Autoimmune post-herpes simplex encephalitis of adults and teenagers
Por:
Armangue, T, Moris, G, Cantarin-Extremera, V, Conde, CE, Rostasy, K, Erro, ME, Portilla-Cuenca, JC, Turon-Vinas, E, Malaga, I, Munoz-Cabello, B, Torres-Torres, C, Llufriu, S, Gonzalez-Gutierrez-Solana, L, Gonzalez, G, Casado-Naranjo, I, Rosenfeld, M, Graus, F, Dalmau, J, Spanish Prospective Multicentric S
Publicada:
17 nov 2015
Resumen:
Objective:To 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.Methods:Prospective observational study of patients diagnosed between June 2013 and February 2015. Immunologic techniques have been reported previously.Results:Among 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).Conclusion:In 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.
Filiaciones:
Armangue, T:
Univ Barcelona, Hosp Clin Barcelona, Neuroimmunol Program, E-08007 Barcelona, Spain
Moris, G:
Hosp Univ Cent Asturias, Dept Neurol, Oviedo, Spain
Cantarin-Extremera, V:
Hosp Univ Nino Jesus, Dept Pediat Neurol, Madrid, Spain
Conde, CE:
Hosp Univ Hernando Moncaleano Perdomo, Dept Neurol, Neiva, Colombia
Rostasy, K:
Univ Witten Herdecke, Childrens Hosp Datteln, Dept Pediat Neurol, Witten, Germany
Erro, ME:
Complejo Hosp Navarra, Dept Neurol, Pamplona, Spain
Portilla-Cuenca, JC:
Hosp San Pedro de Alcantara, Dept Neurol, Caceres, Spain
Turon-Vinas, E:
Hosp Santa Creu & Sant Pau, Pediat Neurol Unit, Barcelona, Spain
Malaga, I:
Hosp Univ Cent Asturias, Pediat Neurol Unit, Dept Pediat, Oviedo, Spain
Munoz-Cabello, B:
Hosp Univ Virgen del Rocio, Pediat Neurol Unit, Seville, Spain
Torres-Torres, C:
Hosp Gen La Mancha Ctr, Dept Pediat Neurol, Alcazar De San Juan, Spain
Llufriu, S:
Univ Barcelona, Hosp Clin Barcelona, Neuroimmunol Program, E-08007 Barcelona, Spain
Univ Barcelona, Hosp Clin Barcelona, August Pi Sunyer Biomed Res Inst IDIBAPS, E-08007 Barcelona, Spain
Univ Barcelona, Hosp Clin Barcelona, Dept Neurol, E-08007 Barcelona, Spain
Gonzalez-Gutierrez-Solana, L:
Hosp Univ Nino Jesus, Dept Pediat Neurol, Madrid, Spain
Gonzalez, G:
Hosp Univ Hernando Moncaleano Perdomo, Dept Neurol, Neiva, Colombia
Casado-Naranjo, I:
Complejo Hosp Navarra, Dept Neurol, Pamplona, Spain
Rosenfeld, M:
Univ Barcelona, Hosp Clin Barcelona, Neuroimmunol Program, E-08007 Barcelona, Spain
Graus, F:
Univ Barcelona, Hosp Clin Barcelona, Neuroimmunol Program, E-08007 Barcelona, Spain
Univ Barcelona, Hosp Clin Barcelona, August Pi Sunyer Biomed Res Inst IDIBAPS, E-08007 Barcelona, Spain
Univ Barcelona, Hosp Clin Barcelona, Dept Neurol, E-08007 Barcelona, Spain
Dalmau, J:
Univ Barcelona, Hosp Clin Barcelona, Neuroimmunol Program, E-08007 Barcelona, Spain
Univ Penn, Dept Neurol, Philadelphia, PA 19104 USA
Catalan Inst Res & Adv Studies ICREA, Barcelona, Spain
Green Published
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