Objective: The aim of this study is to estimate survival among patients with multiple system atrophy-parkinsonian type (MSA-P) or cerebellar type MSA (MSA-C) in relation to blood pressure (BP) measurements, by sex. Methods: A cohort of 99 MSA patients was studied retrospectively. Their BP measurements were obtained during prolonged (40 min, vertical position) drug-free tilt testing. We used K-M survival curves and Cox regression to calculate adjusted (to age of onset) hazard ratios (HRs) of BP measurements on time to death by MSA subtype and sex. Results: Fifty-two MSA patients were males and 47 were females. Sixty-three of them had MSA-P and 36 had MSA-C. The mean age at motor symptom onset was 61.1 ± 10.4 years, and mean disease duration at the time of BP assessment was 8.0 ± 4.7 years. The 2 study groups (MSA-P and MSA-C) did not differ significantly in age at MSA onset, sex ratio, or disease duration. Survival time did not differ between the groups {medians: 12 years (95% confidence interval [CI]: 8–28) and 10 years (95% CI: 8–13), respectively}. The MSA-P group showed a trend towards better survival for males (log-rank p = 0.0925). The maximal diastolic orthostatic BP decline during tilt testing had a borderline positive association with death risk among MSA-C males (adjusted HR = 1.18, p = 0.0665), and systolic BP after 10 min in a supine position had a significant positive association with death risk among MSA-P males (adjusted HR = 1.06, p = 0.0354). Conclusions: The findings of a sex-based difference in the effect of BP on death risk may be important for adjusting the therapeutic approach to MSA patients.

1.
Coon
EA
,
Sletten
DM
,
Suarez
MD
,
Mandrekar
JN
,
Ahlskog
JE
,
Bower
JH
, et al.
Clinical features and autonomic testing predict survival in multiple system atrophy
.
Brain
.
2015
;
138
(
Pt 12
):
3623
31
. .
2.
Goldstein
DS
,
Holmes
C
,
Sharabi
Y
,
Wu
T
.
Survival in synucleinopathies: a prospective cohort study
.
Neurology
.
2015
;
85
(
18
):
1554
61
. .
3.
Low
PA
,
Reich
SG
,
Jankovic
J
,
Shults
CW
,
Stern
MB
,
Novak
P
, et al.
Natural history of multiple system atrophy in the USA: a prospective cohort study
.
Lancet Neurol
.
2015
;
14
:
710
9
.
4.
Watanabe
H
,
Saito
Y
,
Terao
S
,
Ando
T
,
Kachi
T
,
Mukai
E
, et al.
Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients
.
Brain
.
2002
;
125
(
Pt 5
):
1070
83
. .
5.
Wenning
GK
,
Geser
F
,
Krismer
F
,
Seppi
K
,
Duerr
S
,
Boesch
S
, et al.
The natural history of multiple system atrophy: a prospective European cohort study
.
Lancet Neurol
.
2013
;
12
(
3
):
264
74
. .
6.
Calandra-Buonaura
G
,
Guaraldi
P
,
Sambati
L
,
Lopane
G
,
Cecere
A
,
Barletta
G
, et al.
Multiple system atrophy with prolonged survival: is late onset of dysautonomia the clue?
Neurol Sci
.
2013
;
34
(
10
):
1875
8
. .
7.
Kim
HJ
,
Jeon
BS
.
Multiple system atrophy with prolonged survival
.
Mov Disord
.
2012
;
27
(
14
):
1834
. .
8.
Petrovic
IN
,
Ling
H
,
Asi
Y
,
Ahmed
Z
,
Kukkle
PL
,
Hazrati
LN
, et al.
Multiple system atrophy-parkinsonism with slow progression and prolonged survival: a diagnostic catch
.
Mov Disord
.
2012
;
27
(
9
):
1186
90
. .
9.
Colosimo
C
,
Berardelli
A
.
Autonomic failure and reduced survival in multiple system atrophy
.
Brain
.
2015
;
138
(
Pt 12
):
3466
8
. .
10.
Figueroa
JJ
,
Singer
W
,
Parsaik
A
,
Benarroch
EE
,
Ahlskog
JE
,
Fealey
RD
, et al.
Multiple system atrophy: prognostic indicators of survival
.
Mov Disord
.
2014
;
29
(
9
):
1151
7
. .
11.
Coon
EA
,
Nelson
RM
,
Sletten
DM
,
Suarez
MD
,
Ahlskog
JE
,
Benarroch
EE
, et al.
Sex and gender influence symptom manifestation and survival in multiple system atrophy
.
Auton Neurosci
.
2019
:
49
52
.
12.
Freeman
R
,
Wieling
W
,
Axelrod
FB
,
Benditt
DG
,
Benarroch
E
,
Biaggioni
I
, et al.
Consensus statement on the definition of orthostatic hypotension, neurally mediated syncope and the postural tachycardia syndrome
.
Clin Auton Res
.
2011
;
21
(
2
):
69
72
. .
13.
Fanciulli
A
,
Jordan
J
,
Biaggioni
I
,
Calandra-Buonaura
G
,
Cheshire
WP
,
Cortelli
P
, et al.
Consensus statement on the definition of neurogenic supine hypertension in cardiovascular autonomic failure by the american autonomic society (AAS) and the european federation of autonomic societies (EFAS)
.
Clin Auton Res
.
2018
;
28
:
355
62
.
14.
