$320 Wednesday, November 9, 2005 Poster Abstracts

Case report: A 57 year-old woman presented a progressive ataxic gait, palilalia, skeletal deformities (pes cavum, spinal scoliosis) and osteotendinous arreflexia, from the 25 years of age. In the last five years, dysarthria, dystorfic postures of right limbs and distal muscular atrophy of legs were added. Electroneurograptfic exam showed normal motor and sensitive conduction velocities, and the temporal summa- tion of muscular activity diminished.

MRI demonstrated the deposition of iron in the globus pallidus. Genectic study revealed a homzygofic mutation N2941 in the gene encoding PANK2. Discussion: Our patient presents atypical clinical features in Hallevorden-Spatz syndrome, such as light and not progressive impairment of inferior motorneuron, and an absence of cortico- spinal tract damage and cognitive deterioration. It is important to highlight the palilalia presence, as a precocious sign in these atypical fo tins.

0863 MRI/A study of VLM compression and hypertension in hemifacial spasm

Chan, L, Tan, E. Singapore General Hospital," National Neuroseience Institute

Background: Essential hypertension has been associated with hemi- facial spasm (HFS). However, the relationslfip between rostral ventro- lateral medulla (VLM) compression and essential hypertension has not been examined in HFS. Objective: Utilizing multiplannar 3-dimensional Time-of-flight Magnetic resonance angiography (3-D TOF MRA) and constructive interference at steady state (CISS) techniques, we investigated the prevalence and severity of VLM compression in hypertensive HFS and matched normotensive HFS patients. Method: We recruited consecutive HFS patients who were diagnosed with essential hypertension from the movement disorder clinic at a tertiary referral center. For every hypertensive HFS patient, we sought for a non-hypertensive patient of similar age, gender, body mass index (BMD and duration of disease. A multiplannar 3-D TOF MRA and CISS examination was carried out for every patient. Results: A total of 80 (40 hypertensive and 40 non-hypertensive) HFS patients were included. The two groups of patients were matched for age, gender, duration and severity of HFS and BMI. The prevalence of VLM compression was significantly lfigher in hypertensive HFS compared to non-hypertensive patients (175"/o vs 53"/o, p -- 0.02). In hypertensive HFS, there was a greater prevalence of left sided VLM compression, while about equal prevalence was found in normotensive group. The mean VLM compression severity score was greater in the hypertensive group (12.02 ± 0.8 vs 1.68 ± 0.85) compared to normotensive patients. Conclusion: We found a higher prevalence of VLM compression in hypertensive HFS compared to normotensive HFS patients. This finding may have implications for the clinical management of some HFS patients.

0864 Cognitive disturbances in presymptomatic carriers of Huntington's disease

Cras, pL2, Dobbelaere, K 1, de Wilde, S 1, Van Wallendael, K ~, Clincke, B ~. 1University Hospital of Antwerp, Edegem, Belgium; 2Born Bunge Institute, University of Antwerp, Wilrijk, Belgium

Background: Huntington disease (HD) is an autosomal dominant disease that gives rise to progressive, selective neural cell death associated with choreic movements and dementia. We studied presymptomatic HD-carriers for subtle cognitive changes using a novel computerized assessment. Method: Presymptomatic carriers (IN - 10, age 39.0 ± 11.4 yrs, scolarity 15.7 ± 2.0 yrs), early HD patients (N - 5, age 41.3 ± 6.8, scolarity 14.8 ± 2.8 yrs) and sex and age matched, healthy volunteers

0 N -- 10, age 39.1 ± 8.5 yrs, scolarity 14.9 ± 2.1 yrs) were subjected to a Brainspeech ~ computerized cognitive battery consisting of the following tasks: word-list learning (WLL) task-inmtediate and delayed recall, recognition, continuous performance (CPT), paced serial addition (PaSAT) and Stroop test. Additionally, Beck depression inventory (BDD and mini mental status examination were performed. Patients were examined clinically and the Unified Huntington's Disease rating Scale was used to assess disease severity. Results: All presymptomatic disease carriers and healthy volunteers showed a normal UHDRS score. Processing time in word-colour part of Stroop test and CPT reaction time was prolonged in presympto- marie carriers as compared to healthy controls. Also, accuracy was reduced in CPT and WLL, delayed recall. Early HD patients showed similar but more pronounced defects. Conclusion: These findings are compatible with an early disturbance of frontal cognitive control processes in presymptomatic HD mutation carriers. A computerized cogtfitive measure could be useful in evaluating neuroprotective strategies.

0865 The prevalence of restless leg syndrome in Macedonian primary care population

Dhnova, A 1, Nedanovska, B 2, Cicic, V 1 . 1Medical Center "'Dr. T.Panovski", Bitola, Macedonia; 2Health Center, Slropje, Macedonia

Introduction: One of the most common sleep and movement disorder Restless legs sytrdrome (RLS), is still undiagnosed or misdiagnosed in those who present for treatment. RLS is characterized by four essential diagnostic criteria. These symptoms often result in disturbance of sleep, daytime tiredness and import on quality of life. The aim of this study is to evaluate prevalence of RLS in primary care population. Material and Method: Self-modified questionnaire was completed by 243 patients ( 119 female and 124 male), visiting the general practitioner for any reason (original cohort). Questionnaire consist four parts; in the first part diagnostic questions to screen for RLS, second part demogra- phic characteristics and co morbidity, third part questions to assess symptoms of RLS and in fourth questions about previous treatment. Results: Twenty-two (9.0%) patients respond positive to all four questions in the fist part of the questionnaire (screen positive for RLS) and were defined as RLS cohort. Out of 22, 12 (54.5%) were female and 10 male (45.5%). Most common diagnoses in the RLS cohort were: hypertension (nine patients), diabetes mellitus (nine), generalized arthrosis (eight), iron deficiency (six). Positive familiar history reported 5 patients (22.7%). Vitamin supplements were previously administered in 16 patients (72.7%), analgesics in 12 (54.5%). Conclusion: Prevalence of RLS in primary care population 9.0% in Macedonia's is similar with consensus from published studies. A higher proportion of female is confirmed. As Conclusion: RLS as condition seems to be still unrecognized, which result with appropriate treatment, and impact on quality of life.

0866 Randomized cross over study of limb cooling in essential tremor

Virgilio Gerald H. Evidente l, Cynthia Cooper 2, Susan Bernsteni ~, Jose Luis Hernandez 4, Joseph Hentz 4. 1Mayo Clinic, Dept. Of Neurology, USA; 2Mayo Clinic, Occupational Therapy Section, USA," 3Mayo Clinic, Clinical Studies Unit, USA," 4Mayo Clinic, Biostatistics Section, USA

Background: We previously reported the effect of upper limb (UL) cooling and warming on tremor and hand function in 20 Essential Tremor (ET) and 20 Parkinson's disease (PD) patients. We noted that neither warm nor cold affected PD, wlfile limb cooling improved hand tremor & function in ET. We aimed to confirm our earlier findings in ET.