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  • Composite autonomic severit...
    Tamuli, Dibashree; Kaur, Manpreet; Jaryal, Ashok K.; Srivastava, Achal K.; Deepak, Kishore K.

    Journal of clinical neuroscience, July 2023, 2023-Jul, 2023-07-00, 20230701, Letnik: 113
    Journal Article

    •Composite Autonomic Severity Score (CASS) was assessed for the first time in SCA1 and SCA2 patients.•SCA patients were found to have majorly a moderate autonomic failure.•There was a significant impairment in the baroreflex dependent autonomic loop with a predominantly normal functioning non – baroreflex dependent autonomic loop in SCA1 and SCA2 patients.•Sudomotor abnormalities was found to be more in SCA1 than SCA2 patients. The aim of the present study was to evaluate the integrity of autonomic nervous system in spinocerebellar ataxia (SCA) type 1 and 2 patients using battery of autonomic function tests and their comprehensive scoring using composite autonomic severity score (CASS). Battery of autonomic function test comprising of cardiovascular (baroreflex dependent and non-baroreflex dependent) and sudomotor functions were assessed in age and gender-matched SCA1 (n = 31), SCA2 (n = 40) patients along with healthy controls (n = 40). To assess the grade of autonomic abnormalities, the composite autonomic severity score (CASS) was computed using the results of the standard autonomic function tests. We found reduction in baroreflex dependent autonomic reactivity parameters predominantly a significant fall of systolic blood pressure (<0.001) and lower 30:15 ratio (<0.001) during head up tilt in both the SCA1 and SCA2 as compared to controls. On sudomotor assessment, distal leg latency of sweat response was prolonged in SCA1 than SCA2 patients. Moderate generalized autonomic failure was commonly found amongst SCA1 (80.65%) and SCA2 (85%) patients. Severe autonomic failure was found to be more in SCA1 (6.45%) than SCA2 (2.50%) patients. Cardiovascular autonomic function assessment in SCA1 and SCA2 patients revealed a significant impairment in the baroreflex loop integrity. Severity scoring using CASS suggests the existence of moderate autonomic failure in majority of both SCA1 and SCA2 patients.