Department of Neurosurgery, M.A.A. Acibadem University, Maslak Hospital, Istanbul, Turkey.
Department of Medical Biochemistry, M.A.A. Acibadem University, Istanbul, Turkey.
Clin Neurol Neurosurg. 2021 Jan;200:106342. doi: 10.1016/j.clineuro.2020.106342. Epub 2020 Nov 1.
Dural thickening is observed in lymphoma, dural carcinomatosis, meningioma, tuberculosis, and autoimmune diseases. We encountered a patient with dural thickening and complaints of neck and back pain, numbness and loss of strength in the hands. The patient also suffered from polychondritis and had previously received steroid and methotrexate treatment for this indication. The patients' serum was also positive for ANA, yet she did not have any other findings suggesting lupus. Our radiological and pathological analysis revealed IHSP (IgG4-related hypertrophic sclerosing pachymeningitis). In this review study, we provided a detailed literature survey to increase the awareness about IHSP in the neurosurgical community.
MRI (magnetic resonance imaging)-based radiological analyses revealed a posterior extramedullary spinal mass extending from C2 to T2-T3 level. The dural mass was surgically excised and a broad panel of immunohistochemical markers including S100, EMA, CD246/ALK-1, CD45, CD20, CD79a, CD138, CD68, CD1a and CD34 was studied. Immunoglobulin heavy chain/kappa chain gene rearrangement analysis was performed which ruled out a lymphoproliferative disorder.
MRI and pathological findings suggested IHSP. As the disease relapsed with a new anterior extramedullary multilobulated lesion extending from C5 to T1 level, the patient is now closely monitored for further medical and surgical treatment.
IHSP is a relatively novel entity of hypertrophic pachymeningitis and should be included in the differential diagnosis of dural thickening. The fibrosis accompanying IHSP may not respond to medical treatment, which includes steroids and immunosuppressive agents. Additionally, neurological deficits, seizures, spinal decompression, hydrocephalus, or brainstem compression necessitate early surgical intervention. A continued vigilance is also necessary as the disease may relapse long-term following surgical treatment.
硬脑膜增厚可见于淋巴瘤、硬脑膜癌病、脑膜瘤、结核和自身免疫性疾病。我们遇到一位有硬脑膜增厚和颈背部疼痛、手部麻木和无力症状的患者。该患者还患有多发软骨炎，此前曾因该疾病接受类固醇和甲氨蝶呤治疗。患者的血清也抗核抗体（ANA）阳性，但她没有任何其他提示狼疮的发现。我们的放射学和病理学分析显示为 IHSP（IgG4 相关肥厚性硬脑膜炎）。在这项综述研究中，我们进行了详细的文献调查，以提高神经外科学界对 IHSP 的认识。
基于磁共振成像（MRI）的放射学分析显示，一个从 C2 延伸至 T2-T3 水平的椎管外硬脑膜肿块。硬脑膜肿块被手术切除，并进行了广泛的免疫组织化学标志物分析，包括 S100、EMA、CD246/ALK-1、CD45、CD20、CD79a、CD138、CD68、CD1a 和 CD34。免疫球蛋白重链/κ 链基因重排分析排除了淋巴增生性疾病。
MRI 和病理学发现提示为 IHSP。由于疾病复发，出现了一个新的从前向后的椎管外多叶病变，从 C5 延伸至 T1 水平，因此患者现在需要密切监测以进行进一步的医疗和手术治疗。
IHSP 是一种相对较新的肥厚性硬脑膜炎实体，应纳入硬脑膜增厚的鉴别诊断。伴随 IHSP 的纤维化可能对包括类固醇和免疫抑制剂在内的药物治疗无反应。此外，神经功能缺损、癫痫发作、脊髓减压、脑积水或脑干压迫需要早期手术干预。由于疾病在手术后长期可能会复发，因此还需要保持警惕。
