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Parkinsonian and Cerebellar Phenotypes of Probable MSA: An Insight in to Prognostic Factors Based on Autonomic Functions.
Annals of Indian Academy of Neurology ( IF 1.9 ) Pub Date : 2020-05-01 , DOI: 10.4103/aian.aian_34_19 Malligurki Raghurama Rukmani 1 , Ravi Yadav 2 , Binukumar Bhaskarapillai 3 , Pramod Kumar Pal 2 , Talakad N Sathyaprabha 1
Background: Multiple system atrophy is an adult-onset, sporadic, neurodegenerative disorder with parkinsonian (MSA-P) and cerebellar (MSA-C) subtypes. Objective: We aimed to elucidate differences in prognostic factors between MSA subtypes. Methods: The study population comprised 45 probable MSA patients (MSA-P = 22; MSA-C = 23) and 45 healthy controls. Clinical parameters, heart rate variability (HRV), and conventional cardiac autonomic function testing (AFT) were the study tools. Results: Mean age of onset of MSA was 54.7 ± 9 years for MSA-P and 51.9 ± 7 years for MSA-C subgroups. Median disease duration was 2 years in both subgroups. A greater percentage of MSA-P patients (45.5%) had beneficial response to levodopa (P < 0.01). Patients in both subgroups reported significant autonomic disturbances, such as postural symptoms, bladder disturbances, and erectile dysfunction. MSA-P patients had a trend for a greater number of falls and bladder disturbances than MSA-C patients (P = 0.05). Cardiac AFT showed that in MSA-P, 22.2% had definitive and 77.7% had severe autonomic dysfunction, whereas in MSA-C, 9.5% had early, 28.5% had definitive, and 57.1% had severe autonomic dysfunction. HRV analysis showed significant reduction in overall HRV, sympathetic activity, and parasympathetic activity in MSA patients as compared with controls (P < 0.0001). The sympathetic limb was more severely affected in MSA-P patients as compared with MSA-C patients (P < 0.05). Conclusion: Autonomic dysfunction and postural instability, negative prognostic markers, were relatively more common in the MSA-P than in the MSA-C patients. This implies that MSA-P patients have poorer prognosis as compared with MSA-C. Dopaminergic medications can be beneficial in MSA-P patients.
中文翻译：
可能的 MSA 的帕金森病和小脑表型：基于自主神经功能对预后因素的洞察。
背景：多系统萎缩是一种成人发病的散发性神经退行性疾病，具有帕金森病 (MSA-P) 和小脑 (MSA-C) 亚型。目的：我们旨在阐明 MSA 亚型之间预后因素的差异。方法：研究人群包括 45 名可能的 MSA 患者（MSA-P = 22；MSA-C = 23）和 45 名健康对照。临床参数、心率变异性 (HRV) 和常规心脏自主功能测试 (AFT) 是研究工具。结果： MSA-P 亚组的 MSA 平均发病年龄为 54.7 ± 9 岁，MSA-C 亚组为 51.9 ± 7 岁。两个亚组的中位病程为 2 年。更大比例的 MSA-P 患者（45.5%）对左旋多巴有有益反应（P< 0.01)。两个亚组的患者都报告了明显的自主神经紊乱，例如姿势症状、膀胱紊乱和勃起功能障碍。与 MSA-C 患者相比，MSA-P 患者有更多跌倒和膀胱功能障碍的趋势（P = 0.05）。心脏 AFT 显示，在 MSA-P 中，22.2% 有明确的自主神经功能障碍，77.7% 有严重的自主神经功能障碍，而在 MSA-C 中，9.5% 有早期，28.5% 有明确的自主神经功能障碍，57.1% 有严重的自主神经功能障碍。HRV 分析显示，与对照组相比，MSA 患者的总体 HRV、交感神经活动和副交感神经活动显着降低（P < 0.0001）。与MSA-C患者相比，MSA-P患者的交感神经受累更为严重（P < 0.05）。结论：自主神经功能障碍和姿势不稳（阴性预后标志物）在 MSA-P 患者中比在 MSA-C 患者中更为常见。这意味着与 MSA-C 相比，MSA-P 患者的预后较差。多巴胺能药物可能对 MSA-P 患者有益。
