The International Child Neurology Association is pleased to announce the names of those selected to receive the ICNA 2018 Awards at the 15th International Child Neurology Congress to be held in Mumbai, India November 15-18, 2018.

Helen Cross, United Kingdom

 Frank Ford Award 2018

Professor Helen Cross is The Prince of Wales's Chair of Childhood Epilepsy and Honorary Consultant in Paediatric Neurology at UCL Institute of Child Health, Great Ormond Street Hospital for Children NHS Trust, London, and Young Epilepsy, Lingfield.
Professor Cross epitomises the spirit of the Frank Ford award. Her research has focused on improving outcomes for children with early onset epilepsy. Her early research was into improving imaging techniques to determine areas of likely seizure onset in children with drug resistant focal epilepsy and has developed an epilepsy surgery programme based on her research. Professor Cross has published widely on seizure, neuropsychological and behavioural outcomes in children who have undergone surgical resection for treatment of their epilepsy. 

She conducted the first randomized controlled trial of the ketogenic diet in the treatment of children with drug resistant focal epilepsy and is endeavouring to conduct the same in the very young and adults. Recognising there was little in the way of control data with regard to neurodevelopmental progress, she initiated the North London Epilepsy in Infancy study, where a cohort of children was recruited at diagnosis in the first two years of life, and has been followed to at least three years. She is now aiming to conduct a similar study over a wider geographical area, also examining phenotypes and genotypes.

Professor Cross is currently an ICNA Board Member, Clinical Advisor to the Children's Epilepsy Surgery Services (CESS) (2012-present), Chair of the Medicines for Children Research Network Neurosciences Clinical Study Group (2012-present), Chair of the Evidence Update of the NICE Guidelines for Epilepsy (2013) and Secretary General of the ILAE (2013-2017). She is also on the Editorial Board of Epileptic Disorders, Epilepsy Research, Developmental Medicine Child Neurology and European Journal of Paediatric Neurology. Besides her well-recognised contributions to child neurology, Professor Cross has been passionate about spreading the word of child neurology by teaching around the world including Uganda, Kenya and South Africa.
About the Frank Ford Award

Manju Kurian, United Kingdom

John Stobo Prichard Award 2018 

Dr Kurian is an outstanding early career clinician scientist, who has already made a significant contribution to the care and treatment of children with severe neurological disorders over a relatively short period of time. Her programme has led to the further understanding of several complex neurological disorders, utilising gene discovery to further understand the mechanisms underlying the disease, and subsequently exploring the possibility of therapy. She has over 70 publications including work published in Nature Genetics, Nature Communications and Lancet Neurology. 

Furthermore, she has developed strong leadership skills as an independent principal investigator, establishing links with key collaborators nationally and internationally in her field. Her initial work, as her PhD, resulted in the identification of the first parkinsonian disorder associated with mutations in the SLC6A3 dopamine transporter gene. Since then she has developed a strong expertise in genetics, and has identified a number of new genetic diseases in movement disorders, early onset epilepsies and neurodegenerative disorders. Delineation of the disease phenotypes for genotype phenotype correlation has been a considerable strength of her programme. To further progress possible therapies, she has utilised a number of techniques to elucidate disease mechanisms in these neurological disorders including cell models, neuronal cell models, induced pluripotent stem cell derived dopaminergic cell models and animal models (fly/mouse/zebrafish) to assess the effect of mutations on gene function. More recently her group has been exploring therapeutic options at a number of levels including drug screens in cell and animal models, as well as proof of concept gene therapy trials in murine models of certain diseases.

About the John Stobo Prichard Award

Pauline Samia, Kenya

Sheila Wallace Award 2018 

Dr Pauline Samia has made significant contributions to Paediatric Neurology in Africa, serving on the Paediatric Commission for the International League Against Epilepsy, and has raised public awareness of many neurological conditions in East Africa, through her interaction with the media.For a busy clinician she has an impressive publication record, with papers on epilepsy, HIV and Tuberous Sclerosis. She has written chapters for textbooks on neurology in Africa, two of which are to be published this year. She is organising the first epilepsy training course in East Africa, and leads the neurology section of the Kenya Paediatric Association.

