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[副肿瘤性和自身免疫性感觉神经元病或神经节病。早期检测的重要性]

[Paraneoplastic and disimmune sensory neuronopathies or ganglionopathies. Importance of an early detection].

作者信息

Urdiales-Urdiales J, Tejada-García J, Urdiales-Sánchez S, Hernández-Rodríguez J, Soto-García J, Piquero-Fernández J

机构信息

Complejo Asistencial Universitario de León, 24001 León, España.

Hospital Universitario Marqués de Valdecilla, Santander, España.

出版信息

Rev Neurol. 2019 Dec 1;69(11):435-441. doi: 10.33588/rn.6911.2019141.

Abstract

INTRODUCTION

Sensory ganglionopathies or sensory neuronopathies are subacute acquired diseases of the dorsal root ganglion, frequently associated with disinmune, paraneoplastic and toxic agents. Patients present sensory alteration of asymmetric distribution and early ataxia. Early identification is essential, as they may announce an underlying neoplasia or autoimmune disease.

AIM

To study asymmetries of the sensory nervous action potential (SNAP) of nerve pairs and the relationship amplitude of ulnar sensory/ulnar motor potential (USMAR) with serial electroneurophysiological studies for the early diagnosis of sensory ganglionopathies.

PATIENTS AND METHODS

Six patients with sensory ganglionopathies were retrospectively studied with electroneurophysiological studies: four paraneoplastic cases with positivity for onconeuronal antibodies, one associated with Sjogren's syndrome and two idiopathic.

RESULTS

Electroneurophysiological studies showed axonal sensory involvement in all cases, with asymmetry > 50% in SNAP amplitude in two pairs of nerves in four cases and normal motor with USMAR < 0.71 in five cases. Serial electroneurophysiological studies were essential in the diagnosis of two cases in the beginning of the disease with mild sensory symptoms.

CONCLUSIONS

This work evidences the importance of the study of asymmetries in the amplitude of the SNAP of nerve pairs, the USMAR and the serial electroneurophysiological studies in the early diagnosis of sensory ganglionopathies, to further identification of the disinmune and onconeuronal associated antibodies with the nervous system affection to search for hidden neoplasia.

摘要

引言

感觉神经节病或感觉神经元病是背根神经节的亚急性获得性疾病,常与自身免疫性、副肿瘤性和毒性因素相关。患者表现为不对称分布的感觉改变和早期共济失调。早期识别至关重要,因为它们可能提示潜在的肿瘤或自身免疫性疾病。

目的

研究神经对的感觉神经动作电位(SNAP)的不对称性以及尺神经感觉/尺神经运动电位(USMAR)的关系幅度,通过系列神经电生理研究用于感觉神经节病的早期诊断。

患者与方法

对6例感觉神经节病患者进行回顾性神经电生理研究:4例副肿瘤性病例肿瘤神经元抗体呈阳性,1例与干燥综合征相关,2例为特发性。

结果

神经电生理研究显示所有病例均有轴索性感觉受累,4例中有2例两对神经的SNAP幅度不对称>50%,5例运动功能正常,USMAR<0.71。系列神经电生理研究对疾病初期有轻度感觉症状的2例诊断至关重要。

结论

这项研究证明了研究神经对SNAP幅度的不对称性、USMAR以及系列神经电生理研究在感觉神经节病早期诊断中的重要性,有助于进一步识别与神经系统受累相关的自身免疫性和肿瘤神经元抗体,以寻找隐匿性肿瘤。

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