Session Information
Date: Wednesday, June 7, 2017
Session Title: Ataxia
Session Time: 1:15pm-2:45pm
Location: Exhibit Hall C
Objective: To analyze composite autonomic severity score in patients of spinocerebellar ataxia types 1, 2 and 3.
Background: Spinocerebellar ataxia (SCA) is a progressive neurodegenerative disorder characterized by autonomic failure. There is paucity of literature for quantification of autonomic failure applying validated score in SCA. The Composite Autonomic Severity Score (CASS) was developed for laboratory quantification of autonomic failure as a single validated score. This also standardizes for the confounding effects of age and gender. In this context, we evaluated CASS in patients of SCA1, 2 and 3.
Methods: Continuous HR and BP were recorded to determine autonomic function in genetically confirmed SCA1 (n=31, age=35.3±7.82 yrs), SCA2 (n=40, age=33.25±10.45 yrs) and SCA3 (n=9, age=37.11±9.21 yrs) patients. CASS ranges from 0 to 10 and constitutes of sudomotor (0–3), cardiovagal (0–3), and adrenergic (0–4) subscores. The analysis of CASS was done by using MATLAB (R2015a).
Results: The mean total CASS was 4.45±1.69 in SCA1, 4.6±1.26 in SCA2 and 3.78±1.2 in SCA3. Mild autonomic failure were more in SCA3 (44.44%; 4/9) than SCA1 and SCA2. Most of the patients had moderate autonomic failure such as 80.65% (25/31) in SCA1, 85% (34/40) in SCA2 and 55.55% (5/9) in SCA3. More cases of severe failure were seen in SCA1 (2/31) than SCA2 (1/40) whereas none in SCA3 [Table].
Conclusions: Our data suggests moderate generalized autonomic failure (in SCA1 and SCA2) and mild failure (in SCA3) as determined by CASS. This may be a useful index imparting the clinical diagnosis of SCA1, SCA2 and SCA3.
References: 1. Novak P. Quantitative autonomic testing. JoVE (Journal of Visualized Experiments). 2011 Jul 19(53):e2502-e2502.
2. Low PA. Composite autonomic scoring scale for laboratory quantification of generalized autonomic failure. InMayo Clinic Proceedings 1993 Aug 31 (Vol. 68, No. 8, pp. 748-752). Elsevier.
To cite this abstract in AMA style:
D. Tamuli, A. Godiyal, A. Jaryal, A. Srivastava, K. Deepak. The spectrum of composite autonomic severity score in SCA1, SCA2 and SCA3 patients [abstract]. Mov Disord. 2017; 32 (suppl 2). https://www.mdsabstracts.org/abstract/the-spectrum-of-composite-autonomic-severity-score-in-sca1-sca2-and-sca3-patients/. Accessed June 14, 2025.« Back to 2017 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/the-spectrum-of-composite-autonomic-severity-score-in-sca1-sca2-and-sca3-patients/