Diagnosing Dementia in Multiple System Atrophy with MDS Criteria for Parkinson’s Disease Dementia Nicolas Auzou 1,2, Kathy Dujardin 3,4, Roberta Biundo 5, Alexandra Foubert-Samier 1,6,7,8, Caroline Barth 6, Fanny Duval 1, François Tison 1,6,7,8, Luc Defebvre 3,4, Angelo Antonini 5, Wassilios G Meissner 1,6,7,8. 1Service de Neurologie, CHU de Bordeaux, Bordeaux, France 2Univ. de Bordeaux, Laboratoire de Psychologie, Santé et Qualité de Vie, EA4139, Bordeaux, France 3Service de Neurologie et Pathologies du Mouvement, CHRU de Lille, France 4Inserm U1171, Troubles cognitifs dégénératifs et vasculaires, Université de Lille, Lille, France 5Parkinson and Movement Disorders Unit, Fondazione Ospedale San Camillo, Venice, Italy 6Centre de référence atrophie multisystématisée, CHU de Bordeaux, Bordeaux, France 7Univ. de Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293, Bordeaux, France 8CNRS, Institut des Maladies Neurodégénératives, UMR 5293, Bordeaux, France. INTRODUCTION Dementia is an exclusion criterion for MSA according to current consensus diagnostic criteria1 but has been reported with a prevalence ranging between 14 and 18.2%2-3. A MDS-sponsored task force has established a diagnostic procedure of Parkinson’s disease dementia based on a screening checklist (LEVEL-1) and on a detailed cognitive evaluation (LEVEL-2)4. The aim of this study was to assess the usefulness of MDS PDD procedures for diagnosing dementia in MSA patients.   Bordeaux (n=58) Lille (n=30) Venice (n=23) p value Age, y (SD) 64.6 (8.5) 59.8 (7.5)* 64.2 (6.9) 0.026 Time since diagnosis, y (SD) 2.2 (1.9) Time since first symptom, y (SD) 6.1 (2.6) NA 5.3 (3.1) Sample (% male) 51.7% 60% 34.8% 0.18 MSA-P (%) 58.6% 40%$ 78.2% 0.005 Education level, mean (SD) 2.0 (0.8) 1.6 (0.8) 2.1 (0.9) 0.035 MDRS, mean (SD) 135.5 (7.8) 134.5 (6.7) MMSE, mean (SD) 27.1 (2.2) 27.8 (2.1) 26.7 (3.0) 0.22 FAB, mean (SD) 15.7 (1.9) 15.1 (2.1) 12.5 (3.7)§ 0.003 BDI-2 8.5 (5.7) 14.2 (6.8) MADRS 6.4 (4.7) UMSARS I – activities of daily living 21.9 (9.4) 21.8 (7.4) 26.2 (8.2) 0.47 UMSARS II – motor examination 25.2 (7.6) 21.0 (7.4)* 27.0 (8.3) 0.032 UMSARS IV – disability 2.6 (1.1) 2.6 (1.0) 3.4 (1.1) 0.37 METHODS MDS PDD LEVEL-1 criteria MMSE< 26 and at least 2 impaired short tests among : mont backward (cut-off : wrong sequencing, time > 90s or2 omissions), serial 7 substraction (cut-off : 2 incorrect responses), lexical fluency (≤ 9 words in 60s) or clock drawing test (wrong sequencing and/or time pointing), MMSE pentagon copy (failed if no overlap) and MMSE delayed 3-words recall (cut-off : 1 word missing). Cognitive deficits must be severe enough to impact IADL (Pill questionnaire > 2). MDS PDD LEVEL-2 criteria At least 2 abnormal scores (when available) in more than one cognitive domain among : Memory, Attention/Executive functions, Visuospatial functions and Language.   Bordeaux Lille Venice Memory Free and cued recall (5 or 16) Mattis Dementia Rating Scale recall Rey Osterrieth Complex Figure recall Free and cued recall Rey auditory verbal learning test Prose memory Word paired associated task Attention/executive dysfunction Digit forward/backward Spatial span forward/backward Lexical fluency Frontal Assessment Battery Trail Making Test Stroop Digit cancellation Digit Span Ordering Test-B Corsi’s test Trail Making test Raven’s coloured progressive matrices sets Visuospatial function Free-drawn Clock Drawing Test Rey Osterrieth Complex Figure Copy Rey Osterrieth Complex Figure Copy Visual Object and Space Perception Battery (VOSP) Uncompleted letter Language Category fluency (60s) Category fluency (120s) Category fluency task 60s Novelli’s naming task Mood/behavior Beck Depression Inventory-II Montgomery Asberg Depression Rating Scale State-Trait Anxiety Inventory IADL Pill questionnaire ADL/IADL Table 1: Demographic and clinical scores Table 2: Detailed cognitive evluation for LEVEL-2 assesment n LEVEL-1 LEVEL-2 Sensitivity Spécificity PPV NPV MDS PDD (regardless of BDI/MADRS scores) 111 10 (9%) 13 (11,7%) 53,8% 96,9% 70% 94,1% MDS PDD (BDI/MADRS≤ 14) 79 6 (7,6%) 66,7% 97,3% Dujardin adaptation of MDS PDD (regardless of BDI/MADRS scores) 15 (13,5%) 84,6% 95,9% 73,3% 97,9% Dujardin adaptation of MDS PDD (BDI,MADRS≤14) 83,3% 98,6% Table 3: Frequency of dementia according to MDS PDD criteria and Dujardin adaptation (MMSE<27)5 CONCLUSION MDS PDD procedures seem to be be a useful tool for diagnosing dementia in MSA. A full cognitive evaluation remains necessary for MSA patients with high suspicion of dementia buent not detected by LEVEL-1 screening. Executive dysfunctions seem to be the main cognitive deficit in MSA. These results warrant confirmation in a prospective study. 1. Gilman S, Wenning GK, Low PA, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology 2008;71(9):670-676 2. Kitayama M, Wada-Isoe K, Irizawa Y, Nakashima K. Assessment of dementia in patients with multiple system atrophy. Eur J Neurol 2009;16(5):589-594. 3 Kim HJ, Jeon BS, Kim YE, et al. Clinical and imaging characteristics of dementia in multiple system atrophy. Parkinsonism Relat Disord 2013;19(6):617-621. 4 Dubois B, Burn D, Goetz C, et al. Diagnostic procedures for Parkinson's disease dementia: recommendations from the movement disorder society task force. Mov Disord 2007;22(16):2314-2324 5 . Dujardin K, Dubois B, Tison F, et al. Parkinson's disease dementia can be easily detected in routine clinical practice. Mov Disord 2010;25(16):2769-2776. contact: wassilios.meissner@chu-bordeaux.fr