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双侧面瘫作为神经结节病的首发症状:一例病例报告及文献复习

Bilateral Facial Palsy as the Onset of Neurosarcoidosis: A Case Report and a Revision of Literature.

作者信息

Gallo Chiara, Mazzini Letizia, Varrasi Claudia, Vecchio Domizia, Virgilio Eleonora, Cantello Roberto

机构信息

Department of Neurology, University of Eastern Piedmont, Maggiore della Carità Hospital, 28100 Novara, Italy;

Ph.D. Program in Medical Sciences and Biotechnologies, Department of Translational Medicine, University of Eastern Piedmont, 28100 Novara, Italy.

出版信息

NeuroSci. 2022 May 29;3(2):321-331. doi: 10.3390/neurosci3020023. eCollection 2022 Jun.

Abstract

Unilateral facial nerve palsy (FNP) is one of the most common cranial mononeuropathies. Among rare etiologies, neurosarcoidosis (NS) can cause bilateral involvement (both recurring and simultaneous) only in 15% to 25% of cases. The rarity of this systemic disease and its clinical heterogeneity, due to granulomatous inflammation that may affect many anatomic substrates, frequently make the diagnosis a real challenge for the clinician. Based on laboratory and instrumental tests, a careful diagnostic algorithm must be adopted to avoid misdiagnosis and delay in treatment. We present a 52-year-old woman with an acute onset of unilateral right FNP, rapidly developing contralateral involvement (simultaneous bilateral FNP). Lung findings pointed towards a systemic disease, and then lymph node biopsy confirmed NS. Corticosteroid therapy was started. After three years of follow-up, the patient is still in remission with a low prednisone dose. We discuss the differential diagnosis of bilateral FNP, focusing on clinical presentation, diagnosis, and treatment of NS. We have performed a literature revision, confirming bilateral FNP, outside Heerfordt syndrome, to be rare and sometimes represent the only neurological manifestation of NS onset.

摘要

单侧面神经麻痹(FNP)是最常见的颅单神经病之一。在罕见病因中,神经结节病(NS)仅在15%至25%的病例中可导致双侧受累(复发和同时出现)。这种全身性疾病的罕见性及其临床异质性,由于肉芽肿性炎症可能影响许多解剖学部位,常常使诊断成为临床医生的一项真正挑战。基于实验室和仪器检查,必须采用仔细的诊断算法以避免误诊和治疗延误。我们报告一名52岁女性,急性起病的单侧右侧FNP,迅速发展为对侧受累(同时双侧FNP)。肺部检查结果提示全身性疾病,随后淋巴结活检确诊为NS。开始使用皮质类固醇治疗。经过三年随访,患者仍以低剂量泼尼松维持缓解状态。我们讨论双侧FNP的鉴别诊断,重点关注NS的临床表现、诊断和治疗。我们进行了文献复习,证实除黑尔福特综合征外,双侧FNP罕见,有时是NS发病的唯一神经表现。

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