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自身免疫与神经学的交叉:纹状体狼疮性脑炎病例的见解

The Intersection of Autoimmunity and Neurology: Insights From a Case of Striatal Lupus Encephalitis.

作者信息

Soo Tze Hui, Suppiah Subapriya, Zamri Muhammad Furhan

机构信息

Department of Radiology, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, Serdang, MYS.

出版信息

Cureus. 2024 Sep 5;16(9):e68743. doi: 10.7759/cureus.68743. eCollection 2024 Sep.

Abstract

Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disorder predominantly affecting women, characterized by the production of autoantibodies against various nuclear antigens, leading to widespread immune dysregulation and multisystem involvement. Among its complex manifestations, neuropsychiatric systemic lupus erythematosus (NPSLE) represents a particularly challenging aspect of the disease due to its wide spectrum of neurological and psychiatric symptoms. This case report presents a rare instance of striatal lupus encephalitis, a severe subtype of NPSLE, in a 32-year-old woman, highlighting its distinct clinical and radiological features. The patient initially developed bilateral ocular occlusive vasculitis and later presented with acute right-sided hemiparesis and facial asymmetry. Magnetic resonance imaging (MRI) revealed bilateral symmetrical T2-weighted and fluid-attenuated inversion recovery (FLAIR) hyperintense signals in the basal ganglia, consistent with striatal lupus encephalitis. No white matter hyperintensity or vasculitis changes were seen. Cerebrospinal fluid analysis revealed markedly elevated protein levels, though no infectious organism was identified. The patient was treated with high-dose prednisolone, alongside empirical antibiotic and antiviral therapy to address potential meningoencephalitis. Remarkably, she made a full recovery from her stroke-like symptoms. Despite its rarity, the identification of striatal lupus encephalitis is critical due to the severe and potentially irreversible nature of the neurological damage. This case underscores the importance of a comprehensive diagnostic approach, integrating clinical, serological, and neuroimaging findings to differentiate striatal lupus encephalitis from other neuropsychiatric conditions associated with SLE. Its management typically involves aggressive immunosuppressive therapy, with intravenous methylprednisolone being the first-line treatment. The case also illustrates the potential for recovery with prompt and appropriate treatment, as evidenced by the complete resolution of neurological symptoms and MRI findings at follow-up.

摘要

系统性红斑狼疮(SLE)是一种多因素自身免疫性疾病,主要影响女性,其特征是产生针对各种核抗原的自身抗体,导致广泛的免疫失调和多系统受累。在其复杂的表现中,神经精神性系统性红斑狼疮(NPSLE)由于其广泛的神经和精神症状,是该疾病特别具有挑战性的一个方面。本病例报告呈现了一名32岁女性患纹状体狼疮性脑炎(NPSLE的一种严重亚型)的罕见病例,突出了其独特的临床和放射学特征。患者最初出现双侧眼部闭塞性血管炎,随后出现急性右侧偏瘫和面部不对称。磁共振成像(MRI)显示基底节区双侧对称的T2加权和液体衰减反转恢复(FLAIR)高信号,符合纹状体狼疮性脑炎。未见白质高信号或血管炎改变。脑脊液分析显示蛋白水平显著升高,尽管未发现感染病原体。患者接受了高剂量泼尼松龙治疗,同时给予经验性抗生素和抗病毒治疗以应对潜在的脑膜脑炎。值得注意的是,她从中风样症状中完全康复。尽管纹状体狼疮性脑炎罕见,但由于神经损伤的严重性和潜在的不可逆性,其识别至关重要。本病例强调了综合诊断方法的重要性,整合临床、血清学和神经影像学结果以将纹状体狼疮性脑炎与其他与SLE相关的神经精神疾病区分开来。其治疗通常包括积极的免疫抑制治疗,静脉注射甲泼尼龙是一线治疗方法。该病例还说明了及时和适当治疗后恢复的可能性,随访时神经症状和MRI结果完全缓解证明了这一点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aabb/11454829/b4e3a85fe9a2/cureus-0016-00000068743-i01.jpg

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