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麻风病多药治疗后界限类偏瘤型麻风患者的进行性神经病。

Progressive neuropathy in patients with lepromatous leprosy after multidrug therapy.

机构信息

Universidade Federal do Estado do Rio de Janeiro, Programa de Pós-Graduação em Neurologia, Rio de Janeiro, RJ, Brasil.

Fundação Oswaldo Cruz-Fiocruz, Instituto Oswaldo Cruz, Departamento de Hanseníase, Rio de Janeiro, RJ, Brasil.

出版信息

Mem Inst Oswaldo Cruz. 2023 Jan 16;117:e220150. doi: 10.1590/0074-02760220150. eCollection 2023.

Abstract

BACKGROUND

The lepromatous pole is a stigmatising prototype for patients with leprosy. Generally, these patients have little or no symptoms of peripheral nerve involvement at the time of their diagnosis. However, signs of advanced peripheral neuropathy would be visible during the initial neurological evaluation and could worsen during and after multidrug therapy (MDT). Disabilities caused by peripheral nerve injuries greatly affect these patients' lives, and the pathophysiological mechanisms underlying nerve damage remain unclear.

OBJECTIVES

To evaluate the outcome of peripheral neuropathy in patients with lepromatous leprosy (LL) and persistent neuropathic symptoms years after completing MDT.

METHODS

We evaluated the medical records of 14 patients with LL who underwent nerve biopsies due to worsening neuropathy at least four years after MDT.

FINDINGS

Neuropathic pain developed in 64.3% of the patients, and a neurological examination showed that most patients had alterations in the medium- and large-caliber fibers at the beginning of treatment. Neurological symptoms and signs deteriorated despite complete MDT and prednisone or thalidomide use for years. Nerve conduction studies showed that sensory nerves were the most affected.

MAIN CONCLUSIONS

Patients with LL can develop progressive peripheral neuropathy, which continues to develop even when they are on long-term anti-inflammatory and immunosuppressive therapy.

摘要

背景

瘤型麻风是麻风病患者的一种具有耻辱性的典型。通常,这些患者在诊断时几乎没有或没有周围神经受累的症状。然而,在最初的神经学评估时就可以看到晚期周围神经病变的迹象,并且在多药治疗(MDT)期间和之后可能会恶化。周围神经损伤引起的残疾极大地影响了这些患者的生活,而神经损伤的病理生理机制仍不清楚。

目的

评估完成 MDT 多年后仍有持续性神经病变症状的瘤型麻风(LL)患者周围神经病的结局。

方法

我们评估了 14 例因 MDT 后至少四年神经病变恶化而接受神经活检的 LL 患者的病历。

结果

64.3%的患者出现神经病理性疼痛,神经检查显示大多数患者在开始治疗时就存在中、大纤维改变。尽管接受了完整的 MDT 以及泼尼松或沙利度胺多年治疗,神经症状和体征仍在恶化。神经传导研究显示感觉神经最受影响。

主要结论

LL 患者可出现进行性周围神经病,即使长期接受抗炎和免疫抑制治疗,也会持续发展。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/336f/9870262/4a632d2792c9/1678-8060-mioc-117-e220150-gf1.jpg

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