Victor Horsley Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, London, UK.
Ear Institute, University College London, London, UK.
Neurol Sci. 2022 Sep;43(9):5643-5646. doi: 10.1007/s10072-022-06181-x. Epub 2022 Jun 13.
Classical infratentorial superficial siderosis (iSS) is characterised by repeated insidious bleeding into the subarachnoid space, leading to haemosiderin deposition within the subpial layers of the brainstem, cerebellum and spinal cord, sometimes with supratentorial involvement. Although nearly always associated with a dural defect (usually from previous trauma or neurosurgery) there is little evidence to support definitive investigation and management strategies. Here, we present a novel investigation strategy to identify a dural defect and subsequent successful surgical repair with biochemical resolution of subarachnoid bleeding. CLINICAL PRESENTATION: A 55-year-old gentleman presented with a 15-year progressive history of sensorineural deafness, followed by a slowly worsening gait ataxia. He had previously sustained cranio-spinal trauma. On examination there were features of myelopathy and ataxia. MRI demonstrated classical iSS, affecting cerebellum and cerebral cortices, with a cervicothoracic epidural CSF collection. Lumbar puncture (LP) revealed elevated ferritin 413 ng/mL and red cell count of 4160. Reverse CT myelography, a novel technique involving contrast injection into the collection, delineated a dural defect at the T9/T10 level that was not present on conventional myelography. Following surgical repair, repeat LP twelve months later demonstrated biochemical improvement (ferritin 18 ng/mL, red cells < 1). There was no further neurological deterioration in symptoms during eighteen months follow-up. CONCLUSION: We show the value of a rational targeted investigation pathway in identifying a surgically reparable dural defect underlying classical iSS. We also provide proof of concept that surgical repair can facilitate biochemical resolution of subarachnoid bleeding and might prevent progression of neurological disability.
经典颅后窝表浅性铁沉积症（iSS）的特征是蛛网膜下腔反复出现隐匿性出血，导致脑桥、小脑和脊髓的软脑膜下层出现血铁质沉着，有时伴有幕上受累。尽管几乎总是与硬脑膜缺陷有关（通常来自先前的创伤或神经外科手术），但几乎没有证据支持明确的调查和管理策略。在这里，我们提出了一种新的调查策略，以确定硬脑膜缺陷，并随后通过生化方法解决蛛网膜下腔出血，成功进行手术修复。
一名 55 岁男性，有 15 年渐进性感觉神经性耳聋病史，随后出现逐渐恶化的步态共济失调。他以前曾遭受过颅脊柱创伤。检查时存在脊髓病和共济失调的特征。MRI 显示典型的 iSS，影响小脑和大脑皮质，伴有颈胸段硬膜外 CSF 积聚。腰椎穿刺（LP）显示铁蛋白升高至 413ng/mL，红细胞计数为 4160。反向 CT 脊髓造影，一种新的技术，涉及在积聚物中注射对比剂，描绘了 T9/T10 水平的硬脑膜缺陷，而常规脊髓造影未显示该缺陷。手术后 12 个月重复 LP 显示生化改善（铁蛋白 18ng/mL，红细胞<1）。在 18 个月的随访中，症状没有进一步恶化。
我们展示了一种合理的靶向调查途径在确定经典 iSS 下可手术修复的硬脑膜缺陷方面的价值。我们还提供了概念验证，证明手术修复可以促进生化方法解决蛛网膜下腔出血，并可能防止神经功能障碍的进展。
