PMC6431454_01
Brain MRI of patient with positive Anti-NMDAR encephalitis. Arrow shows cortical hyperintensity in uncus right and hippocampus with T2-FLAIR technique.
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In one case, magnetic resonance imaging (MRI) showed cortical hyperintensity on uncus and right hippocampus suggestive of limbic encephalitis (Figure 1).
Anti-NMDAR antibodies detected by indirect immunofluorescence. (a) Hek293 transfected cells; (b) granular layer of cerebellum in tissue of rat.
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CSF and serum samples were analyzed without dilution and with a 1:10 dilution, respectively (Figure 2).
Treatment grouped by patient. P1, P3, P4, and P5 received methylprednisolone (1 g IV daily) during 5 days; P2 and P6 received methylprednisolone according their weight (1mg/kg) also during 5 days. IVIG was administered in five cases at immunomodulatory doses (400-800mg/kg/day). P2 and P3 needed cyclophosphamide (750 mg/m2 IV) every three weeks during 4 cycles; P4 received cyclophosphamide (750 mg/m2 IV) 2 cycles with 6 weeks between each one. Rituximab was administered every two weeks (375 mg/m2 IV) for 2 cycles in P1, P2, P3, and P4. In P2 it was necessary to add four cycles of chemotherapy for immature teratoma repeated every 21 days with bleomycin (30 IU IV every week), etoposide (100mg/m2 IV daily for 5 days), and cisplatin (20mg/m2 IV daily for 5 days).
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IVIG; four patients received rituximab according to their body surface area with a 375 mg/m2 IV infusion every two weeks for 2 cycles; four patients needed plasma exchange and three cyclophosphamide 750 mg/m2 IV; one case received 2 cycles with 6 weeks between each one and two patients received 4 cycles every three weeks. In the case of immature teratoma it was necessary to add four cycles of chemotherapy repeated every 21 days with bleomycin (30 IU IV every week, for three doses), etoposide (100mg/m2 IV daily for 5 days), and cisplatin (20mg/m2 IV daily for 5 days) (Figure 3).