$290 Wednesday, November 9, 2005 Poster Abstracts

was the commonest cause of dementia among the patients with the onset of symptoms before the age of sixty-five years. Vascular dementia was the commonest cause among patients who had a presenile onset of sylnptoms. One-fourth of the patients in both these groups had vascular dementia. There was significant association between vascular dementia and risk factors like hypertension and diabetes. Eight percent of patients with dementia had treatable causes of dementia. Many patients continued to utilize the follow up services offered by the clinic. Conclusion: It is possible to set up dementia clinics in general hospitals using existing resources. Patients and caregivers will be benefited by this service. Vascular risk factor reduction has an important place in the management of dementia in general hospitals.

0744 Tile Value of Clinical $yInptolns ill tile Prediction of Creutzti~ldt-Jakob Disease

Jansen, GH 1, Sabourin, S 1, McGrath, M 1, Gervais, R 1, Wu, HX 1, Giulivi, A ~, Canadian C.JD Surveillance System. 1Prions Section, CIDPC, Public Health Agency of Canada, Ottawa, Canada

Background: In Creutzfeldt-Jakob disease surveillances around the globe, patients are evaluated for the likelihood of having CID based on symptoms and tests. Mostly over 50% of those patients initially suspected to have CJD prove not to have CJD in the end. The current study is to evaluate the value of clinical symptoms as predictors of CJD. Method: Between April 1, 1998 and April 1,2005, 1022 patients suspect of having CJD were referred to the Canadian CJD surveillance system (CJDSS). By pathological examination 200 of these were established as sporadic C.JD (sCJD) cases and 112 turned out to be non-C.JD cases. Signs and symptoms used in the WHO criteria for CJD were obtained from physicians and the patient's family by questionnaire, and by medical chart review. These were compared between the two groups. Results: Rapidly progressive dementia (RPD), ataxia and myoclonus scored as sensitive, yet not so specific symptoms, while central visual disturbances (CVD) scored as specific yet not so sensitive for the diagnosis sCJD. Akinetic mutism, pyramidal, and extrapyramidal signs were not significantly different between the groups. The combination of RPD with ataxia was overall most sensitive (84"/0) and specific (158"/o), though RPD with either ataxia and CVD, or with myoclonus and CVD proved to be more specific but less sensitive. Conclusion: This study shows that in the CJDSS dataset pyramidal signs, extrapyramidal signs and akinetic mutism are not good predictors of CJD. The combination rapidly progressive dementia and ataxia proved to be the best predictor of disease.

0745 Expression of a piggy-back-gene deterulines Alzhehner disease pathology?

Jellinger, K l, Kienzl, E z, Janetzky, B 3, Attems, j4, Preddie, E 5, Bergllmnn, js, Rattay, F 2. 1Institute of Clinical Neurobiology, I/Tenna, Austria; 2I/Tenna Health Board/TUBioMed, University of Technology tq'enna, Austria; 3Dept. of Neurology, Carl Gustav Carus University Dresden, German),; 40WS, Otto Wagner Hospital Vienna, Austria; SAltegen Inc., Canada

Background: The "amyloid cascade" hypothesis implicates accumu- lated amyloid plaques, fibrils and soluble neurotoxic oligomers (ADDL) of beta amyloid protein (A131-42), as a major cause of Alzheimer Disease (AD). However, the bases of the neurotoxic action of A[3 are still a matter of controversy. Method and Results: The applied method to find alternative genes as putative causative factors was "disease gene discovery by positional searching". ALZAS (ALZheimer ASsociated Protein), a "piggy-back- gene", was found on chromosome 21 within the amyloid precursor protein (APP) region. The promoter region of ALZA8 is located in

intron 15 of the APP gene and the coding includes a part of exon 16, the whole exon 17 and the beginning of intron 17, resulting in a 79 amino acid (aa) protein. ALZAS contains the A131-42 fragment, the APP transmembrane signal, and a unique 12 amino acid C-terminal (ct-12) which is not present in any known allele of APP. Reverse transcription-PCR and qRT-PCR m R N A quantification revealed the transcript of this protein to be expressed in brain regions and lymphocytes of AD patients. Confocal microscopy of autopsy brains confirmed intraneuronal and capillary ALZAS immunostaining correlating significantly with Braak stages of neuritic AD pathology using a specific affinity purified attti-ctl2-antibody. Conclusion: ALZAS is thought to compete or even to outcompete APP for the APP transmembrane site in specific neurons. This altered APP processing resulting in stimulation of the cytoskeletal system followed by tau pathology and dying back of synapses is discussed.

0746 Isotonic Back Extensor Strength and Cognition: modification by the metabolic syndrome

Jeong S.K. ~, Nam H.S. z, Kweon S.S. ~, Shin M.H. 3, Park K.S. ~, Choi J.S 4, Seo M.W. 1, KJm Y.H. 1. 1Department of Neurology, Chonbuk National University School of Medicine, Yeonju, Republic of Korea; :Department of Preventive Medicine, Chungnam National University School of Medicine, Daejeon, Republic of Korea; SDepartment of Preventive Medicine, Seonam University School of Medicine, Namwon, Republic of Korea; 4Department of Preventive Medicine, Chonnam National University School of Medicine, Gwangju, Republic of Korea

Background: The present study aimed to evaluate whether maximal isotonic Back Extensor Strength (miBEST) measurement was asso- ciated with cognitive function, and whether the metabolic, syndrome modified the association. Method: A cross-sectional study involving 1254 participants (472 men and 782 women) aged 60 years and over was performed in Namwon, Jeonbuk in 2004. The miBEST, defined by the static maximmn power of waist muscles, was measured with a potentiometer, and cognition with the Korean version of modified Mini-Mental State Examination (K-mMMSE). Results: About 297 subjects (23.7%) had the K-mMMSE score less than 60 and were defined as cognitively impaired. According to the miBEST increases, median scores of the K-mMMSE were signifi- cantly increased (P < 0.001). Among subjects without the metabolic syndrome, compared to the lowest qnintile of the miBEST, the odds ratios for the cognitive impairment in the highest quintile was 0.33 (95% CI; 0.13-0.84), adjusting for the potential confounders. The likelihood ratio test for trend was also significant with the mJBEST increments (P < 0.01 in multivariate model). In the presence of the metabolic syndrome, the associations were not consistent and the trend test lost significance in the multivariate model. Conclusion: The modifying effect of the metabolic syndrome on the association between cognitive impairment and the miBEST, a surrogate measure for fitness, should be carefully considered for revealing the protective effects of exercise or fitness on cognitive function.

0747 Clinically undetected motor neuron disease in pathologicaUy-proven I?ontotemporal lobar degeneration with motor neuron disease

Josephs, KA 1, Knopman, DS 1, Boeve, BF 1, Parisi, JE 2, Petersen, RC 1 , Dickson, DW 3. 2Department of Neurology, Mayo Clinic, Rochester, MN, USA; 2Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA; 3Department of Neuroscience and Pathology, Mayo Clinic Jacksonville, FL, USA

Background: Frontotemporal lobar degeneration with motor neuron disease (FTLD-MND) is a neurodegenerative disease with mixed