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以孤立性脑病变形式出现的神经结节病

Neurosarcoidosis Presented as an Isolated Brain Lesion.

作者信息

Khawaja Mustafa A, Awesat Bader E, Yasini Mohammad N, Anzeh Shahed A, Sinnokrot Zeina R, Baraghithi Nora I, Alayan Mohammed K, Abbasi Abdelrhman G, Asad Diya M, Owda Anas N

机构信息

Medicine, Palestine Medical Complex, Ramallah, PSE.

Medicine, Al-Quds University, Jerusalem, PSE.

出版信息

Cureus. 2023 Sep 24;15(9):e45837. doi: 10.7759/cureus.45837. eCollection 2023 Sep.

Abstract

Sarcoidosis is a multisystemic, noncaseating granulomatous disease of unknown etiology. Neurosarcoidosis (NS) is the involvement of the central nervous system (CNS) in sarcoidosis, and it occurs in approximately 5%-10% of cases. NS can present with a variety of clinical features, making diagnosis challenging. A comprehensive diagnostic approach is required to obtain a definitive diagnosis. In this case we present a 13-year-old boy with diabetes mellitus presented with acute right-sided weakness, paresthesia, headaches, and episodes of loss of consciousness, followed by confusion and aggressive behavior. Neurological examination revealed right-sided motor and sensory deficits, as well as abnormal reflexes. Cranial imaging revealed a solitary lesion in the left centrum semi-ovale. Cerebrospinal fluid (CSF) analysis showed lymphoblastic leukocytosis, increased CSF angiotensin-converting enzyme (ACE), and a high IgG index. Extensive laboratory and imaging studies ruled out other potential etiologies. This case presented with a unique set of clinical features, including a mass lesion effect and seizures, which are uncommon in isolated NS. The patient responded well to high-dose corticosteroid therapy, with resolution of his symptoms. Levetiracetam was used to effectively manage his seizures.

摘要

结节病是一种病因不明的多系统非干酪样肉芽肿性疾病。神经结节病(NS)是结节病累及中枢神经系统(CNS),约5%-10%的结节病患者会出现这种情况。NS可表现出多种临床特征,这使得诊断具有挑战性。需要采用全面的诊断方法才能做出明确诊断。在本病例中,我们报告一名13岁患糖尿病的男孩,出现急性右侧肢体无力、感觉异常、头痛和意识丧失发作,随后出现意识模糊和攻击性行为。神经系统检查发现右侧运动和感觉功能缺损,以及反射异常。头颅影像学检查显示左侧半卵圆中心有一个孤立性病变。脑脊液(CSF)分析显示淋巴细胞增多、CSF血管紧张素转换酶(ACE)升高和IgG指数升高。广泛的实验室检查和影像学检查排除了其他潜在病因。该病例呈现出一组独特的临床特征,包括占位性病变效应和癫痫发作,这在孤立性NS中并不常见。患者对大剂量皮质类固醇治疗反应良好,症状得到缓解。左乙拉西坦用于有效控制其癫痫发作。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b24/10518056/fa8c645a8d0a/cureus-0015-00000045837-i01.jpg

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