Huntington’s disease (HD) is a
dominantly inherited condition in which there is progressive dysfunction and
loss of cells in the straital nucleus of the basal ganglia. It is increasingly
regarded as a good paradigm for neurodegeneration in the wider sense.
Currently, management of HD is aimed at treating the manifest symptoms,
including choreic and dystonic movements and psychiatric disturbance. However,
many treatments are prescribed ‘offlabel’ and there is currently no
evidence-based treatment strategy. As HD is a relatively uncommon condition,
collaboration amongst centres expert in HD is required to undertake properly
powered clinical studies. This need prompted the founding of the UK HD Network (UKHDN)
in Jan 2003. The UKHDN is a network of clinicians and scientists, coordinated
by the Cardiff Centre, which aims to facilitate understanding of HD, assess
current treatments, and create a platform for new and emerging treatment
trials.
Working with the European HD Network The activities of the UKHDN have
been enhanced by the emergence of the European HD Network (EHDN) toward the end
of 2003, coordinated from Ulm, Germany.
The EHDN is a network of national Networks from several countries around Europe
and acts to facilitate and encourage European collaboration as well as hosting
a database for patients interested in research. The heart of the EHDN is a web
portal that allows data to be entered from any location in Europe
(www.euro-hd.net). All relevant trial information is available for downloading.
The core EHDN study, ‘Registry’, has MREC approval in the UKand
data collection began in 2005. The objective of Registry is to collect
prospective data on the phenotypical characteristics of Huntington's
disease
(HD) gene carriers, in order to obtain natural history data, to
expedite identification
and recruitment of participants for clinical trials and to plan for
future
research studies. All Registry participants undergo standardised
clinical
assessments and are invited to donate biosamples (blood and urine) to
the ‘Registry
Biobank’. These biosamples will be used for studies aiming to
identify genetic
modifiers of HD and to establish and validate biological markers to
track the
course of HD. The latter will be crucial for adequate powering of
treatment trials
in asymptomatic gene carriers. A number of international Working Groups
(WGs)
have been set-up via the EHDN to work on specific research areas and
the
development of existing assessment tools that cover all aspects of HD,
ranging
from ‘Standards of Care’ and ‘Quality of Life’
to ‘Symptomatic Treatments’ and ‘Surgical
Therapy’ (see appendix A for full list of WGs).
The UKHDN Steering Committee
has similar and overlapping aims to the HD CSG and so a recent decision was
taken to merge them into a single committee.The HD CSG aims to:
Use the recently-established HD network in UK
as a platform for research
Consolidate and extend the network of UK
clinical centres
Facilitate studies paving the way for high quality clinical
trials in HD
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