Wang Long, Yuan Xiao-Zheng, Zhao Xue-Min, Wang Fu-Yu, Wang Yu
Department of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei.
Department of Neurology.
Medicine (Baltimore). 2018 Dec;97(49):e13538. doi: 10.1097/MD.0000000000013538.
Autonomic symptoms are not uncommon in chronic inflammatory demyelinating polyneuropathy (CIDP), but they are mostly mild and transient and are overshadowed by somatic manifestations. Here, we report a very unusual case of CIDP with severe autonomic symptom, intestinal obstruction, as initial and persistent symptom which responded well to high-dose glucocorticoid and intravenous immunoglobulin treatment.
We described a patient with CIDP with precedent and long-lasting incomplete intestinal obstruction. Clinical manifestations were precedent and chronic abdominal pain, distension and constipation, and later numbness and weakness of lower and upper limbs. Radiograph showed incomplete intestinal obstruction, cerebrospinal fluid (CSF) showed albuminocytological dissociation and electromyography indicated neurogenic lesion.
CIDP with incomplete intestinal obstruction was diagnosed based on the history, related symptoms, typical abdominal radiograph, CSF albuminocytological dissociation, and electromyographic findings.
The patient was treated with intravenous methylprednisolone and immunoglobulin.
After treatment, the intestinal obstruction disappeared and the somato-symptoms improved greatly and gradually.
This case highlights the need for diagnostic vigilance in cases of incomplete intestinal obstruction of unknown cause. We recommend CSF and electromyography examination in view of rare but possibility of CIDP.
自主神经症状在慢性炎症性脱髓鞘性多发性神经病（CIDP）中并不少见，但大多轻微且短暂，被躯体表现所掩盖。在此，我们报告一例非常罕见的CIDP病例，其以严重的自主神经症状——肠梗阻作为初始且持续的症状，对大剂量糖皮质激素和静脉注射免疫球蛋白治疗反应良好。
我们描述了一名患有CIDP且有先前及长期不完全性肠梗阻病史的患者。临床表现为先前的慢性腹痛、腹胀和便秘，以及后来的下肢和上肢麻木及无力。X线片显示不完全性肠梗阻，脑脊液（CSF）显示蛋白细胞分离，肌电图提示神经源性损害。
根据病史、相关症状、典型的腹部X线片、脑脊液蛋白细胞分离及肌电图检查结果，诊断为CIDP合并不完全性肠梗阻。
患者接受了静脉注射甲泼尼龙和免疫球蛋白治疗。
治疗后，肠梗阻消失，躯体症状大幅且逐渐改善。
该病例凸显了对病因不明的不完全性肠梗阻病例进行诊断时保持警惕的必要性。鉴于CIDP虽罕见但有可能，我们建议进行脑脊液和肌电图检查。
