Department of Neurosurgery, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fuchu, Tokyo, 183-0042, Japan.
J Neurol. 2021 Apr;268(4):1491-1494. doi: 10.1007/s00415-020-10319-2. Epub 2021 Jan 3.
Superficial siderosis of the central nervous system is a rare intractable disease induced by chronic subarachnoid hemorrhage. Neurological deficits, such as cerebellar ataxia and hearing difficulties, gradually progress if left undiagnosed. Hemosiderin deposition is irreversible because standard medical treatment has not yet been established. Interventions at the source of bleeding may be the key to a preferable outcome of treatment for chronic subarachnoid hemorrhage; however, the source is not clear in many cases.
Among the consecutive cases diagnosed with a spontaneous cerebrospinal fluid (CSF) leak, cases of superficial siderosis associated with a CSF leak due to a ventral dural defect were retrospectively analyzed.
Among 77 cases of a CSF leak, 7 cases (9%) of superficial siderosis were identified (median age of 59 years, male, 4 cases). Defects were diagnosed on 1-mm sliced fast imaging employing steady-state acquisition MRI (n = 5), conventional myelographic CT (n = 1), or dynamic myelographic CT (n = 1) at high thoracic levels (T1-T4). All defects were repaired by direct neurosurgery. During surgery, continuous bleeding from the epidural veins of the internal vertebral venous plexus was identified as the source of subarachnoid hemorrhage. Epidural CSF pulsations through the defect prevented clot formation by the epidural veins. Dural repair stopped free communication between the subarachnoid and epidural spaces, leading to the disappearance of chronic subarachnoid hemorrhage.
Bleeding from the epidural venous plexus may be the cause of superficial siderosis associated with ventral dural defects. Neurosurgical repair may stop the progression of this condition.
中枢神经系统表面铁沉积症是一种由慢性蛛网膜下腔出血引起的罕见难治性疾病。如果不进行诊断，神经功能缺损（如小脑共济失调和听力障碍）会逐渐加重。由于尚未制定标准的治疗方法，因此铁沉积是不可逆转的。出血源的干预可能是慢性蛛网膜下腔出血治疗结果更好的关键；然而，在许多情况下，出血源并不明确。
在连续诊断出自发性脑脊髓液（CSF）漏的病例中，回顾性分析了与由于腹侧硬脑膜缺陷导致的 CSF 漏相关的表面铁沉积症病例。
在 77 例 CSF 漏病例中，发现了 7 例（9%）表面铁沉积症（中位年龄 59 岁，男性 4 例）。在 1 毫米切片快速成像采用稳态采集 MRI（n=5）、常规脊髓造影 CT（n=1）或高胸段动态脊髓造影 CT（n=1）上诊断出缺陷。所有缺陷均通过直接神经外科手术修复。在手术中，发现硬膜外静脉丛的硬膜外静脉持续出血是蛛网膜下腔出血的原因。通过缺陷的硬膜外 CSF 搏动阻止了硬膜外静脉的血栓形成。硬脑膜修复阻止了蛛网膜下腔和硬膜外腔之间的自由沟通，导致慢性蛛网膜下腔出血消失。
硬脑膜静脉丛出血可能是与腹侧硬脑膜缺陷相关的表面铁沉积症的原因。神经外科修复可能会阻止病情的进展。
