囊性 IgG4 相关脊髓硬膜肥厚症压迫脊髓导致类似神经囊尾蚴病：病例报告。
Spinal cord compression by cystic IgG4-related spinal pachymeningitis mimicking neurocysticercosis: a case report.
机构信息
Faculty of Medicine, Federal University of Ceara, Alexandre Baraúna St., 949, Fortaleza, 60430-160, Ceara, Brazil.
Department of Neurology, General Hospital of Fortaleza, Ávila Goularte St., 900, Fortaleza, 60150-160, Ceara, Brazil.
出版信息
BMC Neurol. 2024 Sep 5;24(1):318. doi: 10.1186/s12883-024-03817-7.
BACKGROUND
To report a case of IgG4-related pachymeningitis presenting with cystic lesions mimicking neurocysticercosis.
CASE PRESENTATION
A 40-year-old female patient with tetraparesis, dysphagia and dysphonia was evaluated with clinical examination, magnetic resonance imaging, and meningeal biopsy. Magnetic resonance imaging (MRI) revealed diffuse pachymeningeal enhancement involving the cranial, cervical, thoracic, and lumbar segments with spinal cord compression and cystic lesions. CSF immunology was initially positive for cysticercus cellulosae. After disease progression a meningeal biopsy was compatible with IgG4 related disease. The patient had partial response to rituximab and needed multiple surgical procedures for spinal cord decompression and CSF shunting.
CONCLUSIONS
This case highlights the possibility of IgG4-related disease in patients with diffuse pachymeningitis causing spinal cord compression, even with cystic lesions on MRI. Diagnosis of IgG4-related pachymeningitis is paramount due to the possibility of treatment response to immunotherapy, particularly to anti-CD20 agents.
背景
报告一例 IgG4 相关脑脊髓膜炎，表现为囊性病变，类似神经囊虫病。
病例介绍
一位 40 岁女性患者，表现为四肢瘫痪、吞咽困难和发音困难，经临床检查、磁共振成像和脑膜活检进行评估。磁共振成像（MRI）显示弥漫性硬脑膜强化，累及颅颈胸腰段，伴有脊髓压迫和囊性病变。脑脊液免疫学最初呈囊尾蚴细胞阳性。疾病进展后，脑膜活检符合 IgG4 相关疾病。该患者对利妥昔单抗有部分反应，需要多次手术进行脊髓减压和脑脊液分流。
结论
本例强调了 IgG4 相关疾病在引起脊髓压迫的弥漫性硬脑膜炎患者中的可能性，即使 MRI 上有囊性病变。由于免疫治疗，特别是抗 CD20 药物治疗有反应的可能性，IgG4 相关脑脊髓膜炎的诊断至关重要。
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