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Euro Huntington's disease network [logo]
Grupo de Trabajo sobre Nuevas Herramientas de Evaluación Cognitiva -

Cognitive changes are an integral feature of Huntington’s disease that are present early in the course of the condition and become more severe with disease progression. Forgetfulness, difficulty paying attention and maintaining concentration and a general ‘slowing’ of cognition are common early symptoms of HD, all of which can have a big impact on work and everyday life.

If treatments for HD are to be successful, it is important that they address cognitive symptoms as well as the more commonly recognised motor symptoms. In this context, it is crucial to know which aspects of cognition are the most sensitive to HD and to develop accurate and reliable methods of measuring these changes.


The primary aims of the Cognitive Phenotype Working Group are as follows:
  1. To develop a neuropsychological battery to be used in the EHDN REGISTRY Project.
  2. To improve standardisation of neuropsychological test administration in order to increase data reliability.
  3. To develop novel assessment tools and evaluate their sensitivity to the progression of HD for potential use as outcome measures in therapeutic trials.
  4. To carry out collaborative neuropsychological studies aiming to extend the theoretical understanding of HD.
Current projects

An instruction manual has been developed in order to improve the standardisation of neuropsychological test administration. With the help of our working group and other EHDN members, this manual is being translated into various European languages. An important next step is to collect normative data across the different European languages. A pilot study comparing Dutch, English and French has been carried out and a large scale study will soon begin.

We are also in the process of developing a training video to guide the administration and scoring of the REGISTRY neuropsychological assessment. Other study protocols under development include the evaluation of a visual memory test, a syntactic comprehension test and a tapping test.

El Grupo de trabajo del Fenotipo cognitivo tiene aproximadamente unos 25 miembros activos y otros 40 miembros asociados (julio 2008). Pueden ser miembros del este grupo los médicos y científicos con interés en los aspectos neuropsicológicos de la enfermedad de Huntington. El grupo se reúne una o dos veces al año (una de ellas coincide normalmente con la reunión plenaria del EHDN) y se mantiene contacto por correo electrónico. Se anima a los miembros a exponer sus trabajos durante las reuniones y a proponer proyectos de colaboración.

Dr Jennifer Thompson
Neuropsychologist, SBAC, Co-Investigador, Evaluador cognitivo, Delegado del grupo Cognitive Phenotype
Cerebral Function Unit, Greater Manchester Neurosciences Centre, Salford Royal NHS Foundation Trust
Dirección postal: Stott Lane, Salford
M6 8HD Manchester
Teléfono: +44 161 2065622
Correo electrónico: 

Associated Language Area Coordinator (Lanco)

, Spain
Sara Minster, UK