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1型神经纤维瘤病(NF1)与胼胝体肿瘤相关。

Neurofibromatosis type 1 (NF1) associated with tumor of the corpus callosum.

作者信息

Pascual-Castroviejo Ignacio, Pascual-Pascual Samuel-Ignacio

机构信息

Pediatric Neurology Service, University Hospital La Paz, Paseo de Castellana 261, 28046 Madrid, Spain.

出版信息

Childs Nerv Syst. 2012 Dec;28(12):2177-80. doi: 10.1007/s00381-012-1903-9. Epub 2012 Sep 22.

Abstract

INTRODUCTION

Neurofibromatosis type 1 (NF1), one of the most common neurocutaneous disorders, is a multisystemic disease associated with tumors in any organ of the body, especially in the central nervous system and also the peripheral nervous system. Pilocytic astrocytomas have been described in almost all intracranial regions in patients with NF1. However, only a few patients with NF1 and tumor of the corpus callosum have been reported to date.

MATERIAL AND METHODS

An 11-year-old white Spanish boy was evaluated due to a family history of NF1 and low performance test scores in school. He was studied from the neurological and intellectual level points of view.

RESULTS

Magnetic resonance (MR) study revealed a tumor in the anterior-middle portion of the corpus callosum and a Wechsler Intelligence Scale for Children-Revised showed verbal IQ of 92, a performance IQ of 108, and a total IQ of 100. In addition, he showed attention deficit and hyperactivity disorder.

CONCLUSIONS

Tumors of corpus callosum in patients with NF1 are very uncommon. The patient presented in this paper consulted due to family history of NF1, progressive hyperactivity, and below average school performance. The MR study showed tumor in the corpus callosum. Tumor histology was not investigated.

摘要

引言

1型神经纤维瘤病(NF1)是最常见的神经皮肤疾病之一,是一种多系统疾病,与身体任何器官的肿瘤有关,尤其是中枢神经系统和周围神经系统。在NF1患者中,几乎所有颅内区域都曾有过毛细胞型星形细胞瘤的描述。然而,迄今为止,仅有少数NF1合并胼胝体肿瘤的患者被报道。

材料与方法

一名11岁的西班牙白人男孩因有NF1家族史且在学校的测试成绩不佳而接受评估。从神经学和智力水平的角度对他进行了研究。

结果

磁共振(MR)研究显示胼胝体中前部有一个肿瘤,韦氏儿童智力量表修订版显示其言语智商为92,操作智商为108,总智商为100。此外,他还表现出注意力缺陷多动障碍。

结论

NF1患者的胼胝体肿瘤非常罕见。本文所介绍的患者因NF1家族史、进行性多动以及学业成绩低于平均水平前来就诊。MR研究显示胼胝体有肿瘤。未对肿瘤组织学进行研究。

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