Table 3.
Clinical characteristics of the patients with anti-NMDAR encephalitis occurring simultaneously with multifocal or extensive FLAIR-T2 abnormalities
| No | Age, sex (Ethnicity) | Clinical features | Atypical features | mRS max | Immunotherapy | NMDAR ab (CSF / serum) | AQP4 ab (CSF / serum) | MOG ab (CSF / serum) | MRI brain / spinal cord | CSF: WBC per μl / OCB | FU | Last mRS |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 13# | 37, F (J) | fluctuating level of consciousness, change of behavior | bilateral facial & bulbar palsy | 5 | st, PLEX, IVIG, su | + / − | >2 / 80 | − / − | periventricular (3th and 4th ventricle) increased FLAIR abnormalities | 76 /− | 30 | 3 |
| 14 | 32, F (C) | psychiatric symptoms, depression, irritability, apathy, behavioral changes, attention and language deficit, dysphasia and mild distal myoclonus | gait disturbance | 4 | Unknown | + / n.d. | − / 20 | − / − | multiple increased T2 signals in cerebellar peduncles, pons, mid brain, pyramidal tracts, thalamus, internal capsules, and lenticular nuclei, with 2 small periventricular areas of Gd-enhancement / increased T2 signals | < 5 / + | 20 | n.d. |
| 15 | 16, F (C) | headache, diplopia, anisocoria, vertigo, ataxia, intention tremor, memory deficit, anxiety, depression, insomnia; later hemihypesthesia, dyskinesias, and dysarthria; afterwards hemiballismus, hemiparesis, vomiting and flushing | diplopia, anisocoria, ataxia, vertigo, vomitus; later hemihypestesia, hemiparesis,-ballismus | 4 | st, IVIG | + / − | − / 20 | − / − | Multiple increased T2/FLAIR signals in the right mesotemporal lobe, cerebellum and thalamus / normal | 5 / − | 4 | 4 |
| 16* | 32, F (C) | memory and behavioral dysfunction, depression | - | 4 | 1. st, IVIG; 2. st, CTX | + / + | − / 40 | − / − | Increased FLAIR signal in hippocampi and caudate nuclei | < 5 / + | 48 | 0 |
| 17 | 47, F (J) | speech disturbance, behavioral change, orofacial dyskinesias, decreased level of consciousness, seizures, tachycardia and hypoventilation | - | 5 | st, PLEX, IVIG, CTX, RTX | + / + | n.d. / 20 | − / − | Multifocal periventricular, subcortical white matter T2/FLAIR increased signal | 25 / n.d. | 22 | 1 |
| 18 | 4, F(H) | seizures, hemiparesis; later: mutism, chorea, orofacial dyskinesias | Hemiparesis | 5 | st, IVIG | + / n.d. | n.d. / − | 2 / 1280 | Multifocal areas of T2/FLAIR increased signal: periventricular, basal ganglia, cerebellum, and pons | < 5 / + | 7 | 2 |
| 19 | 6, M (H) | irritability, personality change, hypersomnia | ataxia, hyperreflexia, Babinski reflexes | 5 | IVIG | + / − | n.d. / − | >5 / 2560 | Multifocal areas of increased FLAIR signal: cortex, subcortex, thalamus, basal ganglia, cerebellum, brainstem, cervical, thoracic cord, with small areas of Gd-enhancement | 43 / + | 2.5 | 1 |
| 20 | 13, F(H) | confusion, behavioral dysfunction, stupor, orofacial dyskinesias (between DSE 3 and 4) | Leg cramps | 5 | st, IVIG, RTX | + / + | n.d. / − | − / − | Multiple subcortical and periventricular T2/FLAIR increased signal abnormalities | 18 / − | 16 | 0 |
| 21† | 18, F (C) | behavioral and speech dysfunction, orofacial dyskinesias, intubated | 9 months before encephalitis: hypesthesia in the legs | 5 | st, IVIG; RTX, CTX, MMF | + / + | n.d. / − | n.d. / − | Single area of `demyelination' in the right frontal region (discovered 2 months before onset encephalitis) | 40 / + | 34 | 0 |
| 22 | 19, M (C) | seizures, fever (multiple episodes) | Oculogyric crises | 5 | st, AZA | + / n.a. | − / n.a. | − / n.a. | Recurrent left parietal T2/FLAIR abnormality with Gd-enhancement | 36 / + | 36 | 0 |
| 23‡ | 62, F (C) | seizures, dysarthria, hemiparesis | - | 4 | 1. st, IVIG, PLEX, CTX; 2. st, CTX, RTX | + / n.a. | − / n.a. | − / n.a. | Extensive areas of increased T2/FLAIR signal in temporal and frontal lobes | 88 / n.d. | 24 | 3 |
Titers for AQP4 and MOG are based on the cell-based assay and provided as the reciprocal number of the highest sample dilution where reactivity was visible.
had an ovarian teratoma.
also suffered from discoid lupus; developed an identical relapse 15 months later.
has two sisters with relapsing remitting multiple sclerosis.
had a purely psychiatric relapse 10 months later.
mRS modified Rankin Scale; max maximum; AQP4 aquaporin-4; ab antibodies; CSF cerebrospinal fluid; WBC white blood cells; OCB oligoclonal bands; FU follow-up; mo months; F female; M male; J Japanese; C Caucasian; H Hispanic; st steroids; PLEX plasma exchange; IVIG intravenous immunoglobulins; su surgery; CTX cyclophosphamide; RTX rituximab; MMF mycophenolate mofetil; AZA azathioprine; FLAIR fluid attenuation inversion recovery; Gd gadolinium; n.d. not determined.