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儿童期起病的多发性硬化症:台湾21例报告。

Multiple sclerosis with childhood onset: report of 21 cases in Taiwan.

作者信息

Weng Wen-Chin, Yang Chih-Chao, Yu Tsui-Wen, Shen Yu-Zen, Lee Wang-Tso

机构信息

Department of Pediatrics, University Hospital, Taipei, Taiwan.

出版信息

Pediatr Neurol. 2006 Nov;35(5):327-34. doi: 10.1016/j.pediatrneurol.2006.05.002.

Abstract

Multiple sclerosis is an inflammatory demyelinating disease of the central nervous system. However, the clinical features of childhood-onset multiple sclerosis in Asia have been rarely reported. This report presents our experience in 21 patients with multiple sclerosis (15 females, 6 males, mean age 12.4 +/- 4.5 years) in Taiwan with the onset age before 18. The most common presenting symptoms were limb weakness (62%) and visual disturbance (43%). Poly-symptomatic presentations were found in 16 (76%) patients. Magnetic resonance imaging demonstrated basal ganglion involvement in 33.3% of the patients. Neuroimaging and neurophysiologic evaluations revealed optic nerve involvement in 13 (62%) patients. Only one child had the optico-spinal form. Eighteen (86%) patients had a relapsing remission course, whereas three (14%) patients had secondary progressive course. Three (14%) patients initially diagnosed with acute disseminated encephalomyelitis developed multiple sclerosis after 4 months, 2 years, and 6 years, respectively. In conclusion, childhood multiple sclerosis in Taiwan is frequently poly-symptomatic, and is characterized by a higher ratio of optic nerve and basal ganglion involvement. However, the optico-spinal form of multiple sclerosis occurs rarely in children in Taiwan. Our experience suggests that the chance of relapsing should not be overlooked in patients presenting with clinical and neuroimaging findings suggestive of acute disseminated encephalomyelitis.

摘要

多发性硬化症是一种中枢神经系统的炎性脱髓鞘疾病。然而,亚洲儿童期起病的多发性硬化症的临床特征鲜有报道。本报告介绍了我们对台湾地区21例18岁前起病的多发性硬化症患者(15例女性,6例男性,平均年龄12.4±4.5岁)的诊治经验。最常见的首发症状是肢体无力(62%)和视力障碍(43%)。16例(76%)患者有多症状表现。磁共振成像显示33.3%的患者有基底节受累。神经影像学和神经生理学评估显示13例(62%)患者有视神经受累。仅1例儿童为视神经脊髓型。18例(86%)患者呈复发缓解病程,3例(14%)患者呈继发进展型病程。3例最初诊断为急性播散性脑脊髓炎的患者分别在4个月、2年和6年后发展为多发性硬化症。总之,台湾地区儿童期多发性硬化症常为多症状性,其特征是视神经和基底节受累比例较高。然而,视神经脊髓型多发性硬化症在台湾儿童中很少见。我们的经验表明,对于临床表现和神经影像学表现提示急性播散性脑脊髓炎的患者,不应忽视其复发的可能性。

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