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一项针对血液系统和其他癌症患儿神经表现和针对抗原抗体的初步研究。

A pilot study on neurological manifestations and antibodies against antigens in children with hematological and other cancers.

机构信息

National Centre for Rare Diseases, Superior Institute of Health (ISS), Rome, Italy.

出版信息

Eur J Paediatr Neurol. 2013 Jan;17(1):97-101. doi: 10.1016/j.ejpn.2012.06.006. Epub 2012 Aug 11.

Abstract

BACKGROUND

Paraneoplastic neurological syndromes (PNS) are most commonly recognized in adults with cancer and can often be identified by the presence of serum antibodies to neuronal proteins that are also expressed by the associated tumor. In children: (a) little emphasis is given to the possibility of paraneoplastic neurological involvement; and (b) few studies investigated the presence of anti-neuronal antibodies.

OBJECTIVE

To run a pilot study on the spectrum of PNS and presence of antibodies to neural antigens in children with malignancies.

METHODS

23 children (7 boys; 16 girls, aged 4 months to 16 years) with hematological or other cancers were examined for neurological manifestations and for antibodies to the neuronal antigens that are frequently detected in adult patients with PNS.

RESULTS

Ten of the 23 children had neurological symptoms (and/or positive antibodies): in 6/10 neurological manifestations could be explained by tumor invasion or chemotoxicity or were probably incidental; a child with neuroblastoma developed opsoclonus-myoclonus syndrome without detectable anti-neuronal antibodies; antibodies to a Tr-like cerebellar antigen [associated to no neurological signs and later enuresis], to voltage-gated potassium channels [associated to sensory signs] and to glutamic acid decarboxylase [associated to multifocal myoclonus] were found in one child each. Results were compared with age- and sex-matched control groups.

CONCLUSION

These results suggest that PNS, though surprisingly not so uncommon in children, may be associated with immune responses to distinct neuronal antigens. Further work is needed to determine whether screening for new antibodies to neuronal antigens could be a useful aid in the diagnosis and prognosis of neurological syndromes in children.

摘要

背景

副肿瘤神经系统综合征(PNS)最常发生于患有癌症的成年人,通常可以通过存在针对神经元蛋白的血清抗体来识别,这些蛋白也由相关肿瘤表达。在儿童中:(a)很少强调副肿瘤性神经受累的可能性;(b)很少有研究调查抗神经元抗体的存在。

目的

对恶性肿瘤儿童中 PNS 的谱和神经抗原抗体的存在进行试点研究。

方法

对 23 例(7 名男孩;16 名女孩,年龄 4 个月至 16 岁)血液系统或其他癌症患儿进行了神经表现和神经元抗原抗体的检查,这些抗原抗体在患有 PNS 的成年患者中经常被检测到。

结果

23 例患儿中有 10 例(10/23)出现神经系统症状(和/或阳性抗体):在 6/10 例中,神经系统表现可以通过肿瘤侵袭或化疗毒性来解释,或者可能是偶然的;一例神经母细胞瘤患儿出现眼震-肌阵挛综合征,但未检测到抗神经元抗体;在一例患儿中发现了抗 Tr 样小脑抗原(与无神经症状和随后的遗尿有关)、电压门控钾通道(与感觉症状有关)和谷氨酸脱羧酶(与多灶性肌阵挛有关)的抗体。将结果与年龄和性别匹配的对照组进行了比较。

结论

这些结果表明,PNS 虽然在儿童中并不罕见,但可能与针对不同神经元抗原的免疫反应有关。需要进一步研究以确定是否筛查新的神经元抗原抗体可以成为儿童神经综合征诊断和预后的有用辅助手段。

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