$328 Wednesday, November 9, 2005 Poster Abstracts

a series of appropriate holes, calculated the CAG index and scored the HD patients under blind conditions. Results: Intervals for the peg insertion task significantly differed between HD patients and controls, but not between HD gene carriers and controls. CAG index reflected the increase of symptoms in HD. Peg insertion results and scored severity of HD and CAG index significantly correlated to each other. Conclusions: Peg insertion scores are no specific diagnostic marker for HD, but they reflect clinical symptoms of neurodegeneration. Performance of peg insertion involves visuospatial cognition, self- elaboration of internal strategies, sorting and planning of movement. Therefore peg insertion particularly reflects executive dysfunction in early HD and additionally motor impairment in advanced HD.

0894 Low CAG ranges in Huntington's disease - Is 41 better than 45?

Salt, C, Andrich, J, Lauter, T, Kraus, P, Przuntek, H. Department of neurology, Huntington-Center NRW, Boehum, Germany

Background: Age of onset of HD is known to be negatively correlated with an expanded CAG repeat in the HD gene. However, there is a broad mean variation especially in the lower CAG ranges. We investigated 154 patients (74 female, 80 male) previously analysed with CAG repeat nmnbers in a low range between 41 and 45 in order to study differences in main symptoms and age of onset. Methods: Age of onset of first motor and/or psychiatric symptoms was evaluated in every patient. 40 patients could be divided into groups of paternal or maternal transmission. Clinical assessment of symptoms was performed by using a standardised battery of neurological and psychological investigations. Clinical signs were differentiated as symptoms presenting or as main symptoms. Results: Mean age of onset of motor symptoms was 47.47 (-88.8) years, in psychiatric, abnormalities (missing data for 33 patients) 45.87 (-89.0). Age of onset was 3.96 (motor), resp. 3.6 (psyclffatric) years earlier than in parent if there was maternal transmission, but 4.84 (motor), resp. 6.0 (psychiatric) years earlier if there was paternal transnffssion.

97.4% of the patients showed symptoms of chorea, 51.3% suffered from choreatic movements as the main clinical symptom. About 29.9% (2.6% as main symptom) of the patients presented other movement disturbances like dystonia, tremor, myoklonus or amnesia. Major depression was presented by 64.3% (20.1% as main symptom) of all patients. 27.2% of the patients suffered from signs of a psychosis, in 7.1% of the patients psychosis with illusions and hallucinations was the main symptom. Dementia was found in 83.1% (18.8% as main symptom). Conclusion: Remarkably, we found a significant negative correlation in age of onset of HD for motor sigus (r -- 0.521, p -- 0.01), as well as for psychiatric abnormalities (r -- 0.445, p - 0.01) but no difference in the main symptoms in this genetically homogenous collective.

0895 Dose dependent improvement of myodonifonn hyperkinesias in Huntington's Disease with valproate

Salt, C, Andrich, J, Lauter, T, Kraus, P, Przmltek, H , Andrich, JE. Department of Neurology, Huntington-Center NRW, St. Josef Hospital, Boehum, Germany

Background: Huntington's Disease (HD) is usually characterized by choreatic movements. Chorea usually is treated with antidopanffnergic neuroleptics like tiaprid and tetrabenazine. Especially in patients with signs of parkinsonism antidopamniergic medication often leads to worsening of symptoms. In past studies valproate showed no beneficial effect on involuntary movements. Myoclonus is rare in HD and often overseen or misdiagnosed as chorea. It does not sufficiantly respond on atttidopanffnergic medication. Method: In our center we had the chance to accompany about 600 different HD patients during the past 10 years. About 90% of these

showed symptoms of chorea, about 60% suffered from choreatic move- ments as the main clinical symptom. Only seven of the patients suffered from nlyoclonus or from myocloniform hyperkinesia as the main symptom and were treated with sodium valproate. All HD subjects were scored by using UHDRS motor score before and after treatment with valproate. 3 patients accepted to be additionally videotaped. Results: In six cases myoclonus and, therefore, UHDRS scores improved in a dose dependent manner. In three of these patients antidopanffnergic medication could be reduced, in the remaining patients antidopanffnergic treatnlent was basically unchanged. One patient with a daily dose of only 300 mg valproate did not improve. Conclusion: In a subgroup of HD patients suffering from myocloni- form hyperkinesias valproate might be a possibility of alternative treatment. Valproic acid seems to be more efficient than antidopanff- nergics in those patients. Besides, sodium valproat has less side effects on the extrapyranffdal system than neuroleptics. Short videotapes and handwritings of patients will be presented.

0896 UV-B irradiation allenuates dermal efl~cts of bolulintml toxin A: a randoudsed, double blind placebo eonlroUed study

P. Sclmider 1, T. Sycha 2, N. Kotzailias 2, G. Kranz 2 + F. Trautinger 2, * E. Auff 2. 1Department of Neurology, Landesklinik Hoehegg, Europe; 2Department of Neurology, Medical University of Vienna, Europe; 3Department of Dermatology, Ivledieal University of I,)'enna, Europe

Background: Botulinum Toxin (BoNT) is frequently used for the treatment of focal hyperhidrosis and for cosmetical indications. BoNT treatment is relatively easy to perform; however, to achieve optimal, long lasting results, some practical recommendations should be followed that imply instability of its protein structure and the importance of controlling the spread and diffusion of BoNT. Thus, intensive heat and/or sun exposure in the days after intradermal injection could be expected to alleviate BoNT effects. Method: In a randomised, double blinded trial, six healthy volunteers were injected intracutaneously with 100 M U BoNT/A (Dysport ~), Ipsen) on one side and placebo (saline) on the contralateral side. Forty- eight hours later, LrV-B irradiation (3* minimal erythema dose) was applied over the injection sides. Two weeks after the injection procedure the areas of antffdrosis were measured and the saline pretreated side was reinjected with BoNT/A. The respective areas of anhidrosis were estimated 2, 4, 6 and 14 weeks after BoNT/A. Results: BoNT/A treatment led to substantial anhidrotic areas is all cases, however, UV-B irradiation significantly alleviated this effect (iJ - 0.0017). The estimated mean difference [95 % CI] between the anlffdrotic area on the UV-B posttreated side and the UV-B pretrea- ted side was 8.3 cm2 [4.2; 12.4]. Tiffs UV-B-evoked reduction of approximately 30% was constant over 14 weeks after BoNT/A injection. Conclusions: We have demonstrated for the first time that UV-B irradiation substantially reduces the effects of intradetmally applied BoNT/A. Hence, whenever BoNT/A is applied intradermally, exces- sive exposure to UV-B and sunburn should be reconsidered.

0897 Developulent of a Patient Knowledge Quesfio mlaire for Bolulintml Toxin Use in Movement Disorders

Scbotl~r, KL 1, O'Sullivan JD 1'2. ZRoyal Brisbane and Women's Hospital, Brisbane, Australia; 2Department of Ivledieine, University of Queensland, Brisbane, Queensland

Background: Botulnium toxin is commonly used in the management of movement disorders from dystonias to tics, and tiffs study aims to develop the first valid and reliable quesfiommire to assess patient knowledge and expectations of treatment.