Lotan Itay, Bacon Tamar, Kister Ilya, Levy Michael
New York University Langone Medical Center, Multiple Sclerosis Comprehensive Care Center, New York, NY, USA; Department of Neurology and Neuroimmunology Unit, Rabin Medical Center, Beilinson Campus, Petach Tikva, Israel and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
New York University Langone Medical Center, Multiple Sclerosis Comprehensive Care Center, New York, NY, USA.
Mult Scler Relat Disord. 2020 Nov;46:102578. doi: 10.1016/j.msard.2020.102578. Epub 2020 Oct 13.
Paroxysmal symptoms (PS), defined as short-lasting, recurrent, and stereotyped neurological symptoms, are frequently reported by patients with Neuromyelitis Optica Spectrum Disorder (NMOSD). Their prevalence and spectrum of presentations in NMOSD have not been fully characterized.
Patients with NMOSD, who were members of a closed international Facebook Group, were recruited to complete an anonymous survey on REDCap. Participants were queried regarding demographic and NMOSD-related characteristics and PS history.
The sample consisted of 219 responders with self-reported NMOSD, of whom 134 (63.8%) reported testing positive for AQP4 Antibody. 156 responders (71.9%) reported ≥1 type of PS during the disease course. The most common PS were intermittent tingling/numbness sensation (N=106, 67.9%), followed by involuntary muscle contractions/abnormal posture (N=95, 60.9%), hot/cold/burning sensations (N=87, 55.8%), and shock-like sensations along the spine or limbs (N=77, 49.4%). 150 responders (96% of those with PS) reported that PS were painful; in 82 responders (54.6%), the pain intensity reached ≥ 8/10 and in 40 responders (26.0%) - 10/10 level. PS were most commonly aggravated by fatigue (105 responders, 70.0%), physical activity (N=86, 57.3%), and neck flexion (N=39 responders, 26.0%). 82 patients (52.5% of those with PS) reported having been prescribed one or more medications for PS. Less than 50% reported them to be 'very helpful.'
This survey highlights that PS occurs commonly in NMOSD patients. The symptomatology of PS is diverse. PS are often painful and not adequately treated. Our study represents a novel method to learn more about a rare disease from the patient's perspective. Given the fact that the study was conducted using an anonymous questionnaire and the diagnosis of NMOSD was self-reported by the survey participants, its' results should be regarded as a first step towards the understanding of PS in NMOSD, which should be further validated in a larger, controlled study.
发作性症状（PS）被定义为短暂的、反复发作的、刻板的神经症状，视神经脊髓炎谱系障碍（NMOSD）患者经常报告有此类症状。其在NMOSD中的患病率和表现谱尚未得到充分描述。
招募了一个封闭的国际脸书群组中的NMOSD患者，在REDCap上完成一项匿名调查。询问参与者的人口统计学和与NMOSD相关的特征以及PS病史。
样本包括219名自我报告患有NMOSD的受访者，其中134人（63.8%）报告AQP4抗体检测呈阳性。156名受访者（71.9%）报告在病程中出现过≥1种PS。最常见的PS是间歇性刺痛/麻木感（N = 106，67.9%），其次是不自主肌肉收缩/异常姿势（N = 95，60.9%）、热/冷/灼烧感（N = 87，55.8%）以及沿脊柱或四肢的电击样感觉（N = 77，49.4%）。150名受访者（有PS者的96%）报告PS会引起疼痛；82名受访者（54.6%）的疼痛强度达到≥8/10，40名受访者（26.0%）达到10/10的水平。PS最常见的加重因素是疲劳（105名受访者，70.0%）、体力活动（N = 86，57.3%）和颈部屈曲（39名受访者，26.0%）。82名患者（有PS者的52.5%）报告曾因PS被开具一种或多种药物。不到50%的人报告这些药物“非常有帮助”。
这项调查突出表明PS在NMOSD患者中很常见。PS的症状多种多样。PS通常会引起疼痛且治疗不充分。我们的研究代表了一种从患者角度了解罕见疾病的新方法。鉴于该研究是使用匿名问卷进行的，且NMOSD的诊断是由调查参与者自我报告的，其结果应被视为迈向了解NMOSD中PS的第一步，应在更大规模的对照研究中进一步验证。
