Continuum (Minneap Minn). 2020 Jun;26(3):695-715. doi: 10.1212/CON.0000000000000855.
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.
本文概述并更新了结节病的神经表现。
2018 年神经结节病联盟诊断标准强调，活检是诊断的关键，并决定诊断的确信度水平。因此，明确的神经结节病需要进行神经系统活检，可能的神经结节病需要进行神经外组织活检。如果临床、影像学和实验室表现相符且排除其他原因，则无需活检即可诊断可能的神经结节病。最近来自美国和法国的两项大型回顾性研究表明，英夫利昔单抗在其他治疗方法效果不佳时似乎有效。
结节病是一种多系统非传染性肉芽肿性疾病，由免疫介导，反映了对尚未确定的抗原或抗原的反应。神经结节病是指由于结节病而导致的神经系统受累，在 5%的结节病病例中表现为临床症状，多达 1/5 的结节病患者无症状受累。结节病肉芽肿可发生在神经系统的任何解剖部位，引起表现多样的病变，因此神经结节病获得了“伟大模仿者”的称号。然而，中枢神经系统结节病发生在明确的表现中，可以分为颅神经病变、脑膜疾病、脑实质（包括垂体-下丘脑）疾病和脊髓疾病。此外，周围神经系统也会受到影响，表现为周围神经病和肌病。糖皮质激素是治疗的基石，尤其是在急性期，而甲氨蝶呤、霉酚酸酯和硫唑嘌呤等类固醇保留剂则用于延长治疗，以最大限度地减少类固醇毒性。抗肿瘤坏死因子药物可能有助于治疗难治性病例。
