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神经结节病:两例病例的临床表现、诊断与治疗回顾

Neurosarcoidosis: The Presentation, Diagnosis and Treatment Review of Two Cases.

作者信息

Basheer Maamoun, Waked Hamd, Jeries Helana, Azrilin Olga, Paz Dan, Assy Nimer, Naffaa Mohammad E, Badarny Samih

机构信息

Internal Medicine Department, Galilee Medical Center, Nahariya 221001, Israel.

Neurology Department, Galilee Medical Center, Nahariya 221001, Israel.

出版信息

Life (Basel). 2023 Dec 31;14(1):69. doi: 10.3390/life14010069.

Abstract

Sarcoidosis is a chronic granulomatous disease of unknown cause characterized by the presence of non-caseating granulomas. The disease can affect any organ including the nervous system. Neurosarcoidosis occurs in about 5% patients with sarcoidosis. The clinical presentation of neurosarcoidosis is varied, and it can involve the brain, spinal cord and peripheral nervous system, separately or in different combinations. The diagnosis of neurosarcoidosis is challenging, as biopsies from the nervous system are not readily available. Anti-TNFα agents are becoming one of the cornerstone treatments for neurosarcoidosis. In this case-based review, we discuss two cases of neurosarcoidosis with different clinical presentations. The first patient presented with confusion, while the second presented with walking difficulty and neurogenic bladder. Both patients were treated with methylprednisolone pulse therapy with rapid, but non-complete, improvement. Therefore, infliximab was initiated in both cases with subsequent improvement in the clinical manifestations and imaging findings, emphasizing the effectiveness and safety of infliximab in cases of severe neurosarcoidosis. In conclusion, the goal of neurosarcoidosis management is to prevent organ system damage and minimize the toxic cumulative adverse effects of glucocorticoid use. In this case-based review we discuss the various presentations, the diagnosis and the treatment of neurosarcoidosis.

摘要

结节病是一种病因不明的慢性肉芽肿性疾病,其特征是存在非干酪样肉芽肿。该疾病可累及任何器官,包括神经系统。神经结节病约见于5%的结节病患者。神经结节病的临床表现多样,可单独或不同组合地累及脑、脊髓和周围神经系统。神经结节病的诊断具有挑战性,因为难以获取神经系统的活检样本。抗TNFα药物正成为神经结节病的主要治疗方法之一。在本病例回顾中,我们讨论了两例临床表现不同的神经结节病病例。首例患者表现为意识模糊,而第二例表现为行走困难和神经源性膀胱。两名患者均接受了甲泼尼龙冲击治疗,病情迅速但未完全改善。因此,两例患者均开始使用英夫利昔单抗治疗,随后临床表现和影像学检查结果均有所改善,这强调了英夫利昔单抗在严重神经结节病病例中的有效性和安全性。总之,神经结节病管理的目标是预防器官系统损害,并尽量减少糖皮质激素使用的累积毒性不良反应。在本病例回顾中,我们讨论了神经结节病的各种表现、诊断和治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f20f/10820900/63e7dbaa2529/life-14-00069-g001.jpg

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