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系统性红斑狼疮中的横贯性脊髓病:临床特征与功能转归

Transverse myelopathy in SLE: clinical features and functional outcomes.

作者信息

Chan K F, Boey M L

机构信息

Department of Rehabilitation Medicine, Tan Tock Seng Hospital, Singapore.

出版信息

Lupus. 1996 Aug;5(4):294-9. doi: 10.1177/096120339600500409.

DOI:10.1177/096120339600500409
PMID:8869901
Abstract

The objective of the study was to examine the clinical features of lupus patients who present with transverse myelopathy (TM) and ascertain functional outcomes when treated early with high dose corticosteroids and/or cyclophosphamide. Case records of nine patients who developed a total of 14 episodes of TM were retrospectively studied. All the patients were female and their ages ranged from 21 to 59 years. Nine episodes of paraparesis, three of tetraparesis, one of numbness and one of neurogenic bladder were reported early in the diagnosis of SLE (median of two years). Neurogenic bowel and bladder and presence of ANA and ds-DNA were invariable. Urodynamics assessment in six patients showed abnormal detrusor behavior in all. CT scans and myelograms were uninformative and CSF studies were normal. ESR and complement levels were insensitive as markers of disease activity. The treatment regimens included pulses of methylprednisolone and/or cyclophosphamide followed by prednisolone and high dose prednisolone from onset. The functional outcomes were uniformly good-with independent ambulation in all except three (who needed assistive devices) and improvement of motor scores. Acute hospital stays were short (range of three to 45 days) whilst only two were referred for inpatient rehabilitation. Bladder abnormalities persisted despite motor recovery and would require long-term review.

摘要

该研究的目的是检查出现横贯性脊髓病(TM)的狼疮患者的临床特征,并确定早期使用大剂量皮质类固醇和/或环磷酰胺治疗时的功能预后。对9例共发生14次TM发作的患者的病例记录进行了回顾性研究。所有患者均为女性,年龄在21岁至59岁之间。在SLE诊断早期(中位时间为两年)报告了9次截瘫发作、3次四肢瘫发作、1次麻木发作和1次神经源性膀胱发作。神经源性肠和膀胱以及ANA和ds-DNA的存在是不变的。6例患者的尿动力学评估显示所有患者逼尿肌行为均异常。CT扫描和脊髓造影无诊断价值,脑脊液检查正常。ESR和补体水平作为疾病活动的标志物不敏感。治疗方案包括从发病开始使用甲基强的松龙和/或环磷酰胺冲击治疗,随后使用强的松和大剂量强的松。功能预后均良好,除3例(需要辅助装置)外所有患者均可独立行走,运动评分有所改善。急性住院时间较短(3至45天),只有2例患者被转诊进行住院康复治疗。尽管运动功能恢复,但膀胱异常仍然存在,需要长期随访。

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