 | 27 Y/F | None | Repeated impaired vision and hypomnesis, convulsions, increased muscle tone in extremities; positive bilateral ataxia signs | Negative/1:10 | No abnormality | Mild abnormality | Hyperintense signal on T2WI/FLAIR and hypointense signal on T1WI at the bilateral cerebellar hemispheres, frontotemporal, and left parietal lobes, without diffusion restriction | MPPT | MP 40 mg/d, tapered off, low dose for 6 Ms | Cognitive decline after MP therapy discontinuation, however improved after rehospitalization | Incapacitation and poor verbal ability at 26 Ms | None |
| Case 4 | 29 Y/M | Headache and fever | Impaired vision in right eye, convulsions |
Negative/1:32; Serum MOG-Ab 1:32 in the 2nd relapse | No abnormality | Roughly normal EEG | MRI showed hyperintense signal on FLAIR in a sulcus in the right frontotemporal lobe, and right optic nerve thickening with uniform enhancement | MPPT | Low-dose PED for 6 Ms, initial methotrexate 15 mg/W, followed by 12.5 mg/d after the 2nd relapse | One mo after PED discontinuation, and again after 4 Ms | Improved vision at 27 Ms | None |
| Case 5 | 29 Y/M | Low-grade fever and headache | Impaired vision, convulsions, slight neck rigidity, disappearance of pupillary light reflex in the right eye |
Negative/1:32 | CSF WBC, 264 × 106/L; protein 0.954 g/L | Mild abnormality | Initial MRI showed hyperintense signal on FLAIR in the corpus callosum; 3 Ms later, the hyperintense MRI signal nearly disappeared | MPPT | Low-dose PED for 6 Ms, MMF for 8 Ms | None | Normal vision and daily activity at 32 Ms | None |
| Case 6 | 43 Y/M | Coughs, nasal obstruction | Decreased vision in the right eye, weakness and numbness of the lower limbs | 1:32/1:100 | CSF pressure 180 mmH2O, protein 804.6 mg/L | Mild abnormality | Initial brain, optic nerve, and cervicothoracic spine MRI showed hyperintense signal on T2WI and equisignal on T1WI at C3–4. Thoracic spine MRI: (−); 6 Ms later, the hyperintense MRI signal at C3–4 disappeared | MPPT | PED 60 mg/d, tapered off, then low dose for 18 Ms; AZA 100 mg/d after the 1st relapse, and maintained for 25 Ms | 3 Ms after the 1st episode, and again after another 3 Ms | Poor right-eye vision at 36 Ms | VEP at the 1st episode showed right optic nerve damage |
| Case 7 | 65 Y/F | None | Impaired vision in both eyes, droopy right upper eyelid, sluggish, diminished pupillary light reflex in the right eye with impaired adduction | Negative/1:32 | CSF pressure 200 mmH2O, protein 1009.6 mg/L | Mild abnormality | MRI 1 mo after onset showed a small syringomyelia and encephalanalosis; cervical spine MRI: (−) | MPPT | Initial PED 55 mg/d, tapered off, then low dose for 18 Ms with AZA for 44 Ms | None | Impaired right-eye vision at 44 Ms | 16 Ms later, VEP showed amplitude reduction in right eye. OCT showed thinning of PRNFL in left eye |
| Case 8 | 35 Y/F | None | Repeated impaired vision in the right eye, convulsions | Negative/1:32 | No abnormality | No abnormality | Brain and optic nerve MRI showed slightly thickened right optic nerve | MPPT and MMF | Low-dose MP for 10 Ms, MMF for 33 Ms | 2 Ms after MP discontinuation | Slightly decreased vision at 33 Ms | None |
| Case 9 | 4 Y/F | None | Decreased vision in both eyes with right gaze palsy | Negative/1:100; serum MOG-Ab: 1:100 in the 1st relapse | CSF protein 212.1 mg/L | No abnormality | Initial MRI showed multiple intracranial foci. Cervicothoracic spine MRI: (−). Two yr later MRI showed hyperintense signal on T2WI and hypointense signal on T1WI at the bilateral frontotemporal lobes; 8 Ms later, MRI showed that the foci nearly disappeared | MPPT | MP tapered off, followed by low-dose MP for 1 yr; long-term maintenance with AZA 50 mg/d | 7 Ms after initial hospitalization, and 2nd 3 Ms after immunotherapy discontinuation | Improved vision 64 Ms after the 1st onset | None |
