Acute transverse myelitis in demyelinating diseases among the Chinese

Acute transverse myelitis in demyelinating diseases among the Chinese
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DOI:
10.1007/s00415-011-6093-y
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发表时间:
2011-12-01
影响因子:
6
通讯作者:
Hu, X.
Hu, X.
中科院分区:
医学2区
文献类型:
--
作者:
Li, R.;Qiu, W.;Hu, X.

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本研究的目的是描述中国急性横肌萎缩症(ATM)患者的人口统计学、临床和预后特征。对脱髓鞘疾病数据库中ATM患者的临床资料进行回顾性分析。确定了67例随访时间超过2年的ATM患者。在我们的队列中,视神经肌病相关ATM(NMO-ATM)的频率很高(40.3%)。复发性ATM(R-ATM)在特发性ATM中常见(69.0%,20/29),女性占多数。R-ATM合并脊髓纵向广泛性病变(LESCL)者NMO-IgG阳性率高,脊髓病变多累及中央灰质,远期功能障碍发生率高,与NMO-ATM相似。在无LESCL的RTM中,NMO-IgG的低血清阳性、优先累及外周白色物质和相对较好的神经恢复与多发性硬化相关ATM(MS-ATM)一致。急性部分性横肌萎缩(APTM)和急性完全性横肌萎缩(ACTM)患者向MS的转化率不显著(16.7 vs. 6.3%,P = 0.753),而LESCL(OR = 11.4,P = 0.028)与向NMO的转化显著相关。LESCL的存在是显示达到兰金3风险较高的唯一变量(风险比:2.5,95% CI:1.0-6.1)。中国ATM患者的人口统计学、临床和预后特征与西方人群不同。特发性R-ATM在中国很常见,是一种异质性实体,与MS-ATM和NMO-ATM具有部分临床、脊柱MRI和预后特征。脊髓病变的长度,而不是APTM/ACTM,可能是一个预后因素与临床结果和长期残疾在我们的人口。
The aim of the study was to characterize the demographic, clinical, and prognostic features of Chinese patients with acute transverse myelitis (ATM). The clinical data from ATM patients in a demyelinating disease database were analyzed retrospectively. Sixty-seven ATM patients with a follow-up duration longer than 2 years were identified. The frequency of neuromyelitis optica-related ATM (NMO-ATM) was high in our cohort (40.3%). Recurrent ATM (R-ATM), with a female predominance, was common in total idiopathic ATM (69.0%, 20/29). In R-ATM with longitudinally extensive spinal cord lesions (LESCLs), the high seropositivity of NMO-IgG, spinal cord lesions mostly involved the central gray matter and severer long-term disability were similar to NMO-ATM. In RTM without LESCLs, low seropositivity of NMO-IgG, preferentially involvement of the peripheral white matter and relative better neurological recovery were consistent with multiple sclerosis-related ATM (MS-ATM). The transition rates to MS in patients with acute partial transverse myelitis (APTM) and acute complete transverse myelitis (ACTM) were not significant (16.7 vs. 6.3%, P = 0.753), while LESCLs (OR = 11.4, P = 0.028) were significantly correlated with transition to NMO. The presence of LESCLs was the only variable showing a higher risk for reaching Rankin 3 (hazard ratio: 2.5, 95% CI: 1.0-6.1). Chinese patients with ATM had demographic, clinical, and prognostic features different from those in Western populations. Idiopathic R-ATM, common in Chinese, is a heterogeneous entity that shares partial clinical, spinal MRI and prognostic features with MS-ATM and NMO-ATM. The length of spinal cord lesion, rather than APTM/ACTM, may be a prognostic factor associated with clinical outcome and long-term disability in our population.