About the Sheila Wallace Award

In a study published in Current Biology today, researchers from King's College London uncovers the precise mechanisms by which autophagy leads to cell death. The study used a mouse model for the polyglutamine disease dentatorubral-pallidoluysian atrophy (DRPLA), progressive acquirement of an ataxic phenotype linked to severe cerebellar cellular pathology, characterized by nuclear degeneration through nucleophagy-based LaminB1 degradation and excretion. 

DRPLA is caused by the expansion of a CAG stretch in the ATROPHIN-1 (ATN1) gene. Patients display ataxic and choreoathetoid symptoms as well as myoclonus, generalized epilepsy, and dementia with extensive cellular degeneration found in the basal ganglia (e.g., the globus pallidus, GP), brainstem (e.g., the red nucleus, RN), and cerebellum (primarily in the dentate nucleus, DN).

The experiments revealed that a "progressive stall" and then a "chronic block" occur in autophagy, in certain disease-specific areas of the brain. In this "persistent stall," the brain cells get "confused" and try to maintain a normal homeostasis through alternative molecular pathways, or routes. The alternative cell clearance process depletes the cell of material, damaging its nucleus and cytoplasm and leaving behind cell corpses with a fragile nucleus and thin cytoplasmic layer leading to cell atrophy and degeneration.

Citation:
Stall in Canonical Autophagy-Lysosome Pathways Prompts Nucleophagy-Based Nuclear Breakdown in NeurodegenerationBaron, Olga et al.Current Biology , Volume 0 , Issue 0 ,

http://www.cell.com/current-biology/fulltext/S0960-9822(17)31391-X

Further reading:

Ebrahimi-Fakhari D, Wahlster L, Hoffmann GF, Kölker S (2014) Emerging role of autophagy in pediatric neurodegenerative and neurometabolic diseases.Pediatr Res 75 (1-2):217-26. DOI: 10.1038/pr.2013.185 PMID: 24165736. 

 In a study published in The FASEB Journal a team of NIH-funded scientists reports on a potential drug that could be protective in ischemic stroke patients. 

The clue for the study came from observations in hibernating ground squirrels. In these animals there is a dramatic reduction of cerebral blood flow during hibernation, yet they emerge from their sleep without any ill effects at all. 

Reference:
Bernstock et al. Quantitative high-throughput screening identifies cytoprotective molecules that enhance SUMO-conjugation via the inhibition of SUMO-specific protease (SENP)2. The FASEB Journal. November 16, 2017. doi: 10.1096/fj.201700711R.

New research published in Epilepsia, a journal of the International League Against Epilepsy (ILAE), indicates that wristband devices may improve the detection and characterization of seizures in patients with epilepsy.

In their attempts to develop a better monitoring method, Giulia Regalia, PhD and Francesco Onorati, PhD, of Empatica Inc. in Milan, Italy and Cambridge, Massachusetts, and their colleagues examined the potential of automated, wearable systems to detect and characterize convulsive epileptic seizures. The researchers used three different wristbands to record two signals -- called electrodermal activity and accelerometer signals -- that usually exhibit marked changes upon the onset of convulsive seizures, obtaining 5928 hours of data from 69 patients, including 55 convulsive epileptic seizures from 22 patients.

The wristband detectors showed high sensitivity (95% of seizures were detected) while keeping the false alarm rate at a bearable level (on average, one false alarm every four days), which improves a pioneering 2012 study led by MIT professor Rosalind Picard, now chief scientist at Empatica.

In addition to detecting seizures, the method also revealed certain characteristics of the seizures, which may help alert clinicians and patients to seizures that are potentially dangerous and life-threatening. 

"Multi-center clinical assessment of improved wearable multimodal convulsive seizure detectors." Francesco Onorati, Giulia Regalia, Chiara Caborni, Matteo Migliorini, Daniel Bender, Ming-Zher Poh, Cherise Frazier, Eliana Kovitch Thropp, Elizabeth D. Mynatt, Jonathan Bidwell, Roberto Mai, W. Curt LaFrance, Jr., Andrew S. Blum, Daniel Friedman, Tobias Loddenkemper, Fatemeh Mohammadpour-Touserkani, Claus Reinsberger, Simone Tognetti, and Rosalind W. Picard. Epilepsia; Published Online: October 4, 2017 (DOI: 10.1111/epi.13899).

URL: http://doi.wiley.com/10.1111/epi.13899