Coreoatetosis tras encefalitis herpética / Choreoathetosis after Herpes Simplex encephalitis

Tras una encefalitis herpética (EH), una cuarta parte de los pacientes con EH tiene una recaída de la enfermedad que, excepcionalmente, se manifiesta como un síndrome extrapiramidal de tipo coreoatetósico. La base fisiopatológica de este tipo de recaída es desconocida, pero se hipotetiza que puede ser un cuadro inmunitario mediado. Se presenta el caso de un niño de 8 meses con EH por virus del herpes simple tipo 1 que en la tercera semana de evolución tuvo una recaída caracterizada por un síndrome extrapiramidal con coreoatetosis y balismo. El cuadro se interpretó como postinfeccioso, inmunitario mediado. Se trató al paciente con corticoides en altas dosis e inmunoglobulinas, además de un nuevo ciclo de aciclovir. La coreoatetosis fue tan grave y resistente a los fármacos que obligó a sedorrelajar al paciente inicialmente. Posteriormente, se ensayó tratamiento con tetrabenazina. Se estudió a escala molecular una mutación en el receptor tipo Toll 3, que se ha relacionado con la susceptibilidad de presentar la enfermedad, que resultó ser negativa (AU)

After Herpes simplex encephalitis, 25% of cases may have a relapse, rarely as a choreoathetosic movement disorder. The anatomic basis for herpes simplex virus encephalitis-associated movement disorders remains poorly understood, but the hypothesis is that it may be due to a post-infectious immune-mediated process. We report an 8-month-old boy, with herpes simplex encephalitis type 1, who started with an extrapyramidal Syndrome, presenting with choreoathetosis and ballistic movements, three weeks after onset. These new symptoms were attributed to a post-infectious immune-mediated process. We treated our patient with corticosteroids at high dose and gamma-globulins, in addition to a new course of Acyclovir. Sedation was required to control the intense choreoathetosic movements. Tetrabenazine was also tried, unsuccessfully. We studied a mutation on the toll like receptors (TLR3), which has been related to susceptibility for the disease, which was negative (AU)

[Choreoathetosis after Herpes simplex encephalitis]. / Coreoatetosis tras encefalitis herpética.

After Herpes simplex encephalitis, 25% of cases may have a relapse, rarely as a choreoathetosic movement disorder. The anatomic basis for herpes simplex virus encephalitis-associated movement disorders remains poorly understood, but the hypothesis is that it may be due to a post-infectious immune-mediated process. We report an 8-month-old boy, with herpes simplex encephalitis type 1, who started with an extrapyramidal Syndrome, presenting with choreoathetosis and ballistic movements, three weeks after onset. These new symptoms were attributed to a post-infectious immune-mediated process. We treated our patient with corticosteroids at high dose and gamma-globulins, in addition to a new course of Acyclovir. Sedation was required to control the intense choreoathetosic movements. Tetrabenazine was also tried, unsuccessfully. We studied a mutation on the toll like receptors (TLR3), which has been related to susceptibility for the disease, which was negative.