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[慢性炎症性脱髓鞘性多发性神经病中可出现呼吸衰竭:一例报告]

[Respiratory failure can be seen in chronic inflammatory demyelinating polyneuropathy: a case report].

作者信息

Christani Maria

机构信息

överläkare, neurologkliniken, Södra älvsborgs sjukhus, Borås.

出版信息

Lakartidningen. 2022 Feb 3;119:21133.

Abstract

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.

摘要

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。

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