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Professor Hermann Doose, whose name is entwined with Myoclonic Astatic Epilepsy otherwise known as “Doose Syndrome” passed away on April 23rd, 2018 following a brief illness. He was 90yrs old. Professor Doose was a founding member of the neuropediatric society in Germany.Hermann Doose was born on September 1927 in Lübeck, North Germany. His father was a surgeon and mother a gynaecologist. At the age of 16 he was recruited to the army during the last year of the war. A subsequent injury and admission to hospital resulted in him being the lone survivor of the group.
Doose studied medicine at the Christian-Albrechts-University Kiel and the Albert-Ludwigs-University Freiburg where he studied physiology and pathology and then completed a training in pediatrics at the University of Kiel. From 1975 to 1992, Doose was director of the Clinic for Neuropediatrics in Kiel and at the same time the medical director of the North German Epilepsy Center for Children and Adolescents in Raisdorf near Kiel, which was opened on his initiative in 1972. There he headed an epilepsy research center since 1992.
The first EEG laboratory in Kiel was founded by him and his 1963 professorial thesis was on the “Spectrum of Petit-Mal Epilepsies in Childhood”. Professor Doose was head of the Department of Paediatric Neurology in Kiel, form 1975 until his retirement in 1992. In 1972 he founded the North German Epilepsy Centre. He was a physician of great empathy and until the last weeks before his death he continuously received letters and phone calls from his patients. He was a great teacher and had inspired many young doctors to pursue paediatric neurology and epileptology.
During his career he authored many papers, books and book chapters.He described for the first time several epilepsy forms of childhood and adolescence including juvenile absence epilepsy (1965), myoclonic-astatic epilepsy or Doose syndrome (1970), infantile absence epilepsy (1994) and infantile Grand-Mal epilepsy (1998) now known as Dravet syndrome.
His 1967 first self-published textbook epilepsy in childhood and adolescence was long considered a standard work in German-speaking countries. It was last reissued in 1998 and is continued since 2012 by Bernd Neubauer and Andreas Hahn under the title Doose epilepsy in childhood and adolescence which is now in its 13th edition. In 1973 he co-authored an epilepsy memorandum of the Deutsche Forschungsgemeinschaft and in the 1970s co-published several congressional and conference reports. Following the co-edition of a book on the genetics of epilepsy he worked on an EEG textbook for children and adolescents including an English language edition which was his last work.
Professor Doose was President of the German chapter of the ILAE and the Society of Neuropediatrics and in his career won several prizes including the Michael prize in 1963, the Hans Berger Prize of the German EEG Society (since 1996: German Society for Clinical Neurophysiology and Functional Imaging, DGKN) in 1985, Honorary Membership German Society for Epileptology (DGfE) (1994) and the Otfrid Foerster Medal German Society for Epileptology (DGfE) in 2004. The ILAE named him the “Ambassador for Epilepsy” in 1975 and in 1974 he founded the charity “Hilfe für das anfallskranke Kind [Help for the sick child]” to raise funds for research on childhood epilepsies.
He will be remembered as a great scientist, physician and teacher by his colleagues, patients and friends.
Source: STIFTUNG MICHAEL ; ILAE
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In a new study published in The New England Journal of Medicine the researchers, comparing two doses of Cannabidiol (CBD) to a placebo, reported a 41.9 percent reduction in "drop seizures" in patients taking a 20 mg/kg/d Cannabidiol (CBD) regimen, a 37.2 percent reduction in those on a 10 mg/kg/d Cannabidiol (CBD) regimen, and a 17.2 percent reduction in a group given a placebo.
The phase III trial was led by principal investigator and study first co-author Orrin Devinsky, MD, a professor of neurology, neurosurgery, and psychiatry at NYU School of Medicine and director of NYU Langone's Comprehensive Epilepsy Center.
The study included an investigational liquid, oral formulation of CBD called Epidiolex. The product is manufactured by GW Pharmaceuticals, which operates in the U.S. as Greenwich Biosciences; GW Pharmaceuticals funded the clinical trial.
