Behavioural changes are an integral feature of
Huntington‘s disease. Personality changes are often already reported early in
the course of the disease. Frequently reported behavioural symptoms comprise
depression, anxiety, irritability, apathy, compulsive behaviour and thoughts
and - to a lesser extent – psychotic symptoms. Severity and occurrence of these
symptoms vary greatly as well as their development during disease progression.
Although there is currently no treatment that is able to cure HD, many of these
behavioural symptoms can be treated adequately with either medication, or
behavioural psychotherapy or a combination of both.
Research into identifying a successful treatment for HD is ongoing. In order to
test treatments for their effectiveness it is important that assessments are
sensitive and valid for measuring behavioural symptoms, as well as their
changes over the course of HD. Additionally the symptomatic treatments used for
behavioural features are insufficiently documented. The Behavioural Working
Group aims at the development of new assessment tools, improvement of existing
tools and a better documentation of symptomatic treatment options.
aims of the EHDN Behavioural Working Group are:
Development of new more
sensitive behavioural assessment tools to be used in the Registry project.
To improve available
behavioural assessment tools
Introduce certification for
Provide better documentation
of use and efficacy of available symptomatic treatment options.
short behavioural assessment for Registry has been developed. The assessment is
shorter and has clearer scoring criteria and is based on the Psychiatric
Behavioural assessment and the UHDRS behavioural assessment. With the help of
the working group and other EHDN members, this assessment has been translated
into the various European languages in EHDN.
The working group is also active in executing validity studies of available
assessments that are used for measuring behavioural symptoms, specifically for
use in HD. An example is the validation of the HADS and BDI / BDI II for
measuring depression in HD.
Study protocols for data mining projects of behavioural data in the Registry
database are currently being developed and will soon be executed.
Membership is open to scientists
and clinicians with an interest in the Behavioural and psychiatric aspects of
HD. The group meets twice a year, of which usually one time is at the EHDN
plenary meeting and one on another occasion most often in Leiden, the
Netherlands. The group maintains contact by e-mail communication between the
face to face meetings. During these meetings the status and progress of Working
Group activities are discussed and members of the working group have the
opportunity to present their own projects as well as to propose collaborative
working group projects.
Each meeting a representative of the Huntington’s Study Group behavioural
working group is invited to give an update of their activities, which also
opens possibilities for more global collaboration.
Members of the working Group have access to the working group area on the EHDN
website and active members can also upload material at this area for optimal
If you are interested in joining the WG, please contact the Lead Facilitators,
copying in Jenny Callaghan, the Lanco coordinator (email@example.com).
Language Area Coordinator (Lanco)