抗神经束蛋白-186联合中枢和外周脱髓鞘（CCPD）：一个新的实体

IF 1.8 4区医学 Q3 CLINICAL NEUROLOGY

Clinical Neurology and Neurosurgery Pub Date : 2025-03-22 DOI:10.1016/j.clineuro.2025.108856

Aradhya Sekhar Bagchi , A. Nalini , M. Netravathi , Ravi Yadav , J. Saini , C.M. Ravindranadh , Seena vengalil , Pritam Raja

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引用次数: 0

摘要

中央和外周脱髓鞘合并（CCPD）是一种罕见但新颖的疾病，在世界各地的临床实践中越来越得到认可。其发病机制在很大程度上是未知的，但通常与抗副腺蛋白的抗体有关。病例报告：一名28岁的男性患者，最初表现为复发缓解，随后出现进行性神经功能缺损。脑和脊柱MRI显示多发性硬化症的典型病变，伴有多发性颅神经和周围神经增厚和增强。神经传导检查显示脱髓鞘感觉运动多神经病变。抗神经束蛋白抗体组显示抗nf140和抗nf186阳性。结论有中枢神经系统和PNS脱髓鞘征象的患者，应结合MRI和电生理检查检查抗神经束蛋白抗体的存在。

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Anti-neurofascin-186 associated combined central and peripheral demyelination (CCPD): A novel entity

Introduction

Combined Central and Peripheral Demyelination (CCPD) is a rare yet a novel entity that is increasingly being recognized in clinical practice all over the world. The etiopathogenesis is largely unknown, but antibodies against paranodal proteins are usually incriminated.

Case report

A 28-year-old male patient presented with initial relapsing-remitting followed by progressive neurological deficits. MRI of the Brain and spine revealed lesions typical of MS with multiple cranial and peripheral nerve thickening and enhancement. Nerve conduction study revealed demyelinating sensorimotor polyneuropathy. Anti-Neurofascin antibody panel revealed positive anti-NF140 and anti-NF 186.

Conclusion

Cases presenting with features suggestive of CNS and PNS demyelination, should be worked up for the presence of anti-neurofascin antibodies along with MRI and electrophysiological studies.

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来源期刊

Clinical Neurology and Neurosurgery 医学-临床神经学

CiteScore

3.70

自引率

5.30%

发文量

358

审稿时长

46 days

期刊介绍： Clinical Neurology and Neurosurgery is devoted to publishing papers and reports on the clinical aspects of neurology and neurosurgery. It is an international forum for papers of high scientific standard that are of interest to Neurologists and Neurosurgeons world-wide.