Department of Neurosurgery, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
Department of Histopathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
Neurol India. 2022 May-Jun;70(3):1223-1225. doi: 10.4103/0028-3886.349589.
At times, spinal lesions are part of systemic manifestations of autoimmune disease. Awareness regarding their clinicopathological spectrum, particularly the lesions which usually respond to steroids/immunotherapy, is essential to avoid unwanted surgical morbidity. We discuss a case of a young-man presenting with thoracic spinal epidural compressive lesion which was indeed a manifestation of IgG4-related hypertrophic pachymeningitis. The mass was firmly adherent to the dura and extended into left neural foramen/paravertebral space which precluded complete excision. Frozen sections suggested fibro-inflammatory stroma with large areas of fibrosis and lymphoplasmacytic infiltrate. After subtotal excision, the patient improved with medical therapy at 1-year follow-up. Although uncommon, the case highlights the need to consider spinal presentation of this rare entity, especially in the context of autoimmune disorders or even in isolation. In this regard, intraoperative frozen section can hint the underlying inflammatory/autoimmune pathology, guide further course of surgery as well as limit unwarranted operative morbidity.
有时，脊柱病变是自身免疫性疾病全身表现的一部分。了解其临床病理谱，特别是通常对类固醇/免疫疗法有反应的病变，对于避免不必要的手术发病率至关重要。我们讨论了一名年轻男性的病例，他表现为胸段脊柱硬膜外压迫性病变，实际上是 IgG4 相关肥厚性硬脑膜炎的表现。该肿块与硬脑膜紧密相连，并延伸至左侧神经孔/椎旁间隙，无法完全切除。冷冻切片提示纤维炎症基质，有大片纤维化和淋巴浆细胞浸润。次全切除后，患者在 1 年随访时通过药物治疗得到改善。虽然不常见，但该病例强调了需要考虑这种罕见实体的脊柱表现，特别是在自身免疫性疾病的背景下，甚至是孤立的情况下。在这方面，术中冷冻切片可以提示潜在的炎症/自身免疫性病理，指导进一步的手术过程，并限制不必要的手术发病率。
