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[麻风病的神经表现]

[Neurological manifestations of leprosy].

作者信息

Grimaud J, Vallat J-M

机构信息

Service de Médecine Interne, Neurologie et Rhumatologie, Hôpital Louis Pasteur, Chartres.

出版信息

Rev Neurol (Paris). 2003 Nov;159(11):979-95.

Abstract

Leprosy, also known as Hansen's disease, is a chronic, infectious disease caused by Mycobacterium leprae. Bacilli localize preferentially in the skin and peripheral nerves and have a propensity to cause nerve damage. The resulting disability has caused great suffering for victims in many countries. Despite recent advances in the immunopathogenesis, epidemiology and prognostic factors of leprosy nerve damage, many aspects of the disease have remained enigmatic. The spectrum of clinical and pathological manifestations of the disease ranges from lepromatous to tuberculoid, depending on the host's T-cell-mediated immune response. Diagnosis is based on three criteria: characteristic skin lesions in association with thickened nerves, demonstration of acid fast bacilli in slit skin smears, and histopathology of skin biopsies. Nerve biopsy is necessary to establish the diagnosis of pure "neural leprosy". In developed countries, the diagnosis is suspected when a patient who has stayed in an endemic area suffers from a peripheral neuropathy of unknown etiology. To facilitate determination of the appropriate antibiotic regimen, patients are classified as either paucibacillary or multibacillary. Some patients may have multibacillary leprosy in nerves and paucibacillary leprosy in skin, which emphasizes the usefulness of nerve biopsy. The course of the disease is often complicated by immune mediated "reactions", which can rapidly lead to further nerve damage, namely reversal reaction and erythema nodosum leprosy. However, nerves are often functionally impaired before developing obvious symptoms such as skin reactions or nevralgia (silent neuropathy). Early recognition and prompt treatment with corticosteroids of leprous reactions and "silent neuropathies" is very important to prevent disability with all its attendant problems. Research progress from clinical trials may improve current methods of prevention and treatment of nerve damage in leprosy.

摘要

麻风病,又称汉森氏病,是一种由麻风分枝杆菌引起的慢性传染病。杆菌优先定位于皮肤和周围神经,并易于导致神经损伤。由此产生的残疾给许多国家的患者带来了巨大痛苦。尽管最近在麻风病神经损伤的免疫发病机制、流行病学和预后因素方面取得了进展,但该疾病的许多方面仍然不明。根据宿主的T细胞介导的免疫反应,该疾病的临床和病理表现范围从瘤型到结核样型。诊断基于三个标准:伴有神经增粗的特征性皮肤病变、在皮肤涂片检查中发现抗酸杆菌以及皮肤活检的组织病理学检查。神经活检对于确诊单纯“神经型麻风病”是必要的。在发达国家,当曾在流行地区停留的患者患有病因不明的周围神经病变时,会怀疑患有该病。为便于确定合适的抗生素治疗方案,患者被分为少菌型或多菌型。一些患者可能神经为多菌型麻风病而皮肤为少菌型麻风病,这突出了神经活检的有用性。该疾病的病程常因免疫介导的“反应”而复杂化,这些反应可迅速导致进一步的神经损伤,即逆转反应和麻风结节性红斑。然而,在出现明显症状如皮肤反应或神经痛(无症状性神经病变)之前,神经功能往往已经受损。早期识别并及时用皮质类固醇治疗麻风反应和“无症状性神经病变”对于预防残疾及其所有相关问题非常重要。临床试验的研究进展可能会改善目前麻风病神经损伤的预防和治疗方法。

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