Jury report for Prof. M.S. (Marjo) van der Knaap

Professor of Paediatric Neurology, VU University Amsterdam

Professor Van der Knaap receives the NWO/Spinoza Prize 2008 for her research into serious brain disorders in children. She diagnosed various new brain disorders using MRI and unravelled these down to the level of the responsible genes.

Marjo van der Knaap (9 May 1958, Delft) obtained a cum laude degree in medicine from the Erasmus University Rotterdam in 1984. In 1991, she obtained a cum laude PhD at the Department of Paediatric Neurology at Utrecht University in close collaboration with the Department of Neuroradiology at VU University in Amsterdam, where she has been professor of paediatric neurology since 1999. She has received a multitude of scientific prizes and grants. For example, she has twice received a TOP grant from Netherlands Organisation for Health Research and Development (ZonMW), and recently received the Sidney Carter Award of the American Academy of Neurology. She has been a member of the Royal Netherlands Academy of Arts and Sciences (KNAW) since 2006.

Van der Knaap investigates abnormalities of the white matter of the brain. In the middle of the brain there are billions of nerve fibres, which connect the grey cerebral cortex to the rest of the body. These fibres are surrounded by myelin sheaths for insulation. The nerve fibres and the myelin together form the white matter. White matter disorders affect one in every thousand children in the Western world. There are many different white matter disorders and children who suffer from these mostly die at a young age following a period with severe mental and motor disabilities.

During her PhD studies, Van der Knaap discovered that patients with the same white matter disorder have similar abnormalities on MRI scans. She subsequently developed a computer-guided pattern recognition system that made the diagnosis of known diseases both quicker and cheaper.

Subsequently, she committed herself to as yet unknown disorders, as her research had revealed that in more than half of the children with a white matter disorder no specific diagnosis could be established. She identified five new diseases, one of which was even named Van der Knaap disease by her peers, and then focussed on discovering their causes. With her staff she managed to identify many of the genes responsible for these diseases, which made prenatal diagnostics possible.

Her ultimate goal is to find effective treatment for children with white matter disorders. With this in mind she breaks through the boundaries of her discipline to follow a disease from its cause to its treatment. She has a talent for building highly successful strategic networks and is a truly multidisciplinary researcher, integrating the disciplines of radiology, neurology and genetics in her own research. Moreover, she combines her research interests with teaching commitments and clinical practice. By doing this she covers the entire spectrum from personal contact with the family of the young patients to identifying new variants of the disease.

Marjo van der Knaap is an internationally renowned authority. As one of the referees wrote: ‘From the Middle East to China, everyone knows where to find her.' Each year, she receives more than 600 MRI scans from foreign medics for assessment. The textbook that she – with one other author – has written about white matter disorders is an internationally used standard work.

Parents of patients praise the empathic but clear manner in which Van der Knaap describes the future perspectives for their children. Her didactic qualities are considerable. She is a source of inspiration for students in both the Netherlands and further afield. The Spinoza Committee expects that she will make further major discoveries, partly because the techniques she uses are being developed further.

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This jury report served as the basis for the speech given by Prof. Didi Braat at the announcement ceremony for the NWO/Spinoza Prizes 2008 on 2 June 2008.