The investigators enrolled 225 patients (age 2 to 55) with Lennox-Gastaut syndrome across 30 international sites in a randomized, double-blind, placebo-controlled trial to assess the efficacy and safety of two doses of CBD: Seventy-six patients received 20 mg/kg/d CBD, 73 received 10 mg/kg/d CBD, and 76 were given a placebo. All medications were divided into two doses per day for 14 weeks. The number of seizures were monitored beginning four weeks prior to the study for baseline assessment, then tracked throughout the 14-week study period and afterwards for a four-week safety check.
Side effects occurred in 94 per of patients in the 20 mg CBD group, 84 percent in the 10 mg CBD group, and 72 percent of those taking placebo. Side effects were generally reported as mild or moderate in severity and those that occurred in more than 10 percent of patients included: sleepiness, decreased appetite, diarrhea, upper respiratory infection, fever, vomiting, nasopharyngitis, and status epilepticus. Fourteen patients taking CBD experienced dose-related, elevated liver enzymes that were reversible. Seven participants from the CBD group withdrew from the trial due to side effects compared to one participant in the placebo group.
A previous study led by Dr. Devinsky published in last May's New England Journal of Medicine had shown a 39 percent drop in seizure frequency in patients with Dravet syndrome. Those findings represented the first large-scale, randomized clinical trial for the compound. Open label CBD studies led by Dr. Devinsky also have shown positive results for treatment-resistant epilepsies.
In April, a U.S. Food and Drug Administration advisory panel unanimously voted to recommend approval of a new drug application for Epidiolex cannabidiol oral solution. The FDA will decide whether to approve the medication in late June.
However Dr Devinsky points out that more research remains imperative to better determine the effects of CBD and other similar cannabis-derived compounds on other forms of the disease and in more dosing regimens.
Citation: Devinsky O, Patel AD, Cross JH, Villanueva V, Wirrell EC, Privitera M et al. (2018) Effect of Cannabidiol on Drop Seizures in the Lennox-Gastaut Syndrome. N Engl J Med 378 (20):1888-1897. DOI: 10.1056/NEJMoa1714631 PMID: 29768152.
Dr. Devinsky received research funding from GW Pharmaceuticals, but no salary support outside of a one-time consulting fee for participating in the FDA advisory panel. He holds equity interests in Pairnomix, Receptor Life Sciences Rx, Tilray, Rettco and Tevard.
Source: NYU Langone Health / NYU School of Medicine Press Release
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The congress offers a comprehensive discipline by way of the various types of sessions and diverse range of topics. The education and scientific program of the congress will highlight state-of-the-art clinical practice, recent advances, uptodate research and workshops on key topics for the practising neurologist.
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Voting for the ICNA Secretary and Treasurer positions and to the Executive Board was held from 03 April 2018 to 03 May 2018 2400 GMT. All eligible ICNA members were provided with a security code following validation of which, they were able to cast their votes on an online ballot. All active members of the ICNA were eligible to vote in this election. Associate and Junior(trainee) members were not eligible to vote according to the ICNA constitution and bye laws.
Members were requested to cast their votes for the following positions in the International Child Neurology Association:
Secretary (Select one candidate)
Treasurer (Select one candidate)
Executive Board (Select at least two members from each region, up to 21 total across all regions).
