Süt Nurşah Yeniay, Kartal Ayşe Tuğba, Ertem Şeyma, Aydın Fatma, Yıldırım Miraç, Bektaş Ömer, Özçakar Zeynep Birsin, Teber Serap
Department of Pediatric Neurology, Ankara University Faculty of Medicine, Ankara.
Department of Pediatric Rheumatology, Ankara University Faculty of Medicine, Ankara, Türkiye.
Turk J Pediatr. 2023;65(1):155-160. doi: 10.24953/turkjped.2022.460.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a peripheral nervous system disease associated with polyautoimmunity.
We report a previously healthy 13-year old boy who was referred to our outpatient clinic with gait disturbance and distal lower limb weakness that had been increasing for six months. The patient had decreased deep tendon reflexes in the upper extremities and absence in the lower extremities, reduced muscle strength in the distal and proximal lower extremities, muscle atrophy, drop foot, and normal pinprick sensations. The patient was diagnosed with CIDP as a result of clinical findings and electrophysiological studies. Autoimmune diseases and infectious agents were investigated in terms of triggering CIDP. Although there was no clinical sign other than polyneuropathy, he was also diagnosed with Sjögren`s syndrome due to positive antinuclear antibodies and antibodies against Ro52, and with autoimmune sialadenitis. After six months of monthly intravenous immunoglobulin and oral methylprednisolone treatments, the patient was able to dorsiflex his left foot and walk without support.
To our knowledge, our case is the first pediatric case with the coexistence of Sjögrens syndrome and CIDP. Therefore, we suggest investigating children with CIDP in terms of underlying autoimmune diseases such as Sjögrens syndrome.
慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）是一种与多自身免疫相关的周围神经系统疾病。
我们报告一名此前健康的13岁男孩，因步态障碍和下肢远端无力逐渐加重6个月而转诊至我们的门诊。该患者上肢深腱反射减弱，下肢深腱反射消失，下肢远端和近端肌力减弱，肌肉萎缩，足下垂，针刺觉正常。根据临床发现和电生理研究，该患者被诊断为CIDP。对自身免疫性疾病和感染因子进行了调查，以确定其是否引发CIDP。尽管除了多发性神经病外没有其他临床症状，但由于抗核抗体和抗Ro52抗体阳性，他还被诊断为干燥综合征和自身免疫性涎腺炎。经过6个月每月一次的静脉注射免疫球蛋白和口服甲基强的松龙治疗后，患者能够背屈左脚并独立行走。
据我们所知，我们的病例是首例同时存在干燥综合征和CIDP的儿科病例。因此，我们建议对患有CIDP的儿童进行潜在自身免疫性疾病如干燥综合征的调查。
