Tidsskr Nor Laegeforen. 2021 Mar 22;141(5). doi: 10.4045/tidsskr.20.0361. Print 2021 Mar 23.
Neurosarcoidosis is a rare form of sarcoidosis that affects the nervous system. The aim of the study was to survey clinical manifestations, findings from assessments and treatment strategies for patients with neurosarcoidosis.
The study performed a retrospective assessment of 17 patients with definitive, probable and possible neurosarcoidosis diagnosed in the period 2008-2019 at the Department of Neurology, Haukeland University Hospital.
The average prevalence of definitive, probable or possible neurosarcoidosis in Norway's Vestland county was 2.7 per 100 000 inhabitants in the period in question. Onset took the form of central nervous affection (8 of 17), hydrocephalus (5 of 17) and cranial neuropathy (5 of 17). Sarcoidosis-like findings were made in 14 of 17 patients by means of contrast-enhanced magnetic resonance tomography (MRT) of the central nervous system, in 7 of 8 patients by positron emission tomography (PET), and in 12 of 16 patients by computed tomography (CT) of the thorax. There were cerebrospinal fluid abnormalities in 15 of 15 patients, with biopsy verification for 13 of 15. The symptoms of 16 of 17 patients improved or stabilised with prednisolone and/or other immunotherapy.
Neurosarcoidosis affects both the central and the peripheral nervous system. Cerebrospinal fluid analysis and contrast-enhanced MRT are important means of detecting inflammation. A biopsy is necessary for making a definitive diagnosis, but is not always feasible. PET can be used as a supplement to other examinations to assess various organ manifestations and to pinpoint biopsy sites. Corticosteroid therapy, and in some cases other immunotherapy, elicits a good response.
神经结节病是一种罕见的结节病形式，影响神经系统。本研究旨在调查神经结节病患者的临床表现、评估结果和治疗策略。
该研究对 2008 年至 2019 年期间在卑尔根大学附属医院神经科确诊为明确、可能和可能的神经结节病的 17 名患者进行了回顾性评估。
在所研究的时期内，挪威韦斯特兰县明确、可能或可能的神经结节病的平均患病率为每 100000 名居民 2.7 人。发病形式为中枢神经系统受累（17 例中的 8 例）、脑积水（17 例中的 5 例）和颅神经病变（17 例中的 5 例）。17 例患者中有 14 例通过中枢神经系统增强磁共振成像（MRT）、8 例中的 7 例通过正电子发射断层扫描（PET）和 16 例中的 12 例通过胸部计算机断层扫描（CT）发现类结节病表现。15 例患者的脑脊液异常，其中 13 例经活检证实。17 例患者中的 16 例经泼尼松龙和/或其他免疫治疗后症状改善或稳定。
神经结节病影响中枢和周围神经系统。脑脊液分析和增强 MRT 是检测炎症的重要手段。活检对于明确诊断是必要的，但并非总是可行。PET 可作为其他检查的补充，用于评估各种器官表现并确定活检部位。皮质类固醇治疗，在某些情况下还有其他免疫治疗，会产生良好的反应。
