This was my first attendance at the Encephalitis Conference and I immediately knew that the 2017 event would be special when finding an empty seat was a greater challenge than navigating the London underground as a bona fide Northerner. On arrival, I was enthralled by the variety of disciplines and industries which were represented including neurologists, healthcare professionals, clinical psychologists and neuropsychologists, academics, researchers, and lawyers.
The 2017 Encephalitis Society Professional`s Conference took place in front of a packed house at MacFarlane’s LLP in central London, with delegates travelling from the US, the Netherlands, Denmark, UK, and Indonesia. Special thanks were in order for the Encephalitis Society who organised and sponsored the day along with other sponsors, which included MacFarlane`s LLP, Routledge publishers, the Auto-Immune Encephalitis Alliance, the University of Liverpool, and the Health Protection Research Unit for Emerging and Zoonotic Infections.
In his keynote address titled “A curiosity for cures”, Professor Andrew Lees opened with his fantasia on the subject of Encephalitis Lethargica and of his unlikely mentor, William Burroughs, the author of many novels, short stories and essays, perhaps best known for his novel Naked Lunch and the infamous character, Doc Benway. Professor Lees reflected on the anti-bureaucratic stance of his mentor and the Faustian bargain which was later struck, ensuring Professor Lees completed his medical school training. The audience was then enlightened with a historical perspective of Encephalitis Lethargica in England, dating back 100 years to 1918, where it was mysteriously referred to as the “sleepy sickness”. During the first few decades, the source of this mysterious illness was unknown, although doctors were powerless to assist patients, progress was made on ruling out early candidates of its onset, such as Spanish Flu or a cerebral variant of Polio. Within 20 years a hospital, Highlands Hospital in Enfield, North London was opened to care for the most severely affected patients of the early epidemic. This very hospital would later become the backdrop of the 1990 film “Awakenings”, staring Robert De Niro and Robin Williams, drawing upon the personal accounts and experiences of Professor Lees and his patients. (See ACNR’s documentary about Andrew Lees’ work at vimeo.com)
Dr Marienke de Bruijn, of Erasmus University Medical Centre, Rotterdam, presented her work on long-term neuropsychological and behavioural outcomes in paediatric anti-NMDAR encephalitis. Dr de Bruijn reported differing clinical presentations within adult and paediatric anti-NMDAR encephalitis: children and adults present both with behavioural difficulties, additionally adults more with memory difficulties and children with epilepsy or movement disorders. Dr de Bruijn reported good outcomes for more than three quarters of the children in her study, although a sobering reflection was that only 60% of the children returned to school at their previous level, and persistent neuropsychological difficulties were reported.
Following Dr de Bruijn was Dr Jessie Cooper of the School of Health Sciences, University of London, with her presentation “Care beyond the hospital ward: understanding the socio-medical trajectory of HSVE”. Dr Cooper highlighted how the rising number of survivors of Herpes Simplex Virus Encephalitis (HSVE) has led to an increasing number of people facing life with an acquired brain injury. Conducting interviews with participants affected by HSVE, several common themes emerged including how the daily lives of loved ones can be affected following HSVE, how a lack of access to support and structured rehabilitation for neuropsychological changes can make problems worse and how financial changes can affect the survivor and their family. Concluding her presentation, Dr Cooper identified several things that might ameliorate some of these difficulties, including the importance of giving the person and their family information about encephalitis and its sequelae after discharge from hospital and sign-posting to relevant places of support, such as the Encephalitis Society.
Dr Ronan McGinty from the Oxford Autoimmune Neurology Group, Nuffield Department of Clinical Sciences, University of Oxford, presented research on autoantibodies in 232 adults with newly diagnosed focal epilepsy, 10 of whom had autoimmune encephalitis. Of these 10 patients, autoantibodies were detected in 7 (including antibodies to LGI1, CASPR2, GABA-a and GABA-b receptors, and the NMDA receptor), most had difficult-to-treat temporal lobe-onset seizures with inflammatory changes on MRI brain, and 4/6 treated with immunotherapy demonstrated an improvement in functional status. This study suggests that features of encephalitis could be useful clinical indicators of neuronal surface antibodies in adults with new focal-onset epilepsy and prompt early consideration of immunotherapy.
Dr Hannah Brindle of the Institute of Infection and Global Health, University of Liverpool, joined the conference remotely to present her work on Acute Encephalitis Syndrome (AES) from 1992-2015, in Vietnam. Dr Brindle informed the audience of the epidemiology of AES in Vietnam using surveillance data, with Japanese Encephalitis being the largest cause of AES in children; HSVE, dengue and enterovirus as the largest causes in adults; and roughly 50% of AES cases in Vietnam are of an unknown aetiology. Dr Brindle concluded her presentation reporting that peaks in the number of cases reported in northern Vietnam during the summer months are evidenced by a significant positive correlation between latitude and incidence of AES and interestingly that AES incidence is significantly correlated with irrigated land, supporting hypotheses that many cases of AES may be vector-borne.
