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诊断难题:一例无系统性结节病的神经结节病病例

A Diagnostic Dilemma: A Case of Neurosarcoidosis Without Systemic Sarcoidosis.

作者信息

Sedeta Ephrem, Ilerhunmwuwa Nosakhare P, Hindu Pahlani Rachna, Aiwuyo Henry, Wasifuddin Mustafa, Uche Ifeanyi, Hakobyan Narek, Perry Jamal, Terebelo Sima

机构信息

Medicine, Brookdale University Hospital Medical Center, Brooklyn, USA.

出版信息

Cureus. 2023 Aug 2;15(8):e42844. doi: 10.7759/cureus.42844. eCollection 2023 Aug.

DOI:10.7759/cureus.42844
PMID:37664296
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10472481/
Abstract

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology characterized by non-caseating granulomas in involved organs. Approximately 10% of patients with sarcoidosis exhibit central nervous system involvement. However, the occurrence of isolated neurosarcoidosis without concurrent systemic signs is very rare, affecting less than 1% of patients. We report a case of isolated neurosarcoidosis in a previously healthy patient who initially presented with a single episode of seizure and loss of consciousness. Brain MRI showed T2/fluid-attenuated inversion recovery (FLAIR) hyperintense extra-axial soft tissue mass over the left cerebral convexity measuring approximately 14 mm in maximum depth. Excisional biopsy of the brain mass showed chronic non-caseating granulomatous inflammation with epitheloid cells that was consistent with sarcoidosis. Treatment with high dose-steroids led to significant clinical improvement. At a two-year follow-up, there were no signs of systemic disease or recurrence of the meningeal mass. This case emphasizes the rarity of such presentation, diagnostic difficulties, and the importance of high suspicion and timely management to prevent debilitating neurologic complications.

摘要

结节病是一种病因不明的多系统肉芽肿性疾病,其特征是受累器官出现非干酪样肉芽肿。约10%的结节病患者有中枢神经系统受累。然而,无并发全身症状的孤立性神经结节病非常罕见,不到1%的患者会出现。我们报告一例孤立性神经结节病病例,患者此前身体健康,最初表现为单次癫痫发作和意识丧失。脑部MRI显示左侧大脑凸面T2/液体衰减反转恢复(FLAIR)序列高信号的轴外软组织肿块,最大深度约14mm。脑部肿块切除活检显示慢性非干酪样肉芽肿性炎症,伴有上皮样细胞,符合结节病表现。高剂量类固醇治疗使临床症状显著改善。在两年的随访中,没有全身疾病迹象或脑膜肿块复发。该病例强调了这种表现的罕见性、诊断困难以及高度怀疑和及时处理以预防使人衰弱的神经并发症的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d4bb/10472481/9c72ac7c7cbe/cureus-0015-00000042844-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d4bb/10472481/b1d3466d4098/cureus-0015-00000042844-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d4bb/10472481/ef8b263934e0/cureus-0015-00000042844-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d4bb/10472481/9c72ac7c7cbe/cureus-0015-00000042844-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d4bb/10472481/b1d3466d4098/cureus-0015-00000042844-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d4bb/10472481/ef8b263934e0/cureus-0015-00000042844-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d4bb/10472481/9c72ac7c7cbe/cureus-0015-00000042844-i03.jpg

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