Sireesha Yareeda, Uppin Megha S, Ganti Shridhar, Alugolu Rajesh, Mudumba Vijaya Saradhi, Bhattacharjee Suchanda, Neeharika Mathukumalli L, Bastia Jogendra, Kanikannan Meena Angamuthu
Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
Ann Indian Acad Neurol. 2019 Jan-Mar;22(1):73-78. doi: 10.4103/aian.AIAN_283_18.
To study the clinical presentation, radiological findings, and therapy responsiveness of patients with biopsy-proven immunoglobulin G4 (IgG4)-related neurological disease.
The study was conducted between January 2016 and March 2018 from the Department of Neurology and Pathology of Nizam's Institute of Medical Sciences. Patients with neurological symptoms and biopsy suggestive of IgG4-related disease (IgG4-RD) were included. These patients were studied for their demographic pattern and clinical presentation. The presence of serological markers such as vasculitic profile and IgG4 levels was analyzed. Radiological findings were studied in detail. Therapeutic agents used and the response to therapy were assessed.
There were six cases with IgG4-related neurological disease which were all hypertrophic pachymeningitis. The age ranged from 35 to 64 (mean = 46) years. The clinical presentation was acute in one, subacute in two, and chronic in three patients. The most common presenting symptom was headache (4), followed by gait and/or urinary disturbances (2), paraparesis (1), and diplopia (1). IgG4 levels were elevated in 50% of them. Pseudotumor-like mass and sinovenous thrombosis, not described previously, were seen in one patient. All the patients were treated with oral or intravenous steroid. Rituximab was given in three patients; azathioprine was the steroid-sparing agent in one patient. Those with acute/subacute onset of presentation had an excellent response to steroids. All the patients with a chronic duration of their symptoms received empirical anti-tuberculous therapy before a definitive diagnosis of Ig G4-RD was made.
The characterization of patients with IgG4-related neurological disease based on the understanding of the clinical spectrum increases the confidence in the clinician to resort to early immunosuppression, thereby having prognostic implications.
研究经活检证实的免疫球蛋白G4（IgG4）相关神经系统疾病患者的临床表现、影像学表现及治疗反应。
该研究于2016年1月至2018年3月在尼扎姆医学科学研究所神经科和病理科进行。纳入有神经系统症状且活检提示IgG4相关疾病（IgG4-RD）的患者。对这些患者的人口统计学特征和临床表现进行研究。分析血清学标志物如血管炎指标和IgG4水平的存在情况。详细研究影像学表现。评估所用治疗药物及治疗反应。
有6例IgG4相关神经系统疾病患者，均为肥厚性硬脑膜炎。年龄范围为35至64岁（平均46岁）。1例患者临床表现为急性，2例为亚急性，3例为慢性。最常见的首发症状是头痛（4例），其次是步态和/或排尿障碍（2例）、双下肢轻瘫（1例）和复视（1例）。其中50%的患者IgG4水平升高。1例患者出现了此前未描述过的假瘤样肿块和静脉窦血栓形成。所有患者均接受口服或静脉注射类固醇治疗。3例患者使用了利妥昔单抗；1例患者使用硫唑嘌呤作为类固醇减量药物。急性/亚急性起病的患者对类固醇治疗反应良好。所有症状持续时间较长的患者在IgG4-RD确诊前均接受了经验性抗结核治疗。
基于对临床谱的理解对IgG4相关神经系统疾病患者进行特征描述，可增加临床医生早期进行免疫抑制治疗的信心，从而对预后产生影响。
