Department of Neurology, University of Miami, Miami, FL, USA, 1120 NW 14(th) Street, 13th Floor, Miami FL 33136, USA; Jacobs Multiple Sclerosis Center for Treatment and Research, Department of Neurology, Jacobs School of Medicine and Biomedical Sciences, State University of New York (SUNY), Buffalo, NY, USA, 1010 Main St, 2nd Floor, Buffalo NY 14202, USA.
Department of Neurology, University of Miami, Miami, FL, USA, 1120 NW 14(th) Street, 13th Floor, Miami FL 33136, USA.
Mult Scler Relat Disord. 2021 Nov;56:103254. doi: 10.1016/j.msard.2021.103254. Epub 2021 Sep 4.
Peripheral nervous system (PNS) abnormalities in Multiple Sclerosis (MS) have been reported in case reports and small case series over the past several decades. Little is known, however, about the prevalence of electrodiagnostic abnormalities in patients with MS, including not only demyelinating neuropathies such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) but also axonal peripheral neuropathy and sympathetic dysfunction.
This is an observational, cross-sectional study with the objective of identifying the prevalence of the electrodiagnostic abnormalities in predominantly Hispanic MS patients in Miami, Florida. Electrodiagnostic data including nerve conduction study (NCS), electromyography (EMG) and sympathetic skin response (SSR) information was prospectively collected in 18 patients (16 females; 43.7±15.2 years) with a diagnosis of MS compared to 18 healthy (16 females; 39.9±11 years), age- and height-matched controls. The study was offered to all-comers in the MS Clinic over a period of 3 months, regardless of clinical suspicion for an underlying neuropathic process, in an effort to estimate the prevalence of abnormalities. Demographic data including age, sex, race/ethnicity was evaluated in addition to MS-specific characteristics including MS subtype, duration of disease, duration of therapy, clinical symptoms and laboratory data.
There were no significant differences in baseline characteristics of patients and controls for age (p=0.4) and height (164.0±6.4 vs 162.3±4.6 centimeters; p=0.3). The mean disease duration was 106±27 months (median 107 months; range 5-336 months). The mean Expanded Disability Status Scale (EDSS) was 2.4±1.87 (median: 2.5; range 1.0-6.5). The ethnicity of patients (15 Hispanic, 3 non-Hispanic) and controls (13 Hispanic, 5 non-Hispanic; p=0.56) was similar. The frequency of electrophysiological axonal polyneuropathy (PN) was 77.8% (14/18 patients), and 85.6% of these patients had clinical sensory symptoms. Interestingly, 1 patient had previously unrecognized CIDP. All 18 patients displayed prolonged SSR latencies consistent with autonomic dysfunction. Thirteen patients (72.2%) reported autonomic symptoms such as bladder abnormalities and blood pressure fluctuations.
The prevalence of electrodiagnostic abnormalities, especially axonal polyneuropathy, in the MS population may be higher than traditionally considered. The relationship between axonal polyneuropathy and central axonopathy in the context of neurodegeneration in MS should be further explored. Analytic studies may identify common symptomatic and pathophysiologic etiologies to further understanding and potentially guide treatment of MS subtypes with PNS involvement.
过去几十年来，已有病例报告和小病例系列研究报道多发性硬化症（MS）患者存在周围神经系统（PNS）异常。然而，人们对 MS 患者电诊断异常的发生率知之甚少，包括脱髓鞘性神经病（如慢性炎症性脱髓鞘性多发性神经病[CIDP]），还包括轴索性周围神经病和自主神经功能障碍。
这是一项观察性、横断面研究，目的是确定佛罗里达州迈阿密以西班牙语为主要语言的 MS 患者电诊断异常的发生率。前瞻性收集了 18 例 MS 患者（16 名女性；43.7±15.2 岁）和 18 例健康对照者（16 名女性；39.9±11 岁）的电诊断数据，包括神经传导研究（NCS）、肌电图（EMG）和交感皮肤反应（SSR）信息。该研究在 3 个月的时间内向 MS 诊所的所有患者提供，无论是否有潜在的周围神经病变的临床怀疑，以估计异常的发生率。除了 MS 特异性特征（如 MS 亚型、疾病持续时间、治疗持续时间、临床症状和实验室数据）外，还评估了年龄、性别、种族/民族等人口统计学数据。
患者和对照组的基线特征在年龄（p=0.4）和身高（164.0±6.4 与 162.3±4.6 厘米；p=0.3）方面无显著差异。平均疾病持续时间为 106±27 个月（中位数为 107 个月；范围为 5-336 个月）。平均扩展残疾状况量表（EDSS）评分为 2.4±1.87（中位数：2.5；范围为 1.0-6.5）。患者（15 名西班牙裔，3 名非西班牙裔）和对照组（13 名西班牙裔，5 名非西班牙裔；p=0.56）的种族相似。电生理学轴索性多发性神经病（PN）的发生率为 77.8%（18 例患者中的 14 例），这些患者中有 85.6%存在临床感觉症状。有趣的是，1 例患者存在先前未被识别的 CIDP。所有 18 例患者的 SSR 潜伏期均延长，提示存在自主神经功能障碍。13 例（72.2%）患者报告有自主神经症状，如膀胱异常和血压波动。
MS 患者的电诊断异常发生率，尤其是轴索性多发性神经病，可能高于传统观点。在 MS 神经退行性变的背景下，轴索性多发性神经病与中枢轴索病之间的关系应进一步探讨。分析性研究可能会发现常见的症状和病理生理病因，从而进一步了解和可能指导有周围神经系统受累的 MS 亚型的治疗。
