儿童慢性炎症性脱髓鞘性多发性神经病的临床特征及肌肉回声强度与疾病严重程度的相关性分析

目的 探讨儿童慢性炎症性脱髓鞘性多发性神经病(Chronic inflammatory demyelinating polyneuropathy,CIDP)的临床特征及肌肉回声强度与疾病严重程度的相关性。方法 收集2015～2022年本院诊断为肯定型和可能CIDP患儿23例,其中男13例,女10例; 根据诊断年龄分为早期起病组8例(诊断年龄<4岁)和晚期起病组15例(诊断年龄≥4岁); 根据起病到病程高峰期时间分为慢性起病CIDP组(起病到病程高峰期时间≥2个月)和急性和亚急性起病CIDP组(起病到病程高峰期时间<2个月); 10例CIDP进行肌肉超声检查,肌肉超声回声强度分级按照赫克马特分级,并与患儿疾病严重程度进行相关性分析。结果 入组CIDP中首次诊断分别为CIDP7例,吉兰-巴雷综合征13例,运动发育迟缓1例,遗传代谢病1例、分离转换障碍1例; 早期起病CIDP和晚期起病CIDP组间临床特征无明显差异(P>0.05); 与慢性起病CIDP组比较,急性和亚急性起病CIDP组缓解一复发例数更多(χ²=10.879,P=0.001),起病到确诊时间更短(χ²=-11.340,P=0.000),误诊率更高(χ²=6.188,P=0.013); 肌肉超声中胫前肌和股内肌肌肉回声强度分级与疾病严重程度呈正相关(r=0.832,P=0.003)。结论 儿童CIDP最易误诊为GBS; CIDP肌肉超声回声强度与疾病严重程度呈正相关。

Clinical features of childhood chronic inflammatory demyelinating polyneuropathy and the correlation between muscle echointensity and severity of disease

ObjectiveTo explore clinical features of childhood chronic inflammatory demyelinating polyneuropathy(CIDP)and the correlation between muscle echointensity and the severity of this disease.Methods The clinical dates of 23 children, including 13 males and 10 females, with positive and possible CIDP diagnosed by our hospital from 2015 to 2022 were collected. We established two groups of patients based on age at onset of CIDP, including 8 cases from early onset group(diagnosis age <4 years)and 15 cases from late onset group(diagnosis age ≥4 years). According to the time from onset to nadir, the patients were divided into chronic onset group(the time from the onset to nadir ≥2 months)and acute or subacute onset group(the time from the onset to nadir <2 months). Ten patients with CIDP were examined by muscle ultrasound. The muscle echointensity was visually graded with the Heckmatt scale, and the correlation between muscle echointensity and the severity of this disease was analyzed.Results In CIDP group, the first diagnosis was Guillain-barre syndrome(GBS)in 13 children, CIDP in 7 children, motor retardation in 1 child, hereditary disease in 1 child and dissociative conversion disorder in 1 child. There was no significant difference between early onset group and late onset group on clinical features of children with CIDP(P>0.05). Compared to chronic onset group, acute or subacute onset group had shorter time from onset to diagnosis(χ²=-11.340, P=0.000), more relapsing-remitting(χ²=10.879, P=0.001)and higher misdiagnosis rate(χ²=6.188, P=0.013). In muscle ultrasoundof10 patients with CIDP, the grade of echointensity of tibialis anterior muscle and intramedullary muscle was positively correlated with the severity of the disease(r=0.832, P=0.003).Conclusions Conclusions CIDP in childhood was often misdiagnosis as GBS. Increased muscle echointensity was positively correlated with the severity of this disease.