Kamer Carolina, Pretto Barbara Janke, Livramento Carlos Rafael, da Silva Rafael Carlos
Department of Internal Medicine, School of Medicine, Centro Universitário para o Desenvolvimento do Alto Vale do Itajaí, Rio do Sul, Santa Catarina, Brazil.
Department of Rheumatology, Rheumatology and Immunotherapy Center of Alto Vale, Rio do Sul, Santa Catarina, Brazil.
Surg Neurol Int. 2021 Nov 30;12:581. doi: 10.25259/SNI_1012_2021. eCollection 2021.
Brain paracoccidioidomycosis (PCM) or neuroparacoccidioidomycosis (NPCM) is a fungal infection of the central nervous system (CNS) caused by , a dimorphic fungus. The CNS involvement is through bloodstream dissemination. The association between NPCM and systemic lupus erythematous (SLE) is rare. However, SLE patients are under risk of opportunistic infections given their immunosuppression status.
The aim of this case report is to present a 37-year-old female with diagnosis of SLE who presented with progressive and persistent headache in the past 4 months accompanied by the right arm weakness with general and neurologic examination unremarkable. The computerized tomography of the head showed left extra-axial parietooccipital focal hypoattenuation with adjacent bone erosion. The brain magnetic resonance imaging reported left parietooccipital subdural collection associated with focal leptomeningeal thickening with restriction to diffusion and peripheral contrast enhancement. The patient underwent a left craniotomy and dura mater biopsy showed noncaseous granulomatosis with multinucleated giant cells with rounded birefringent structures positive for silver stain, consistent with PCM. Management with itraconazole 200 mg daily was started with a total of 12 months of treatment, with patient presenting resolution of headache and right arm weakness.
The diagnosis of NPCM is challenging and a high degree of suspicious should be considered in patients with persistent headache and immunosuppression.
脑副球孢子菌病（PCM）或神经副球孢子菌病（NPCM）是由一种双相真菌引起的中枢神经系统（CNS）真菌感染。中枢神经系统受累是通过血液传播。NPCM与系统性红斑狼疮（SLE）之间的关联很少见。然而，SLE患者因其免疫抑制状态而有机会性感染的风险。
本病例报告的目的是介绍一名37岁诊断为SLE的女性，她在过去4个月中出现进行性持续性头痛，并伴有右臂无力，全身和神经系统检查无异常。头部计算机断层扫描显示左侧轴外顶枕部局灶性低密度影，伴有相邻骨质侵蚀。脑部磁共振成像报告左侧顶枕部硬膜下积液，伴有局灶性软脑膜增厚，扩散受限和外周对比增强。患者接受了左开颅手术，硬脑膜活检显示非干酪性肉芽肿，有多核巨细胞，有圆形双折射结构，银染色阳性，符合PCM。开始每日用200毫克伊曲康唑治疗，共治疗12个月，患者头痛和右臂无力症状消失。
NPCM的诊断具有挑战性，对于持续头痛和免疫抑制的患者应高度怀疑。
