• Thaís Armangue, Germán Moris, Verónica Cantarín-Extremera, Carlos Enrique Conde, Kevin Rostasy, Maria Elena Erro, Juan Carlos Portilla-Cuenca, Eulàlia Turón-Viñas, Ignacio Málaga, Beatriz Muñoz-Cabello, Carmen Torres-Torres, Sara Llufriu, Luis González-Gutiérrez-Solana, Guillermo González, Ignacio Casado-Naranjo, Myrna Rosenfeld, Francesc Graus, Josep Dalmau, and Spanish Prospective Multicentric Study of Autoimmunity in Herpes Simplex Encephalitis.
• From the Neuroimmunology Program (T.A., S.L., M.R., F.G., J.D.), August Pi Sunyer Biomedical Research Institute (IDIBAPS), and the Department of Neurology (S.L., F.G), Hospital Clínic, University of Barcelona; the Department of Neurology (G.M.) and the Pediatric Neurology Unit, Pediatrics Department (I.M.), Hospital Universitario Central de Asturias, Oviedo; the Department of Pediatric Neurology (V.C.-E., L.G.-G.-S.), Hospital Universitario Niño Jesús, Madrid, Spain; the Department of Neurology (C.E.C., G.G.), Hospital Universitario Hernando Moncaleano Perdomo, Neiva, Colombia; the Department of Pediatric Neurology (K.R.), Children's Hospital Datteln, Witten/Herdecke University, Witten, Germany; the Department of Neurology (M.E.E., I.C.-N.), Complejo Hospitalario de Navarra, Pamplona; the Department of Neurology (J.C.P.-C.), Hospital San Pedro de Alcántara, Cáceres; the Pediatric Neurology Unit (E.T.-V.), Hospital de la Santa Creu i Sant Pau, Barcelona; the Pediatric Neurology Unit (B.M.-C.), Hospital Universitario Virgen del Rocio, Sevilla; the Department of Pediatric Neurology (C.T.-T.), Hospital General La Mancha Centro, Alcázar de San Juan, Spain; the Department of Neurology (J.D.), University of Pennsylvania, Philadelphia; and the Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
• Neurology. 2015 Nov 17; 85 (20): 1736-43.

ObjectiveTo report 14 patients with immune-mediated relapsing symptoms post-herpes simplex encephalitis (HSE) and to compare the clinical and immunologic features of the teenage and adult group with those of young children.MethodsProspective observational study of patients diagnosed between June 2013 and February 2015. Immunologic techniques have been reported previously.ResultsAmong the teenage and adult group (8 patients, median age 40 years, range 13-69; 5 male), 3 had an acute symptom presentation suggesting a viral relapse, and 5 a presentation contiguous with HSE suggesting a recrudescence of previous deficits. Seven patients developed severe psychiatric/behavioral symptoms disrupting all social interactions, and one refractory status epilepticus. Blepharospasm occurred in one patient. Five patients had CSF antibodies against NMDA receptor (NMDAR) and 3 against unknown neuronal cell surface proteins. In 5/6 patients, the brain MRI showed new areas of contrast enhancement that decreased after immunotherapy and clinical improvement. Immunotherapy was useful in 7/7 patients, sometimes with impressive recoveries, returning to their baseline HSE residual deficits. Compared with the 6 younger children (median age 13 months, range 6-20, all with NMDAR antibodies), the teenagers and adults were less likely to develop choreoathetosis (0/8 vs 6/6, p < 0.01) and decreased level of consciousness (2/8 vs 6/6, p < 0.01) and had longer delays in diagnosis and treatment (interval relapse/antibody testing 85 days, range 17-296, vs 4 days, range 0-33, p = 0.037).ConclusionIn teenagers and adults, the immune-mediated relapsing syndrome post-HSE is different from that known in young children as choreoathetosis post-HSE and is underrecognized. Prompt diagnosis is important because immunotherapy can be highly effective.© 2015 American Academy of Neurology.

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