Lazzeri Giulia, Franco Giulia, Difonzo Teresa, Carandina Angelica, Gramegna Chiara, Vergari Maurizio, Arienti Federica, Naci Anisa, Scatà Costanza, Monfrini Edoardo, Dias Rodrigues Gabriel, Montano Nicola, Comi Giacomo P, Saetti Maria Cristina, Tobaldini Eleonora, Di Fonzo Alessio
Neurology Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.
Centro Dino Ferrari, Neuroscience Section, Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy.
Front Neurol. 2022 Jun 16;13:912820. doi: 10.3389/fneur.2022.912820. eCollection 2022.
Multiple System Atrophy (MSA) is a rare neurodegenerative disease, clinically defined by a combination of autonomic dysfunction and motor involvement, that may be predominantly extrapyramidal (MSA-P) or cerebellar (MSA-C). Although dementia is generally considered a red flag against the clinical diagnosis of MSA, in the last decade the evidence of cognitive impairment in MSA patients has been growing. Cognitive dysfunction appears to involve mainly, but not exclusively, executive functions, and may have different characteristics and progression in the two subtypes of the disease (i.e., MSA-P and MSA-C). Despite continued efforts, combining imaging studies as well as pathological studies, the physiopathological bases of cognitive involvement in MSA are still unclear. In this view, the possible link between cardiovascular autonomic impairment and decreased cognitive performance, extensively investigated in PD, needs to be clarified as well. In the present study, we evaluated a cohort of 20 MSA patients (9 MSA-P, 11 MSA-C) by means of a neuropsychological battery, hemodynamic assessment (heart rate and arterial blood pressure) during rest and active standing and bedside autonomic function tests assessed by heart rate variability (HRV) parameters and sympathetic skin response (SSR) in the same experimental session. Overall, global cognitive functioning, as indicated by the MoCA score, was preserved in most patients. However, short- and long-term memory and attentional and frontal-executive functions were moderately impaired. When comparing MSA-P and MSA-C, the latter obtained lower scores in tests of executive functions and verbal memory. Conversely, no statistically significant difference in cardiovascular autonomic parameters was identified between MSA-P and MSA-C patients. In conclusion, moderate cognitive deficits, involving executive functions and memory, are present in MSA, particularly in MSA-C patients. In addition, our findings do not support the role of dysautonomia as a major driver of cognitive differences between MSA-P and MSA-C.
多系统萎缩（MSA）是一种罕见的神经退行性疾病，临床上由自主神经功能障碍和运动受累共同定义，可主要为锥体外系型（MSA-P）或小脑型（MSA-C）。尽管痴呆通常被认为是MSA临床诊断的警示信号，但在过去十年中，MSA患者存在认知障碍的证据不断增加。认知功能障碍似乎主要但并非仅涉及执行功能，并且在该疾病的两种亚型（即MSA-P和MSA-C）中可能具有不同的特征和进展。尽管经过持续努力，结合影像学研究以及病理学研究，MSA认知受累的生理病理基础仍不清楚。从这个角度来看，在帕金森病中广泛研究的心血管自主神经功能损害与认知能力下降之间的可能联系也需要阐明。在本研究中，我们通过神经心理测验组、静息和主动站立时的血流动力学评估（心率和动脉血压）以及在同一实验环节中通过心率变异性（HRV）参数和交感皮肤反应（SSR）评估的床边自主神经功能测试，对一组20例MSA患者（9例MSA-P，11例MSA-C）进行了评估。总体而言，大多数患者的蒙特利尔认知评估量表（MoCA）评分所显示的整体认知功能得以保留。然而，短期和长期记忆以及注意力和额叶执行功能受到中度损害。比较MSA-P和MSA-C时，后者在执行功能和言语记忆测试中得分较低。相反，MSA-P和MSA-C患者之间在心血管自主神经参数方面未发现统计学上的显著差异。总之，MSA存在中度认知缺陷，涉及执行功能和记忆，尤其是在MSA-C患者中。此外，我们的研究结果不支持自主神经功能障碍作为MSA-P和MSA-C之间认知差异的主要驱动因素的作用。