更新日期：2020-05-01
Annals of Indian Academy of Neurology ( IF 1.9 ) Pub Date : 2020-05-01 , DOI: 10.4103/aian.aian_34_19 Malligurki Raghurama Rukmani 1 , Ravi Yadav 2 , Binukumar Bhaskarapillai 3 , Pramod Kumar Pal 2 , Talakad N Sathyaprabha 1
Affiliation
Background: Multiple system atrophy is an adult-onset, sporadic, neurodegenerative disorder with parkinsonian (MSA-P) and cerebellar (MSA-C) subtypes. Objective: We aimed to elucidate differences in prognostic factors between MSA subtypes. Methods: The study population comprised 45 probable MSA patients (MSA-P = 22; MSA-C = 23) and 45 healthy controls. Clinical parameters, heart rate variability (HRV), and conventional cardiac autonomic function testing (AFT) were the study tools. Results: Mean age of onset of MSA was 54.7 ± 9 years for MSA-P and 51.9 ± 7 years for MSA-C subgroups. Median disease duration was 2 years in both subgroups. A greater percentage of MSA-P patients (45.5%) had beneficial response to levodopa (P < 0.01). Patients in both subgroups reported significant autonomic disturbances, such as postural symptoms, bladder disturbances, and erectile dysfunction. MSA-P patients had a trend for a greater number of falls and bladder disturbances than MSA-C patients (P = 0.05). Cardiac AFT showed that in MSA-P, 22.2% had definitive and 77.7% had severe autonomic dysfunction, whereas in MSA-C, 9.5% had early, 28.5% had definitive, and 57.1% had severe autonomic dysfunction. HRV analysis showed significant reduction in overall HRV, sympathetic activity, and parasympathetic activity in MSA patients as compared with controls (P < 0.0001). The sympathetic limb was more severely affected in MSA-P patients as compared with MSA-C patients (P < 0.05). Conclusion: Autonomic dysfunction and postural instability, negative prognostic markers, were relatively more common in the MSA-P than in the MSA-C patients. This implies that MSA-P patients have poorer prognosis as compared with MSA-C. Dopaminergic medications can be beneficial in MSA-P patients.
中文翻译：
可能的 MSA 的帕金森病和小脑表型：基于自主神经功能对预后因素的洞察。
背景：多系统萎缩是一种成人发病的散发性神经退行性疾病，具有帕金森病 (MSA-P) 和小脑 (MSA-C) 亚型。目的：我们旨在阐明 MSA 亚型之间预后因素的差异。方法：研究人群包括 45 名可能的 MSA 患者（MSA-P = 22；MSA-C = 23）和 45 名健康对照。临床参数、心率变异性 (HRV) 和常规心脏自主功能测试 (AFT) 是研究工具。结果： MSA-P 亚组的 MSA 平均发病年龄为 54.7 ± 9 岁，MSA-C 亚组为 51.9 ± 7 岁。两个亚组的中位病程为 2 年。更大比例的 MSA-P 患者（45.5%）对左旋多巴有有益反应（P< 0.01)。两个亚组的患者都报告了明显的自主神经紊乱，例如姿势症状、膀胱紊乱和勃起功能障碍。与 MSA-C 患者相比，MSA-P 患者有更多跌倒和膀胱功能障碍的趋势（P = 0.05）。心脏 AFT 显示，在 MSA-P 中，22.2% 有明确的自主神经功能障碍，77.7% 有严重的自主神经功能障碍，而在 MSA-C 中，9.5% 有早期，28.5% 有明确的自主神经功能障碍，57.1% 有严重的自主神经功能障碍。HRV 分析显示，与对照组相比，MSA 患者的总体 HRV、交感神经活动和副交感神经活动显着降低（P < 0.0001）。与MSA-C患者相比，MSA-P患者的交感神经受累更为严重（P < 0.05）。结论：自主神经功能障碍和姿势不稳（阴性预后标志物）在 MSA-P 患者中比在 MSA-C 患者中更为常见。这意味着与 MSA-C 相比，MSA-P 患者的预后较差。多巴胺能药物可能对 MSA-P 患者有益。
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