Acute disseminated encephalomyelitis - A follow-up study of 40 adult patients
Acute disseminated encephalomyelitis - A follow-up study of 40 adult patients
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DOI：
10.1212/wnl.56.10.1313
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发表时间：
2001-05-22
期刊：
影响因子：
9.9
通讯作者：
Storch-Hagenlocher, B
中科院分区：
文献类型：
--
作者：
Schwarz, S;Mohr, A;Storch-Hagenlocher, B
目的：描述急性播散性脑脊髓炎（ADEM）患者的临床、脑脊液和影像学表现以及长期随访，并确定进展为ms的可能预后因素。方法：分析40名诊断为ADEM的成年人（28名女性，平均年龄33.5岁）。通过图表回顾分析临床症状、颅MRI和脑脊液检查结果以及对疾病急性期标准化治疗的反应。随访8 ~ 137个月，最终诊断为ADEM或临床明确的MS。比较ADEM和MS患者两组间的差异。结果：15例患者既往感染（n = 14）或免疫（n = 1）。最常见的临床症状是运动障碍（80%），其次是感觉障碍、脑干症状和共济失调。脑脊液的结果变化很大；20%的患者结果正常。65%的患者寡克隆带阳性。95%的患者在急性期病情有所好转。在26例最终诊断为ADEM的患者中，2例死亡，9例轻度缺陷，3例中度缺陷，12例无剩余症状。最终诊断为ADEM的患者年龄较大，既往感染较多，临床体征为脑干受累，脑脊液白蛋白分数较高，幕下病变较多。结论：许多最初诊断为ADEM的患者在长期随访后发展为临床明确的MS。作者没有发现鉴别多发性硬化症首发与单相ADEM的有用诊断标准。术语ADEM仍然可以作为临床综合征的描述，但在制定可靠的诊断标准之前不应作为一个单独的实体使用。
Objectives: To describe the clinical, CSF, and radiologic findings and long-term follow-up in a cohort of patients with acute disseminated encephalomyelitis (ADEM), and to determine possible prognostic factors for progression to MS. Methods: Forty adults (28 women, mean age 33.5 years) diagnosed with ADEM were analyzed. Clinical symptoms, cranial MRI and CSF findings, and the response to a standardized treatment during the acute phase of the disease were analyzed by chart review. The final diagnosis of ADEM or clinically definite MS was established upon follow-up examination after 8 to 137 months. The patients with ADEM and MS were compared to detect differences between the two groups. Results: Fifteen patients had a preceding infection (n = 14) or immunization (n = 1). The most frequent clinical signs were motor deficit (80%), followed by sensory deficits, brainstem signs, and ataxia. CSF findings were highly variable; normal results were present in 20% of patients. Oligoclonal bands were positive in 65% of patients. Ninety-five percent of all patients improved during the acute phase of the disease. Upon follow-up, 14 patients had developed clinically definite MS. Of the 26 patients with the final diagnosis of ADEM, two patients had died, nine had minor deficits, three had moderate deficits, and 12 patients had no remaining symptoms. Patients with the final diagnosis of ADEM were older, and more often had a preceding infection, clinical signs of brainstem involvement, a higher CSF albumin fraction, and infratentorial lesions. Conclusions: Many patients initially diagnosed with ADEM develop clinically definite MS upon long-term followup. The authors found no useful diagnostic criteria for the differentiation of a first episode of MS from monophasic ADEM. The term ADEM may still be employed as a description of a clinical syndrome, but should not be used as a distinct entity until reliable diagnostic criteria have been developed.