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Autoimmune post-herpes simplex encephalitis of adults and teenagers
Thaís Armangue1, Germán Moris1, Verónica Cantarín-Extremera1
1From 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.
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.
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