Department of Graduate Medical Education, Virginia Mason Medical Center, Seattle, Washington, USA.
Section of Rheumatology, Virginia Mason Medical Center, Seattle, Washington, USA.
World Neurosurg. 2019 Oct;130:65-70. doi: 10.1016/j.wneu.2019.06.130. Epub 2019 Jun 27.
Back pain is a leading reason for patients to seek medical attention. Although musculoskeletal causes are common, patients can also present with rarer etiologies.
A 50-year-old man presented with 2 months of isolated upper back pain initially suspected to be secondary to overuse muscular strain. During the next 3 months, his pain worsened, and he developed lower extremity dysesthesia and subjective weakness, despite normal neurological examination findings. Nonrevealing laboratory workup included normal muscle enzymes, C-reactive protein, urinalysis, and human leukocyte antigen B27. Magnetic resonance imaging revealed a normal brain but a hypointense C7-T5 epidural mass, prompting a neurosurgical recommendation for laminectomy with evacuation of the suspected hematoma. His symptoms fully and promptly resolved after a 5-day course of prednisone 40 mg. When his symptoms recurred within 2 months, he underwent T4-T5 laminectomy with biopsy of a mass confluent with the dura mater. Initial pathological examination revealed fibrotic tissue of unclear etiology with polyclonal lymphoid infiltrate but no malignant cells, vasculitis, or granulomas. After months of recurrent, steroid-responsive symptoms, he presented to the rheumatology clinic. Repeat spinal magnetic resonance imaging demonstrated progression of epidural thickening with suspected spinal cord compression. Previous biopsy samples were then immunostained for IgG4, revealing focally dense IgG4-positive plasma cells, up to 29 cells per high power field, consistent with spinal IgG4-related hypertrophic pachymeningitis. He began rituximab therapy with a prednisone taper and demonstrated symptomatic and neurologic improvement with successful withdrawal from corticosteroids.
To the best of our knowledge, the present case represents the 12th reported case of spinal IgG4-related hypertrophic pachymeningitis. An early diagnosis and treatment could prevent progression to permanent neurological impairment and functional disability.
背痛是患者寻求医疗关注的主要原因。尽管肌肉骨骼原因较为常见，但患者也可能出现罕见病因。
一名 50 岁男性因孤立性上背痛就诊，最初怀疑为过度使用肌肉劳损所致。在接下来的 3 个月中，尽管神经检查结果正常，但其疼痛恶化，并出现下肢感觉异常和主观无力。非特异性实验室检查包括正常的肌肉酶、C 反应蛋白、尿液分析和人类白细胞抗原 B27。磁共振成像显示大脑正常，但 C7-T5 硬膜外有低信号肿块，促使神经外科建议行椎板切除术以清除疑似血肿。他在接受 5 天 40mg 泼尼松治疗后症状完全迅速缓解。当他的症状在 2 个月内再次复发时，他接受了 T4-T5 椎板切除术和与硬脑膜融合的肿块活检。最初的病理检查显示病因不明的纤维组织，伴有多克隆淋巴细胞浸润，但无恶性细胞、血管炎或肉芽肿。在反复发作、类固醇反应性症状数月后，他到风湿病诊所就诊。重复脊柱磁共振成像显示硬膜外增厚进展，疑似脊髓受压。随后对以前的活检样本进行 IgG4 免疫染色，显示局灶性密集 IgG4 阳性浆细胞，高倍视野下多达 29 个细胞，符合脊柱 IgG4 相关肥厚性硬脑膜炎。他开始接受利妥昔单抗治疗和泼尼松减量，并在成功停用皮质类固醇后表现出症状和神经改善。
据我们所知，本病例代表第 12 例报告的脊柱 IgG4 相关肥厚性硬脑膜炎。早期诊断和治疗可防止进展为永久性神经损伤和功能障碍。
