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International Dementia with Lewy Bodies Conference Fort Lauderdale, Florida, USA; December 1 – 4, 2015

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International Dementia with Lewy Bodies Conference Fort Lauderdale, Florida, USA; December 1 – 4, 2015 Am J Neurodegener Dis 2015;4(Supplementary Issue 1): 1-178 www.ajnd.us /ISSN:2165-591X/2015 International DLB Conf. International Dementia with Lewy Bodies Conference Fort Lauderdale, Florida, USA; December 1 – 4, 2015 Table of Contents Oral Presentations Day 1 DLB Review & Update (Tuesday Dec. 1) Session 1: DLB Update – Clinical (O.1 - O.3) ........................................................ page 3 - 5 Session 2: DLB Update – Imaging/Biomarkers (O.4 - O.6) .................................... page 6 - 8 Session 3: DLB Update – Neuropathology/Genetics/Basic (O.7 - O.10) ................ page 9 - 12 Session 4: DLB Update – Therapeutics (O.11 - O.13) ........................................... page 13 - 15 Day 2 New DLB Research I (Wednesday Dec. 2) Session 5: Clinical I (O.14 - O.18).......................................................................... page 16 - 20 Session 6: Clinical II (O.19 - O.23) ......................................................................... page 21 - 25 Session 7: Imaging (O.24 - O.28) .......................................................................... page 26 - 30 Day 3 New DLB Research II (Thursday Dec. 3) Session 8: Neuropathology/Molecular Biology (O.29 - O.33) ................................. page 31 - 35 Session 9: Genetics/Biofluid Markers (O.34 - O.39) ............................................... page 36 - 41 Session 10: Novel Therapeutics (O.40 - O.43)....................................................... page 42 - 45 Day 4 Hot Topics/Controversies/Harmonization (Friday Dec. 4) Session 11: Hot Topics (O.44 - O.47) .................................................................... page 46 - 49 Session 12: Controversies (O.48 - O.51) ............................................................... page 50 - 53 Session 13: Toward Global DLB Research Harmonization (O.52 - O.55) .............. page 54 - 57 1 Am J Neurodegener Dis 2015;4(Suppl 1): 1-178 Am J Neurodegener Dis 2015;4(Supplementary Issue 1): 1-178 www.ajnd.us /ISSN:2165-591X/2015 International DLB Conf. Poster Presentations Tuesday Dec. 1 and Wednesday Dec. 2 Biofluid Biomarkers (P.1 - P.5) ............................................................................... page 58 - 62 Cognitive, Neuropsychiatric, Motor, Sleep and Autonomic (P.6 - P.47) .................. page 63 - 101 Genetics (P.48 - P.57) ........................................................................................... page 102 - 111 Molecular Biology and Models (P.58 - P.59) .......................................................... page 112 - 113 Neuroimaging (P.60 - P.86) ................................................................................... page 114 – 140 Neuropathology (P.87 - P.99) ................................................................................ page 141 - 153 Neuropsychology and Cognitive Neuroscience (P.100 - P.105) ............................. page 154 - 159 Non-Pharmacologic, Psychosocial, Education, Cognitive, and Movement (P.106 - P.107) .............................................................................. page 160 -161 Patient and Care Partner Education (P.108 - P.110) ............................................. page 162 - 164 Treatment (P.111 - P.116) ..................................................................................... page 165 - 170 Late Breaking (P.117) ............................................................................................ page 171 AUTHOR INDEX ................................................................................................... page 172 - 178 2 Am J Neurodegener Dis 2015;4(Suppl 1): 1-178 Am J Neurodegener Dis 2015;4(Supplementary Issue 1): 1-178 www.ajnd.us /ISSN:2165-591X/2015 International DLB Conf. TUESDAY DECEMBER 1, 2015 Session 1: DLB Update – Clinical O.1 DLB History, Differential Diagnosis and Diagnostic Features Ian McKeith Newcastle University, Newcastle, UK Although case reports of dementia associated with LB pathology can be traced back to the early part of the twentieth century, its recognition as a common and possibly, separate entity was much more recent. The DLB Consortium first met in 1995 to bring together the handful of researchers interested in the disorder, to harmonise the various competing appellations and to evolve the DLB concept. A brief historical account of this and subsequent developments will be offered as a starting point for the current meeting. DLB cases can be usefully divided into typical and atypical presentations. Typical cases correspond to the DLB profile described by Consensus criteria with one or more of fluctuating confusion, recurrent visual