movement-based priming strategy designed to ‘‘re-balance” M1activity by reducing intracortical inhibition and increasingipsilesional M1 excitability. We aimed to determine if APBTprior to physical therapy improves clinical outcome in strokepatients.

Methods: Thirty-two patients (20 men, mean age 55.3 years)with upper limb weakness at least 6 months after stroke were stud-ied. Patients were randomized to a one-month intervention of self-directed upper limb motor practice (MP) (control group) or to APBTfor 10–15 minutes prior to the same motor practice (APBT group).Upper limb function was assessed at baseline, and immediatelyand one-month after the intervention. Transcranial magnetic stimu-lation was used to assess M1 function.

Results: Affected upper limb function improved in both the con-trol and APBT groups immediately after the intervention (p < 0.005).After one-month, the APBT group demonstrated better upper limbfunction than control patients (p < 0.05). The APBT group also hadincreased ipsilesional M1 excitability (p < 0.025), increased transcal-losal inhibition (p < 0.01), and increased inhibition within contrale-sional M1 (p < 0.005).

Conclusions: APBT produced sustained improvements in upperlimb function in chronic stroke patients and induced specific andsustained changes in motor cortex function. We speculate that APBTmay have facilitated plastic reorganization in the brain in responseto motor therapy. The utility of APBT as an adjuvant to physical ther-apy warrants further consideration in larger randomized controlledtrials.

doi:10.1016/j.jocn.2008.07.014

116. A novel moderate-affinity metal binding ligand confersneuroprotection against 6-hydroxydopamine mediated chroniccell death and improves rotational behavior in a mouse model ofparkinson’s diseaseSimon Wilkins, Karoline Deleva, Mikhalina Cortes, Mila Nurjono,Irene Volitakis, Jessica George, I David Finkelstein, Robert A. Cherny

The Mental Health Research Institute of Victoria

There is evidence that aberrant interactions between metals andand bioavailable reductants are involved in the destruction of sub-stantia nigra (SN) cells in Parkinson’s Disease (PD). Previously weshowed that long term pre-treatment with clioquinol (CQ) a mono-dentate antioxidant with moderate affinity for copper, zinc and iron,reduced the susceptibility of SN neurons to the neurotoxin MPTP(Neuron, 2003;37:889–909). Because 6-hydroxydopamine (6-OHDA)is an oxidative metabolite of dopamine implicated in pathogenicpathways in PD we examined the effects of CQ using this nigraltoxin. Initial studies showed administration of CQ commencing onthe same day as 6-OHDA injection significantly diminished lesionsize. We concluded that redox-active metals mediate both MPTPand 6-OHDA-induced neurotoxicity and because no pretreatmentwith CQ was required, neuroprotection was likely due more to itsredox-neutralising effects than bulk metal chelation. To determinewhether this class of drug might be effective when nigral degenera-tion was already advanced, we developed an experimental paradigmwhereby drug is only administered in the days following initiation ofthe acute phase of nigral death by 6-OHDA. Drug efficacy is assessedby rotational behaviour in response to amphetamine challenge andby stereoscopic counting of residual nigral cells. A novel bioavailableanalogue of CQ tested in this model (provided by Prana Biotechnol-ogy Ltd) significantly reduced post-amphetamine rotational behav-ior and preserved more SN neurons than in either CQ-treated oruntreated lesioned animals. We conclude that these compounds

merit further investigation for their potential as a novel therapyfor PD.

doi:10.1016/j.jocn.2008.07.015

117. Clinical, neurophysiological and genetic observations inkufor-rakeb syndrome: report of a new kindred with compoundheterozygous mutations in the ATP13A2 GeneVictor S.C. Fung a, Prachi Mehta b, Neil Manwaring b, Carolyn M. Sue b,Christian Kubisch c

a Movement Disorder Unit, Department of Neurology, Westmead Hos-pital, Sydneyb Department of Neurogenetics, Kolling Institute, Royal North ShoreHospital, Sydneyc Institute of Human Genetics, University Hospital, Cologne, Germany

Objective: Kufor-Rakeb syndrome (KRS) is a form of autosomalrecessive juvenile onset Parkinsonism first reported in 1994. Linkageto chromosome 1p36 was shown in 2001 and the locus designatedPARK9. Mutations in ATP13A2 were first reported in 2006. We reporta new kindred with KRS.

Case Histories: A Chinese male was seen in 2003 and his sister in2007. The proband had normal motor milestones but mild develop-mental delay. He presented at age 17 with social anxiety requiringtreatment with SSRIs. He developed bilateral Parkinsonism fromage 19 with akinesia, rigidity, upper limb action tremor without resttremor and postural instability. There were atypical Parkinsonianfeatures of upward supranuclear gaze palsy, facial action tremor,mild upper limb dystonia, lower limb hyperreflexia and cognitivedysfunction. He had a good response to levodopa/benserazide 200/500 tds. His sister had an identical presentation from age 18, exceptthat she also had mild cervical dystonia.

Results: Surface EMG recordings showed a 10Hz action tremor ofthe face and upper limbs. NCS and median SEPs were normal and Creflexes absent. Cerebral MRI showed no definite abnormalities.Genetic analysis in both siblings showed compound heterozygositywith novel c.3176T>G/c.3253delC mutations that were not presentin over 150 Asian and 95 German controls.

Conclusion: Our findings extend the clinical spectrum of KRSwith a later age of onset, prominent anxiety as the presenting symp-tom and relatively slower progression. This is the first report ofneurophysiological findings in this syndrome. Two novel pathogenicmutations are described.

doi:10.1016/j.jocn.2008.07.016

118. Impact of changes in the consensus diagnostic criteria fordementia with lewy bodiesGlenda M. Halliday, Amy King, Heather McCann

Prince of Wales Medical Research Institute and University of New SouthWales

Objective: To examine the impact of changes in the consensuscriteria for the diagnosis of dementia with Lewy bodies (DLB).

Methods: 195 longitudinally studied autopsy cases with demen-tia and/or Parkinson’s disease (PD) recruited through a regional braindonor program were selected with a-synuclein-positive brainstemLewy bodies (with or without additional Alzheimer pathology) inthe absence of other neuropathologies. Clinicopathological diagnoseswere compared using both the new and old criteria for DLB.

466 Abstracts / Journal of Clinical Neuroscience 16 (2009) 462–481