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Sarcoidosis and the Nervous System.

机译：结节病和神经系统。

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This article provides an overview and update on the neurologic manifestations of sarcoidosis. The 2018 Neurosarcoidosis Consortium diagnostic criteria emphasize that biopsy is key for diagnosis and determines the level of diagnostic certainty. Thus, definite neurosarcoidosis requires nervous system biopsy and probable neurosarcoidosis requires biopsy from extraneural tissue. Without biopsy, possible neurosarcoidosis can be diagnosed if the clinical, imaging, and laboratory picture is compatible and other causes are ruled out. Recent large retrospective studies from the United States and France established that infliximab appears to be efficacious when other treatments are inadequate. Sarcoidosis is a multisystem noninfectious granulomatous disorder that is immune mediated, reflecting the response to an as-yet unidentified antigen or antigens. Neurosarcoidosis refers to neurologic involvement due to sarcoidosis that clinically manifests in 5% of cases of sarcoidosis, with asymptomatic involvement in as many as another one in five patients with sarcoidosis. Sarcoid granulomas can occur in any anatomic substrate in the nervous system, causing protean manifestations that have earned neurosarcoidosis the sobriquet the great mimic. Nevertheless, central nervous system sarcoidosis occurs in well-defined presentations that can be classified as cranial neuropathies, meningeal disease, brain parenchymal (including pituitary-hypothalamic) disease, and spinal cord disease. In addition, the peripheral nervous system is affected in the form of peripheral neuropathy and myopathy. Glucocorticoids are the cornerstone of treatment, especially in the acute stage, whereas steroid-sparing agents such as methotrexate, mycophenolate mofetil, and azathioprine are used for prolonged therapy to minimize steroid toxicity. Anti-tumor necrosis factor agents may help in refractory cases.
机译：本文提供了关于结节病的神经系统表现的概述和更新。 2018年神经皮病联盟联盟诊断标准强调，活检是诊断的关键，并确定诊断确定性水平。因此，明确的神经皮病症需要神经系统活组织检查，并且可能的神经颈病症需要来自外膜组织的活组织检查。如果没有活组织检查，如果临床，成像和实验室图片兼容，则可以诊断出可能的神经颈病症，并排除了其他原因。来自美国和法国的最近大型回顾性研究确定，当其他治疗不充分时，英夫利昔单抗似乎是有效的。结节病是一种多洋组织无排感肉芽肿病症，其免疫介导，反映对对尚未识别的抗原或抗原的反应。神经颈痘是指因结节病引起的神经系统参与，临床表现在临床上表现为5％的结节病病例，无症状受累，在5例患有的结节病患者中有多种。在神经系统中的任何解剖学底物中可能发生肌肉肉芽肿，导致蛋白质的蛋白质表现出来的令人毛骨悚然的巨大模仿。然而，中枢神经系统的结节病发生在明确的呈现中，可以归类为颅神经病，脑膜病，脑实质（包括垂体下丘脑）疾病和脊髓疾病。此外，外周神经系统受外周经神经病变和肌病的形式受到影响。糖皮质激素是治疗的基石，特别是在急性阶段，而类固醇味剂如甲氨蝶呤，霉酚酸酯，和氮嘌呤用于延长治疗，以最大限度地减少类固醇毒性。抗肿瘤坏死因子剂可能有助于难治性情况。

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