Emekli Ahmed Serkan, Gündüz Tuncay, Samancı Bedia, Kürtüncü Murat
Department of Neurology, Istanbul University Faculty of Medicine, Istanbul, Türkiye.
Noro Psikiyatr Ars. 2025 Jun 16;62(2):156-163. doi: 10.29399/npa.28649. eCollection 2025.
Neurosarcoidosis (NS) represents the most severe complication of sarcoidosis. NS exhibits a diverse array of clinical and radiological features that mimic many inflammatory, infectious, and neoplastic neurological disorders. In our study, we evaluated the demographic, clinical, laboratory, and imaging features of patients with NS.
In this retrospective observational study, we included patients with definite and probable NS with at least 6-months of follow-up. Diagnostic tests, including cerebrospinal fluid analysis and imaging findings, lesion localization, therapeutic interventions, and disease course were evaluated. The modified Rankin scale was employed as a metric to assess the severity of the disease.
Fourteen patients met the inclusion criteria; two were definitively diagnosed with NS through brain biopsy, while twelve received a probable NS diagnosis based on biopsies of non-neuronal tissues. The predominant initial manifestation of NS was cranial neuropathy (64.3%), with facial palsy emerging as the most prevalent subset (35.7%). Five patients exhibited parenchymal involvement, while leptomeningeal involvement was noted in two. All patients were treated with corticosteroids, with nine individuals (64.3%) necessitating additional immunosuppressive interventions. Stable disease or improvement was observed in the majority of patients (85.7%), albeit one case resulted in mortality.
We observed favorable outcomes in the majority of patients. Nevertheless, it's imperative to acknowledge that NS can lead to both mortality and severe morbidity. Recognizing clinical patterns is vital for accurate diagnosis and effective treatment. However, there is an existing gap in management, underscoring the necessity for randomized controlled trials aimed at elucidating optimal treatment strategies.
神经结节病（NS）是结节病最严重的并发症。NS表现出各种各样的临床和放射学特征，可模仿许多炎症性、感染性和肿瘤性神经系统疾病。在我们的研究中，我们评估了NS患者的人口统计学、临床、实验室和影像学特征。
在这项回顾性观察研究中，我们纳入了确诊和疑似NS且至少随访6个月的患者。评估了诊断测试，包括脑脊液分析和影像学检查结果、病变定位、治疗干预措施和疾病进程。采用改良Rankin量表作为评估疾病严重程度的指标。
14例患者符合纳入标准；2例通过脑活检确诊为NS，12例根据非神经组织活检获得疑似NS诊断。NS的主要初始表现为颅神经病变（64.3%），其中面神经麻痹最为常见（35.7%）。5例患者出现实质受累，2例出现软脑膜受累。所有患者均接受了皮质类固醇治疗，9例（64.3%）患者需要额外的免疫抑制干预。大多数患者（85.7%）病情稳定或有所改善，尽管有1例死亡。
我们观察到大多数患者预后良好。然而，必须认识到NS可导致死亡和严重的发病率。识别临床模式对于准确诊断和有效治疗至关重要。然而，目前在管理方面存在差距，这凸显了开展随机对照试验以阐明最佳治疗策略的必要性。