Gilman
S
,
Wenning
GK
,
Low
PA
,
Brooks
DJ
,
Mathias
CJ
,
Trojanowski
JQ
, et al.
Second consensus statement on the diagnosis of multiple system atrophy
.
Neurology
.
2008
;
71
(
9
):
670
6
. .
15.
Goldstein
DS
,
Pechnik
S
,
Holmes
C
,
Eldadah
B
,
Sharabi
Y
.
Association between supine hypertension and orthostatic hypotension in autonomic failure
.
Hypertension
.
2003
;
42
(
2
):
136
42
. .
16.
Fedorowski
A
,
Stavenow
L
,
Hedblad
B
,
Berglund
G
,
Nilsson
PM
,
Melander
O
.
Orthostatic hypotension predicts all-cause mortality and coronary events in middle-aged individuals (the malmo preventive project)
.
Eur Heart J
.
2010
;
31
(
1
):
85
91
. .
17.
Liu
R
,
Umbach
DM
,
Peddada
SD
,
Xu
Z
,
Tröster
AI
,
Huang
X
, et al.
Potential sex differences in nonmotor symptoms in early drug-naive Parkinson disease
.
Neurology
.
2015
;
84
(
21
):
2107
15
. .
18.
Loke
H
,
Harley
V
,
Lee
J
.
Biological factors underlying sex differences in neurological disorders
.
Int J Biochem Cell Biol
.
2015
;
65
:
139
50
. .
19.
Ndayisaba
JP
,
Fanciulli
A
,
Granata
R
,
Duerr
S
,
Hintringer
F
,
Goebel
G
, et al.
Sex and age effects on cardiovascular autonomic function in healthy adults
.
Clin Auton Res
.
2015
;
25
(
5
):
317
26
. .
20.
Ong
KL
,
Tso
AW
,
Lam
KS
,
Cheung
BM
.
Gender difference in blood pressure control and cardiovascular risk factors in Americans with diagnosed hypertension
.
Hypertension
.
2008
;
51
(
4
):
1142
8
. .
21.
Gibbons
CH
,
Freeman
R
.
Clinical implications of delayed orthostatic hypotension: a 10-year follow-up study
.
Neurology
.
2015
;
85
(
16
):
1362
7
. .
22.
Pavy-Le
TA
,
Piedvache
A
,
Perez-Lloret
S
,
Calandra-Buonaura
G
,
Cochen-De Cock
V
,
Colosimo
C
, et al.
New insights into orthostatic hypotension in multiple system atrophy: a European multicentre cohort study
.
J Neurol Neurosurg Psychiatry
.
2016
;
87
:
554
61
.
23.
Schrag
A
,
Wenning
GK
,
Quinn
N
,
Ben-Shlomo
Y
.
Survival in multiple system atrophy
.
Mov Disord
.
2008
;
23
(
2
):
294
6
. .
24.
Media Center World Health Statistics. Large gains in life expectancy. New release. 15-5-2014. WHO
.
25.
Fanciulli
A
,
Wenning
GK
. Treatment. In:
Wenning
GK
,
Fanciulli
A
, editors.
Multiple system atrophy
.
Vienna
:
Springer-Verlag
;
2014
. p.
169
94
.
26.
Roncevic
D
,
Palma
JA
,
Martinez
J
,
Goulding
N
,
Norcliffe-Kaufmann
L
,
Kaufmann
H
.
Cerebellar and parkinsonian phenotypes in multiple system atrophy: similarities, differences and survival
.
J Neural Transm
.
2014
;
121
(
5
):
507
12
. .
27.
O’Sullivan
SS
,
Massey
LA
,
Williams
DR
,
Silveira-Moriyama
L
,
Kempster
PA
,
Holton
JL
, et al.
Clinical outcomes of progressive and multiple system atrophy
.
Brain
.
2008
;
131
(
Pt 5
):
1362
72
.
28.
Ben-Shlomo
Y
,
Wenning
GK
,
Tison
F
,
Quinn
NP
.
Survival of patients with pathologically proven multiple system atrophy: a meta-analysis
.
Neurology
.
1997
;
48
(
2
):
384
93
.
29.
Kim
HJ
,
Jeon
BS
,
Lee
JY
,
Yun
JY
.
Survival of Korean patients with multiple system atrophy
.
Mov Disord
.
2011
;
26
(
5
):
909
12
. .
30.
Iodice
V
,
Lipp
A
,
Ahlskog
JE
,
Sandroni
P
,
Fealey
RD
,
Parisi
JE
, et al.
Autopsy confirmed multiple system atrophy cases: mayo experience and role of autonomic function tests
.
J Neurol Neurosurg Psychiatry
.
2012
;
83
(
4
):
453
9
. .
31.
Norcliffe-Kaufmann
L
,
Kaufmann
H
.
Is ambulatory blood pressure monitoring useful in patients with chronic autonomic failure?
Clin Auton Res
.
2014
;
24
(
4
):
189
92
. .
32.
Palma
JA
,
Gomez-Esteban
JC
,
Norcliffe-Kaufmann
L
,
Martinez
J
,
Tijero
B
,
Berganzo
K
, et al.
Orthostatic hypotension in parkinson disease: how much you fall or how low you go?
Mov Disord
.
2015
;
30
(
5
):
639
45
. .
33.
Colosimo
C
,
Tiple
D
,
Wenning
GK
.
Management of multiple system atrophy: state of the art
.
J Neural Transm
.
2005
;
112
(
12
):
1695
704
. .
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.