中华医学杂志
中華醫學雜誌
중화의학잡지
National Medical Journal of China
2011年
35期
2464-2467
,共4页
张伟赫%矫毓娟%焦劲松%刘尊敬%汪仁斌
張偉赫%矯毓娟%焦勁鬆%劉尊敬%汪仁斌
장위혁%교육연%초경송%류존경%왕인빈
脊髓炎,横贯性%视神经脊髓炎%临床特点
脊髓炎,橫貫性%視神經脊髓炎%臨床特點
척수염,횡관성%시신경척수염%림상특점
Myelitis,transverse%Neuromyelitis optica%Clinical features
目的 分析超长节段横贯性脊髓炎的临床特点。方法 对2009年9月至2011年3月在中日友好医院神经内科住院的4例首次发病的超长节段横贯性脊髓炎患者的临床、影像学特点以及治疗和预后进行回顾性分析。结果 男1例，女3例，发病年龄为29 ～ 33岁。本病多以肢体瘫痪伴尿便障碍为首发，临床表现包括视力异常、运动、感觉以及尿便障碍等视神经和脊髓受累症状和体征，3例患者血尿酸降低，血清白身免疫抗体谱及NMO-IgG可阳性，头颅MRI无典型脱髓鞘病灶，脊髓MRI表现为长T1，长T2病灶几乎累及脊髓全长，可伴脊髓肿胀，其中2例患者确诊为干燥综合征伴超长节段横贾性脊髓炎。4例患者均经大剂量激素、丙种球蛋白以及免疫抑制剂治疗，3例获得不同程度缓解，1例死亡。结论 超长节段横贯性脊髓炎好发于青年女性，以脊髓受累多见，可合并视神经受累及其他自身免疫性疾病，颅内多无病灶，对激素等免疫抑制剂敏感。
目的 分析超長節段橫貫性脊髓炎的臨床特點。方法 對2009年9月至2011年3月在中日友好醫院神經內科住院的4例首次髮病的超長節段橫貫性脊髓炎患者的臨床、影像學特點以及治療和預後進行迴顧性分析。結果 男1例，女3例，髮病年齡為29 ～ 33歲。本病多以肢體癱瘓伴尿便障礙為首髮，臨床錶現包括視力異常、運動、感覺以及尿便障礙等視神經和脊髓受纍癥狀和體徵，3例患者血尿痠降低，血清白身免疫抗體譜及NMO-IgG可暘性，頭顱MRI無典型脫髓鞘病竈，脊髓MRI錶現為長T1，長T2病竈幾乎纍及脊髓全長，可伴脊髓腫脹，其中2例患者確診為榦燥綜閤徵伴超長節段橫賈性脊髓炎。4例患者均經大劑量激素、丙種毬蛋白以及免疫抑製劑治療，3例穫得不同程度緩解，1例死亡。結論 超長節段橫貫性脊髓炎好髮于青年女性，以脊髓受纍多見，可閤併視神經受纍及其他自身免疫性疾病，顱內多無病竈，對激素等免疫抑製劑敏感。
목적 분석초장절단횡관성척수염적림상특점。방법 대2009년9월지2011년3월재중일우호의원신경내과주원적4례수차발병적초장절단횡관성척수염환자적림상、영상학특점이급치료화예후진행회고성분석。결과 남1례，녀3례，발병년령위29 ～ 33세。본병다이지체탄탄반뇨편장애위수발，림상표현포괄시력이상、운동、감각이급뇨편장애등시신경화척수수루증상화체정，3례환자혈뇨산강저，혈청백신면역항체보급NMO-IgG가양성，두로MRI무전형탈수초병조，척수MRI표현위장T1，장T2병조궤호루급척수전장，가반척수종창，기중2례환자학진위간조종합정반초장절단횡가성척수염。4례환자균경대제량격소、병충구단백이급면역억제제치료，3례획득불동정도완해，1례사망。결론 초장절단횡관성척수염호발우청년녀성，이척수수루다견，가합병시신경수루급기타자신면역성질병，로내다무병조，대격소등면역억제제민감。
Objective To analyze the clinical features of ultra longitudinally extensive transverse myelitis (uLETM). Methods Four first-onset uLETM patients hospitalized during September 2009 and March 2011 were recruited and retrospectively analyzed for clinical and MRI ( magnetic resonance imaging)features, as well as therapeutic profiles and prognoses. Results The male-to-female ratio was 1∶3 and the age-of-onset 29-33 years old. Extremity paralysis and intrinsic sphincter disorders were initially observed. The clinical manifestations include visual, motor, sense and intrinsic sphincter disorders caused by optic nerve and spinal cord lesions. Uric acid decreased in 3 cases. Seropositivity for autoimmune antibody spectrum and NMO-IgG ( neuromyelitis optica-immunoglobulin G) was found in some patients. Spinal MRI showed overall hypointense T1 and hyperintense T2 lesions in spinal cord with partial swelling and negative in brain MRI. Sjogren's syndrome associated with uLETM was diagnosed in 2 patients. Three cases improved after treatment with high-dose corticosteroids, intravenous immunoglobulin and other immunosuppressive agents. One patient died. Conclusion uLETM is commonly found in young women. Spinal cord is frequently affected. And it may occur concurrently with optical abnormalities and other autoimmune diseases, Intracranial parenchyma is rarely affected. The therapy of corticosteroids is recommended.
