Joseph Y Choi, Shantelle A Graff, Hasita V Nalluri, Martha M Quezado, Pinar E Zerk, Dragan Maric, Dorian B McGavern, John D Heiss
Arachnoiditis develops from inflammatory processes in the intrathecal space and can cause syringomyelia, myelopathy, radiculopathy, and chronic pain. Understanding inflammatory and fibrotic mechanisms may identify therapeutic targets. We performed a retrospective study of 60 adults who had undergone surgery for arachnoiditis-associated syringomyelia. Clinical status was assessed preoperatively and at 3 to 4 months post-surgery. Cerebrospinal fluid (CSF) was obtained preoperatively, 1-week post-operatively, and at 3 to 4 months. Operative arachnoid adhesion specimens underwent histopathologic review and immunofluorescence staining with quantitative assessment of fibrosis, inflammation, and human endogenous retrovirus group K (HERV-K) antigens. Lesions had mid-thoracic predominance and heterogeneous etiologies. Median CSF white cell counts were low preoperatively (1 cell/µL, range: 0 to 11 cells/µL). Tissue profiling supported a fibroblast-associated, scar-forming lesion (mean density of CRABP2 = 434 cells/mm2, ER-TR7 = 430 cells/mm2) with moderate collagen I deposition (8.1% of specimens), less adaptive immune signals (9 to 180 cells/mm2), and minimal HERV-K staining (1.1% of tissue). In arachnoiditis-associated syringomyelia, preoperative CSF had few white blood cells; 1-week postoperative CSF had RBC and WBC counts consistent with postoperative meningeal irritation. Chronic fibrosis characterized arachnoid tissue removed at surgery. Histology and immunohistochemical staining suggested that arachnoiditis manifested as a fibroproliferative, adhesive phenotype within the subarachnoid space.