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英国儿童获得性横贯性脊髓病的 2 年前瞻性研究。

Acquired transverse myelopathy in children in the United Kingdom--a 2 year prospective study.

机构信息

Department of Paediatric Neurology, Royal Preston Hospital, Sharoe Green Lane, Preston PR2 9HT, UK.

出版信息

Eur J Paediatr Neurol. 2010 Nov;14(6):479-87. doi: 10.1016/j.ejpn.2009.12.002. Epub 2010 Jan 25.

DOI:10.1016/j.ejpn.2009.12.002
PMID:20089428
Abstract

AIMS

To define the incidence, describe presentation, management and outcome and identify prognostic factors in Acquired Transverse Myelopathy (ATM) in children under 16 years.

METHODS

A prospective population-based surveillance study, involving all consultant paediatric neurologists in the United Kingdom from 1 July 2002 to 30 June 2004.

RESULTS AND DISCUSSION

Response rate was 91%, and 60 children were reported, of whom 41 were included. Median age was 9 years. The incidence of ATM in children under 16 years in confirmed cases is at least 1.72 per million children per year. There was a previously unrecognised male predominance (M:F 25:16). Early evaluation of bladder function is sometimes omitted. MR imaging should include whole spine and brain to maximise diagnostic information. Despite the use of high dose steroids, 25% of cases were left with significant sequelae. Outcome data was available for 36 children in whom recovery was defined as 'complete' in 19, 'good' in 8, 'fair' in 3 and 'poor' in 6. Significant positive prognostic factors were preceding infection, start of recovery within a week of onset, age less than 10 years, and lumbosacral spinal level on clinical assessment. Significant negative predictors were flaccid legs at presentation, sphincter involvement and rapid progression from onset to nadir within 24h.

摘要

目的

定义发病率,描述表现、处理和结果,并确定 16 岁以下儿童获得性横贯性脊髓病(ATM)的预后因素。

方法

一项前瞻性基于人群的监测研究,涉及 2002 年 7 月 1 日至 2004 年 6 月 30 日期间英国所有顾问儿科神经科医生。

结果和讨论

应答率为 91%,报告了 60 例患儿,其中 41 例被纳入。中位年龄为 9 岁。经证实的 16 岁以下儿童 ATM 的发病率至少为每年每百万儿童 1.72 例。存在先前未被认识到的男性优势(M:F 25:16)。膀胱功能的早期评估有时会被忽略。磁共振成像(MRI)应包括整个脊柱和大脑,以最大限度地获取诊断信息。尽管使用了大剂量类固醇,仍有 25%的病例遗留严重后遗症。36 例患儿的预后数据可用,其中 19 例恢复定义为“完全”,8 例为“良好”,3 例为“一般”,6 例为“差”。显著的阳性预后因素是发病前感染、发病后 1 周内开始恢复、年龄小于 10 岁、临床评估时腰骶脊髓水平。显著的负预测因素是发病时出现弛缓性瘫痪、括约肌受累和在 24 小时内从发病到最低点迅速进展。

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