Rukmani Malligurki Raghurama, Yadav Ravi, Bhaskarapillai Binukumar, Pal Pramod Kumar, Sathyaprabha Talakad N
Department of Neurophysiology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, Karnataka, India.
Department of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, Karnataka, India.
Ann Indian Acad Neurol. 2020 May-Jun;23(3):289-295. doi: 10.4103/aian.AIAN_34_19. Epub 2020 Jun 10.
Multiple system atrophy is an adult-onset, sporadic, neurodegenerative disorder with parkinsonian (MSA-P) and cerebellar (MSA-C) subtypes.
We aimed to elucidate differences in prognostic factors between MSA subtypes.
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.
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 ( < 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 ( = 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 ( < 0.0001). The sympathetic limb was more severely affected in MSA-P patients as compared with MSA-C patients ( < 0.05).
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.
多系统萎缩是一种成人起病、散发性神经退行性疾病，有帕金森型（MSA-P）和小脑型（MSA-C）两种亚型。
我们旨在阐明多系统萎缩各亚型在预后因素方面的差异。
研究人群包括45例可能的多系统萎缩患者（MSA-P = 22例；MSA-C = 23例）和45例健康对照者。临床参数、心率变异性（HRV）和传统心脏自主神经功能测试（AFT）为研究工具。
MSA-P亚组多系统萎缩的平均发病年龄为54.7±9岁，MSA-C亚组为51.9±7岁。两个亚组的疾病中位持续时间均为2年。更大比例的MSA-P患者（45.5%）对左旋多巴有良好反应（<0.01）。两个亚组的患者均报告有明显的自主神经功能障碍，如姿势症状、膀胱功能障碍和勃起功能障碍。MSA-P患者比MSA-C患者有更多跌倒和膀胱功能障碍的趋势（=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%有严重自主神经功能障碍；而在MSA-C中，9.5%有早期自主神经功能障碍，28.5%有明确自主神经功能障碍，57.1%有严重自主神经功能障碍。HRV分析显示，与对照组相比，多系统萎缩患者的总体HRV、交感神经活动和副交感神经活动显著降低（<0.0001）。与MSA-C患者相比，MSA-P患者的交感神经部分受影响更严重（<0.05）。
自主神经功能障碍和姿势不稳作为不良预后标志物，在MSA-P患者中比在MSA-C患者中相对更常见。这意味着与MSA-C患者相比，MSA-P患者预后较差。多巴胺能药物对MSA-P患者可能有益。
