[Acute encephalitis anti-ionotropic glutamate receptor activated N-methyl-D-aspartate (NMDAR): analysis of eleven pediatric cases in Argentina (Benito Yelín Award)].
Encephalitis are an inflammatory processes of various origin, among which include autoimmune origin. The identification of antibodies against the N-methyl-D- aspartate, allowed clinical immunological characterization of an entity susceptible to immunomodulatory therapy. Originally described in young women associated with ovarian teratoma, is now a recognized entity in children even in the absence of detectable tumors. The aim of the study was conducted through review of medical records, was to describe the clinical, developmental and findings in further studies of eleven children with confirmed diagnosis of this entity through identification of specific antibodies. All debuted with psychiatric symptoms in nine associating seizures, and two extrapyramidal movements. In the evolution of language all had commitment nine severe autonomic symptoms, one with hypoventilation and requirements of ARM. Brain MRI was abnormal in three. Eight had voltage EEG asymmetry and / or amplitude, three of them had spikes. Six had CSF pleocytosis and three of seven positive oligoclonal bands. Five IgM serology for mycoplasma were positive. CPK increase occurred in conjunction with antisychotics in five. With immunomodulatory treatment, five had complete recovery three behavioral disorders / cognitive deficits and one severe. A patient's clinical picture resolved without treatment. In any associated tumor was detected. We conclude that in front of a child with acute encephalopathy and clinical support this entity after infectious cause were ruled out, immunomodulatory therapy should be started early, avoid the use of antipsychotic drugs and search for possible hidden tumors.
Servicio de Neurología, Hospital de Pediatría Prof. Dr. J. P. Garrahan, Buenos Aires, Argentina.
Elisa
E
Pérez
Víctor
V
Ruggieri
Soledad
S
Monges
Mariana
M
Loos
Roberto
R
Caraballo
Andrés
A
Yerga
Carlos
C
Rugilo
Angela
A
Vincent
Hugo A
HA
Arroyo
D000208:Acute Disease; D060426:Anti-N-Methyl-D-Aspartate Receptor Encephalitis / Q000175:diagnosis / Q000276:immunology* / Q000503:physiopathology / Q000628:therapy; D000906:Antibodies / Q000276:immunology*; D001118:Argentina; D001921:Brain / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006801:Humans; D056747:Immunomodulation; D008279:Magnetic Resonance Imaging; D008297:Male; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D012189:Retrospective Studies; D012640:Seizures / Q000503:physiopathology; D013997:Time Factors; D016896:Treatment Outcome
D000906:Antibodies; D016194:Receptors, N-Methyl-D-Aspartate
acute encephalitis; anti­NMDAR encephalitis; immune-mediated encephalitis; limbic encephalitis