Interrelationships between survival, sex and blood pressure in patients with multiple system atrophy

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.

Download Full-text

Pulsed Wave Doppler Ultrasound Is Useful to Assess Vasomotor Response in Patients with Multiple System Atrophy and Well Correlated with Tilt Table Study

The Scientific World JOURNAL ◽

10.1100/2012/548529 ◽

2012 ◽

Vol 2012 ◽

pp. 1-8 ◽

Cited By ~ 1

Author(s):

Ke-Vin Chang ◽

Wen-Shiang Chen ◽

Ruey-Meei Wu ◽

Ssu-Yuan Chen ◽

Hsiu-Yu Shen ◽

...

Keyword(s):

Blood Pressure ◽

Heart Rate ◽

Correlation Analysis ◽

Multiple System Atrophy ◽

Cardiovascular Response ◽

Vasomotor Response ◽

Tilt Table ◽

Hemodynamic Variables ◽

Pulsed Wave Doppler ◽

Head Up Tilt

The study aim was to assess sympathetic vasomotor response (SVR) by using pulsed wave Doppler (PWD) ultrasound in patients with multiple system atrophy (MSA) and correlate with the tilt table study. We recruited 18 male patients and 10 healthy men as controls. The SVR of the radial artery was evaluated by PWD, using inspiratory cough as a provocative maneuver. The response to head-up tilt was studied by a tilt table with simultaneous heart rate and blood pressure recording. The hemodynamic variables were compared between groups, and were examined by correlation analysis. Regarding SVR, MSA patients exhibited a prolonged latency and less heart rate acceleration following inspiratory cough. Compared with the tilt table test, the elevation of heart rate upon SVR was positively correlated to the increase of heart rate after head-up tilt. The correlation analysis indicated that the magnitude of blood pressure drop from supine to upright was positively associated with the SVR latency but negatively correlated with the heart rate changes upon SVR. The present study demonstrated that blunted heart rate response might explain MSA's vulnerability to postural challenge. PWD may be used to predict cardiovascular response to orthostatic stress upon head-up tilt in MSA patients.

Download Full-text

Twenty-four-hour ambulatory blood pressure and heart rate profiles in diagnosing orthostatic hypotension in Parkinson's disease and multiple system atrophy

European Journal of Neurology ◽

10.1111/ene.13135 ◽

2016 ◽

Vol 24 (1) ◽

pp. 90-97 ◽

Cited By ~ 16

Author(s):

E. Vichayanrat ◽

D. A. Low ◽

V. Iodice ◽

E. Stuebner ◽

E. M. Hagen ◽

...

Keyword(s):

Blood Pressure ◽

Parkinson’S Disease ◽

Heart Rate ◽

Parkinson's Disease ◽

Multiple System Atrophy ◽

Orthostatic Hypotension ◽

Ambulatory Blood Pressure

Download Full-text

Vertical pons hyperintensity and hot cross bun sign in cerebellar-type multiple system atrophy and spinocerebellar ataxia type 3

10.21203/rs.2.21614/v3 ◽

2020 ◽

Author(s):

Atsuhiko Sugiyama ◽

Hajime Yokota ◽

Yoshitaka Yamanaka ◽

Hiroki Mukai ◽

Tatsuya Yamamoto ◽

...

Keyword(s):

Blood Pressure ◽

Multiple System Atrophy ◽

Spinocerebellar Ataxia ◽

High Intensity ◽

Spinocerebellar Ataxia Type ◽

Motor Symptoms ◽

Spinocerebellar Ataxia Type 3 ◽

Early Stages ◽

Cerebellar Type ◽

Type 3

Abstract Background The “hot cross bun” (HCB) sign, a cruciform hyperintensity in the pons on magnetic resonance imaging (MRI), has gradually been identified as a typical finding in multiple system atrophy, cerebellar-type (MSA-C). Few reports have evaluated the sensitivity of an HCB, including a cruciform hyperintensity and vertical line in the pons, which precedes a cruciform hyperintensity, in the early stages of MSA-C. Moreover, the difference in frequency and timing of appearance of an HCB between MSA-C and spinocerebellar ataxia type 3 (SCA3) has not been fully investigated.Methods This study investigated the time at which an HCB and orthostatic hypotension (OH) appeared in 41 patients with MSA-C, based on brain MRI and head-up tilt test. The MRI findings were compared with those of 26 patients with SCA3. The pontine signal findings on T2-weighted MRI were graded as 0 (no change), 1 (a vertical T2 high-intensity line), or 2 (a cruciform T2 high-intensity line), with grades 1 or 2 considered as an HCB. OH 30/15 was defined as a decrease in systolic blood pressure of > 30 mmHg or diastolic blood pressure of > 15 mmHg.Results Among the 24 patients with MSA-C within 2 years from the onset of motor symptoms, an HCB was detected in 91.7%, whereas OH 30/15 was present in 60.0%. Among the 36 patients with MSA-C within 3 years from the onset of motor symptoms, a grade 2 HCB was detected in 66.7% of those with MSA-C but in none of those with SCA-3.Conclusions HCB is a highly sensitive finding for MSA-C, even in the early stages of the disease. A grade 2 HCB in the early stage is an extremely specific finding for differentiating MSA-C from SCA-3.

Download Full-text

The vertical pons hyperintensity and hot cross bun sign in cerebellar-type multiple system atrophy and spinocerebellar ataxia type 3

10.21203/rs.2.21614/v1 ◽

2020 ◽

Author(s):

Atsuhiko Sugiyama ◽

Hajime Yokota ◽

Yoshitaka Yamanaka ◽

Hiroki Mukai ◽

Tatsuya Yamamoto ◽

...

Keyword(s):

Blood Pressure ◽

Multiple System Atrophy ◽

Spinocerebellar Ataxia ◽

High Intensity ◽

Spinocerebellar Ataxia Type ◽

Motor Symptoms ◽

Spinocerebellar Ataxia Type 3 ◽

Early Stages ◽

Cerebellar Type ◽

Type 3

Abstract Background The “hot cross bun” (HCB) sign, a cruciform hyperintensity in the pons on magnetic resonance imaging (MRI), has gradually been identified as a typical finding in multiple system atrophy, cerebellar-type (MSA-C). Few reports have evaluated the sensitivity of an HCB including a cruciform hyperintensity and vertical line in the pons, which precedes a cruciform hyperintensity, in the early stages of MSA-C. Moreover, the difference in frequency and timing of appearance of an HCB between MSA-C and spinocerebellar ataxia type 3 (SCA3) has not been fully investigated. Methods This study investigated the time at which an HCB and orthostatic hypotension (OH) appeared in 41 patients with MSA-C, based on brain MRI and head-up tilt test. The MRI findings were compared with those of 26 patients with SCA3. The pontine signal findings on T2-weighted MRI were graded as 0 (no change), 1 (a vertical T2 high-intensity line), or 2 (a cruciform T2 high-intensity line), with grades 1 or 2 considered as an HCB. OH 30/15 was defined as a decrease in systolic blood pressure of > 30 mmHg or diastolic blood pressure of > 15 mmHg. Results Among the 24 patients with MSA-C within 2 years from the onset of motor symptoms, an HCB was detected in 91.7%, whereas OH 30/15 was present in 60.0%. Among the 36 patients with MSA-C within 3 years from the onset of motor symptoms, a grade 2 HCB was detected in 66.7% of those with MSA-C but in none of those with SCA-3. Conclusions HCB is a highly sensitive finding for MSA-C, even in the early stages of the disease. A grade 2 HCB in the early stage is an extremely specific finding for differentiating MSA-C from SCA-3.

Download Full-text