Christani Maria
överläkare, neurologkliniken, Södra älvsborgs sjukhus, Borås.
Lakartidningen. 2022 Feb 3;119:21133.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune inflammatory polyneuropathy characterized by a progressive or relapsing course. It has been reported that approximately 16% of CIDP patients may present with an acute clinical onset much like Guillain-Barré syndrome (GBS) but with a subsequent chronic progression. These patients are classified as acute-onset CIDP (A-CIDP). In CIDP, bulbar and ventilator involvement is uncommon. We report a case of a 56-year-old man in previous good health who developed progressive paresthesia and weakness in the limbs within 2 weeks. The diagnosis of A-CIDP was made. The patient's course fluctuated greatly over 5 months and he developed oropharyngeal weakness and ventilatory insufficiency that led to a tracheostomy. He slowly improved after IVIG treatment in combination with rituximab but required mechanical ventilatory support for 3 months. One month later he was able to perform all activities of daily living independently.
慢性炎症性脱髓鞘性多发性神经病（CIDP）是一种获得性自身免疫性炎性多发性神经病，其特征为病程呈进行性或复发性。据报道，约16%的CIDP患者可能表现为急性临床起病，很像吉兰-巴雷综合征（GBS），但随后呈慢性进展。这些患者被归类为急性起病型CIDP（A-CIDP）。在CIDP中，延髓和呼吸机受累情况并不常见。我们报告一例56岁男性病例，该患者既往身体健康，在2周内出现进行性肢体感觉异常和无力。诊断为A-CIDP。患者病程在5个月内波动很大，出现口咽肌无力和通气不足，导致行气管切开术。在静脉注射免疫球蛋白（IVIG）联合利妥昔单抗治疗后，他逐渐好转，但需要机械通气支持3个月。1个月后，他能够独立进行所有日常生活活动。
