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28例慢性炎症性脱髓鞘性多发性神经病的单中心研究:突尼斯的首例研究。

Monocentric study of 28 cases of chronic inflammatory demyelinating polyneuropathy: first Tunisian study.

作者信息

Mansour Malek, Rachdi Amine, Baradai Nesrine, Kacem Amel, Bedoui Ines, Mrissa Ridha

机构信息

Department of Neurology, Military Hospital, Tunis, Tunisia.

Department of Medicine, Regional Hospital of Jendouba, Jendouba, Tunisia.

出版信息

Neurol Sci. 2022 Jan;43(1):565-571. doi: 10.1007/s10072-021-05153-x. Epub 2021 May 4.

Abstract

BACKGROUND

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare affection of the peripheral nervous system. Its diagnostic criteria have evolved since 1975. The aim of our work is to study the epidemiological, clinical, and paraclinical aspects of CIDP.

METHODS

We conducted a retrospective study of 28 CIDP patients of the neurology department of the military hospital of Tunis between January 2000 and December 2017. All these patients met the European Federation of Neurological Societies/Peripheral Nerve Society(EFNS/PNS)2010 diagnostic criteria for definite CIDP.

RESULTS

The average age was 50 years with a gender ratio of 1.57. We found sensitivomotor symptoms in 66% of patients. Neurological assessment showed a proximal and distal motor weakness in 50% of cases, the involvement of superficial and deep sensory systems in 44% of patients with a generalized areflexia in all patients. Median Inflammatory Neuropthy Cause and Treatment (INCAT) score was 7. Concerning electrophysiology, all our patients met the EFNS/EPS 2010 diagnostic criteria for a definite CIDP. Screening for concurrent pathologies was positive in 11 patients. On the therapeutic side, there was no superiority of intravenous immunoglobin compared with pulsed methylprednisolone. Oral steroids were used as backup in about 50% of patients. There were good outcomes in 72% of patients who improved very well after treatment.

CONCLUSION

CIDP is a rare and polymorphic disorder with a variety of concurrent pathologies. Our study is the first study in Tunisia and in Maghreb countries which included the most big series of patients. Our results were similar to literature. A multicentral study would be better profitable.

摘要

背景

慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)是一种罕见的周围神经系统疾病。自1975年以来,其诊断标准不断演变。我们这项研究的目的是探讨CIDP的流行病学、临床及辅助检查方面的情况。

方法

我们对2000年1月至2017年12月期间突尼斯军事医院神经科的28例CIDP患者进行了回顾性研究。所有这些患者均符合欧洲神经病学学会联合会/周围神经学会(EFNS/PNS)2010年明确CIDP的诊断标准。

结果

平均年龄为50岁,性别比为1.57。我们发现66%的患者有感觉运动症状。神经学评估显示,50%的病例存在近端和远端肌无力,44%的患者感觉系统(浅感觉和深感觉)受累,所有患者均有全身性反射消失。炎症性神经病病因与治疗(INCAT)评分中位数为7分。在电生理方面,所有患者均符合EFNS/EPS 2010年明确CIDP的诊断标准。11例患者并发疾病筛查呈阳性。在治疗方面,静脉注射免疫球蛋白与脉冲式甲基强的松龙相比并无优势。约50%的患者将口服类固醇作为备用治疗。72%的患者治疗后改善良好,预后较好。

结论

CIDP是一种罕见的多形性疾病,伴有多种并发疾病。我们的研究是突尼斯和马格里布国家首个纳入最大患者系列的研究。我们的结果与文献报道相似。多中心研究可能会更有成效。

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