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[1例慢性炎症性脱髓鞘性多发性神经根神经病合并膈神经麻痹]

[A case of chronic inflammatory demyelinating polyradiculoneuropathy complicated by phrenic nerve palsy].

作者信息

Kawakami T, Sekijima Y, Tokuda T, Yamazaki M, Yanagisawa N

机构信息

Department of Medicine (Neurology), Shinshu University School of Medicine.

出版信息

Rinsho Shinkeigaku. 1998 Feb;38(2):118-21.

PMID:9619073
Abstract

We report on a patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) complicated by phrenic nerve palsy. A 50-year-old man was admitted to our hospital due to dyspnea and numbness in his limbs. On admission, severe muscle atrophy, weakness, and sensory disturbance were seen in the distal part of his extremities. Deep tendon reflexes were absent in all the limbs. Routine blood examinations showed no abnormal findings except for mild hyperglycemia (fasting blood sugar 145 mg/dl). Right phrenic nerve palsy was observed on a chest roentgenogram and % vital capacity (%VC) was 56%. Cerebrospinal fluid showed elevated levels of total protein (116 mg/dl) and IgG (16.9 mg/dl). Sural nerve biopsy revealed severe loss of myelinated fibers with demyelination and onion-bulb formation. After immunoadsorption plasmapheresis, the phrenic nerve palsy improved rapidly and %VC recovered to 76%. Although phrenic nerve palsy is rare in patients with CIDP, it is important to take notice of this condition, because phrenic nerve palsy is critical when it occurs bilaterally or when it develops in patients who have suffered from respiratory diseases. Immunoadsorption plasmapheresis is one of the effective treatments for CIDP, especially in patients with serious complications such as phrenic nerve palsy.

摘要

我们报告一例慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)合并膈神经麻痹的患者。一名50岁男性因呼吸困难和肢体麻木入院。入院时,其四肢远端可见严重肌肉萎缩、无力及感觉障碍。四肢腱反射均消失。常规血液检查除轻度高血糖(空腹血糖145mg/dl)外无异常发现。胸部X线片显示右侧膈神经麻痹,肺活量百分比(%VC)为56%。脑脊液显示总蛋白(116mg/dl)和IgG(16.9mg/dl)水平升高。腓肠神经活检显示有髓纤维严重丢失伴脱髓鞘及洋葱球形成。免疫吸附血浆置换术后,膈神经麻痹迅速改善,%VC恢复至76%。尽管膈神经麻痹在CIDP患者中罕见,但注意这种情况很重要,因为双侧发生或在患有呼吸系统疾病的患者中发生时,膈神经麻痹很危急。免疫吸附血浆置换是CIDP的有效治疗方法之一,尤其是对于伴有膈神经麻痹等严重并发症的患者。

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