1. 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):
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):
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):
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):
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):
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