Emeritus Professor, Faculty of Brain Sciences, University College London

Visiting Professor, Dept of Experimental Psychology, Oxford.

Fellow of the British Academy, the Academy of Medical Sciences and the Academia Europaea.  

Jan Atkinson has co-directed the Visual Development Unit, first in Cambridge and then in University College London and Oxford. Her extensive research has covered many different areas of vision, visual brain development and visually controlled behaviour. Her work includes studies of typical visual and cognitive development in infants and children, common visual disorders such as strabismus, amblyopia and cerebral visual impairment, visual, visuomotor and visuocognitive development in infants and children, many with developmental disorders including Williams Syndrome (WS).  She has investigated the brain networks underpinning both typical and atypical visual development, directed infant vision screening programmes of total populations and developed new techniques for measuring paediatric visual difficulties. She has devised vision tests (ophthalmological and paediatric) for assessing and diagnosing vision and attentional problems not only in WS individuals but also those with premature birth histories, brain injury around birth related to cerebral palsy and Down’s Syndrome. She has summarized much of her research and assessment in her book ‘The Developing Visual Brain’ (Oxford University Press).  A recent overview of her work is in her Teller Award lecture at  https://jov.arvojournals.org/article.aspx?articleid=2613613&resultClick=1

 With the help of the Williams Syndrome Foundation, her group has carried out research assessments on over 200 WS children since the early 1990’s. 

A fuller biography and complete list of Janette’s scientific publications is at https://iris.ucl.ac.uk/iris/browse/profile?upi=JATKI15.  Please e-mail her if you would like copies of any of the paperslisted below She also has a Wikipedia entry at https://en.wikipedia.org/wiki/Janette_Atkinson

Highlights of findings on studies of Williams Syndrome from her team, the Visual Development Unit include [with references to original research articles  below]

• WS people have a specific, lasting problem with the “dorsal-stream” brain system that processes visual and spatial information for visuomotor actions [1,4, 5, 7]. She has called this ‘dorsal stream vulnerability’. Examples of common WS spatial problems are in many everyday tasks and actions that require planning and visuomotor coordination, such as handwriting, dressing by putting your clothes on in the correct order, putting together a lego model, reaching out for a jug and pouring a glass of water, finding your way around and remembering a route, getting on and off escalators, standing on a moving bus, judging the speed of oncoming traffic when trying to cross the road, climbing on a climbing frame or jumping and balancing on a trampoline,  or riding a bike.
• WS individuals are more prone to visual problems such as squint (called ‘strabismus’ by medical professionals or ‘lazy eye’) and short or long- sight (called ‘refractive errors’ and including ‘astigmatism’) which may require glasses, but poor vision is not the main source of visuo-motor problems  and  spatial difficulties [3]
• They also have problems with the frontal brain systems that regulate behaviour, but these show up most strongly with control of spatially directed actions [4, 7]
• WS people have specific difficulties with some aspects of attention, but on some tasks which they understand and like, they can focus their attention and maintain attention to the level which is in line with their general intellectual abilities [10, 12]
• They often have difficulties with walking up and down stairs as they do not anticipate how to place their feet when stepping down. [9, 11]
• They use different systems from other children in remembering where an object is hidden and are easily disrupted by a new viewpoint [6,11]
• There are some differences in early brain development to typical brain development in toddlers with WS which we have  found using brain imaging methods (MRI)  (2). This may be related to the basis of some of the common ‘dorsal stream’ problems in Williams syndrome.   

So WS people need special strategies to cope with their difficulties with spatial information, attention, and controlling actions. Sometimes these difficulties lead to anxiety in particular situations. However, with practice and special strategies, many individuals with Williams syndrome can overcome or reduce their difficulties. There are always exceptions – one WS child tested in the VDU is a brilliant skier and another is a very proficient ballet dancer. However, many WS children seem to prefer musical activities, drama, computer games or collecting things, rather than sports activities and that is fine – we wouldn’t all want to be a ‘Lionel Messi’!!

At present Jan Atkinson , Oliver Braddick  (husband)  and their collaborators are testing an app for use on an iPad which has been devised by them  in the Visual Development Unit to measure different aspects of visual and auditory attention. It is called the ‘ECAB’, Early Child Attention Battery, and consists of 4-8 subtests, each lasting a few minutes, some for visual attention and some for auditory attention.   An example of these short tests requires the child to spot certain objects in a visual display, such as finding the red apples in amongst red strawberries and white apples (selective attention),  or spotting an animal each time it appears on the screen and carrying on looking for animals for 3- 5 minutes (sustained attention). We have started using these attention tests not only with WS children but also children who have had brain damage at birth and children born very prematurely. We plan to extend the WS group we are testing with the ECAB over the next few years.

