Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea; Neuroscience Center, Samsung Medical Center, Seoul, Republic of Korea; Edmond J. Safra Program in Parkinson's Disease, Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital, UHN, Division of Neurology, University of Toronto, Toronto, Ontario, Canada.
Parkinson's Disease and Movement Disorders Unit, IRCCS Mondino Foundation, Pavia, Italy; Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.
J Neurol Sci. 2022 Feb 15;433:120019. doi: 10.1016/j.jns.2021.120019. Epub 2021 Oct 1.
Although various motor manifestations can be seen in patients with cerebrospinal fluid (CSF) disorders, such as hydrocephalus or intracranial hypotension, the clinical presentation with parkinsonism is not clearly elucidated.
We searched the literature for studies describing the occurrence of parkinsonism in subjects with normal pressure hydrocephalus (NPH), obstructive hydrocephalus, and intracranial hypotension. We analyzed the clinical presentation (particularly with respect to bradykinesia, rigidity, rest tremor, and gait disturbance/postural instability) as well as the response to treatment.
Parkinsonism was most commonly reported in NPH patients. Although gait disturbance/postural instability is a well-known motor symptom of NPH, other cardinal signs include upper limb involvement or asymmetric presentation. As for obstructive hydrocephalus, parkinsonism was mainly observed in subjects with aqueductal stenosis and more often after shunt surgery. Patients with NPH or obstructive hydrocephalus rarely improved with levodopa therapy, while most subjects only improved with shunt surgery. Although the mechanism is still controversial, a functional involvement of nigrostriatal pathway has been hypothesized based on imaging studies and case reports. Brain imaging is also helpful for atypical cases of intracranial hypotension presenting with parkinsonism. Parkinsonism improved after treatment in such cases as well.
Studies exploring the relationship between CSF disorders and parkinsonism are mainly descriptive and their quality is generally poor. However, considering that these disorders can be treated, clinicians' awareness of the differential diagnosis is important and future studies better exploring the underlying pathophysiological mechanisms are warranted. This article is part of the Special Issue "Parkinsonism across the spectrum of movement disorders and beyond" edited by Joseph Jankovic, Daniel D. Truong and Matteo Bologna.
尽管脑脊液（CSF）紊乱患者可能出现各种运动表现，如脑积水或颅内低血压，但帕金森病的临床表现尚不清楚。
我们检索了描述正常压力脑积水（NPH）、梗阻性脑积水和颅内低血压患者发生帕金森病的文献。我们分析了临床表现（特别是运动迟缓、僵硬、静止性震颤和步态障碍/姿势不稳）以及对治疗的反应。
帕金森病最常发生于 NPH 患者。虽然步态障碍/姿势不稳是 NPH 的一种众所周知的运动症状，但其他主要体征包括上肢受累或不对称表现。对于梗阻性脑积水，帕金森病主要发生在导水管狭窄的患者中，且更多见于分流术后。NPH 或梗阻性脑积水患者对左旋多巴治疗很少有效，而大多数患者仅对分流手术有效。尽管机制仍存在争议，但基于影像学研究和病例报告，提出了黑质纹状体通路的功能受累假说。脑成像也有助于诊断不典型的颅内低血压伴帕金森病。此类病例经治疗后帕金森病也有所改善。
探索 CSF 紊乱与帕金森病之间关系的研究主要是描述性的，其质量普遍较差。然而，鉴于这些疾病可以治疗，临床医生对鉴别诊断的认识很重要，需要进一步研究更好地探索潜在的病理生理机制。本文是由 Joseph Jankovic、Daniel D. Truong 和 Matteo Bologna 编辑的“跨越运动障碍谱系及其他”特刊的一部分。
