Neurology Unit, Department of Systems Medicine, University of Roma Tor Vergata, via Montpellier 1, 00133, Rome, Italy.
Neuroradiology Unit, Department of Biomedicine and Prevention, University of Roma Tor Vergata, Rome, Italy.
Neurol Sci. 2021 Mar;42(3):905-910. doi: 10.1007/s10072-020-04985-3. Epub 2021 Jan 14.
Recognition of secondary movement disorders (SMDs) is fundamental either to alleviate disabling disturbances or to treat potentially life-threatening conditions, such as brain tumors. Primary CNS lymphoma (PCNSL) is a rare form of CNS cancer that is often located in subcortical areas, accounting for both neuropsychiatric and motoric disorders. Nevertheless, an overview on PCNSL-related movement disorders (MDs) phenomenology has not been provided yet.
To outline the main features of PCNSL-related MDs.
A retrospective analysis was conducted on a cohort of patients with PCNSL presenting with MDs, including all existing cases identified by a systematic literature review (source: Medline; period: 1946-2020) and two unreported cases. Data on phenomenology, neuroimaging, histology, and clinical course were collected.
A total cohort of fifteen subjects was defined, enrolling thirteen previously described patients extracted from eleven published studies, and our two unreported cases. A parkinsonian syndrome appearing at about 60 years of age, unresponsive to levodopa, associated to other neurological signs, resulted as the most common presentation of PCNSL-related MD. Chorea, dystonia, and dyskinesia occurred less frequently, with some degree of responsiveness to symptomatic treatments. Basal ganglia were involved in most cases and motoric disturbances often ameliorated after tumor mass reduction.
This study identified those features of PCNSL-related MDs that could support an appropriate approach to such a rare condition. In fact, while the outcome remains still poor, the therapeutic scenario of PCNSL is changing; an early diagnosis together with an adequate management will be thus crucial for timely and successful interventions.
识别继发性运动障碍（SMD）对于缓解致残性障碍或治疗潜在危及生命的疾病（如脑肿瘤）至关重要。原发性中枢神经系统淋巴瘤（PCNSL）是一种罕见的中枢神经系统癌症，通常位于皮质下区域，既有神经精神障碍，也有运动障碍。然而，尚未对 PCNSL 相关运动障碍（MD）的表现进行综述。
概述 PCNSL 相关 MD 的主要特征。
对一组表现为 MD 的 PCNSL 患者进行回顾性分析，包括通过系统文献回顾（来源：Medline；时间：1946-2020 年）确定的所有现有病例以及两例未报告的病例。收集了关于表现、神经影像学、组织学和临床病程的数据。
共定义了十五例患者，包括从十一项已发表的研究中提取的十三例以前描述过的患者，以及我们的两例未报告的病例。以帕金森综合征为主要表现，发病年龄约 60 岁，对左旋多巴无反应，伴有其他神经体征，这是 PCNSL 相关 MD 最常见的表现。较少出现舞蹈症、肌张力障碍和运动障碍，对对症治疗有一定的反应。基底节在大多数情况下受累，肿瘤体积缩小后运动障碍常改善。
本研究确定了那些支持对这种罕见疾病进行适当治疗的 PCNSL 相关 MD 特征。事实上，尽管预后仍然很差，但 PCNSL 的治疗方案正在发生变化；早期诊断和适当的管理对于及时和成功的干预至关重要。