As always, the main aim of our work is to not only identify specific problems but to devise methods and strategies so that WS individuals can start to reduce their problems and enjoy life to the full.

 

Some scientific publications from Jan Atkinson’s team on Williams Syndrome [web links are to abstracts available on PubMed]

[1] J Atkinson, J King, O Braddick, L Nokes, S Anker, F Braddick. “A specific deficit of dorsal stream function in Williams’ syndrome”. NeuroReport  8, 1919-1922 (1997)  http://www.ncbi.nlm.nih.gov/pubmed/9223077

[2] E Mercuri, J Atkinson, O Braddick, M Rutherford, F Cowan, S Counsell, L Dubowitz, G Bydder. “Chiari I malformation and white matter changes in asymptomatic young children with Williams syndrome: clinical and MRI study” .European Journal of Paediatric Neurology  5/6 177-181 (1997) http://www.ncbi.nlm.nih.gov/pubmed/10728215 

[3]  J Atkinson, S Anker, O Braddick, L Nokes, A Mason, F Braddick.  “Visual and visuo-spatial development in young Williams Syndrome children”. Developmental Medicine and Child Neurology, 43: 330-337 (2001).  http://www.ncbi.nlm.nih.gov/pubmed/11368486

[4] J Atkinson, O Braddick, S Anker, W Curran, R Andrew (2003). “Neurobiological models of visuo-spatial cognition in young Williams Syndrome children: Measures of dorsal-stream and frontal function”. Developmental Neuropsychology, 23: 141-174.  http://www.ncbi.nlm.nih.gov/pubmed/12730023

[5] Atkinson, J., Braddick, O., Rose, F. E., Searcy, Y. M., Wattam-Bell, J., & Bellugi, U. (2006).  Dorsal-stream motion processing deficits persist into adulthood in Williams Syndrome.  Neuropsychologia, 44, 828-833.  http://www.ncbi.nlm.nih.gov/pubmed/16168445

[6] M Nardini, J Atkinson, O Braddick, N Burgess (2008). Developmental trajectories for spatial frames of reference in Williams syndrome. Developmental Science 11(4): 583-595.  http://www.ncbi.nlm.nih.gov/pubmed/18576966

[7]  Atkinson J, Braddick O (2011) From genes to brain development to phenotypic behavior: “dorsal-stream vulnerability” in relation to spatial cognition, attention, and planning of actions in Williams syndrome (WS) and other developmental disorders.  Progress in Brain Research. 189: 261-83.  http://www.ncbi.nlm.nih.gov/pubmed/21489394

[8]  Atkinson J, Braddick O (2011). Linked brain development for vision, visual attention and visual cognition in typical development and developmental disorders.  Ch.19, pp.247-270 in Riva D, C. Njiokiktjien C, Bulgheroni S (eds.) Brain Lesion Localization and Developmental Function: Frontal Lobes, Limbic System, Visuo-Cognitive System.  Mariani Foundation Paediatric Neurology Series 26, John Libbey Eurotext Ltd. 

[9]  Cowie D, Braddick O, Atkinson J. (2012)   Visually guided step descent in children with Williams syndrome.  Developmental Science, 15: 74-86.  http://www.ncbi.nlm.nih.gov/pubmed/22251294

[10]  Atkinson J, Braddick O. (2012) Visual attention in the first years: typical development and  developmental disorders.  Devel Med Child Neurol 54: 589–595  http://www.ncbi.nlm.nih.gov/pubmed/22568833

[11] Atkinson J, Braddick O. Spatial cognition, visuomotor action and attention. pp 249-262   In: Farran E, Karmiloff-Smith A, eds. Neurodevelopmental Disorders across the Lifespan.. Oxford University Press, 2012.

[12] Breckenridge K, Braddick O, Anker  S, Woodhouse M, Atkinson J (2013)  Attention in Williams syndrome and Down’s syndrome:  Performance on the new Early Childhood Attention Battery (ECAB).  British Journal of Developmental Psychology, 31: 257-269.   http://www.ncbi.nlm.nih.gov/pubmed/23659894

[13] Atkinson J (2017) Visual Brain Development: A review of “Dorsal Stream Vulnerability”—motion, mathematics, amblyopia, actions, and attention.  Journal of Vision,17:26. doi:10.1167/17.3.26