hallucinations, mild extrapyramidal features, RBD, autonomic dysfunction or neuroleptic sensitivity. In such cases the differential diagnosis includes Alzheimer’s disease, Parkinson’s disease with dementia, vascular dementia or delirium. Although guidelines recommend neuroimaging investigations to aid diagnostic discrimination, since >80% of clinically typical cases are found to have cortical LB disease at autopsy, current biomarkers can add only a limited amount. More problematic are atypical presentations with complaints of memory dysfunction, apathy, low mood and general functional decline but lacking core features of DLB and corresponding to what was previously called the Lewy body variant of Alzheimer’s disease. Cognitive testing reveals deficits across multiple domains and in the absence of further history taking, the most frequent diagnosis made is of Alzheimer’s disease or mild cognitive impairment (MCI). It is in such cases that systematic questioning about prodromal features suggestive of LB disease are most helpful and these should form part of the routine history taking of all patients being assessed for the cause of cognitive decline. It is also in this group that neuroimaging and other biomarkers may most increase diagnostic confidence. 3 Am J Neurodegener Dis 2015;4(Suppl 1): 1-178 Am J Neurodegener Dis 2015;4(Supplementary Issue 1): 1-178 www.ajnd.us /ISSN:2165-591X/2015 International DLB Conf. O.2 Key Clinical, Epidemiologic, Neuropsychologic and Imaging Features of DLB Bradley F Boeve Mayo Clinic, Rochester, MN Dementia with Lewy bodies (DLB) is a clinical syndrome characterized by dementia associated with two or more of the following features: recurrent fully-formed visual hallucinations, fluctuations in cognition, spontaneous parkinsonism and REM sleep behavior disorder (RBD). The evolving data indicates that the clinical manifestations of DLB encompass many other features spanning the cognitive, neuropsychiatric (e.g., delusions, apathy, depression, etc.), motor, sleep (e.g., hypersomnia, fragmented nocturnal sleep, etc.), autonomic (e.g., constipation, orthostatic hypotension, sexual dysfunction) and sensory (e.g., reduced smell and color vision) domains. DLB is not only the second most common cause of degenerative dementia syndrome (after Alzheimer’s disease or AD), some of the clinical features are similar to both AD and Parkinson’s disease, underscoring the relatively high prevalence as well as the complexity in terms of diagnosis and phenomenology. Neuropsychological assessment typically demonstrates impairment in attention, executive functioning and visuospatial functioning, with more preserved functioning in memory and confrontation naming – this profile is different than that typically present in individuals with AD. Neuroimaging studies are also useful in differentiating DLB from other disorders, with the characteristic findings being preserved hippocampal volumes on MRI, posterior cerebral hypometabolism and the “posterior cingulate island sign” on FDG-PET imaging, reduced striatonigral uptake on ioflupane SPECT imaging, and reduced cardiac uptake on MIBG imaging. Medical management can be particularly challenging in DLB, as certain medications which are effective for some of the manifestations can worsen other features, yet a comprehensive approach addressing the most problematic features can result in significant clinical improvement and quality of life in some patients. Characterizing prodromal DLB is a high priority as early diagnosis may lead to management strategies being used that could impact symptoms and clinical course more. Prodromal DLB patients will also be attractive candidates for clinical trials as potential disease modifying therapies are refined. 4 Am J Neurodegener Dis 2015;4(Suppl 1): 1-178 Am J Neurodegener Dis 2015;4(Supplementary Issue 1): 1-178 www.ajnd.us /ISSN:2165-591X/2015 International DLB Conf. O.3 DLB Tools for the Clinician James E Galvin Florida Atlantic University, Boca Raton, FL Introduction: Dementia with Lewy bodies (DLB) is a challenge to diagnose with long delays leading to significant patient and caregiver burden. We developed two brief instruments to assist diagnosis and staging of dementia in the clinic setting. The Quick Dementia Rating Systems (QDRS) is a 10-item questionnaire provides a rapid assessment of the presence and severity of dementia. The Lewy Body Composite Risk Score (LBCRS) was developed from autopsy-verified cases to improve the ability to detect DLB as the cause of dementia. Methods: The QDRS and LBCRS were validated in a consecutive series of patients compared with the Clinical Dementia Rating (CDR) and gold standard measures of cognition, motor, function, and behavior. Psychometric properties including floor and ceiling effects; validity, and internal consistency were determined. Receiver operator characteristic curves were used to test discrimination
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