Department of Neurology, University of New Mexico, Albuquerque, NM.
McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX.
Neurologist. 2021 Sep 7;26(5):161-166. doi: 10.1097/NRL.0000000000000333.
Movement disorders (MDs) have been described in demyelinating diseases (DDs). However, data is lacking in the effective treatment of these MD as well as in a potential correlation between DD lesions localization and the phenomenology of the MD and its response to treatment.
Retrospective review of 185 patients with MD and DD seen at our center over a period of 7 years. Clinical imaging, medications, and therapeutic responses to both MD and DD treatments were reviewed.
Of the 185 patients, 62 were excluded because of a diagnosis of spasticity without any other MD. One hundred twenty three patients with DD (75% female, age 48.8±12.8 y) had one or more MD. The most common MD was ataxia followed by isolated tremor. Forty-two patients (34%) received any treatment for MD, 29 (69%) of which responded at least partially to a first MD agent and 78.6% responded at least partially to a second or third agent. Responders to the first MD therapy were more likely to have a lesion in the basal ganglia or the cerebellum, and less likely to have a lesion in the brainstem or the spinal cord, but these results could be biased by a lower-than-expected frequency of tonic spasms in our series. No correlation between DD lesions localization and the phenomenology of the MD was discovered.
MD are common in DD and are frequently overlooked or undertreated. MD in this sample have a 69% therapeutic response to a first trial. Greater awareness of potential therapeutic options is needed to decrease disability.
脱髓鞘疾病（DD）中已描述到运动障碍（MD）。然而，这些 MD 的有效治疗方法以及 DD 病变定位与 MD 的表现及其对治疗的反应之间的潜在相关性的数据尚缺乏。
对我们中心在 7 年期间诊治的 185 例 MD 和 DD 患者进行回顾性分析。回顾了临床影像学、药物以及 MD 和 DD 治疗的反应。
185 例患者中，有 62 例因诊断为痉挛而无其他 MD 被排除在外。123 例患有 DD（75%为女性，年龄 48.8±12.8y）的患者有 1 种或多种 MD。最常见的 MD 是共济失调，其次是孤立性震颤。42 例（34%）患者接受了任何 MD 治疗，其中 29 例（69%）至少对首剂 MD 药物有部分反应，78.6%至少对第二或第三剂药物有部分反应。对首剂 MD 治疗有反应的患者更有可能有基底节或小脑病变，而不太可能有脑干或脊髓病变，但这些结果可能因本研究中痉挛性抽搐的频率低于预期而存在偏差。未发现 DD 病变定位与 MD 表现之间存在相关性。
MD 在 DD 中很常见，但常被忽视或治疗不足。本研究中，MD 对首剂试验的治疗反应率为 69%。为减少残疾，需要更充分地认识潜在的治疗选择。
