Background

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.

Objective

To outline the main features of PCNSL-related MDs.

Methods

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.

Results

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.

Conclusions

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.

中文翻译：

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原发性中枢神经系统淋巴瘤的运动障碍：2例未报告的病例和文献复习

背景

识别继发性运动障碍（SMD）是缓解残疾障碍或治疗可能威胁生命的疾病（例如脑瘤）的基础。原发性中枢神经系统淋巴瘤（PCNSL）是中枢神经系统癌症的一种罕见形式，通常位于皮层下区域，导致神经精神病和运动障碍。但是，尚未提供PCNSL相关运动障碍（MDs）现象学的概述。

目的

概述与PCNSL相关的MD的主要功能。

方法

回顾性分析了一群患有MDs的PCNSL患者，包括通过系统的文献综述确定的所有现有病例（来源：Medline；时期：1946-2020）和两个未报告的病例。收集有关现象学，神经影像学，组织学和临床过程的数据。

结果

定义了总共15名受试者，招募了13名先前描述的患者，这些患者选自11项已发表的研究，以及2例未报告的病例。帕金森综合症出现在大约60岁，对左旋多巴无反应，与其他神经系统症状有关，是PCNSL相关MD的最常见表现。舞蹈病，肌张力障碍和运动障碍的发生频率较低，对症状治疗有一定程度的反应。在大多数情况下，基底神经节受累，运动障碍通常在肿瘤减轻后得到缓解。

结论

这项研究确定了PCNSL相关MD的那些特征，这些特征可以支持针对这种罕见情况的适当方法。实际上，尽管结果仍然很差，但PCNSL的治疗方案正在发生变化。因此，早期诊断以及适当的管理对于及时，成功地进行干预至关重要。