| Case 10 | 16 Y/F | Low-grade fever, general fatigue | Raving, drowsiness, disappearance of pharyngeal reflex | Negative/1:10 | CSF protein 352.3 mg/L | Mild-to-moderate abnormality | Initial MRI showed abnormal signals in the medulla oblongata; 6 Ms later, MRI showed abnormal signals in the area postrema, left basal ganglia, and around the third ventricle; 30 Ms later, MRI showed softening foci in the basal ganglia and area postrema | MPPT and AZA | Initial PED 60 mg/d, tapered off, low dose for 24 Ms and AZA for 48 Ms | None | Resumed good general condition at 48 Ms | None |
| Case 11 | 41 Y/F | None | Dizziness, walk unsteadily, positive Romberg sign, left sided positive finger- nose test |
Negative/1:10 | No abnormality | No abnormality | Brain and cervicothoracic spine MRI: (−). Optic nerve MRI showed bilateral optic nerve swelling. | MPPT | Without immunotherapy | None | Freedom of movement at 40 Ms | None |
| Case 12 | 27 Y/M | Cold symptoms | Limb numbness, decreased muscle strength in lower extremities, increased muscle tone in the extremities, bilateral positive Babinski sign | 1:10/1:32 | CSF WBC 82 × 106/L | Not done | MRI showed hyperintensity on T2WI and isointense signal on T1WI at C3–6. Brain and thoracic spine MRI: (−) | MPPT | Initial PED 60 mg/d, tapered off, low dose for 6 Ms; MMF 0.5 g/d for 18 Ms | None | Return to normal life and work at 29 Ms | None |
| Case13 | 22 Y/M | None | Left eye swelling with impaired vision, limited outreach in both eyes |
Negative/1:10 | No abnormality | Mild-to-moderate abnormality | Brain, optic nerve, and cervicothoracic spine MRI: (−) | MPPT | Initial PED 60 mg/d, tapered off, low dose for 1 yr | None | Near complete vision recovery at 37 Ms | None |
| Case 14 | 6 Y/M | None | Decreased vision in both eyes | Negative/1:10 | No abnormality | Not done | Brain MRI: (−) | MPPT | Initial PED 60 mg/d, tapered off, low dose for 6 Ms | None | Return to normal vision at 36 Ms | None |
| Case 15 | 32 Y/M | None | Decreased vision in both eyes, visual field defect in the right eye | Negative/1:32 | No abnormality | Not done | Brain, optic nerve, and cervicothoracic spine MRI: (−) | MPPT | Initial PED 60 mg/d, tapered off, low dose for 9 Ms, MMF 1 g/d for 11 Ms | None | Good vision recovery at 24 Ms without affecting normal work | VEP: P100 latency with moderate amplitude reduction in right eye, and severe amplitude reduction in left eye. 1 yr later, OCT: thinning of PRNFL in both eyes |
(−) = no abnormality, ADC = apparent diffusion coefficient, AZA = Azathioprine, C = cervical vertebra, CSF = cerebrospinal fluid, EEG = Electroencephalogram, F = female, FLAIR = fluid-attenuated inversion-recovery, IVIG = intravenous immunoglobulin, M = male, MMF = mycophenolate mofetil, MOG = myelin oligodendrocyte glycoprotein, MOG-Ab = myelin oligodendrocyte glycoprotein antibody, MP = methylprednisolone, MPPT = methylprednisolone pulse treatment, MRI = magnetic resonance imaging, Ms = months, OCT = optical coherence tomography, PED = prednisone, PRNFL = peripapillary retinal nerve fiber layer, VEP = Visual evoked potential, W = week, WBC = white blood cell, WI = weighted imaging, Y = year, Yrs = years.
*
CSF protein reference range: 150–450 mg/L.
†
CSF WBC reference range: 0–8 × 106/L.
‡
Tapered off, decrease of prednisone by 5 mg/wk or methylprednisolone by 4 mg/wk.
§
Low dose, 5–10 mg/d prednisone or 4 mg/d methylprednisolone.