Short communication Anti-brain but not celiac disease antibodies in Landau-Kleffner Syndrome and related epilepsies S. Boscolo a , V. Baldas b , G. Gobbi c,1 , L. Giordano d,1 , G. Cioni e,1 , T. Not b , A. Ventura b , E. Tongiorgi a, * a BRAIN Centre for Neuroscience, Department of Biology, University of Trieste, Via L. Giorgieri 10-34127, Trieste, Italy b Pediatric Clinic and Transfusional centre of the IRCCS Burlo Garofolo, Via dell’Istria 65, Trieste, Italy c U.O.NPI Dep. Materno Infantile Ospedale Maggiore, L.go Nigrisoli 2, Bologna, Italy d Neurological Pediatric Clinic, Spedali Riuniti, P.le Spedali Civili 1, Brescia, Italy e Dpt. Developmental Neuroscience, Stella Maris Scientific Institute, University of Pisa, Via dei Giacinti, 2, Italy Received 7 July 2004; received in revised form 22 October 2004; accepted 22 October 2004 Abstract The Landau-Kleffner syndrome, the continuous spikes-waves during slow sleep syndrome and the benign epilepsy of childhood with rolandic spikes are rare childhood epilepsies with unknown etiology. Improvement in patients treated with immunoglobulin suggests an involvement of the immune system. We provide immunohistochemical evidence of autoantibodies against rat brain auditory cortex, brainstem and cerebellum, in children suffering with one or more of these syndromes. Only 1/14 patient was celiac. D 2004 Elsevier B.V. All rights reserved. Keywords: Autoimmunity; Pediatric epilepsy; Anti-brain autoantibodies 1. Introduction The Landau-Kleffner syndrome (LKS), the continuous spikes-waves during slow sleep syndrome (CSWS) and the benign epilepsy of childhood with rolandic spikes (BRE) are distinct syndromes (ILAE, 1989). However, these epileptic disorders may be considered as part of a continuous pathological spectrum with BRE as a mild disorder, on one end, and CSWS as a more severe disorder, on the other end. The LKS, is a rare, age-related (onset 4–7 years) epileptic aphasia occurring in children who lose previously acquired speech and language abilities (Landau and Kleffner, 1957) and may occur without seizures being recorded. LKS Variant (LKSV) includes behavioral features of autistic regression (Mantovani, 2000). All patients with Landau- Kleffner syndrome show, at some time during its course, the sleep-EEG pattern of continuous spike and wave discharges (Tassinari et al., 2002). CSWS is characterized by variable clinical manifesta- tions and an EEG consisting of an almost continuous presence of spike waves discharges in slow sleep, persisting in three or more records over a period of at least one month. On the other hand, the EEG pattern consisting of CSWS may simply be a transient phase which influences the long outcome of the BRE and LKS (Veggiotti et al., 1999) and which characterizes the LKSV. BRE occurs in neurologically and cognitively normal children who usually present with a nocturnal partial seizure and with typical EEG showing centrotemporal and multi- focal or generalized sharp slow waves (Beaussart, 1972). BRE accounts for 6–16% of all non-febrile seizures in children and, in 40% of patients, has a familial occurrence. All these epileptic syndromes appear in young children in the absence of obvious structural lesions suggesting that 0165-5728/$ - see front matter D 2004 Elsevier B.V. All rights reserved. doi:10.1016/j.jneuroim.2004.10.031 * Corresponding author. Tel.: +39 040 558 3864; fax: +39 040 568855. E-mail address: tongi@units.it (E. Tongiorgi). 1 These authors have equally contributed. Journal of Neuroimmunology 160 (2005) 228 – 232 www.elsevier.com/locate/jneuroim