Poster Abstracts Wednesday, November 9, 2005 $323

findings were reviewed by an experienced neuroradiologist who was blinded to the side of hernichorea. Results: There was one female and two male patients with an age range of 75 to 90 years. Their blood glucose ranged from 13.5 to 27 mmol/1 and their serum osmolarity ranged from 306 to 310 llmlOl/1. All 3 patients had contralateral lentifoml nucleus hyperdensity on computed tomography and hyperintensity on MRI-T1. M R spectro- scopy showed elevated choline and reduced N-acetylaspartate in two patients and a significantly elevated myoinositol peak in one patient. All 3 patients had clinical and/or neuroimaging evidence of prior ischemia on the side ipsilateral to the choreoathetosis. Conclusions: Hyperglycemic hemichorea, is associated with signal changes in the contralateral lentiform nucleus. M R spectroscopy evidence of elevated myoinositol in the lentiform nucleus may be an osmotic marker associated with tiffs condition. We also hypothesize that hyperglycemia only resulted in unilateral chorea because the opposite basal ganglia neural circuit was disrupted by previous ischernic insults.

0875 Consequences of Chelating Therapy Discontinuation in Wflson's Disease

Kozic, D. Institute of Oncology, Semska Kamenica, Yugoslavia

Introduction: The aim of the report is to show the consequences of chelating therapy discontinuation in patient with neurologic presenta- tion of Wilson's disease (WD). Methods: Initial M R scanning of the brain was perfomled in 1997 wlffle M R reexanffnation was done in 2003, after one year long chelating therapy discontinuation (patient was not able to purchase required medications). Latest cranial MRI was performed in 2004, one year after readministration of appropriate therapy. Results: M R examination of the brain performed after one year long chelating therapy discontinuation showed significant progression of T2W signal abnormalities in midbrain and central aspect of the pons and involvement of thalami that were unaffected on initial M R scanning. Improvement was noted one year later after readministra- tion of appropriate therapy. Discussion: Nunlerous authors found partial or complete, relatively slow treatment-related reversal of changes in the brain parenchyma in patients with WD. Some of the changes are unfortunately consistent with gliosis, resistant to chelating therapy. It is known that the progression of disease can be halted with adequate treatment. Our report however shows marked, relatively prompt deterioration if for any reason chelating treatment is discontinued. The main morphologic substrate of the brain parenchimal lesions in WD appears not to be copper deposition, but demyelination, possibly caused by generated toxic radicals due to impaired process of copper detoxification.

0876 Lesions of dentate fascia in multiple sTstem atrophy

Liang Wang 1'2, Yoslffo Haslffzume i, Mad Yoslffda 1. lInstitute for Medical Science of Aging, Aichi Medical University, Nagakute-cho,

2 Aiehi-gun, Japan.." Institute of Neurology, Huanshan Hospital, Fudan University, Shanghai, P.R. China

Objective: Cases of marked temporal lobe atrophy and numerous neuronal cytoplasnffc inclusions (NCIs) in the granule cells of the lffppocampus had been reported. To study the positive rate of NCIs in the dentate fascia of multiple system atrophy (MSA), and the relation between NCIs and glial cytoplasmic inclusions (GCIs), 54 MSA cases including 3 with marked temporal lobe atrophy were histopathologi- cally investigated. Methods: All cases were studied with Gallyas-Braak staining and inmmnotffstochemical staining with ~-sytmclein (~S, C-20), ubiquitin, and AT-8 antibodies. Several selected cases were further studied with

c¢-synuclein (c¢S, N-I 9) and Tau-2 antibodies staining, (zS (C-20) and AT-8 double immunostaining. Results: Various numbers of GCIs were found in all cases. NCIs- positive granule cells were detected in more than half o f MSA cases. The morphological expressions of NCIs were ring-like or C-shaped encircling the nuclei; some were spherical in cases with marked temporal atrophy, but the number of NC.Is varied from one case to the next. The number of NCIs in granule cells was significantly correlated with that o f GCIs in white matter near the dentate fascia. The longer the duration of MSA disease is, the more numerous the NCIs are. There were more NCIs in cases with olivopontocerebellar atrophy (OPCA)/striatonigral degeneration (SND) than in cases with SND or Shy-Drager syndrome (SDS). Contusion: Our results demonstrated that a dynamic process of NCIs in the granule cells could be an indicator of the severity for the involvement of the temporal lobe in MSA.

0877 Tau Haplotypes Regulate Tau Expression in Human Brains

Loy, CT, Kwok, JBJ, Blair, IP, Hallupp, M, Todd, E, Schofield, PR. Neurobiology Program, Garvan Institute of Medical Research, Sydney, Australia

Background: A primary haplotype (HI) of the Microtubule-Associated Protein Tau (MAPT) is associated with tauopathies such as Corticobasal Degeneration and Progressive Supranuclear Palsy. We hypothesized that tiffs haplotype increases Tau expression in the hunlan brain. Method: We examined 116 brain bank sanlples with no evidence of tauopathy. Total RNA was extracted from the cerebellum. Tau transcript was amplified from brain eDNA using primers flanking the constitutively spliced Exon 7 sequence. Tau expression was measured twice for each sample using Real-Time PCR (SYBR-Green Chem- istry). Tiffs was then nomlalized by comparison with transcripts from the housekeeping gene GAPDH, to take into account variation in total RNA levels among samples. Tau haplotype was determined by a MspI restriction digest assay which detects the -373 T/C single nucleotide polymorphism within the haplotype. Results: 66, 43 and 7 samples were found to have the H1H1, H1 H2 and H2H2 haplotypes respectively. Brain samples with the H1H1 haplotype had significantly lffgher Tau expression compared to samples with the H2H2 haplotype (p - 0.006, two-sample t-test), showing a 3.21-fold increase in mean normalised value. When compared to samples with the H1H2 haplotype, samples with the H1H1 haplotype showed a 1.78-fold increase in mean normalised value but the difference did not reach significance (p -- 0.092, two-sample t-test). Contusions: This provides in vivo evidence that the HIH1 haplotype increases Tau expression in human brains, suggesting that elevation of Tau expression may underlie the pathogenesis of tauopathies.

0878 Coffee drinking anti cigarette Suloldng in prhllal-y adult-onset dyslonia: a illUlticenter ca~-control investigation

Martino, D ~, Abbvuzzese, G 2, Defazio, G a, Ghirlanda, p3, Avanzino, L 2, Buccafusca, M 3, Colosimo, C 4, Fabbrini, G 4, Majorana, G ~, Marinelli, L 2, Berardelli, A 4. 1Department of Neurological and Psychiatric Sciences, University of Bari, Bari, Italy," 2Department of Neurosciences, Ophthalmology and Genetics, University of Genoa, Genoa, Italy; 3Department of Neuroseienee, Psychiatric and Anes thesiologieal Sciences, Messina, Ita[y.." 4Depar tmen t of Neurological Sciences ("Rome) and lnstitute NEUROMED (Pozzilli, IS), University of Rome "La Sapienza", Rome, Italy

Background: Primary adult-onset dystonia (PAOD) has an undefined aetiology. Preliminary reports support an involvement of both genetic