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No new research programmes were initiated in 2010, but expenditure continued on the national surveillance programme and the SCT Ph.D. Studentship. Total expenditure on research and medical studies, including equipment, since the trust was formed will have been more than a quarter of a million pounds. Developments in 2010 are summarized below.

“Encephalitis Lethargica: During and After the Epidemic”

Prof. Joel Vilensky’s book, dedicated to Sophie and with a Prologue by SCT, as well as a preface by Dr Oliver Sacks, was published by Oxford University Press in New York at the end of 2010.

EL Surveillance Programme

The EL surveillance program, led by Prof. Gavin Giovannoni, has now been in place for nearly 3 years. Initially, it was expected that the incidence would be about 20 cases per year, but as mentioned in the previous research update, a number of the initially reported cases turned out not to be EL. Up to October 2010, only 13 cases of EL have been confirmed, as reported by David Holden. One of Gavin’s EL patients has started a Facebook site called Encephalitis Forum; this is a self-help site, which should be very helpful and informative for encephalitis patients, not limited to EL patients.

SCT Ph.D. Studentship

Our Ph.D. student, Priya Dua, is in her final year. She gave a talk about her research at the previous AGM. The main focus of Priya’s research is to establish a working model to study EL and related post-streptococcal movement disorders, and particularly to establish the role of streptococcal infections and the functional significance of the anti-neuronal antibodies studied in work that we previously funded at the Institute of Neurology. She has written a summary report on her progress to date.

Richard Steele
April 2011


Since its formation, the trust has spent or agreed to spend about £215,000 to promote research and other activities related to encephalitis lethargica (EL); details are shown here. I am pleased to say that 2008 saw tremendous progress, and it is not too much to say that it represents a turning point towards achieving some of SCT’s main educational and medical aims, This has been consolidated in 2009. In summary, the major developments in projects that we are funding are as follows.

Firstly, the start of the national surveillance programme led by our Scientific Adviser, Professor Gavin Giovannoni, means that every neurologist in the UK is now being asked regularly about EL in the British Neurological Surveillance Unit’s monthly questionnaire. This is a major step towards our aim of increasing awareness of EL among the medical profession. Over the next few years, the surveillance program will provide detailed information about the current incidence of EL in the UK, and collect samples that will be useful in future research. In addition, every patient identified during this programme will receive a letter from the Sophie Cameron Trust about the information and support we can offer. As described in the “Latest News” section, entry of patients into the database is under way

Secondly, Oxford University Press, a major academic publisher, has signed a contract to publish a textbook on EL by Professor Vilensky. This book, which is expected to be called "Encephalitis Lethargica: During and After the Epidemic", should appear in early 2011, and will further increase awareness of EL among the medical profession in the UK and internationally. It will be a convenient source of information for the general public too; no comprehensive book about medical aspects of EL has been available for decades. The trust will contribute a foreword for the book, as an acknowledgement of our financial support for its preparation. Professor Vilensky has also kindly agreed that the book will be dedicated to the memory of Sophie Cameron. The final manuscript is expected to be sent to Oxford University Press by April 2010

Thirdly, we are funding a 3-year Ph.D. Studentship, as well as other research on the causes of EL, at Queen Mary University London. There is a lot of interest internationally among medical researchers about anti-basal ganglia antibodies (ABGA), which may be related to a wide range of diseases, including Sydenham’s chorea, PANDAS, Tourette’s syndrome, obsessive compulsive disorder and EL. Our funding will help to ensure that the possible link to EL will be one of the main focuses of AGBA research in the UK. In particular, the Ph.D. student will be trying to develop a model of EL – if successful, this would be useful for understanding the mechanism of the disease, and for testing possible treatments. The Studentship has been awarded to Priyamvada Dua, who gave a talk about the initial results of her research at the 2009 Sophie Cameron Trust AGM.

Finally, the website has been greatly expanded and updated in 2008. It now includes much more information about the history and symptoms of EL. Professor Vilensky kindly provided an abbreviated version of his lecture in London and other material, Professor Giovannoni and David Holden have written detailed reports on the surveillance program and their other research activities, and Royce Menezes has allowed us to include his results on physiotherapy. The site now includes a search facility. In addition, site statistics are available on a password-protected page. In 2009, there were between four and five thousand unique visitors to the site from more than 60 countries.

More details of progress in 2008/2009 are given below.

National surveillance programme for encephalitis lethargica in the UK

The UK national surveillance programme for EL, headed by Professor Gavin Giovannoni and partly funded by the Trust, is now well under way. The paediatric surveillance programme commenced in December 2007, and is being conducted through the British Pediatric Neurological Surveillance Unit (BPNSU). The adult surveillance programme was delayed pending resolution of a query sent by the British Neurological Surveillance Unit (BNSU), but commenced in April 2008.

The EL database is being maintained at Queen Mary University London on a secure University server. As of October 2008, 15 paediatric EL cases have been notified through the BPNSU scheme and confirmed through the surveillance programme – a feature of both the BPNSU and BNSU schemes is that a number of erroneous cases are initially reported. Four adult EL cases have been notified via the BNSU scheme, one of which has been confirmed to date. The current notification rate is approaching the expected 20 cases per year; however, this is likely to include a backlog of cases from 2007 and previous years. A letter of support from the Sophie Cameron Trust has been included with the information sent to patients identified by the programme.

