Journal of the Neurological Sciences 341 (2014) 173

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Response to letter to the Editor Bickerstaff encephalitis and atypical features — Bickerstaff's papers revisited We thank Dr Wong et al. for their interest in our paper regarding Bickerstaff brainstem encephalitis and value their description of the spectrum of presenting features of Bickerstaff encephalitis, thus further highlighting the importance of recognizing atypical presentations of rare disorders [1]. Although dysarthria, dysphagia, ophthalmoplegia and diplopia, are uncommon in anti-NMDA receptor encephalitis these clinical features have been identified in some patients [2]. We agree with Dr Wong et al. that there remains considerable uncertainties concerning Bickerstaff brainstem encephalitis (BBE). Although it is theoretically possible that multiple auto-immune disorders and several antibodies may co-occur it would seem unlikely that both may clinically present concurrently. As BBE is frequently associated with an infectious trigger, while anti-NMDA encephalitis is commonly associated with a tumour (e.g. ovarian teratoma), the chance of co-incidental co-occurrence seems low. The diagnosis of the 3rd patient described in the original 1951 manuscript of Bickerstaff remains unclear and Dr. Wong and colleagues do not provide a convincing argument supporting that this patient had the same syndrome as the other two patients. The circumstances of symptoms occurring temporally related to ovarian cystectomy and the tempo of recovery with a prominent psychiatric component, keeps anti-NMDA receptor encephalitis as a potential diagnosis. Keen observation and recording of clinical detail, coupled with storage of samples where diagnostic uncertainty exists, may provide the best opportunity to gain future insights into antibody associated neurological disorders, especially as both Bickerstaff encephalitis and anti-NMDA receptor encephalitis are rare but potentially treatable disorders. Disclosures Dr Merwick reports unrestricted educational grants towards travel/ accommodation costs for attendance at conferences from Boehringer Ingelheim, Pfizer Healthcare, Lunbeck and Biogen Idec. Prof. Delanty is a Consultant Neurologist, and a Director of the Epilepsy Service at Beaumont Hospital in Dublin, and is an Honorary Associate Professor at the Royal College of Surgeons in Ireland. Prof. Delanty has received honoraria for serving on national and international advisory boards of UCB Pharma, Eisai Pharmaceuticals, Janssen-Cilag, and GSK Ltd; and has received honoraria for speaking at symposia sponsored by UCB Pharma, Eisai Pharmaceuticals, and GSK Ltd. Prof. Delanty serves on the Independent Data Monitoring Committee of a clinical trial sponsored by Lundbeck Inc. Dr. Delanty is the principle investigator of the Irish component of the UK and Irish Epilepsy and Pregnancy Register

http://dx.doi.org/10.1016/j.jns.2014.03.050 0022-510X/© 2014 Elsevier B.V. All rights reserved.

which has received funding from UCB Pharma, Eisai Pharmaceuticals, Janssen-Cilag, GSK Ltd, Pfizer Inc. and Sanofi Aventis. The Epilepsy Research Programme at Beaumont Hospital has received financial support from the Higher Education Authority of Ireland, the Irish Health Research Board, Science Foundation Ireland, and Brainwave, The Irish Epilepsy Association. Prof. Dalmau is a Professor at Institució Catalana de Recerca i Estudis Avançats (ICREA) in IDIBAPS/Hospital Clínic, Barcelona, and serves on the editorial board of Neurology®; receives royalties from the editorial board of Up-To-Date; has filed a patent application for the use of LGI1 as a diagnostic test; has received royalties from Athena Diagnostics, Inc. for a patent re: Ma2 autoantibody test and has patents pending re: NMDA and GABAB receptor autoantibody tests (license fee payments received from EUROIMMUN AG); and receives research support from funding from EUROIMMUN AG, the NIH/NCI, FIS/FEDER, and a McKnight Neuroscience of Brain Disorders award.

References [1] Merwick A, Dalmau J, Delanty N. Insights into antibody associated encephalitis — Bickerstaff's papers of the 1950's revisited. J Neurol Sci 2013;334(1–2):167–8. [2] Titulaer MJ, McCracken L, Gabilondo I, Armangue T, Glaser C, Iizuka T, et al. Treatment and prognostic factors for long-term outcome in patients with anti-N-Methyl-DAspartate (NMDA) receptor encephalitis: a cohort study. Lancet Neurol 2013;12: 157–65.

A. Merwick Department of Neurology, Beaumont Hospital, Dublin 9, Ireland Charles Dent Metabolic Unit, National Hospital for Neurology & Neurosurgery, Queen Square, London WC1N 3BG, UK Corresponding author at: Department of Neurology, Beaumont Hospital, Dublin 9, Ireland. Tel.: 00353 1 8093000; fax: 00353 1 8092895. E-mail address: [email protected]. J. Dalmau Institució Catalana de Recerca i Estudis Avançats (ICREA) at Institut d'Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Service of Neurology, Hospital Clínic, University of Barcelona, Barcelona, Spain Department of Neurology, University of PA, Philadelphia, USA N. Delanty Department of Neurology, Beaumont Hospital, Dublin 9, Ireland Department of Molecular and Cellular Therapeutics, Royal College of Surgeons in Ireland, Dublin, Ireland 22 February 2014

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