Last modified: 2014-04-03
Abstract
Introduction: Five cases of Anti-NMDAR encephalitis described with variable manifestations, and good response to immunotherapy.
Case 1: 4 years-old girl presented with fever, acute encephalopathy (A), seizures (B), and later dystonias and choreo-athetoid movements (C), perioral dyskinesias (D), urinary retention and episodes of alternating hypertension and hypotension, cardiac arrhythmias, recurrent cardiac asystole-all attributed to severe autonomic dysfunction. After failure of IV Methyl prednisolone (IVMP) and IVIg, she responded well to Rituximab.
Case 2: 5 years-old boy (Nigerian ethnicity); had symptoms A, B, C, and D as case 1. After failure of IVMP, he responded well to Rituximab given 2 months after onset.
Case 3: 5 months-old boy presented with symptoms A, B, C, and D as case 1, 3 weeks after febrile illness. He had suboptimal response to IVMP and plasmapheresis, but responded well to Rituximab.
Case 4: 8 years-old boy had A, B, C, and D as case 1, but also had severe catatonia and mutism. He had poor response to IVMP and IVIg (3rd week of illness), but excellent response to Rituximab.
Case 5: 8 year-old boy had acute-onset visual hallucinations and seizures. He improved spontaneously within 48 hours. Anti-NMDAR Ab positive in CSF but negative in serum.
Investigations: MRI-Brain was normal. CSF showed mildly elevated proteins; positive Anti NMDAR Ab in CSF and serum in all (except case 5-only CSF positive).
Follow up and conclusion: All had good recovery except case1 (partial recovery), and no relapse. High index of suspicion and early Rituximab therapy improves outcome.