Red flags for multiple system atrophy.
The clinical diagnosis of multiple system atrophy (MSA) is fraught with difficulty and there are no pathognomonic features to discriminate the parkinsonian variant (MSA-P) from Parkinson's disease (PD). Besides the poor response to levodopa, and the additional presence of pyramidal or cerebellar signs (ataxia) or autonomic failure as major diagnostic criteria, certain other clinical features known as "red flags" or warning signs may raise the clinical suspicion of MSA. To study the diagnostic role of these features in MSA-P versus PD patients, a standardized red flag check list (RFCL) developed by the European MSA Study Group (EMSA-SG) was administered to 57 patients with probable MSA-P and 116 patients with probable PD diagnosed according to established criteria. Those red flags with a specifity over 95% were selected for further analysis. Factor analysis was applied to reduce the number of red flags. The resulting set was then applied to 17 patients with possible MSA-P who on follow-up fulfilled criteria of probable MSA-P. Red flags were grouped into related categories. With two or more of six red flag categories present specificity was 98.3% and sensitivity was 84.2% in our cohort. When applying these criteria to patients with possible MSA-P, 76.5% of them would have been correctly diagnosed as probable MSA-P 15.9 (+/-7.0) months earlier than with the Consensus criteria alone. We propose a combination of two out of six red flag categories as additional diagnostic criteria for probable MSA-P.
Köllensperger
Martin
M
Section for Clinical Neurobiology, Department of Neurology, Innsbruck Medical University, Austria.
Geser
Felix
F
Seppi
Klaus
K
Stampfer-Kountchev
Michaela
M
Sawires
Martin
M
Scherfler
Christoph
C
Boesch
Sylvia
S
Mueller
Joerg
J
Koukouni
Vasiliki
V
Quinn
Niall
N
Pellecchia
Maria Teresa
MT
Barone
Paolo
P
Schimke
Nicole
N
Dodel
Richard
R
Oertel
Wolfgang
W
Dupont
Erik
E
Østergaard
Karen
K
Daniels
Christine
C
Deuschl
Günther
G
Gurevich
Tanya
T
Giladi
Nir
N
Coelho
Miguel
M
Sampaio
Cristina
C
Nilsson
Christer
C
Widner
Håkan
H
Sorbo
Francesca Del
FD
Albanese
Alberto
A
Cardozo
Adriana
A
Tolosa
Eduardo
E
Abele
Michael
M
Klockgether
Thomas
T
Kamm
Christoph
C
Gasser
Thomas
T
Djaldetti
Ruth
R
Colosimo
Carlo
C
Meco
Giuseppe
G
Schrag
Anette
A
Poewe
Werner
W
Wenning
Gregor K
GK
D000368:Aged; D002524:Cerebellar Ataxia / Q000175:diagnosis; D015331:Cohort Studies; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000145:classification / Q000175:diagnosis*; D009460:Neurologic Examination / Q000706:statistics & numerical data*; D010300:Parkinson Disease / Q000145:classification / Q000175:diagnosis*; D020734:Parkinsonian Disorders / Q000145:classification / Q000175:diagnosis*; D012680:Sensitivity and Specificity; D012791:Shy-Drager Syndrome / Q000175:diagnosis