Conference at Bari – April 2008

Mr David Holden and Professor Gavin Giovannoni attended a symposium on the Role of Autoimmunity in Tourette’s Syndrome and Related Disorders, as part of the Italian Movement Disorders National Congress, in Bari April 4-5 2008. David Holden presented a poster entitled “Encephalitis lethargica: surveillance and immunological studies”. This described the EL surveillance programme, and related investigations into the immunological basis of this disease, both of which are supported by funding from the Trust. The presentation of an EL poster at this meeting had the effect of raising awareness of the disease, and placing it in the context of related neuropsychiatric disorders. A flyer describing the surveillance project was distributed at the meeting.

Sophie Cameron Trust Ph.D. Studentship

The Trust is funding a 3-year Ph.D. studentship at Queen Mary University London (QMUL), supervised by Professors Giovanonni and Baker at the Institute of Cell and Molecular Science. This grant, called The Sophie Cameron Trust Ph.D. Studentship, will allow a research student to work full-time on a project entitled “Functional effects of anti-basal ganglia antibodies in patients with encephalitis lethargica and related disorders associated with streptococcal infection”. The research will focus on the hypothesis that autoimmune mechanisms play an important role in EL, as they are thought to do in other neuropsychiatric disorders, including Sydenham’s chorea, PANDAS, Tourette’s syndrome and obsessive compulsive disorder. The SCT PhD Studentship has been awarded to Ms Priya Dua, who recently received an M.Sc. in integrative neuroscience from Imperial College, London. She will try to set up a model of encephalitis lethargica based on the streptococcal hypothesis. Ms. Dua spoke about her research at the 2009 Sophie Cameron Trust AGM.

Photo: Priya with some of SCT’s trustees following her presentation. Left to right: Sue Lucas, Dr Bernard Whiteside, Sandy Joakim, Dr Richard Wharton,
Priyamvada Dua, Richard Steele, Michael Robson.

Research on the targets of anti-basal ganglia antibodies

The Neuroimmunology Unit at ICMS-QMUL is investigating the molecules in the brain that are targeted by anti-basal ganglia antibodies (ABGA), and trying to establish their functional significance in causing disease. More biological samples for testing will be obtained via the EL surveillance programme. The SCT grant has so far contributed to supporting Joseph Cohen, an MBBCh-BSc student, who did his laboratory project on this topic, and has written a report of his results, and Kathrin Hoffman, a German MSc student, who has been taken on for a further 8 months to extend Joseph’s work.

New textbook on encephalitis lethargica

The Trust has funded an editorial assistant and other expenses (including translation) to help with the preparation of a new textbook on EL, which will be edited by Professor Vilensky, and will include contributions from eminent physicians and scientists, including Professor Giovannoni, our scientific adviser. This book, “Encephalitis Lethargica: During and After the Epidemic" is to be published by Oxford University Press, probably in early 2011. It will be a detailed, up-to-date examination of the clinical and pathological features of EL, based on a careful analysis of the thousands of scientific reports and books published in the pandemic period, as well as the considerable number published more recently. This will be the first comprehensive textbook on EL for many years, and we hope that it will become the definitive work on EL. The book will be dedicated to Sophie Cameron, and will have a foreword written by Richard Steele on behalf of the trust. Judith Cameron has helped with editing some translated case reports, and will be acknowledged too.

Seminar about encephalitis lethargica by Professor Vilensky

On 17th June 2008, Richard Steele and Richard Wharton, on behalf of the Trust, visited the Institute of Neurology in Queen Square, London. The Trust had invited Professor Joel Vilensky of Indiana State University, USA to London to speak about EL. Professor Vilensky has developed an encyclopaedic knowledge of the history and current state of EL, and has unique access to records, documents and film footage from the epidemic in the 1920s. He gave a seminar entitled “Sleeping Princes and Princesses: The Encephalitis Lethargica Epidemic of the 1920s and a Contemporary Evaluation of the Disease” at the Wolfson Lecture Theatre, National Hospital for Neurology and Neurosurgery, Queen Square, and this was attended by about 80 people including a number of eminent neurologists. It was co-chaired by Professors Andrew Lees and Roger Lemon. Prof. Lees was a co-author of an important paper on the diagnostic criteria for EL in the 1980s. Prof. Vilensky covered a huge amount in an hour, expounding the history, showing film of cases, and summarising the present status of cases and research. An abbreviated version of his presentation is available here.

Meeting at the Institute of Neurology following Dr Joel Vilensky’s talk at the Wolfson Lecture Theatre, National Hospital for Neurology and Neurosurgery, Queen Square, London. Left to right: Richard Steele, Dr Anette Schrag, Professor Joel Vilensky, Professor Gavin Giovannoni and David Holden. Photo courtesy of Dr Richard Wharton.

Richard Steele – November 2009



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