Johns Hopkins University, Department of Neurology, Baltimore, MD, United States of America.
Johns Hopkins University, Department of Neurology, Baltimore, MD, United States of America.
J Neuroimmunol. 2022 Jul 15;368:577871. doi: 10.1016/j.jneuroim.2022.577871. Epub 2022 Apr 18.
Neurosarcoidosis affects 5-26% of patients with systemic sarcoidosis and can be the first or only manifestation of the disease. Neurosarcoidosis can affect any part of the nervous system with heterogeneous clinical manifestations and imaging appearances that overlap with many infectious, inflammatory, and neoplastic disorders, making its diagnosis challenging. In the absence of a reliable biomarker to confirm neurosarcoidosis, the diagnosis is based on identifying a compatible clinical and imaging profile and identifying pathological evidence of non-caseating granulomas by biopsy of other organs or, if needed, in the nervous system, with the exclusion of other causes of granulomatous disease and possible neuroinfectious and neuroinflammatory disorder mimics. This review focuses on the clinical features of neurosarcoidosis with an emphasis on the recognition of the main presentation phenotypes and the initial diagnostic approach and differential diagnosis of neurosarcoidosis.
神经结节病影响 5-26%的系统性结节病患者，并且可以是疾病的首发或唯一表现。神经结节病可以影响神经系统的任何部位，具有不同的临床表现和影像学表现，与许多感染性、炎症性和肿瘤性疾病重叠，因此诊断具有挑战性。在缺乏可靠的生物标志物来确诊神经结节病的情况下，诊断基于确定相容的临床和影像学特征，并通过对其他器官进行活检来确定非干酪样肉芽肿的病理学证据，如果需要，也可以在神经系统中进行活检，同时排除其他肉芽肿性疾病和可能的神经传染性和神经炎症性疾病的类似物。本文重点介绍神经结节病的临床特征，强调识别主要表现表型以及神经结节病的初始诊断方法和鉴别诊断。
