Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Bologna, Italy.
Neurology Unit, "M. Bufalini" Hospital, AUSL Romagna, Cesena, Italy.
J Neurol. 2023 Jan;270(1):109-129. doi: 10.1007/s00415-022-11347-w. Epub 2022 Aug 30.
The degree of involvement of the autonomic nervous system in progressive supranuclear palsy (PSP) has been investigated in several studies, often providing conflicting results. There is a need for a better characterization of autonomic dysfunction in PSP, to enhance our understanding of this highly disabling neurodegenerative disease including patients' needs and possibly be of value for clinicians in the differential diagnosis among Parkinsonian syndromes.
We applied a systematic methodology to review existing literature on Pubmed regarding autonomic nervous system involvement in PSP.
PSP reported quite frequently symptoms suggestive of autonomic dysfunction in all domains. Cardiovascular autonomic testing showed in some cases a certain degree of impairment (never severe). There was some evidence suggesting bladder dysfunction particularly in the storage phase. Dysphagia and constipation were the most common gastrointestinal symptoms. Instrumental tests seemed to confirm sudomotor and pupillomotor disturbances.
PSP patients frequently reported visceral symptoms, however objective testing showed that not always these reflected actual autonomic impairment. Further studies are needed to better delineate autonomic profile and its prognostic role in PSP.
多项研究已经对进行性核上性麻痹（PSP）中自主神经系统的参与程度进行了研究，这些研究的结果往往相互矛盾。因此，我们需要更好地描述 PSP 中的自主功能障碍，以增强我们对这种高度致残的神经退行性疾病的理解，包括患者的需求，并可能对帕金森综合征的鉴别诊断中的临床医生有价值。
我们采用系统的方法，在 Pubmed 上检索了有关 PSP 中自主神经系统参与的现有文献。
PSP 报告了在所有领域中都有相当频繁的自主功能障碍症状。心血管自主神经测试在某些情况下显示出一定程度的损害（但从未严重）。有一些证据表明，在储存阶段膀胱功能障碍更为常见。吞咽困难和便秘是最常见的胃肠道症状。仪器测试似乎证实了汗腺和瞳孔运动障碍。
PSP 患者经常报告内脏症状，但客观测试表明，并非所有这些症状都反映了实际的自主神经损伤。需要进一步研究以更好地描述 PSP 中的自主神经特征及其预后作用。
