Department of Neurosurgery, Allegheny General Hospital, Pittsburgh, PA, USA.
Department of Neurological Surgery, Rush University Medical Center, Chicago, IL, USA.
Neurol India. 2022 May-Jun;70(3):1180-1186. doi: 10.4103/0028-3886.349607.
IgG4-related disease is a rare, recently recognized chronic inflammatory disease. IgG4-related hypertrophic pachymeningitis (IgG4-RHP) of the central nervous system predominantly involves the cranial meninges. Spinal involvement remains rare.
We report a case of recurrent cervicothoracic IgG4-RHP and review the surgical literature.
A 35-year-old woman presented with a 6-month history of neck and right shoulder pain, progressive right triceps weakness and paresthesias in the right C8 and T1 dermatomes. MRI demonstrated a T2 hypointense epidural soft tissue mass extending from C6-T1. The patient underwent C6-T1 laminoforaminotomy and partial resection with near complete symptom resolution. Histopathology was consistent with diagnosis of IgG4-RHP. Eighteen months postoperatively, she experienced symptom recurrence necessitating re-operation and adjuvant postoperative prednisone with complete resolution at 40-months' follow-up.
Of the now nineteen confirmed cases of IgG4-RHP, fifteen underwent surgery. A majority achieved partial resection. Three surgical patients did not receive adjuvant therapy with symptomatic recurrence between 2 and 18 months.
IgG4 相关疾病是一种罕见的、最近才被认识到的慢性炎症性疾病。中枢神经系统 IgG4 相关肥厚性脑脊膜炎（IgG4-RHP）主要累及颅脑膜。脊柱受累仍较为罕见。
我们报告一例复发性颈胸 IgG4-RHP，并复习手术文献。
一名 35 岁女性因颈痛和右肩部疼痛、进行性右肱三头肌无力和右 C8 和 T1 皮节感觉异常，病程 6 个月就诊。MRI 显示 C6-T1 连续的 T2 低信号硬膜外软组织肿块。患者接受 C6-T1 椎板切开术和部分切除术，症状几乎完全缓解。组织病理学符合 IgG4-RHP 的诊断。术后 18 个月，症状复发，再次手术，术后给予泼尼松辅助治疗，40 个月随访时完全缓解。
在目前已确诊的 19 例 IgG4-RHP 中，有 15 例行手术治疗。大多数患者行部分切除术。3 例手术患者未接受辅助治疗，症状复发时间为 2-18 个月。
