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[儿童和青少年多发性硬化症中神经系统的受累类型]

[The type of involvement of the nervous system in multiple sclerosis in children and adolescents].

作者信息

Gergont Aleksandra, Nowak Agnieszka, Piotrowska Agata, Kaciński Marek

机构信息

Katedra Neurologii Dzieci i Młodziezy i Klinika Neurologii Dzieciecej, Uniwersytet Jagielloński Collegium Medicum, Kraków.

出版信息

Przegl Lek. 2008;65(11):789-94.

Abstract

INTRODUCTION

The multiple sclerosis (MS) is characterized by variable clinical symptomatology, neuroradiological changes and neuropathological features, what influence the variable course of disease.

THE AIM

It was to describe the type of nervous system involvement in children with MS, considering the clinical manifestation and neuroradiological changes.

MATERIAL AND METHODS

9 children with multiple sclerosis hospitalized in the Department of Pediatric Neurology Chair of Pediatric and Adolescent Neurology, in 2006 and 2007 year were included. There were 5 girls aged 14-17 years and 4 boys aged 10-17 years. The history, clinical examination as well as head MRI were performed in all children, and in 2 children the spinal cord MRI as well. McDonald's criteria were employed. In 8 children oligoclonal bands in the cerebrospinal fluid were examined, in 6 children VEP and in 4 BAEP as well.

RESULTS

The most common clinical manifestations of MS were superficial sensory deficits, vertigo and diplopia. In 4 children with two attacks of symptoms disseminated in OUN, the head MRI revealed disseminated demyelination in the brain and in 1 child also demyelination in the cervical spinal cord. In 3 of patients oligoclonal bands were detected, and also in 3 children pathological VEP. In 2 other children only 1 attack was observed with symptoms disseminated in CNS, correlating with neuroradiological picture, within in 1 of them clinically silent progression of demyelination was revealed in the second MRI. In other children hospitalized after first attack with clinically isolated syndrome, demyelination was disseminated CNS, and in the youngest male patients oligoclonal bands were positive.

CONCLUSIONS

The most common clinical manifestations of MS were superficial sensory deficits, vertigo and visual disturbances, the least typical was organic based psychotic manifestation. The most common MR changes were located in the white matter of centrum semiovale and in corona radiata.

摘要

引言

多发性硬化症(MS)具有多样的临床症状、神经放射学改变和神经病理学特征,这些因素影响着疾病的不同病程。

目的

根据临床表现和神经放射学改变,描述儿童多发性硬化症患者神经系统受累的类型。

材料与方法

纳入2006年和2007年在儿童与青少年神经学系小儿神经学教研室住院的9例多发性硬化症患儿。其中有5名年龄在14 - 17岁的女孩和4名年龄在10 - 17岁的男孩。对所有患儿进行了病史采集、临床检查以及头部MRI检查,2名患儿还进行了脊髓MRI检查。采用了麦克唐纳标准。对8名患儿检测了脑脊液中的寡克隆带,6名患儿检测了视觉诱发电位(VEP),4名患儿检测了脑干听觉诱发电位(BAEP)。

结果

MS最常见的临床表现为浅感觉障碍、眩晕和复视。4例有两次症状发作且病变在空间上散在分布(OUN)的患儿,头部MRI显示脑内有散在脱髓鞘病变,1例患儿颈椎脊髓也有脱髓鞘病变。3例患者检测到寡克隆带,3例患儿VEP异常。另外2例患儿仅观察到1次发作,症状在中枢神经系统(CNS)呈散在分布,与神经放射学表现相符,其中1例在第二次MRI检查时发现有临床无症状的脱髓鞘进展。其他首次发作后以临床孤立综合征入院的患儿,脱髓鞘病变散在于CNS,最年幼的男性患者寡克隆带呈阳性。

结论

MS最常见的临床表现为浅感觉障碍、眩晕和视觉障碍,最不典型的是器质性精神症状表现。最常见的MR改变位于半卵圆中心和放射冠的白质。

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