Report on the 14th International Meeting of the International Huntington Association, held in Denmark, August 2OO1
Dorothy Tortell, Social Worker, Wellington Huntington's Disease Association

I was very privileged to represent the New Zealand Huntington's associations at the 14th meeting of the International Huntington Association in August in Denmark, with the support of a Travel Grant from the Neurological Foundation of New Zealand. The 19th International Meeting of the World Federation of Neurology Research Group on Huntington Disease took place at the same time over the first 3 days. One session was concurrent and some papers were presented to both meetings.

There were approximately 86 participants from 27 countries at the meeting and over 2O papers were presented. Topics ranged from research updates, to understanding challenging behaviour, medication for psychiatric/behavioural problems, predictive, prenatal and pre-implantation testing,the unique combination of risks and stress faced by staff working in Huntington's Units, the relationship between caregiver burden and the severity of Huntington's Disease, dental care,eating and swallowing difficulties, nutrition, juvenile HD, family communication about HD risk, and Percutaneous Endoscopic Feeding (PEG). I presented a paper on 'Initiatives for children and young people' in which I discussed the camp for young people which the Wellington Association runs annually and I also discussed the value of the book "Huntington 's and Me - a guide for young people" by Alison Gray which was published by our Association in 2OOO.

Dr Karl Kieburtz, from the University of Rochester, USA discussed drug trials which were conducted by the HD Study Group in North America on Coenzyme Q1O and Remacemide. The trial was set up to determine whether the use of Coenzyme Q1O and Remacemide would slow HD. Coenzyme Q1O is a nutritional substance which can be bought over the counter and is taken in capsule form twice a day. The results showed that both drugs were generally well tolerated. Remacemide tended to decrease chorea for persons in the early stages of HD whereas Coenzyme Q10 tended to reduce functional decline. Further studies are required to establish whether or not Coenzyme Q1O does slow the decline in total functional capacity for a person in the early stages of HD.

Dr Julie Snowden from the Cerebral Function Unit, at the Greater Manchester Neuroscience Centre, Hope Hospital, presented a paper on understanding challenging behaviour in HD. She spoke about how behaviour is often the most distressing part of the disease and creates the greatest challenge for the carers. Difficult behaviours are often directed at family members and may not be noticed at clinics, which could be the reason why behavioural problems are often underestimated by the outsider. Dr Snowden discussed the impact of cognitive change on behaviour and in particular on initiative and drive, thinking ahead, organisational skills, attention, self-monitoring, and mental flexibility. She then went on to discuss mood changes in HD and the frequency of behavioural problems in HD. She concluded by stating that behavioural problems can damage social and family relationships but that it is the disease and not the person that is at fault.

Dr Jochen Jackowski from Germany presented a paper on dental awareness in HD. He said that dental destruction was very common in people who suffer from HD and outlined some of the reasons why. These include oral hygiene, which may be insufficient due to psychological and cognitive impairment. Under these conditions, dental care becomes more difficult as the condition progresses. Because of difficulty in communications, those with HD may have problems with communicating the extent of their dental pain. Uncontrollable movements make it difficult to maintain an acceptable level of hygiene and may complicate diagnosis of dental problems and it is very often difficult to obtain good x-rays. The side effects of drugs and grinding or clenching of the teeth may also lead to dental problems. Unfortunately dentists often only become involved in emergency dental care for those with HD and are therefore not experienced in the care of people with HD. Dr Jackowski reiterated that dental examination needs to become part of the regular care of persons with HD and treatment requires flexible interdisciplinary decision making.

Dr Dirk Gaasbeek from the Netherlands, presented a paper on Percutaneous Endoscopic Gastrostomy (PEG). He discussed the use of PEG feeding when severe swallowing difficulties made it too difficult to eat and drink with resulting weight loss. He explained how early open discussion on the goals of such a treatment is vital and all decisions made must be recorded and should be evaluated at regular intervals. One important issue, which must be discussed, is whether PEG feeding is to be continued until death or discontinued at an earlier stage. The client is involved in all the discussions and relatives are informed of all decisions.

As one participant stated, those hoping for a dramatic breakthrough, may have been a little disappointed, but clearly there is a world-wide body of researchers making steady progress in understanding and finding ways to control HD.