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急性播散性脑脊髓炎(acute disseminated encephalomyelitis, ADEM)是一种中枢神经系统自身免疫性脱髓鞘性疾病,儿童为主要受累者。因本病临床症状非特异且缺乏有效的生化指标,故目前儿童ADEM的诊断缺乏金标准,预后判断困难;既往儿童ADEM的文献多是小样本量和短期随访,本文报道较大样本量和较长随访时间的研究结果,期望进一步阐释儿童ADEM的临床特点,并对随访到的患儿进行分组,对可能影响本病预后的多种因素进行分析。现作报道。
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56例感染后发病,其中上呼吸道感染52例,肺炎3例(包括支原体肺炎1例),腹泻1例;3例有疫苗接种史无感染;4例同时有疫苗接种史及上呼吸道感染诱因;14例(18.2%)无明确诱因。
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诱因:病前感染77.9%,疫苗接种9.1%;临床症状:发热39.0%,肢体活动障碍37.7%,惊厥33.8%,意识障碍24.7%,头痛头晕19.5%,二便障碍10.4%,口眼歪斜7.8%,视物不清5.2%,共济失调5.2%,精神行为异常3.9%;神经系统体征:肌肉减退37.7%,病理征23.4%,脑膜刺激征9.1%。
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69例患儿入院后接受腰穿CSF检查,其中34例(49.3%)有核细胞数增高,16例(23.2 %)蛋白增高。15例CSF与血清同步送检寡克隆区带(OB)、碱性髓鞘蛋白(MBP),仅1例血清和CSF均OB阳性,该例患儿CSF常规、生化正常,其他14例血清和CSF的OB、MBP均为阴性,其中1例存在CSF的IgG鞘内合成。5例检测CSF及血清的髓鞘少突胶质细胞糖蛋白(MOG)抗体,CSF均阴性,2例血清阳性。16例检测血清支原体IgM抗体,其中10例阳性。6例检测CSF支原体IgM抗体,均为阴性。
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55例入院后接受脑电图检查,36例(65.5%)异常,表现为轻重不等的背景慢波,偶见局灶性异常放电。29例接受肌电图检查,异常11例(37.9%),表现为双侧对称性CMAP波幅低,或运动感觉神经传导速度减慢,或F波波幅低或出波率低。15例患儿接受视觉诱发电位检查,7例(46.7%)有异常,表现为P100潜伏期延长或伴波形分化差。
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76例入院后接受头颅MRI检查,66例(86.8%)异常(见图 1),56例接受脊髓MRI检查,27例(48.2%)异常。10例头颅MRI正常患儿,依据脊髓MRI异常信号诊断。
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75例患儿在对症、支持治疗基础上,采用免疫治疗方案,10例患儿仅用糖皮质激素治疗,3例患儿仅用免疫球蛋白治疗,62例患儿同时应用免疫球蛋白联合糖皮质激素治疗。应用糖皮质激素治疗患儿后续泼尼松1~2 mg·kg-1·d-1口服,逐渐减量,总疗程3~5月。
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77例患儿,4例表现为ADEM多相型:第1例病史3年,停药2年半再发;第2例病史半年,停药10 d再发;第3例病史10个月,减药量过程中反复;第4例病史2个月,治疗好转后复查头颅MRI见新病灶。4例中有3例检测了CSF的OB、MBP和IgG鞘内合成,均正常;2例检测了CSF和血清MOG,1例为血清阳性、CSF阴性,另1例血清及CSF均为阴性。
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随访到患儿41例,随访时间为病后6个月至6年6个月,根据随访结果分为2组:痊愈组和不完全痊愈组,其中痊愈组31例,患儿临床症状消失,无治疗;不完全痊愈组包括运动能力下降2例,病后尿床1例,抽搐伴语言运动能力下降1例,头痛1例,无症状、口服泼尼松治疗中1例,病情反复需继续治疗1例,严重双下肢无力、萎缩伴二便障碍1例,死亡2例(1例治疗稳定数月可上学,突发抽搐、呼吸衰竭死亡,另1例病情重,治疗中死亡)。
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2组患儿性别、发病年龄、病前诱因、临床主要症状、CSF白细胞及蛋白水平、四肢肌电图、头颅MRI差异均无统计学意义(P>0.05) (见表 1)。
变量 痊愈组(n=31) 不完全痊愈组(n=10) χ2 P 性别 女 15(48.4) 6(60.0) 0.08 >0.05 男 16(51.6) 4(40.0) 年龄/岁 < 2 5(16.1) 3(30.0) — >0.05* 2~5 10(32.3) 2(20.0) >5 16(51.6) 5(50.0) 肢体活动障碍 11(35.5) 3(30.0) 0.00 >0.05 症状 惊厥 7(22.6) 4(40.0) 0.45 >0.05 意识障碍 7(22.6) 4(40.0) 0.45 >0.05 头痛头晕 7(22.6) 1(10.0) 0.17 >0.05 发热 10(32.3) 2(20.0) 0.12 >0.05 诱因 疫苗接种 1(3.2) 1(10.0) — >0.05* 前驱感染 19(61.3) 10(100.0) 3.76 >0.05 CSF白细胞 异常 15(53.6) 5(55.6) 0.00 >0.05 正常 13(46.4) 4(44.4) CSF蛋白 异常 8(28.6) 4(44.4) 1.15 >0.05 正常 20(71.4) 5(55.6) 四肢肌电图 异常 4(30.8) 3(75) 0.98 >0.05 正常 9(69.2) 1(25) 头颅MRI 有基底核或脑干病灶 11(36.7) 4(40) 0.00 >0.05 无基底核或脑干病灶 19(63.3) 6(60) *示Fisher′s确切概率法P值 表 1 41例随访患儿痊愈组与不完全痊愈组变量比较[n; 百分率(%)]
儿童急性播散性脑脊髓炎临床特点及预后影响因素分析
Clinical analysis and influencing prognosis factors in 77 children with acute disseminated encephalomyelitis
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摘要:
