少年慢性炎性脱髓鞘性多发神经病临床分析

Analysis of the juvenile with chronic inflammatory demyelinating polyneuropathy

目的研究少年慢性炎性脱髓鞘性多发神经病(CIDP)的临床特征。方法对14例少年CIDP患者的临床表现、脑脊液检查、肌电图检查及腓肠神经活检结果进行分析。结果本组患者男女比例3.67∶1,发病前有上呼吸道感染5例;首发症状为双下肢远端无力5例,四肢远端麻木无力4例,四肢无力和双下肢远端麻木无力各2例,四肢肌痛1例;患者都有运动障碍,四肢远端运动和感觉同时明显受累11例,肢体远端肌肉萎缩2例;伴肌痛4例;腱反射减弱6例,消失8例;脑神经损害1例;自主神经受累2例;复发1例;发病高峰期Hughes评分平均3.84分;脑脊液检查蛋白细胞分离9例;肌电图示神经源性损害14例;神经活检示髓鞘脱失5例,炎性细胞浸润5例,髓鞘再生2例;激素治疗12例有效。结论本组患者男性多见;首发症状多为对称性双下肢或四肢远端麻木无力;绝大部分有感觉障碍;脑神经和自主神经损害少见;腓肠神经活检有助于诊断;激素治疗有效。

Objective To study the clinical and pathological features of the juvenile patients with CIDP. Methods The features of clinical manifestation, cerebrospinal fluid, EMG and the biopsy results of sural nerve were presented and analyzed. Results Among this group, the male to female ratio was 3.67 ： 1, five cases were involved with upper respiratory tract infection before the onset. As the initial symptom, there were five cases with distal end of both lower extremities weak, four cases with distal end limb numb and weak, two cases with distal end limb numb, two cases with the distal end of both lower extremities numb and weak, one case with myalgia. Motor disorder was common to all the patients, eleven of which were concomitant with sensory dysfunction, two with limb muscle atrophy, four with myalgia; six with tendon reflexes weakened and eight disappeared, one with cranial nerve damaged, two with the autonomic nerve lesion, one with relapse. The score of the peak incidence as Hughes was 3.84 points on average. Nine cases were of obvious albuminocytolgoic dissociation by the examination of cerebrospinal fluid; Fourteen cases were neurogenic damage by the EMG. The biopsy show that five cases were with amyelination, five with inflammatory cell infiltrated, two cases with obvious remyelination. And the glucoeorticoid therapy was effective for twelve cases. Conclusion Male patients are common to the juvenile with CIDP. The numbness of the distal end limb is the common initial symptom. Most of which are with sensory dysfunction, and rare with cranial nerves and autonomic nerve damaged. The sural nerve biopsy has an important value to the diagnosis. The giucocorticoid therapy is proved to be ef- fective for most patients.