巴西人对神经结节病广谱的看法

Carolina B. Moura , Flávia Aguiar Machado , Johnatan Felipe Ferreira da Conceição , Isadora Gomes Mesquita , Rafael Santos Correia , Rodrigo Cutrim Gaudio , Melina da Silva Bernardes , Lucas F B Horta , Caroline Bittar-Braune
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引用次数: 0

摘要

背景:神经结节病(NS)是一种罕见的结节病,其特点是由于肉芽肿性炎症影响神经系统的不同区域而导致广泛的神经系统表现。NS在巴西的流行情况没有充分的记录,这表明该地区存在诊断不足的风险。病例系列本研究检查了来自巴西的5例活检证实的系统性结节病和可能的NS患者。神经系统表现多种多样,包括纵向广泛的脊髓炎、肌病、小脑受累、眶尖综合征和多发性颅神经病变。结节病的最初表现为NS。诊断通过临床、放射学、实验室和病理结果的详细评估,遵循既定的NS诊断标准。每位患者均接受MRI、脑脊液分析和全身评估,活检证实为全身结节病。治疗主要涉及皮质类固醇,一些患者需要额外的免疫抑制治疗,导致良好的结果。该病例系列突出了NS的诊断挑战,特别是在巴西,那里的认识可能有限。神经科医生应考虑NS病例不明原因的神经症状,并伴有全身体征。及时诊断和治疗对于改善这种可能使人衰弱的疾病的患者预后至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Brazilian perspectives on neurosarcoidosis broad spectrum

Background

Neurosarcoidosis (NS) is a rare form of sarcoidosis, characterized by a wide range of neurological presentations due to granulomatous inflammation affecting different areas of the nervous system. The prevalence of NS in Brazil is not well-documented, suggesting a risk of underdiagnosis in this region.

Case Series

This study examines a series of five patients from Brazil with biopsy-confirmed systemic sarcoidosis and probable NS. Neurological manifestations varied widely, including longitudinally extensive myelitis, myopathy, cerebellar involvement, orbital apex syndrome, and multiple cranial neuropathies. NS was the initial presentation of sarcoidosis in most cases. Diagnoses were confirmed through a detailed assessment of clinical, radiological, laboratory, and pathological findings, following established NS diagnostic criteria. Each patient underwent MRI, cerebrospinal fluid analysis, and systemic evaluations, with systemic sarcoidosis confirmed by biopsy. Treatment primarily involved corticosteroids, with some patients requiring additional immunosuppressive therapy, resulting in favorable outcomes.

Conclusions

This case series highlights the diagnostic challenges of NS, particularly in Brazil, where awareness may be limited. Neurologists should consider NS in cases of unexplained neurological symptoms accompanied by systemic signs. Prompt diagnosis and treatment are essential to improve patient outcomes in this potentially debilitating condition.
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