急性播散性脑脊髓炎与多发性硬化的诊断及鉴别诊断

Diagnosis of acute disseminated encephalomyelitis and multiple sclerosis in childhood and their differential diagnosis

目的 根据急性播散性脑脊髓炎 (ADEM)和多发性硬化 (MS)临床及实验室检查特点 ,探讨二者的诊断和鉴别诊断。方法 制定较严格的诊断标准 ,对符合标准的 34例ADEM和 17例MS的临床表现及实验室检查分析比较。结果 ADEM 34例 ,男 19例 ,女 15例 ;MS 17例 ,男 7例 ,女 10例。ADEM与MS起病发热 (6 4.7% ;2 9.4% ) ;头痛 (5 8.8% ;2 3.5 % ) ;意识障碍 (6 4.7% ;5 .9% ) ,其中昏迷 (17.6 % ;0 % ) ;惊厥 (35 .3 % ;0 % )。ADEM起病为广泛性CNS障碍 ,MS常为单一神经症状 ,如脊髓病 6例 ,偏瘫 5例 ,视神经炎 3例等。CSF免疫学检查差异不明显。MRI显示ADEM与MS丘脑病变 (32 .4% ;0 % )。ADEMEMG检查可有周围神经损伤 ,MS不明显。结论 对有前驱感染或接种史、临床出现广泛的CNS改变 ,特别是意识障碍或惊厥 ,MRI示脑白质多发性病灶 ,特别是伴有丘脑病变者 ,应考虑ADEM ;临床以单一神经症状综合征起病、中枢神经系统 (CNS)中至少有两处不相关联的白质病灶 。

Objective Acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS) are both common demyelinating diseases of the central nervous system(CNS). This paper was to study the clinical and lab test features of ADEM and MS so as to improve clinical and differential diagnosis.Methods The clinical features and experimental results were analyzed in 34 cases with ADEM and 17 cases with MS compatible with the established criteria.Results ADEM cases included 19 boys and 15 girls and MS cases 7 boys and 10 girls. Certain clinical features were helpful to differentiate ADEM and MS, such as fever (64.7 %;29.4 %), headache (58.8 %;23.5 %), consciousness change ( 64.7 %;5.9 %) including coma (17.6 %;0 %); and seizures (35.3 %;0 %). ADEM often produced widespread CNS disturbance, but MS usually presented monosymptomatic syndrome, such as 6 cases of myelopathy,5 hemiplegia , and 33 optic neuritis, etc. It was impossible tomake a certain distinction on CSF immunological examinations. Cranial MRI revealed thalamic involvement (ADEM 32.4 %; MS 0 %); ADEM and EMG examination showed peripheral nerve damage in ADEM whereas not any in MS.Conclusion It is suggested that ADEM be considered in the patients presenting previous history of an infection or vaccination, widespread CNS disturbance and alteration of consciousness or seizures in particular, and multifocal white matter lesion on MRI especially with thalamic involvement and that MS be considered with onset of monosymptomatic syndrome, at least two non-associated lesions in white matter, and relapses and remittence.