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一名患有脑脊髓神经根神经病且临床病程呈复发-缓解型的患者:血清和脑脊液抗中性糖鞘脂抗体与临床复发的相关性

A Patient With Encephalomyeloradiculoneuropathy Exhibiting a Relapsing-Remitting Clinical Course: Correlation of Serum and Cerebrospinal Fluid Anti-Neutral Glycosphingolipids Antibodies With Clinical Relapse.

作者信息

Nanaura Hitoki, Kataoka Hiroshi, Shima Sayuri, Iwasa Naoki, Eura Nobuyuki, Sugie Kazuma, Mutoh Tatsuro, Ueno Satoshi

机构信息

Department of Neurology, Nara Medical University, Kashihara, Japan.

Department of Neurology, Fujita Health University School of Medicine, Toyoake, Japan.

出版信息

Front Neurol. 2018 Apr 4;9:206. doi: 10.3389/fneur.2018.00206. eCollection 2018.

Abstract

Several patients who had a progressive clinical course involving both the central and peripheral nervous systems have been reported, but the diagnostic marker has been remained uncertain. More recently, such patients were reported to have namely "encephalomyeloradiculoneuropathy (EMRN)" associated with anti-neutral glycosphingolipid (GSL) antibodies. These antibodies were reported to disappear from the serum in the recovery phase, but whether this finding applies to the cerebrospinal fluid (CSF) remains uncertain. We describe a 67-year-old man with EMRN in whom we measured anti-neutral GSL antibodies in serial serum and CSF samples. During the disease course, the optical densities of the positive band against the background intensity ratio (-<0.3; ±≥0.3 to <0.6; +≥0.6 to <1.0; 2+≥1.0 to <2.0; 3 +≥2.0) for serum and CSF anti-lactosylceramide (LacCer) antibodies were found to be as follows: 2+ and 1+ at the first admission, ± and - when the consciousness level improved after immunotherapy, - and 1+ at clinical relapse, and ± and - when the consciousness level improved after immunotherapy. This is the first time to document that clinical relapse occurred in EMRN, and at this time the negative anti-LacCer antibodies in CSF after the first course of immunotherapy turned positive, but this was not seen in serum samples.

摘要

已有数例临床病程呈进行性、累及中枢和周围神经系统的患者被报道,但诊断标志物仍不明确。最近,有报道称这类患者患有与抗中性糖鞘脂(GSL)抗体相关的“脑脊髓神经根神经病(EMRN)”。据报道,这些抗体在恢复期会从血清中消失,但这一发现是否适用于脑脊液(CSF)仍不确定。我们描述了一名67岁的EMRN男性患者,我们对其连续的血清和脑脊液样本中的抗中性GSL抗体进行了检测。在疾病过程中,血清和脑脊液抗乳糖基神经酰胺(LacCer)抗体相对于背景强度比的阳性条带光密度(-<0.3;±≥0.3至<0.6;+≥0.6至<1.0;2+≥1.0至<2.0;3+≥2.0)如下:首次入院时为2+和1+,免疫治疗后意识水平改善时为±和-,临床复发时为-和1+,免疫治疗后意识水平改善时为±和-。这是首次记录到EMRN发生临床复发,此时首次免疫治疗后脑脊液中抗LacCer抗体阴性转为阳性,但血清样本中未出现这种情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4fb1/5893640/20938e45c91e/fneur-09-00206-g001.jpg

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