Clinical presentation and therapeutic outcomes of anti-N-methyl-D-aspartate receptor encephalitis in children

Abstract

Objective To explore the clinical characteristics, diagnosis, treatment and outcomes of anti-N-methyl-D-aspartate receptor(anti-NMDAR) encephalitis in children. Methods Six children diagnosed as anti-NMDAR encephalitis were recruited at the Department of Neurology, Capital Institute of Pediatrics, from December 2011 to April 2013.The data of clinical characteristics and laboratory examinations were retrospectively analyzed.All the children had long-term follow-ups and the prognosis was assessed. Results (1)Age and course of the disease at the time of the admission: the mean age of the 6 patients(2 female) was 3 years and 5 months, ranging from 2 years and 2 months to 6 years and 8 months.The course of the disease at the time of the admission ranged from 15 to 80 days, with a mean time of 39 days.(2)Clinical characteristics: 5 cases had afebrile convulsion and 1 case had speech impairment at the onset of disease.Convulsion occurred in all the 6 cases, 4 cases of whom had persistent convulsion, and 5 cases had impaired consciousness.All the 6 cases exhibited aphasia, and complicated with mental or emotional abnormalities, irritability or shouting.Five cases developed into sleep disorders such as sleep deprivation.Five cases had limb and facial involuntary movement, in which 2 cases had stereotyped action.Prominent autonomic nervous dysfunction including hidrosis was found in 1 case.(3)Laboratory examination: cerebrospinal fluid test was normal in 6 cases, and 1 case had slightly increased white blood cell level.Specific anti-NMDAR antibody was positive in serum and/or cerebrospinal fluid in the 6 cases.Electroencephalograph of the 6 cases showed slow wave background during lucid interval, and 5 cases had interictal epileptiform discharges.The skull MRI showed cerebral atrophy 4 cases, and 2 cases of them were complicated with encephalomalacia.No tumor was found in the patients.(4)Treatment and follow-ups: 6 cases received gamma globulin or methylprednisolone or other immunotherapy.Three cases received combined therapy with Rituximab, 1 case received plasmapheresis, and 1 case received Cyclophosphamide.Follow-ups lasted for 2 to 31 months.Three patients had clinical recovery, and varying degrees of neurological complications were found in 3 cases. Conclusions (1)Anti-NMDAR encephalitis is common in children.(2)The specificity of its clinical symptoms is not strong.The incidence of convulsion is high, and different degrees of consciousness disorders may occur in some of the severe patients.Degeneration of language function and emotional changes can be observed.Most pediatric patients have abnormal movement, and the symptoms of automatic nervous system are not prominent.(3)The disease can be confirmed by the specific anti-NMDAR antibody in the spinal fluid or plasma.(4)The time of clinical recovery is long, and an early immunotherapy is associated with a better prognosis. Key words: Anti-N-methyl-D-aspartate receptor; Child; Encephalitis