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全身性紅斑性狼瘡併發大範圍脊髓侵犯之急性橫斷性脊髓炎:病例報告

Diffused Involvement of the Spinal Cord in a Patient with Lupus-Related Acute Transverse Myelitis:A Case Report

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摘要


急性橫斷性脊髓炎 ( acute transverse myelitis; ATM ) 是全身性紅斑性狼瘡 ( systemic lupus erythematosus; SLE ) 罕見但嚴重的併發症。磁振造影是診斷與追蹤急性橫斷性脊髓炎最好的工具。近年來,由於早期診斷,以及早期給予高劑量類固醇與細胞毒殺劑治療 ( high dose corticosteroid plus cytotoxic agents ),患者的治療成效已有顯著進步。 本個案為21歲女性,診斷為全身性紅斑性狼瘡已有4年,89年7月初發生急性雙下肢無力,雙下肢感覺異常以及尿液滯留。胸腰椎磁振造影診斷為急性橫斷性脊髓炎;而且受影響的脊髓,起自第3胸髓,終至脊髓圓椎 ( conus medullaris )。個案於發病後第2天,開始接受2次高劑量類固醇,以及1次細胞毒殺劑的治療,並於發病後第18天轉入復健科,開始復健治療。經過2個月的治療與訓練,個案雙下肢的肌力由0分進步至4-5分,可以拿四腳柺杖走路,不需他人扶持,並且日常生活能夠完全自理。膀胱功能由原來不反應性膀胱,進步到可以靠恥骨上輕敲刺激與括約肌肌肉鬆弛劑來幫助解尿,個案最後餘尿小於50mL。但是仍有頻尿與尿失禁的情形,因此以抑制逼尿肌高張反射的藥物來增加膀胱容量,減少解尿頻率,並且改善逼尿肌高張反射造成的尿失禁情形。 急性橫斷性脊髓炎一般只侵犯1至4節的胸髓或頸髓。本病例報告一位全身性紅斑性狼瘡患者,併發罕見的大範圍的急性橫斷性脊髓炎,經由積極的治療與復健之後,最後仍然有令人滿意的復原結果。

並列摘要


Acute transverse myelitis ( ATM ) is a rare but severe complication of systemic lupus erythema-tosus ( SLE ). Magnetic resonance imaging ( MRI ) is the leading imaging study for diagnosing ATM and monitoring the treatment and/or the progression of the disease. Lesions of the spinal cord of ATM are usually less than 4 spinal segments. Currently, high dose corticosteroid plus cytotoxic agents is recommended to obtain the maximal therapeutic efficacy. A case of lupus-related ATM with diffuse spinal cord involvement is presented. A 21-year-old woman had a 4-year history of SLE. Acute onset of weakness and numbness of bilateral lower limbs were found since July 1, 2000. Neurological examination was suggestive of thoracic spinal cord lesion with signs of both upper and lower motor neuron disorders. MRI of the spine revealed cord swelling and hypersignal lesions with diffuse involvement from T3 to conus medullaris on T2 weighted images. Lupus-related ATM was diagnosed and two courses of pulse corticosteroid therapy and one course of pulse cytotoxic agent therapy were given. She was transferred to our rehabilitation unit 18 days after onset of ATM. With participation in a comprehensive rehabilitation program for 2 months, she experienced marked improvement in clinic symptoms, motor functions, bladder functions and independence in her activities of daily living. The muscle power of bilateral lower limbs improved from grade 2 to grade 4-5. She could walk with a quad cane independently. We concluded that early aggressive therapy with high dose corticosteroid plus cytotoxic agents and a comprehensive rehabilitation program should be prescribed for patients with lupus-related ATM.

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