Candidates
SECRETARYDr Elaine C Wirrell, United States
Prof Pratibha Dutta Singhi, India
Dr Hans Hartmann, Germany
TREASURER
Dr Lieven Lagae, Belgium
Dr Michael Shevell, Canada
Africa
Dr Richard Iwa Idro, Uganda
Dr Edward Nkingwa Kija, Tanzania
Prof Ahmed Raouf Ibrahim, Egypt
Dr Pauline W. Samia, Kenya
Prof Chahnez Charfi Triki, Tunisia
East Asia - Oceania
Dr Russell Dale, Australia
Dr Yu-Wu Jiang, China
Dr Mitsuhiro Kato, Japan
Dr Lakshmi Nagarajan, Australia
Dr Takao Takahashi, Japan
West Asia
Dr Anaita Hegde, India
Prof Pratibha Dutta Singhi, India
Dr Viraj V Sanghi, India
Prof Haluk Topaloğlu, Turkey
Europe
Dr Thaís Armangue, Spain
Dr Jaime Campistol, Spain
Prof Judith Helen Cross, United Kingdom
Dr Linda De Meirleir, Belgium
Dr Bernard Dan, Belgium
Dr Antigone S Papavasiliou, Greece
Dr Michèl Willemsen, The Netherlands
North America
Dr David Bearden, United States
Dr Adam Kirton, Canada
Dr Michael V Johnston, United States
Dr Kenneth Mack, United States
Dr Elaine C Wirrell, United States
Dr Jonathan Mink, United States
Dr Sakkubai Naidu, United States
Dr Karen L Skjei, United States
Dr Jorge A Vidaurre, United States
Dr Ganeshwaran Mochida, United States
Dr Mahendranath Moharir, Canada
Central-South America
Dr Jaime Carrizosa Moog, Columbia
Dr Ana Carolina Coan, Brazil
Dr Sergio Rosemberg, Brazil
Dr Silvia Tenembaum, Argentina
Dr Kette D Valente, Brazil
Dr Vanessa van der Linden, Brazil
Regulations:
Only active members of ICNA were eligible to vote in this election. Trainee and Associate members were not eligible
Every ballot was confidential
Members were requested to select one candidate each for Secretary and Officer positions or choose to abstain
Members were requested to select AT LEAST two candidates from each of the six regions for the Executive Board.
Members were able to select more than two but the total number across all regions should not have exceeded Twenty One. In the event of the total number of candidates exceeding 21, the ballot was considered spoilt and void.
Polls closed at 2400 GMT on 3 May 2018.
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Natalio Fejerman was born in Buenos Aires on November 8th, 1933. He earned his medical degree from Medical School in Buenos Aires in 1957 and started his professional training at the Children's Hospital Ricardo Gutierrez in Buenos Aires and later at Boston Children's Hospital under Prof. Cesare Lombroso. He was the head of the Department of Neurology at the General Children's Hospital Pedro de Elizalde in Buenos Aires form 1980 to 1987 and from 1987 until his retirement at the National Pediatric Hospital Garrahan, also in Buenos Aires. He was a pioneer in pediatric neurology in Argentina and Latin America, introducing different new as well as older, forgotten, treatments, such as the ketogenic diet. His extraordinary knowledge on childhood epilepsy was of importance not only for Latin America but for the world.
Both nationally and internationally, Dr. Fejerman contributed to organizational work. Among other positions, he was the president of the Argentine League against Epilepsy for two terms and vice-president of the International Child Neurology Association (ICNA) for three terms. He was also elected to various committees of the International League Against Epilepsy (ILAE), such as the Committee on Classification of Epilepsy. In 1995, in recognition of his work, he received the Ambassador Award for Epilepsy, which is conferred together by the ILAE and the International Bureau for Epilepsy for outstanding activities in the field of epilepsy. He was on the ILAE Executive Committee for 8 years, serving two terms, as vice-president 1997-2001 and as secretary general 2001-2005. In 1992 he organized the 6th International ICNA Congress and in 2001 the 24th International Epilepsy Congress in Buenos Aires.
He published more than 150 scientific papers in international journals, contributed many chapters to international textbooks on epilepsy, and published many books on epilepsy and child neurology. His book on pediatric neurology is still the main textbook used in Argentina and in other Spanish-speaking countries.
Lastly, and maybe most importantly, Natalio was an extraordinary mentor. He taught generations of Argentine neurologists in child neurology as well as epilepsy. Over the last years, coming to our grand rounds, he continued to inspire even the youngest of us. He will be deeply missed by his many trainees, colleagues and friends.
Lastly, and maybe most importantly, Natalio was an extraordinary mentor. He taught generations of Argentine neurologists in child neurology as well as epilepsy. Over the last years, coming to our grand rounds, he continued to inspire even the youngest of us. He will be deeply missed by his many trainees, colleagues and friends.
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