急性播散性脑脊髓炎的临床特征及实验室检查

Clinical Characteristics and Laboratory Dection on Acute Disseminated Encephalomyelitis in Children

目的探讨急性播散性脑脊髓炎(ADEM)患儿的临床和实验室特点。方法对22例有典型临床表现,MRI异常,通过激素治疗明显有效的ADEM患儿的临床表现、实验室检查及预后进行回顾性分析。结果有前驱病史14例(63.6%),以出疹性病毒感染为主;临床表现为共济失调17例(77.3%),意识障碍16例(72.7%);脑脊液异常11例,蛋白轻度升高9例,淋巴细胞升高2例。22例MRI均异常,中丘脑病变占40.9%。16/18例脑电图(EEG)异常(88.9%)。2/4例视神经诱导电位双侧异常。病例均予激素治疗,2例使用静脉丙种球蛋白。死亡率4.5%,后遗症发生率27.2%(6/22例)。结论ADEM临床表现多样,MRI检查对ADEM诊断具有特征性,激素治疗有效,严重病例常有后遗症。应结合病史、年龄、病理组织学、临床表现、MRI病灶位置、范围及形态、预后等与病毒性脑炎、MS加以鉴别。

Objective To analyze the clinical and laboratory features in children with acute disseminated encephalomyelitis （ADEM）. Methods The clinical manifestations, laboratory examination and prognosis of 22 children with ADEM in our hospital from January 2000 to September 2004 were reviewed retrespectively,and these 22 patients＇s had typical clinical manifestation with MRI abnormal and corticost-riod was effective for treatment. Resets Of 22 patients included in this study, 14 patients（63.6% ） had forerunner case history, 17 had atsxia（77.3%） and 16 had conscious disturbance（72.7%）. Eleven cases had abnormal eerebrospinal fluid（CSF） examinations, 9 cases had mild raised protein, and 2 cases were had lymphocytic pleocytosis. Abnormal MRI occurred in all cases （22cases）, thalamic involvement was found in 9 cases （40.9%） ,and 18 patients（88.9% ） had normal EEG. All patients were administered with corticoatfiod, and 2 cases were followed by treatments of intravenous immunoglobulin（IVIG）. The death rate was 4.5%, about 27.2% of the patients had sequal. Conelusiom ADEM is a common inflammation dymyelinating disease in nervous system. It has various clinical manifestations. MRI examination is the specific diagnosis means for ADEM. Corticoatriod is effective for treatment of ADEM, and has a satisfactory prognosis. But sequals usually occurs in some serious cases, even death. ADEM is easily confused with viral encephalitis and multiple sclerosis. Generally, ADEM has forerunner case history of viral infection, ataxia in early-onset, and MRI shows extensive mulifocality lesion, deep gray matter usually is involved. The absence of oligoclonal bands is always the hint of ADEM.