Improve knowledge of behavioural symptoms of HD
- Review and develop measures to assess behavioural symptoms of HD (e.g. the Problem Behaviours Assessment – short version (PBA-s))
- Identify gaps in assessment of HD behavioural symptoms (eg perseveration) and longitudinal change, relationship with imaging, cognitive symptoms etc.
- Provide training to the HD research community on the assessment of behavioural symptoms and the administration of research measures such as the PBA-s.
- Develop a system of annual certification for PBA-s raters in order to ensure consistent data quality and enhance the confidence of behavioural interviewers (e.g. in Enroll-HD).
Behavioural data analyses
- Use data from observational studies and clinical trials to further validate the psychometric properties of behavioural assessment tools such as the PBA-s, for use in HD, and publish the results of such analyses.
- Carry out and publish data mining projects using Registry/Enroll data to enhance understanding of how behavioural symptoms develop and affect HD patients and families.
Development of guidelines
- Develop symptomatic treatment guidelines (in collaboration with our colleagues from the Behavioural Working Group of the Huntington Study Group) with a view to providing practical guidance for non-specialists in the clinical care of HD behavioural symptoms.
- Collaborate with other EHDN Working Groups (e.g. Symptomatic Treatment, Genetic Modifiers) to develop and conduct research projects which further elucidate the behavioural phenotype of HD.
- Collaborate with other HD research networks globally (e.g. HSG Behavioural WG)
- Provide a platform for researchers and clinicians to share knowledge; to allow early-career researchers access to expertise in HD behavioural symptoms.
The WG welcomes contact from EHDN members and associate members.
- Callaghan J, Stopford C, Arran N, Boisse MF, Coleman A, Santos RD, Dumas EM, Hart EP, Justo D, Owen G, Read J, Say MJ, Durr A, Leavitt BR, Roos RA, Tabrizi SJ, Bachoud-Levi AC, Bourdet C, van Duijn E, Craufurd D. Reliability and Factor Structure of the Short Problem Behaviors Assessment for Huntington’s Disease (PBA-s) in the TRACK-HD and REGISTRY studies. J Neuropsychiatry Clin Neurosci. 2015 Winter; 27(1): 59-64. PMID: 25716488
- van Duijn E, Craufurd D, Hubers AA, Giltay EJ, Bonelli R, Rickards H, Anderson KE, van Walsem MR, van der Mast RC, Orth M, Landwehrmeyer GB; the European Huntington’s Disease Network Behavioural Phenotype Working Group. Neuropsychiatric symptoms in a European Huntington’s disease cohort (REGISTRY). Journal of Neurology, Neurosurgery & Psychiatry 2014 Dec; 85(12): 1411-8). PMID: 24828898
- Groves M, van Duijn E, Anderson K, Craufurd D, Edmondson MC, Goodman N, van Kammen DP, Goodman L. An international survey-based algorithm for the pharmacologic treatment of Irritability in Huntington’s disease. PLoS Currents Huntington Disease 2011 Sep 2. Edition 1. doi: 10.1371/currents.RRN1259. PMID: 21975525
- Anderson K, Craufurd D, Edmondson MC, Goodman N, Groves M, van Duijn E, van Kammen DP, Goodman L. An international survey-based algorithm for the pharmacologic treatment of Obsessive-compulsive behaviours in Huntington’s disease. PLoS Currents 2011 Sep 20; 3: RRN1261. PMID: 21947193
- Hubers AAM, van Duijn E, Roos RAC, Craufurd D, Rickards H, Landwehrmeyer GB, van der Mast RC, Giltay EJ, and the REGISTRY investigators of the European Huntington’s Disease Network: Suicidal ideation in a European Huntington’s disease population. Journal of Affective Disorders 2013; 151: 248-258. PMID: 23876196
- In collaboration with the EHDN Genetic Modifiers WG we are looking at genetic modifiers of onset of behavioural symptoms in HD.
- Longitudinal analysis of behavioural data from the REGISTRY study.
- Development of treatment guidelines for Anxiety, sleep disorders, agitation, apathy, psychosis.
- Development of training materials on behavioural symptoms.
- Development of PBA-s specific training materials
Jenny de Souza, PhD
University of Birmingham
Mayke Oosterloo, MD
Maastricht University Medical Center
Maastricht, The Netherlands