目的总结儿童急性播散性脑脊髓炎(acute disseminated encephalomyelitis,ADEM)的临床特点并分析影响预后的因素。 方法回顾性分析77例ADEM患儿的临床特点。随访到的患儿分为痊愈组和不完全痊愈组,对2组患儿的发病年龄、性别、诱因、主要临床症状、脑脊液白细胞及蛋白水平、四肢肌电图、头颅MRI进行统计分析,探寻预后影响因素。 结果77例中男44例,女33例,发病年龄4月14天至14岁。病前60例有感染诱因,7例有疫苗接种史,14例无明确诱因。主要临床症状:肢体活动障碍、惊厥、意识障碍、头痛头晕等,常伴非特异症状发热。头颅和脊髓MRI检查:76例接受头颅MRI检查,66例异常;56例接受脊髓MRI检查,27例异常;头颅MRI正常患儿脊髓MRI见病灶。随访到41例患儿,随访时间病后6个月至6年6个月,其中痊愈组31例,不完全痊愈组10例。2组患儿年龄、性别、诱因、主要临床症状、脑脊液白细胞及蛋白水平、四肢肌电图、头颅MRI的差异均无统计学意义(P>0.05)。 结论儿童ADEM,急性或亚急性起病,脑病样表现,头颅或脊髓MRI异常为其主要诊断依据,多数预后良好。性别、发病年龄、病前诱因、主要临床症状、脑脊液一般检查、四肢肌电图及头颅MRI差异对预后无影响。 Abstract:ObjectiveTo summarize the clinical features and analyze the factors of influencing prognosis in children with acute disseminated encephalomyelitis(ADEM). MethodsThe clinical features of children with ADEM retrospectively analyzed.The children patients were divided into the complete healing group and incomplete healing group.The age, gender, inducements, clinical symptoms, white cell and albumen level in cerebrospinal fluid, electromyogram in extremities and encephalic MRI were compared between two groups, and the factors of influencing prognosis were explored. ResultsA total of 77 patients(44 male and 33 female), aged range from 4 months 14 days to 14 years old, were investigated.Sixty cases with infection history, 7 cases with vaccination history and 14 cases without definite inducement were found.The major clinical symptoms included the motility disorders of extremities, eclampsia, conscious disturbance, headache and dizziness, and the patients often accompanied by non-specific symptoms of fever.The results of encephalic MRI in 76 cases showed that 66 cases were abnormity, and the results of spinal cord MRI in 56 cases showed that 27 cases were abnormity.The lesion in spinal cord was found in the patients with normal encephalic MRI.Forty-one children(including 31 cases of complete healing group and 10 cases of incomplete healing group) were followed up for 6 months to 6 years after illness.The differences of the age, gender, inducements, clinical symptoms, cerebrospinal fluid white blood cell count and protein level, electromyography of limbs and encephalic MRI between two groups were not statistically significant(P>0.05). ConclusionsThe acute or subacute onset, encephalopathy, MRI abnormalities in the cranial or spinal cord are the main diagnosis criteria, and the prognosis in most patients are good.The gender, age of onset, predisposition, main clinical symptoms, general examination of cerebrospinal fluid, electromyography of limbs and head MRI do not affect on the prognosis. -
Key words:
- acute disseminated encephalomyelitis /
- children /
- clinical features /
- follow-up /
- prognosis
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表 1 41例随访患儿痊愈组与不完全痊愈组变量比较[n; 百分率(%)]
变量 痊愈组(n=31) 不完全痊愈组(n=10) χ2 P 性别 女 15(48.4) 6(60.0) 0.08 >0.05 男 16(51.6) 4(40.0) 年龄/岁 < 2 5(16.1) 3(30.0) — >0.05* 2~5 10(32.3) 2(20.0) >5 16(51.6) 5(50.0) 肢体活动障碍 11(35.5) 3(30.0) 0.00 >0.05 症状 惊厥 7(22.6) 4(40.0) 0.45 >0.05 意识障碍 7(22.6) 4(40.0) 0.45 >0.05 头痛头晕 7(22.6) 1(10.0) 0.17 >0.05 发热 10(32.3) 2(20.0) 0.12 >0.05 诱因 疫苗接种 1(3.2) 1(10.0) — >0.05* 前驱感染 19(61.3) 10(100.0) 3.76 >0.05 CSF白细胞 异常 15(53.6) 5(55.6) 0.00 >0.05 正常 13(46.4) 4(44.4) CSF蛋白 异常 8(28.6) 4(44.4) 1.15 >0.05 正常 20(71.4) 5(55.6) 四肢肌电图 异常 4(30.8) 3(75) 0.98 >0.05 正常 9(69.2) 1(25) 头颅MRI 有基底核或脑干病灶 11(36.7) 4(40) 0.00 >0.05 无基底核或脑干病灶 19(63.3) 6(60) *示Fisher′s确切概率法P值 -
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