Professor Leonard Van Den Berg, coordinator of the JPND-supported SOPHIA project, gave a brief insight into current progress on this biomarker project

Read more about the SOPHIA Project here

What are your key project achievements to date?

At this moment, the key project objective is to finalise development of the web-based platform including a virtual biobank to integrate the core clinical dataset from patients from all participating centres with biomarker data obtained from different biosamples. The core clinical dataset has been defined and the required fields for data collection of neuropathology biomarkers, wet biomarkers (in CSF, blood) and imaging biomarkers (MRI, MUNIX) are being set up. Standard Operating Procedures (SOPs) for sample collection and biomarker measurement have already been developed for some of the biomarkers. Other SOPs are still work in progress using the results from variability and reliability analysis on small sample datasets. The SOPs will also include a monitoring and quality control mechanism. Longitudinal data will be collected and analysed once the system is up and running. In the future new biomarkers can be quickly optimized and/or harmonized using a standard approach, if required.

Recently it became clear that the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) would be an excellent addition to the core clinical dataset that is collected from ALS patients: Collecting longitudinal reliable neuropsychology data would be useful to detect the specific profile of cognition and behaviour changes in ALS and to differentiate it from other disorders. Using the SOPHIA consortium, developers of ECAS are able to roll out and validate the screen throughout Europe within a short timeframe. The establishment of a consortium like SOPHIA has been instrumental to this effort, and will be just that for future biomarkers.

Where is the SOPHIA consortium making a real difference?

The European ALS community has established an active consortium ( which holds several 2-3 day workshops per year among others about European collaboration on databases/biobanks, molecular biomarkers, and neuroimaging, and members have generated a series of consensus statements and standard operating procedures for biomarkers. These types of initiatives are an excellent foundation for European collaboration but more focused projects and funding is required for larger international collaborations to obtain the numbers of samples necessary to perform large biomarker validation studies in ALS and other motor neuron diseases. This is where the SOPHIA consortium is making the difference, as the results of its works will be a common European strategy for the prioritization and selection of candidate biomarker domains for optimization and harmonization and a permanent interactive European ALS biomarker tool for all researchers to enable optimization/harmonization of novel biomarkers using an integrated and robust pan-European ALS methodology.

In your opinion, what is the added-value of JPND support for this project?

The benefit of JPND support for the SOPHIA project lies in communication of project results to the wider ND disease society, which is essential for sharing all methods, database, biomarker essays, and biomarkers in SOPHIA with other members of the scientific community devoted to ALS and to other neurodegenerative diseases. The concept of this project (pan-European SOPs) can be expanded to other neurodegenerative disease areas resulting in a driving force for shared biomarker research.

What has been your experience of this research collaboration to date?

One year into the project it is very clear that this is a group of highly motivated investigators, keen to set up a platform to promote biomarker research for neurodegenerative diseases. All involved feel this can only be truly accomplished at a European level and are therefore eager to collaborate. Each partner in the project is actively involving his/her colleagues in order to make sure they have the right people working on each of the different sub-projects. This has been very helpful in progressing the tasks that the consortium has set itself and will result in deliverables of high standard.

The SOPHIA project website is available at

September 17, 2013