Dr Julianne Brown, Clinical Scientist and Virologist at Great Ormond Street Hospital for Children, then presented “Should deep sequencing be routinely used for diagnosis of encephalitis?”. Dr Brown highlighted the pros (relative speed and sensitivity) and cons (insufficient CSF specimen for multiple tests of multiple targets) of the routine diagnostic method of encephalitis, polymerase chain reaction (PCR), contrasting this with another method, known as “deep sequencing” (DS) which uses RNAseq to identify the causes of encephalitis. Dr Brown’s recent work investigating the use of DS as an emerging application in identifying encephalitis has led to Dr Brown and her team being awarded a grant from Great Ormond Street Hospital Children’s Charity, in order to set up a new diagnostic service for encephalitis.
After a light lunch kindly sponsored by the Autoimmune Encephalitis Alliance, Dr Anusha Yeshokumar of the Icahn School of Medicine at Mount Sinai, NYC, outlined her study investigating long-term neurobehavioural impairment in patients with autoimmune encephalitis (AE). Several important conclusions were drawn from her study. While many AE patients may continue to experience poor outcomes following hospital discharge, patients with anti-NMDAR encephalitis may have better outcomes in neurologic disability and adaptive function than people affected by other forms of autoimmune encephalitis. Also, the persistent deficits experienced by patients may not be adequately captured by routine neurologic assessments alone.
Next, Maarten Titulaer, Assistant Professor at Erasmus University Medical Centre, The Netherlands, gave an elucidating keynote address titled, “Anti-GABAbr encephalitis: status epilepticus and beyond”. Dr Titulaer highlighted that over 90% of patients in his study showed behaviour and cognitive changes and a similar percentage having seizures; of which 100% were tonic-clonic seizures, with over 40% of patients developing refractory status epilepticus (necessitating ICU admission). Several important take home messages from Dr Titulaer’s keynote address were that Anti-GABAbr encephalitis may present as a rapidly progressing dementia, that MRI of the brain can be normal and that limbic encephalitis can often present with refractory seizures.
Following the keynote address, Dr James Varley of the Oxford autoimmune neurology group presented his work on contactin associated protein-2 antibody mediated neurological conditions. Dr Varley highlighted several CASPR-2 antibody associated features, the three most common of which were, cognitive symptoms, psychiatric symptoms and seizures, with cognitive changes and neuropathic pain being reported as the most troublesome during recovery. Concluding his presentation, Dr Varley highlighted a clinical pattern across cohorts in CASPR-2 mediated neurological diseases and that despite generally good recovery rates, some persistent sequelae are the norm, rather than the exception.
Dr Sophie Binks, an academic clinical fellow at the Nuffield Department of Clinical Neurosciences, Oxford, rounded off the speaker presentations for the day with her work on long-term cognitive, psychiatric and quality of life sequelae in autoimmune and infectious encephalitides. Investigating LGI1 antibody autoimmune encephalitis and infective causes of encephalitis, Dr Binks underlined some early findings from her study which corroborate with other speakers from today; changes in cognition, behaviour and mood following encephalitis can affect the person as well as the families and carers of the patient, and also that these effects can be long-lasting following discharge from hospital.
After a short break and book signings of Life after Encephalitis by Encephalitis Society CEO, Dr Ava Easton and Roald Dahl’s Marvellous Medicine by Professor Tom Solomon, a wonderful day of informative keynote addresses and presentations from leading researchers across many institutions and several countries, concluded with Phillipa Chapman of the Encephalitis Society, who presented the Society’s work this year in a video titled “Year in Review”. This really accentuated the amazing work done by the Society`s ambassadors, the families and survivors of those affected by encephalitis, and the Society itself. Recognising the role of the many representatives of the Society, Professor Barbara Wilson OBE, was presented with an award for 10 years of service as the Society’s President, with a handsome limited edition book by her favourite artist, Bob Dylan. A Lifetime Achievement Award was presented to Professor Angela Vincent for her work that has resulted in improved outcomes for patients and decreased mortality and disability following encephalitis. Humbly accepting her award Professor Vincent stated how “very lucky (she) has been to have been in a such a great team for so long”.
Conference awards were then bestowed on two worthy winners, for best poster and best speaker, from all the erudite work presented on the day. Dr Adam al Diwani was awarded the prize for best poster and Dr Julianne Brown for best oral presentation. Finally, Dr Tehmina Bharucha received the 2017 Johnny Sutton Travel Bursary.
In the final scheduled event for the day, Michael Milligan, a playwright and actor from the USA who has performed on Broadway and at the Edinburgh Fringe Festival, performed the UK premiere of his one-man play, “Side Effects”. This play had the packed house gripped throughout and enacts a burned-out, disenchanted and increasingly distrustful Doctor, navigating the layers of bureaucracy which obfuscate his passion for providing the level of care which his father delivered, and which he otherwise wishes that he could offer.
The Encephalitis Conference 2017 concluded with a cheese and wine reception which provided an additional and welcome time for the speakers and presenters of the day to discuss their projects, research and potential future collaborations together, in a wonderfully engaging, supportive and scientific milieu.